Madjid Samii

Venelin Gerganov

Surgery of
Cerebellopontine
Lesions

123
Surgery of Cerebellopontine Lesions
Madjid Samii • Venelin Gerganov

Surgery of
Cerebellopontine
Lesions
With Chapters on Microsurgical Anatomy by
A.L. Rhoton and Neuropathology by G.F. Walter
Madjid Samii Venelin Gerganov
International Neuroscience Institute INI Department of Neurosurgery
Hannover International Neuroscience Institute
Germany Hannover
Germany

ISBN 978-3-642-35421-2 ISBN 978-3-642-35422-9 (eBook)

DOI 10.1007/978-3-642-35422-9
Springer Heidelberg New York Dordrecht London

Library of Congress Control Number: 2013934833

© Springer-Verlag Berlin Heidelberg 2013

This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or
part of the material is concerned, specifically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way,
and transmission or information storage and retrieval, electronic adaptation, computer software,
or by similar or dissimilar methodology now known or hereafter developed. Exempted from this
legal reservation are brief excerpts in connection with reviews or scholarly analysis or material
supplied specifically for the purpose of being entered and executed on a computer system, for
exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is
permitted only under the provisions of the Copyright Law of the Publisher’s location, in its
current version, and permission for use must always be obtained from Springer. Permissions for
use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable
to prosecution under the respective Copyright Law.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this
publication does not imply, even in the absence of a specific statement, that such names are
exempt from the relevant protective laws and regulations and therefore free for general use.
While the advice and information in this book are believed to be true and accurate at the date of
publication, neither the authors nor the editors nor the publisher can accept any legal responsibility
for any errors or omissions that may be made. The publisher makes no warranty, express or
implied, with respect to the material contained herein.

Printed on acid-free paper

Springer is part of Springer Science+Business Media (www.springer.com)

To my family for their support and to all my patients for their
confidence in me.
Madjid Samii
To my beloved family - my wife Lyuba for her love,
understanding and support, and our wonderful children, Zara
and Marco, and to my parents.
Venelin M. Gerganov
Authors

Madjid Samii, MD, PhD; Prof.

President, International Neuroscience Institute, Hannover, Germany
President, China International Neuroscience Institute at the Capital Medical
University, Beijing, China
President, Iran International Neuroscience Institute at Tehran University of
Medical Sciences, Tehran, Iran
Rudolf Pichlmayr Str. 4, 30625 Hannover, Germany
Telephone number: 0049 511 27092 700
Fax number: 0049 511 27092 706
E-mail address: samii@ini-hannover.de
Venelin M. Gerganov, MD, PhD; Assoc. Prof.
Consultant neurosurgeon
Department of Neurosurgery, International Neuroscience Institute
Hannover, Germany
Rudolf Pichlmayr Str. 4, 30625 Hannover, Germany
Telephone number: 0049 511 27092 835
Fax number: 0049 511 27092 706
E-mail address: vgerganov@gmail.com
Albert L. Rhoton, Jr., M.D.
R.D. Keene Family Professor and Chairman Emeritus Department of
Neurosurgery
University of Florida P.O. Box 100265
Gainesville, Florida 32610
Telephone number: 352-273-9000
Fax number: 352-392-8413
E-mail address: rhoton@neurosurgery.ufl.edu
Gerhard Franz Walter, MD, PhD, Prof.
Dean, PhD-Programme Clinical Neurosciences
International Neuroscience Institute, Hannover, Germany
Rudolf Pichlmayr Str. 4, 30625 Hannover, Germany
Telephone number: 0049 511 27092 700
Fax number: 0049 511 27092 706
E-mail address: gf.walter@ini-hannover.de

vii
Contents

1 History of Cerebellopontine Angle Surgery .............................. 1

Description of CP Angle Tumors and First Attempts
at Surgery ....................................................................................... 1
Improvement of the Surgical Outcome and Introduction
of the Standard of Complete Tumor Removal ............................... 3
Development of Operative Techniques to Preserve
the Facial Nerve ............................................................................. 4
Translabyrinthine Approach ....................................................... 4
Transpetrosal Approach ............................................................. 5
Middle Fossa Approach ............................................................. 5
Modern Period of Functional Preserving Safe CP Angle
Surgery ........................................................................................... 5
Hearing Preservation .................................................................. 6
Radiosurgery .............................................................................. 6
References ...................................................................................... 6
2 Microsurgical Anatomy of the Cerebellopontine Angle
by the Retrosigmoid Approach ................................................... 9
Posterior Cranial Fossa .................................................................. 9
The Cerebellopontine Angle .......................................................... 9
Cerebellar Surfaces .................................................................... 11
Cerebellar Brain Stem Fissures .................................................. 14
Cerebellar Arteries ..................................................................... 16
The Fourth Ventricle ...................................................................... 18
Three Neurovascular Complexes ................................................... 19
Upper Neurovascular Complex ...................................................... 19
Trigeminal Root Anatomy ......................................................... 20
Anatomy of Vascular Compression in the Upper
Neurovascular Complex ............................................................. 24
Arterial Relationships................................................................. 26
Venous Relationships ................................................................. 29
Suprameatal Extension of the Retrosigmoid Approach ............. 32
Middle Neurovascular Complex .................................................... 34
Anatomy of Acoustic Neuromas ................................................ 34
Meatal Relationships .................................................................. 36
Retrosigmoid Approach ............................................................. 37
Brain Stem Relationships ........................................................... 39
Summary: Anatomy of Acoustic Neuromas .............................. 45
ix
x Contents

Anatomy of Vascular Compression in the Middle

Neurovascular Complex ................................................................. 50
Cochlear and Vestibular Nerve Compression Syndromes ......... 55
Geniculate Neuralgia .................................................................. 58
Lower Neurovascular Complex ..................................................... 59
Neural Relationships .................................................................. 60
Anatomy of Glossopharyngeal Neuralgia .................................. 61
Vascular Relationships ............................................................... 63
Vascular Compression in the Lower
Neurovascular Complex ................................................. 63
Tumors Involving Multiple Neurovascular Complexes ................. 63
Telovelar Approach to Fourth Ventricle ......................................... 65
References ...................................................................................... 70
3 Neuropathology of the Cerebellopontine Angle
and Its Surroundings ................................................................... 73
Introductory Remarks .................................................................... 73
Pathology of the Peripheral Nervous System ................................ 73
Schwannoma .............................................................................. 74
Malignant Peripheral Nerve Sheath Tumor (MPNST) .............. 76
Pathology of the Autonomous Nervous System ............................ 77
Jugulotympanic Paraganglioma ................................................. 77
Pathology of the Meninges ............................................................ 78
Meningioma ............................................................................... 78
Hemangiopericytoma ................................................................. 81
Hemangioblastoma ..................................................................... 82
Leptomeningeal Melanocytic Lesions ....................................... 83
Meningiosis and Solid Metastatic Tumors ................................. 83
Acute Bacterial Sive Purulent Meningitis .................................. 84
Tuberculous Meningitis.............................................................. 85
Sarcoidosis ................................................................................. 86
Aspergillosis ............................................................................... 86
Cryptococcosis ........................................................................... 88
Cysticercosis............................................................................... 89
Echinococcosis ........................................................................... 89
Pathology of the Osseous Skull Base............................................. 89
Fibrous Dysplasia ....................................................................... 90
Aneurysmal Bone Cyst .............................................................. 91
Osteochondroma......................................................................... 91
Chondroblastoma ....................................................................... 91
Chondrosarcoma......................................................................... 91
Chordoma ................................................................................... 91
Endolymphatic Sac Tumor ......................................................... 92
Pathology of the Sellar Region ...................................................... 93
Pituitary Adenoma ..................................................................... 93
Pituitary Carcinoma ................................................................... 95
Spindle Cell Oncocytoma of the Adenohypophysis .................. 96
Pituicytoma................................................................................. 96
Granular Cell Tumor of the Neurohypophysis........................... 97
Contents xi

Craniopharyngioma .................................................................... 97
Xanthogranuloma of the Sellar Region ...................................... 98
Pathology of Regional Glial Tumors ............................................. 99
Pilocytic Astrocytoma ................................................................ 99
Pilomyxoid Astrocytoma ........................................................... 99
Ependymoma.............................................................................. 100
Pathology of the Choroid Plexus of the Fourth Ventricle .............. 100
Choroid Plexus Papilloma .......................................................... 100
Choroid Plexus Carcinoma ........................................................ 101
Pathology of the Hematopoietic System ........................................ 101
Primary CNS Lymphomas ......................................................... 102
Langerhans Cell Histiocytosis.................................................... 102
Non-Langerhans Cell Histiocytosis ........................................... 103
Pathology of Germ Cells ................................................................ 104
Germ Cell Tumors ...................................................................... 104
Tumorlike Dysontogenetic Cysts ............................................... 105
Pathology of Blood Vessels and Circulation .................................. 105
Arteriovenous Malformation ...................................................... 105
Saccular Aneurysm .................................................................... 107
Ischemic Brain Infarct ................................................................ 109
Hypertensive Angiopathy and Brain Hemorrhage ..................... 109
Vasculitis .................................................................................... 110
References .................................................................................. 112
4 Approaches to the Cerebellopontine Angle ............................... 115
Middle Fossa Approach ................................................................. 115
Standard MF Approach .............................................................. 115
Extended MF Approach ................................................................. 118
Translabyrinthine Approach and Its Variations ............................. 119
Operative Technique................................................................... 119
Mastoidectomy ........................................................................... 119
Labyrinthectomy ........................................................................ 120
Surgical Outcome ....................................................................... 120
Retrolabyrinthine Approach........................................................... 121
Partial Translabyrinthine Approach ............................................... 121
Transcochlear Approach ................................................................ 121
Combined Approaches ................................................................... 121
Combined Translabyrinthine–Retrosigmoid Approach ............. 122
Translabyrinthine–Transtentorial Approach .............................. 122
Retrosigmoid Approach ................................................................. 122
Benefits of the Approach ............................................................ 122
Patient Positioning and Anesthesia Considerations ................... 123
Anesthetic Considerations ............................................................. 123
The Semi-sitting Position ........................................................... 126
Standard Instrument Sets Used in All CP Angle Surgeries ....... 128
Skin Incision and Craniotomy.................................................... 128
Dura Incision .............................................................................. 131
Hemostasis, dural closure and mastoid occlusion...................... 132
Reconstruction of the Posterior Skull Base................................ 134
xii Contents

Drawbacks of the RS Approach ..................................................... 134

Limited Exposure of the Fundus ................................................ 134
Need of Cerebellar Retraction.................................................... 136
Postoperative Headache ............................................................. 136
Pneumocephalus and Tension Pneumocephalus ........................ 137
References ...................................................................................... 141
5 Vestibular Schwannomas............................................................. 147
Epidemiology ................................................................................. 147
Biology of VS ................................................................................ 148
Molecular Genetics .................................................................... 148
The NF2 Protein ......................................................................... 148
Pathogenesis of VS ........................................................................ 149
Origin of VS ............................................................................... 149
Classification .................................................................................. 152
Neuroimaging ................................................................................ 156
Clinical Presentation ...................................................................... 156
Management of Patients with VS .................................................. 162
Conservative Management ......................................................... 162
Surgical Treatment of VS ........................................................... 163
Radiosurgical Management of VS ............................................. 282
References ...................................................................................... 304
6 Neurofibromatosis Type 2 and Other Bilateral
Cerebellopontine Angle Tumors ................................................. 315
Neurofibromatosis Type 2 .............................................................. 315
Criteria for Diagnosis ................................................................. 315
Genetics ...................................................................................... 315
Clinical Presentation .................................................................. 316
General Management ................................................................. 321
Large VSs ................................................................................... 328
Smaller VS, Useful Hearing....................................................... 328
Surgery on the Only Hearing Side ............................................. 332
Personal Series ........................................................................... 337
Other Bilateral CP Angle Tumors .................................................. 352
Hearing Rehabilitation ............................................................... 364
References ...................................................................................... 371
7 Meningiomas ................................................................................ 375
Cerebellopontine Angle Meningiomas .......................................... 375
Epidemiology ............................................................................. 375
Pathogenesis and Pathology ....................................................... 375
Classification .............................................................................. 376
Neuroimaging ............................................................................. 380
Clinical Presentation .................................................................. 390
Management ............................................................................... 392
Petroclival Meningiomas ............................................................... 436
General Features......................................................................... 436
Management ............................................................................... 438
Foramen Magnum Meningiomas ................................................... 483
Contents xiii

General Features......................................................................... 483

Management ............................................................................... 483
References ...................................................................................... 502
8 Cerebellopontine Angle Epidermoids ........................................ 509
General Features ............................................................................ 509
Neuroimaging ................................................................................ 509
Clinical Presentation ...................................................................... 511
Management................................................................................... 511
Outcome ..................................................................................... 515
Recurrences ................................................................................ 515
References ...................................................................................... 545
9 Nonvestibular Schwannomas ...................................................... 547
Trigeminal Schwannomas .............................................................. 547
General Features......................................................................... 547
Neuroimaging ............................................................................. 547
Clinical Presentation .................................................................. 549
Management ............................................................................... 549
Oculomotor Nerve Schwannoma ................................................... 577
Trochlear Schwannomas ................................................................ 577
Abducens Nerve Schwannoma ...................................................... 580
Facial Schwannomas ...................................................................... 583
Cochlear Schwannomas ................................................................. 604
Lower Cranial Nerve Schwannomas.............................................. 604
References ...................................................................................... 604
10 Jugular Foramen Tumors............................................................ 609
General Features ............................................................................ 609
Neuroimaging ................................................................................ 609
Clinical Presentation and Diagnosis .............................................. 610
Management................................................................................... 611
Operative Approaches ................................................................ 611
Endoscope-Assisted or Pure Endoscopic Tumor Removal ........ 617
Extended Endoscopic Endonasal Approach .............................. 625
Postoperative Care and Outcome ................................................... 625
Lower Cranial Nerves Dysfunction............................................ 625
Postoperative Care ...................................................................... 625
Radiotherapy/Radiosurgery........................................................ 626
Jugular Foramen Schwannomas..................................................... 627
Classification .............................................................................. 627
Neuroimaging ............................................................................. 629
Management ............................................................................... 637
Hypoglossal Nerve Schwannomas ................................................. 664
Glomus Jugulare Tumors ............................................................... 664
General Features......................................................................... 664
Pathogenesis and Classification ................................................. 664
Clinical Presentation .................................................................. 666
Management ............................................................................... 668
Meningiomas of the Jugular Foramen ........................................... 686
xiv Contents

General Features......................................................................... 686

Neuroimaging ............................................................................. 686
Clinical Manifestation ................................................................ 691
Operative Treatment ................................................................... 691
References ...................................................................................... 698
11 Other Cerebellopontine Angle Tumors ...................................... 703
Intra-Axial Tumors Extending Secondarily to the
Cerebellopontine Angle ................................................................. 703
Brain Stem Gliomas ................................................................... 703
Choroid Plexus Papillomas ........................................................ 714
Ependymomas ............................................................................ 718
Subependymomas ...................................................................... 726
Medulloblastomas ...................................................................... 730
Lymphomas ................................................................................ 730
Rare Extra-axial Cerebellopontine Angle Lesions ........................ 730
Arachnoid Cysts ......................................................................... 731
Dermoids .................................................................................... 740
Hamartomas ............................................................................... 744
Lipomas ...................................................................................... 748
Neurenteric Cysts ....................................................................... 749
Hemangioblastomas ................................................................... 757
CP Angle Metastases.................................................................. 764
Collision Tumors ........................................................................ 767
Vascular Lesions......................................................................... 773
Neurocysticercosis ..................................................................... 788
Temporal Bone and Petrous Apex Lesions .................................... 788
Cholesterol Granulomas of the Petrous Apex ............................ 788
Chordomas ................................................................................. 798
Chondrosarcomas ....................................................................... 807
Chondroblastomas ...................................................................... 818
Endolymphatic Sac Tumors ....................................................... 818
Fibrous Dysplasia ....................................................................... 834
Osteomas .................................................................................... 839
Pleomorphic Adenomas ............................................................. 840
Intralabyrinthine Schwannomas ................................................. 844
References ...................................................................................... 844
12 Management of Facial Palsy ....................................................... 855
Restoration of Facial Nerve Function ............................................ 855
Principles of Nerve Anastomosis ............................................... 855
Facial Nerve Reconstruction ...................................................... 855
Facial Nerve Reanimation with a Donor Nerve
(or Indirect Facial Nerve Reconstruction).................................. 862
Plastic Surgical Procedures ............................................................ 868
Physiotherapy ................................................................................. 869
Eye Care ......................................................................................... 869
References ...................................................................................... 870
Index ..................................................................................................... 873
 History of Cerebellopontine
Angle Surgery
During different historical phases, the tumors
originating from the eighth nerve have been
referred to as perineural fibroblastomas, neuri-
lemoma, schwannomas, acoustic neurinomas,
acoustic neuromas, and vestibular schwanno-
mas (VS). The last term corresponds most pre-
cisely to the pathogenetic tumor features and is
currently generally accepted. Although it was
introduced in the early twentieth century, we will
use it throughout the text, even in the histori-
cal part. The term “acoustic neuroma,” which is
popular in North America, is a misnomer—the
tumor does not originate from the acoustic nerve
and neuromas arise only from a cut peripheral
nerve. In 1902, Henneberg and Koch introduced
the definition cerebellopontine angle (CP angle)
or “Kleinhirnbrückenwinkel” (in the German
nomenclature), when presenting two patients
with bilateral vestibular schwannomas.
The history of CP angle tumors’ surgery can
be divided in several periods:
I period—description of CP angle tumors and
first attempts at surgery
II period—improvement of the surgical outcome
and introduction of the standard of complete
tumor removal
III period—development of operative techniques
to preserve the facial nerve
IV period—modern period of function preserving
safe CP angle surgery
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_1, © Springer-Verlag Berlin Heidelberg 2013
1
Description of CP Angle Tumors
and First Attempts at Surgery
Eduard Sandifort—a professor of anatomy in
Leyden—made the first description of presump-
tive VS in 1777. He described at autopsy a small
firm tumor adherent to the acoustic nerve, com-
pressing the brain stem and extending into the
IAC: an “acoustic neuroma” [1, 49]. In 1822, the
Scottish physician J. H. Wishart reported the first
case of bilateral VSs [57]. He published a case
report about a patient with blindness, deafness,
facial twitching, and unrelenting vomiting and
headaches. The patient died during surgery at the
age of 21 years. At autopsy, Wishart found sev-
eral skull base tumors, including two attached to
both seventh cranial nerves. In 1830, Sir Charles
Bell made the first definitively accurate postmor-
tem description of VS [2, 29].
During these initial years, the correct diagno-
sis of CP angle tumors was possible only at
autopsy. Jean Cruveilhier—professor of patho-
logical anatomy in Paris—was the first to describe
in detail the progression of clinical symptoms in
a 26-year-old patient with such tumor [8, 43]. He
believed that the lesion was located at the skull
base but was unable to localize it correctly until
autopsy. The autopsy findings were consistent
with a VS. Oppenheim in Germany was the first
to make the correct diagnosis antemortem based
1
2 1
on the typical clinical symptoms in 1890 [34].
In the second half of the nineteenth century, the
neurologists begun to understand that patients
with unilateral deafness, facial numbness, and
progressive blindness most probably have a CP
angle tumor. Only in the last two decades of the
century, however, the diagnosis became accurate
enough to justify surgery [5]. In 1910, the
Swedish pathologist Folke Henschen described
the involvement of the IAC by VSs [21].
Von Bergmann in Germany undertook the
earliest attempt at CP angle surgery in 1890. He
performed an unsuccessful attempt to remove a
tumor, which at autopsy turned out to be VS. One
year later, Charles McBurney of New York, after
whom the appendectomy incision is named,
performed an exploratory posterior fossa craniec-
tomy for a presumed cerebellar tumor [28]. The
first successful complete removal of a CP angle
tumor was performed by Sir Charles Balance, a
classmate and coworker of Viktor Horsley at the
Brown Institute and the National Hospital, Queen
Square in London. A 49-year-old woman pre-
sented with 1-year history of vertigo, headache,
vomiting, gait instability, progressive visual loss,
and unilateral deafness [56]. Presumptive diag-
nosis of acoustic tumor was made and she has
been operated in two stages: on November 19,
1894, a right posterior fossa craniectomy was
done, and 1 week later, the tumor was approached
and removed with the finger inserted in an unster-
ile fashion between the pons and the petrous
bone. According to the original description, the
tumor was well encapsulated, firm, and had a
wide attachment to the dura of the petrous bone
[4]. The patient recovered from surgery and was
alive for at least 18 years. She had facial anesthe-
sia and complete facial palsy, which led to ulcer-
ation and ultimately to removal of her right eye.
Later on, a controversy ensued on the issue of the
pathological nature of this tumor. Dandy was
convinced that the tumor was an acoustic tumor,
while Cushing contended that it was rather a
meningioma, basing his opinion on the assump-
tion that the patient had normal preoperative
auditory function [9, 37]. In 1923, Balance him-
self, as President of the Section of Otology of the
Royal Society of Medicine, related that he had
History of Cerebellopontine Angle Surgery
seen his first case of acoustic tumor in 1887 at
St. Thomas’ Hospital, although did not even think
of surgery at that time [29].
The credit for the first removal of a VS belongs
probably to Thomas Annandale of Edinburgh
[48]. In 1895, he succeeded in removing a tumor
“the size of a pigeon’s egg” in a young woman
via a unilateral suboccipital craniotomy. Although
her clinical history and the operative findings
suggested that the lesion must have been a VS,
some authors argued later that it has been actu-
ally a cerebellar tumor.
Victor Horsley in 1905 performed a gross total
removal of a VS at the National Hospital in
London. The patient lived for several years but
was seriously crippled due to a supposed brain
stem ischemia resulting from injury to the AICA.
In the same year, Borchardt performed the first
“transsigmoid” VS surgery [7]. Fedor Krause
was chief of surgery in the Augusta Hospital in
Berlin and professor at Friedrich-Wilhelms
University. The approach to the CP angle, which
he described in 1903, was a unilateral suboccipi-
tal osteoplastic flap and is considered the
predecessor of the current retrosigmoid approach
[31]. Krause reported that VSs could be removed
by surgery but described them as the most prob-
lematic of all brain tumors [52]. Interestingly, he
was the first to use faradic stimulation to differen-
tiate the facial from the audiovestibular nerve in a
case of tinnitus when he performed an eighth cra-
nial nerve section [53]. Garré in 1903 and Biggs
in 1909 were the first to attempt surgery for bilat-
eral VSs, with dismal outcome in both cases [1].
In 1913 at the International Congress of Medicine
in London, three of the most outstanding European
surgeons—Horsley of London, von Eiselsberg of
Vienna, and Krause of Berlin—reported their early
experience with VS surgery. The mortality rates
were 67 % (in the Horsley’s series of 15 patients),
77 % (in the Eiselsberg’s series of 17 patients), and
84 % (in the Krause’s series of 31 patients) [2].
They used similar technique of unilateral suboc-
cipital approach and the index finger or a spatula
to remove the tumor quickly. The speed of tumor
removal was of primary importance because of
the profuse bleeding and the limited possibilities
for hemostasis (obtained by packing the CP angle
Improvement of the Surgical Outcome and Introduction of the Standard of Complete Tumor Removal
with gauze). The CP angle earned later the name
“bloody angle” by Harvey Cushing [9, 10]. Some
decades later, Horrax introduced another famous
description of VSs: the “yellow monsters” [22].
The survivors usually suffered severe neurological
deficits. Eiselsberg and Horsley attempted later a
2-stage removal of VS without better success.
The reasons for the poor initial results were
the limited technical abilities, lack of reliable
anesthesia, and delayed tumor diagnosis. Most of
the patients presented late with very large tumors,
had severe deficits and hydrocephalus, and were
moribund [29]. The increased intracranial pres-
sure could be hardly controlled and “the surgeon
often struggled to achieve sufficient tumor expo-
sure using rudimentary methods of illumination
and hemostasis” [2]. Frequently, the cerebellum
swelled so massively that it was necessary “to
shave off the excess.”
Gradually, the knowledge of the anatomy and
pathology increased and new operative approaches
and techniques have been introduced. De Martel,
a pioneer of French neurosurgery, introduced the
sitting position for posterior fossa surgery [29].
In 1916, F. Henschen published his major contri-
bution to the histology and pathogenesis of CP
angle tumors, in particular of a clinical series
including 245 verified cases of unilateral and 24
bilateral VSs [21]. At autopsy, he discovered that
VSs originate from the vestibular portion of the
acoustic nerve, lying in the porus acusticus [1].
H. Stenvers of Utrecht (1917) and E. Towne of
San Francisco (1926) described the enlargement
of the porus of the IAC, seen on conventional
X-ray examinations.
Improvement of the Surgical Outcome
and Introduction of the Standard
of Complete Tumor Removal
Harvey Cushing who established new standards
of surgery in the posterior fossa and reduced to
an acceptable level the complications and the
mortality rates of VS surgery made the next
major contribution to CP angle surgery. In 1917,
he published a description of his experience with
3
30 operated patients with VS cases in his famous
monograph Tumors of the Nervus Acusticus and
the Syndrome of the Cerebellopontine Angle, in
which he reported remarkably lower mortality
rates than those of his predecessors [9]. In 1917,
his mortality rate was 20 % and was further
reduced to 4 % by 1936. Cushing considered
that the safe complete removal of VS is an unat-
tainable goal with the available anesthesia, diag-
nostic, and operative techniques and focused on
the need for decompression of the brain stem
and avoidance of medullary compression dur-
ing surgical manipulations. As usual for that
period, all patients were diagnosed late, all had
signs of increased intracranial pressure, and half
were blind. Cushing emphasized the importance
of controlling intracranial pressure by using a
ventricular tap. He performed a large curvilin-
ear incision extending between the two mastoid
processes, followed by a midline incision down
to the spinous process of the upper cervical ver-
tebrae [34]. Then, he made bilateral suboccipi-
tal craniectomy that allowed him to explore both
CP angles, considering the frequently unreliable
diagnostic findings. Furthermore, an osseous
suboccipital decompression was achieved. The
bone opening allowed him to access early the
cerebellomedullary cistern to drain CSF and to
perform decompression of the tonsils. Cushing
was the first to apply gentle and meticulous
technique and to monitor the changes of vital
signs during surgery. He operated consciously
without unjustified emphasis on the speed and
paid great attention to hemostasis, using bone
wax, silver vessel clips, and electrocautery.
Despite the good early results, the intracapsular
removal was associated with an expectedly high
tumor recurrence rate and the 5-year mortality
rate reached 54 % [9, 10].
Walter Dandy, a student of Cushing, consid-
ered this outcome in case of benign tumors as
unacceptable and favored total VS removal
[11, 12]. He believed that the residual tumor cap-
sule could be removed, thus achieving better
long-term results. Dandy used initially the bilat-
eral suboccipital craniectomy of Cushing and
performed extensive intracapsular decompres-
sion, followed by careful excision of the tumor
4 1
capsule. He emphasized the importance of metic-
ulous clipping of the veins and arteries surround-
ing the capsule in order to avoid hemorrhagic
complications. Dandy was the first surgeon to
achieve total removal of VS with relatively low
operative mortality.
This next stage in the history of VS surgery
was possible due to the advances in intraopera-
tive technology and the earlier detection of such
tumors: the syndrome of the CP angle was widely
recognized and the introduced by Dandy in 1918
air ventriculography allowed neurosurgeons to
detect smaller tumors [2]. His preliminary report
on five patients was published in 1922 and was
followed 3 years later by a detailed follow-up,
including an extensive discussion on the benefits
of total resection [11, 12]. Dandy continued to
improve his technique of tumor removal and in
1934 described a less invasive unilateral suboc-
cipital approach, actually similar to the one of
Krause [13]. In 1941, he reported on 46 patients
with VSs, removed totally with an operative
mortality rate of 10.87 % [14].
Interestingly, Cushing never agreed officially
with the concept of Dandy, probably due to their
untoward personal relationship. He maintained
his belief that the mortality rate is too high to jus-
tify total VS resection. Horrax and Poppen applied
the technique of Dandy and in 1949 published
their experience, reporting a 5-year mortality rate
of 12.7 % [22]. They could preserve the facial
nerve just in one of the 46 operated cases. The
technique of Dandy was refined further by
McKenzie and Alexander, who placed the patients
in a seated position [35]. Adson and Bucy propa-
gated a straight lateral incision, avoiding the bulky
musculocutaneous flap of the Dandy [5].
Development of Operative
Techniques to Preserve the Facial
Nerve
W. Dandy considered the facial palsy as “a neces-
sary sequel of the operation.” The next major
breakthrough in CP angle tumor surgery was the
successful facial nerve preservation. In 1931, Sir
Hugh Cairns of London became the first surgeon
History of Cerebellopontine Angle Surgery
to preserve the function of the facial nerve while
performing total VS removal. Herbert Olivecrona
emphasized the importance of facial nerve pres-
ervation. He routinely performed electrical stim-
ulation to identify the nerve and had a special
nurse to observe the patient for facial contrac-
tions [28, 55]. In 1939 Olivecrona presented his
initial results, demonstrating that the facial nerve
was preserved in an astonishing for the time rate
of 65 % of the patients [39]. Later on, he described
a series of 300 patients with VSs: half of the
tumors were removed totally; the facial nerve
function was preserved in 69 of them [40]. The
mortality rate in the Olivecrona series, however,
was still very high—29 %.
One reason for these poor results was the
lack of sufficient knowledge of the anatomi-
cal structure of the CP angle, especially that of
its vasculature. In 1949, Atkinson published his
study on the distribution of the AICA and—in
particular—on its relation to VSs [3]. He stressed
the importance of its preservation and described
the dramatic sequelae of its injury.
Translabyrinthine Approach
Parallel to the development of the suboccipital
approach, alternative techniques have been devel-
oped in an attempt to avoid the complications
related to cerebellar retraction and the difficulty
to work at the long working distance with poor
illumination and visualization. The anatomy of
the bony labyrinth was extensively studied in the
late nineteenth century and the otologists became
increasingly familiar with it [37]. The first laby-
rinthectomy was performed in 1895 by the
German otologist Albert Jansen for a suppurative
disease. Rudolf Panse from Dresden developed
the concept of using this operative approach to
expose the CP angle [37] and published it in 1904
[41]. Although his proposal was to chisel out the
mastoid bone and to remove the cochlea, he never
performed such surgery personally. Charles
Elsberg of New York performed cadaver dissec-
tions and measurements and found that the
shortest route to the CP angle started 1–2 cm
behind the ear: the route through the mastoid.
Modern Period of Functional Preserving Safe CP Angle Surgery
Franciscus H. Quix of Utrecht is credited for
the first translabyrinthine removal of a VS [47]. The
surgery was performed in two stages because
the initial operation had to be aborted due to
strong bleeding from the superior petrosal sinus.
The tumor could not be removed completely. The
patient survived the surgery but died 6 months
later. Later on in 1911 and 1912, Werner Kummel
and Ernst Schmiegelow reported their experience
with the approach [2].
The translabyrinthine approach could not be
established at that time because of the lack of
adequate surgical instruments and technique.
Furthermore, the leading neurosurgeons of the
time—such as Cushing and Dandy—found
it absolutely inappropriate. They stressed on
the poor surgical exposure provided by this
approach and the difficult reconstruction with
the related high likelihood of meningitis [37].
The approach was reintroduced by William F.
House in the 1960s who was unaware of the earlier
descriptions [25].
Transpetrosal Approach
Moritz Borchardt of Berlin in 1905 was the first to
realize the wide exposure gained by an extensive
removal of the petrous bone [7]. As recounted by
Ngyuen, he attempted to remove a cerebellar
tumor via the suboccipital approach but had to
stop because of excessive bleeding [37]. In order
to avoid the same delicate area, he resected the
temporal bone deep to the IAC. Once again, he had
to discontinue the surgery but at the third attempt
was able to remove the entire tumor. Later on, von
Eiselsberg of Vienna used similar combined sub-
occipital and transpetrosal approach in one patient
but could remove only a portion of the tumor.
Middle Fossa Approach
The middle fossa (MF) approach to the IAC
was described in 1904 by Parry who used it
for sectioning of the auditory nerve in a patient
with vertigo and tinnitus [42]. Later on—in
1938—Putman reported its utilization for the
5
treatment of recurrent vertigo [46], while in 1947,
Gardner used a similar technique to resect the
GSPN in a patient with unilateral headache [20].
Modern Period of Functional
Preserving Safe CP Angle Surgery
The greatest breakthrough in the surgical treat-
ment of VS that allowed achieving the current
standards was the introduction of the surgical
microscope and of the microsurgical technique. In
1957, Theodore Kurze resected for the first time a
VS with an operating microscope at the University
of Southern California. In 1958, William House
began using the microsurgical technique for tem-
poral bone dissections and applied it in 1961 dur-
ing a translabyrinthine surgery of a VS. His major
contribution was the utilization of the operating
microscope and high-speed drill. William House
himself learned microsurgery from Wullstein in
Siegen, who inaugurated microsurgery of oto-
sclerosis (Wullstein, personal communication
1971). This new technique allowed Dr. House to
remove the required amount of the petrous bone
safely and to expose widely the IAC. He refined
and established the translabyrinthine approach as
a technique to expose tumors of the CP angle and
IAC. In 1964, he published his experience with
the transtemporal bone microsurgical removal of
VSs [24, 25]. W. House elaborated and refined
the middle fossa technique in the late 1950s and
described systematically the relevant anatomical
landmarks [36]. In January 1960, he operated his
first VS using the middle fossa approach. Using
the MF approach in small tumors, House could
reduce the mortality to 5.4 % and preserve the
facial nerve in 95 % [23].
Pool and Pava in 1957 were the first to empha-
size the importance of tumor removal from the
IAC [45]. Rand and Kurze developed a technique
to remove the posterior meatal wall. In 1965, they
presented their experience on the microsurgical
suboccipital transmeatal approach, achieving
complete removal of the VS and preservation of
the cranial nerves [50]. In 1967, C. G. Drake
reported on his experience with the complete
6 1
removal of even large VS [17, 18]. He described
his techniques for preservation of the facial nerve
and for its reconstruction [17]. As stated by W.
Pellet, the transmeatal technique quickly gained
popularity among neurosurgeons as a means to
achieve complete tumor removal [44, 54]. The
opening or “trepanation” of the meatal wall was
performed with a gauge or drill.
M. G. Yasargil studied the microsurgical
anatomy of the CP angle and showed that during
the extension of VSs into the CP angle, a double
arachnoid membrane envelops the tumor. He
emphasized the significance of these arachnoid
layers as a safe dissection plane that allows for
preservation of even the finest structures [58, 59].
Major breakthrough was the introduction of intra-
operative facial nerve monitoring by Delgado
et al. in 1979, which opened the way for func-
tional preserving CP angle surgery [15]. The later
progress in the management of CP angle tumors
was related to the introduction of new diagnos-
tic modalities (CT and MRI), which allowed
earlier diagnosis, precise operative planning,
and detection of postoperative complications;
developments in neuroanesthesia and neuroin-
tensive care; establishing of reliable techniques
of continuous electrophysiological monitoring of
the function of all relevant cranial nerves; increas-
ing the knowledge of the normal and pathological
anatomy of the CP angle; and establishment of
the microneurosurgery. The competition between
surgeons using different approaches stimulated
the refinement of the operative techniques and
further improvement of the outcome.
Hearing Preservation
In 1954, Elliot and McKissick were the first to
report hearing preservation in two of three
patients after gross VS removal via suboccipital
approach [19]. Eleven years later—in 1965—
McKissick reported preserved hearing in 5 of 270
operated patients. Hullay and Tomits in 1965
could preserve hearing in 1/50 cases [27]. In
1968, Rand and Kurze presented two cases with
preserved hearing after complete tumor removal
History of Cerebellopontine Angle Surgery
using the microsurgical technique [51]. Rand was
the first to mention hearing preservation as treat-
ment goal. In 1978, M. G. Yasargil reported on
preserved hearing in 2/164 cases. In the same
year, Di Tullio et al. reported one case of hearing
preservation out of 79 surgeries [16].
W. House used the MF approach to preserve
hearing [44]. His first successful attempt was in
1964. In 1968, he reported on 7 cases of hear-
ing preservation from 19 attempts [26]. However,
House was against the attempts at systematic hear-
ing preservation in patients with VSs. Hearing
preservation candidates were selected carefully,
considering factors such as tumor size, position
of cranial nerves, and hearing level on the oppo-
site side. In 1982, at one conference, he reported
on a series of 200 patients operated via the MF
approach: he regarded as hearing-preservation
candidates only 10 and could preserve hearing
in 4 of them. At the same conference, the senior
author (MS) presented his initial experience with
200 patients with VSs with a hearing preserva-
tion rate of approximately 30 %.
Radiosurgery
In 1951, Lars Leksell described a new technique
for stereotactically focused irradiation as a method
to destroy intracranial targets—the gamma knife
[32]. He and E. O. Backlund performed the first
gamma knife procedure in 1967. In 1969, Leksell
performed the first gamma knife radiosurgical
procedure for a VS in a patient with NF2 [30, 33, 38].
Since then, radiation techniques and protocols
have continued to evolve; currently, radiosurgery
is increasingly used in patients with VS [6].
References
1. Ahn MS, Jackler RK, Lustig LR (1996) The early his-
tory of the neurofibromatosis. Evolution of the con-
cept of neurofibromatosis type 2. Arch Otolaryngol
Head Neck Surg 122(11):1240–1249
2. Akard W, Tubbs RS, Seymour ZA, Hitselberger WE,
Cohen-Gadol AA (2009) Evolution of techniques for
the resection of vestibular schwannomas: from saving
life to saving function. J Neurosurg 110(4):642–647
References
3. Atkinson WJ (1949) The anterior inferior cerebellar
artery; its variations, pontine distribution, and
significance in the surgery of cerebello-pontine angle
tumours. J Neurol Neurosurg Psychiatry 12(2):
137–151
4. Ballance C (1907) Some points in the surgery of the
brain and its membranes. Macmillan and Co, Ltd.,
London
5. Bambakidis NC, Kakarla UK, Kim LJ, Nakaji P,
Porter RW, Daspit CP, Spetzler RF (2007) Evolution
of surgical approaches in the treatment of petroclival
meningiomas: a retrospective review. Neurosurgery
61(5 Suppl 2):202–209
6. Bassim MK, Berliner KI, Fisher LM, Brackmann DE,
Friedman RA (2010) Radiation therapy for the
treatment of vestibular schwannoma: a critical evalua-
tion of the state of the literature. Otol Neurotol 31(4):
567–573
7. Borchardt M (1906) Ueber Operationen in der hin-
teren Schaedelgrube inkl. der Operationen der
Tumoren am Kleinhirnbrueckenwinkel. Arch Klin
Chir 81:386–432
8. Cruveilhier J (1829–1842) Anatomie pathologique du
corps humain, ou Descriptions, avec figures
lithographiées et coloriées, des diverses alterations
morbides dont le corps humain est susceptible, vol 2.
Bailière, Paris. Liv 32, Pl 2, pp 19–24; Liv 38, Pl 5,
pp 1–4
9. Cushing H (1917) Tumors of the nervus acusticus and
the syndrome of the cerebellopontine angle. WB
Saunders Co, Philadelphia
10. Cushing H (1921) Further concerning the acoustic
neuromas. Laryngoscope 31:209–228
11. Dandy W (1922) An operation for the total extirpation
of tumors in the cerebello-pontine angle. A prelimi-
nary report. Bull Johns Hopkins Hosp 33:344–345
12. Dandy W (1925) An operation for the total removal of
cerebellopontine (acoustic) tumors. Surg Gynecol
Obstet 41:129–148
13. Dandy W (1934) Removal of cerebellopontile (acous-
tic) tumors through a unilateral approach. Arch Surg
29:337–344
14. Dandy W (1941) Results of removal of acoustic
tumors by the unilateral approach. Arch Surg 42:
1026–1033
15. Delgado TE, Bucheit WA, Rosenholtz HR, Chrissian
S (1979) Intraoperative monitoring of facila muscle
evoked responses obtained by intracranial stimulation
of the facila nerve: a more accurate technique for
facila nerve dissection. Neurosurgery 4(5):418–421
16. DiTullio MV Jr, Malkasian D, Rand RW (1978) A
critical comparison of neurosurgical and otolaryngo-
logical approaches to acoustic neuromas. J Neurosurg
48(1):1–12
17. Drake CG (1967) Surgical treatment of acoustic neu-
roma with preservation or reconstitution of the facial
nerve. J Neurosurg 26(5):459–464
18. Drake CG (1967) Total removal of large acoustic neu-
romas. A modification of the McKenzie operation
7
with special emphasis on saving the facial nerve. J
Neurosurg 26(5):554–561
19. Elliott FA, McKissick KW (1954) Acoustic neuroma;
early diagnosis. Lancet 267(6850):1189–1191
20. Gardner WJ, Stowell A, Dutlinger R (1947) Resection
of the greater superficial petrosal nerve in the treat-
ment of unilateral headache. J Neurosurg 4(2):
105–114
21. Henschen F (1916) Zur Histologie und Pathogenese
der Kleinhirnbrüchenwinkeltumoren. Arch Psychiatr
Nervenkr 56:21–122
22. Horrax GPJ, Poppen JL (1949) The end results of
complete versus intracapsular removal of acoustic
tumors. Ann Surg 130:567–575
23. House WF (1963) Middle cranial fossa approach to
the petrous pyramid. Report of 50 cases. Arch
Otolaryngol 78:460–469
24. House WF (1964) Transtemporal bone microsurgical
removal of acoustic neuromas. Evolution of transtem-
poral bone removal of acoustic tumors. Arch
Otolaryngol 80:731–742
25. House WF (1964) Transtemporal bone microsurgical
removal of acoustic neuromas. Report of cases. Arch
Otolaryngol 80:617–667
26. House WF (1968) Acoustic neuroma. Case summa-
ries. Arch Otolaryngol 88(6):586–591
27. Hullay J (1958) Variety in the site of acoustic tumors
in the pontile angle; a preliminary report. Acta
Neurochir (Wien) 6(1–2):115–120
28. Jackler RK, Whinney D (2001) A century of eighth
nerve surgery. Otol Neurotol 22(3):401–416
29. Koerbel A, Gharabaghi A, Safavi-Abbasi S, Tatagiba
M, Samii M (2005) Evolution of vestibular schwan-
noma surgery: the long journey to current success.
Neurosurg Focus 18(4):e10. doi:180410 [pii]
30. Kondziolka D, Lunsford LD, Flickinger JC (2004)
Acoustic neuroma radiosurgery. Origins, contempo-
rary use and future expectations. Neurochirurgie
50(2–3 Pt 2):427–435
31. Krause F (1903) Zur Freilegung der hinteren
Felsenbeinflache und des Kleinhirns. Beitr Klin Chir
37:728–764
32. Leksell L (1951) The stereotaxic method and radio-
surgery of the brain. Acta Chir Scand 102(4):
316–319
33. Leksell L (1971) A note on the treatment of acoustic
tumours. Acta Chir Scand 137(8):763–765
34. Machinis TG, Fountas KN, Dimopoulos V, Robinson
JS (2005) History of acoustic neurinoma surgery.
Neurosurg Focus 18(4):e9
35. McKenzie KG, Alexander E Jr (1954) Acoustic neu-
roma. Clin Neurosurg 2:21–36
36. Monfared A, Mudry A, Jackler R (2009) The history
of middle cranial fossa approach to the cerebellopon-
tine angle. Otol Neurotol 31(4):691–696
37. Nguyen-Huynh AT, Jackler RK, Pfister M, Tseng J
(2007) The aborted early history of the translabyrin-
thine approach: a victim of suppression or technical
prematurity? Otol Neurotol 28(2):269–279
8 1
38. Niranjan A, Lunsford LD (2000) Radiosurgery: where
we were, are, and may be in the third millennium.
Neurosurgery 46(3):531–543
39. Olivecrona H (1939) Acoustic tumors. In: Winther
KKK (ed) III Congrès Neurologique International,
Copenhague, 21/8–25/8 1939. Comptes Rendus des
Séances. Einar Munksgaard, Copenhangue, pp 761–771
40. Olivecrona H (1950) Analysis of results of complete
and partial removal of acoustic neuromas. J Neurol
Neurosurg Psychiatry 13(4):271–272
41. Panse R (1904) Ein Gliom des Acusticus. Arch
Ohrenheilkd 61:251–255
42. Parry D (1904) A case of tinnitus treated by division of
the auditory nerve. J Laryngol Otol 19(19):402–406
43. Pearce JM (2003) Cruveilhier and acoustic neuroma.
J Neurol Neurosurg Psychiatry 74(8):1015
44. Pellet W (2008) History of vestibular schwannoma
surgery. Prog Neurol Surg 21:6–23
45. Pool JL, Pava AA (1957) The early diagnosis and
treatment of acoustic nerve tumors. Charles C Thomas,
Springfield
46. Putnam T (1938) Treatment of recurrent vertigo
(Meniere’s syndrome) by subtemporal destruction of
the labyrinth. Arch Otolaryngol 27:161–168
47. Quix F (1912) Ein fall von translabyrintharisch operier-
tem tumor acusticus. Verh Dtsch Otol Ges 21:245–255
48. Ramsden R (1995) ‘A brilliant surgical result, the first
recorded’: Annandale’s case, 3 May 1895. J Laryngol
Otol 109:369–373
49. Ramsden RT (1995) The bloody angle: 100 years of
acoustic neuroma surgery. J R Soc Med 88(8):
464P–468P
History of Cerebellopontine Angle Surgery
50. Rand RW, Kurze T (1965) Micro-neurosurgical resec-
tion of acoustic tumors by a transmeatal posterior
fossa approach. Bull Los Angel Neuro Soc 30:17–20
51. Rand RW, Kurze T (1968) Preservation of vestibular,
cochlear, and facial nerves during microsurgical
removal of acoustic tumors. Report of two cases.
J Neurosurg 28(2):158–161
52. Reidel C, Post KD (1993) Complications in the treat-
ment of acoustic neuromas. In: Post KD, Friedman E,
McCormick P (eds) Postoperative complications in
intracranial neurosurgery. Thieme, New York, pp
91–110
53. Rosegay H (1992) The Krause operations. J Neurosurg
76(6):1032–1036
54. Rougerie J, Guyot JF (1964) Attempted preservation
of the facial nerve in the ablation of neurinomas of the
cerebellopontile angle. Neurochirurgie 10:13–21
55. Sampath P, Holliday MJ, Brem H, Niparko JK, Long
DM (1997) Facial nerve injury in acoustic neuroma
(vestibular schwannoma) surgery: etiology and pre-
vention. J Neurosurg 87(1):60–66
56. Stone JL (1999) Sir Charles Ballance: pioneer British
neurological surgeon. Neurosurgery 44(3):610–631
57. Wishart J (1822) Case of tumours in the skull, dura
mater, and brain. Edinb Med Surg J 18:393
58. Yasargil M (1996) Acoustic neuromas. In: Microneuro-
surgery, vol IVb. Thieme, New York, pp 100–123
59. Yasargil MG (1999) A legacy of microneurosurgery:
memoirs, lessons, and axioms. Neurosurgery 45(5):
1025–1092
 Microsurgical Anatomy
of the Cerebellopontine Angle
by the Retrosigmoid Approach
Posterior Cranial Fossa
The posterior cranial fossa, the largest and deepest
of the three cranial fossae, contains the most com-
plex of the intracranial anatomy. It extends from
the tentorial incisura, through which it communi-
cates with the supratentorial space, to the foramen
magnum, through which it communicates with
the spinal canal. The upper surface of the cerebel-
lum is separated from the supratentorial space by
the tentorium cerebelli. Its intracranial surface is
penetrated by the jugular foramen, internal acous-
tic meatus, hypoglossal canal, the vestibular and
cochlear aqueducts, and several venous emissary
foramina. Here, in approximately one-eighth the
intracranial space, are found the pathways regulat-
ing consciousness, vital autonomic functions, and
motor activities and sensory reception for the head,
body, and extremities, in addition to the centers for
controlling balance and gait. Only two of the 12
pairs of cranial nerves are located entirely outside
the posterior fossa (Fig. 2.1). The posterior fossa
is strategically situated at the outlet of the cerebro-
spinal fluid flow from the ventricular system. The
arterial relationships are especially complex with
the vertebral and basilar arteries having relatively
inaccessible segments deep in front of the brain
stem and the major cerebellar arteries coursing in
relation to multiple sets of cranial nerves before
reaching the cerebellum. The most commonly
utilized operative route to the posterior fossa is a
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_2, © Springer-Verlag Berlin Heidelberg 2013
2
retrosigmoid approach directed through the cer-
ebellopontine angle.
The Cerebellopontine Angle
The cerebellopontine angle is located between the
superior and inferior limbs of the cerebellopon-
tine fissure, a V-shaped angular cleft, formed by
the cerebellum folding around the pons and mid-
dle cerebellar peduncle (Fig. 2.1) [33, 38, 40].
The cerebellopontine angle faces the posterior
surface of the temporal bone. The middle cerebel-
lar peduncle fills the interval between the superior
and inferior limbs of the angle (fissure). The apex
of the angle is located laterally where the superior
and inferior limbs meet. The fourth through the
eleventh cranial nerves are located near or within
the angular space between the two limbs (Figs. 2.1,
2.2, and 2.3). The trochlear and trigeminal nerves
are located near the angle’s superior limb, and the
glossopharyngeal, vagus, and accessory nerves
are located near the inferior limb. The facial and
acousticovestibular nerves arise near the central
part of the CP angle. The abducens nerve is
located near the base of the fissure, along a line
connecting the anterior ends of the superior and
inferior limbs. The lateral recess and the foramen
of Luschka open into the middle part of the infe-
rior limb. Other structures located along the mid-
dle part of the inferior limb are the flocculus, the
9
10 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a b
Fig. 2.1 (a) The petrosal surface of the cerebellum faces
forward toward the posterior surface of the temporal
bone. It is the surface that is elevated to reach the cerebel-
lopontine angle. The cerebellopontine fissure, also called
the cerebellopontine angle, a V-shaped fissure formed by
the cerebellum wrapping around the pons and middle cer-
ebellar peduncle, has superior and inferior limbs that
define the margins of the cerebellopontine angle. Cranial
nerves V–XI arise in or near the cerebellopontine fissure
or angle. The superior limb extends above the trigeminal
nerve, and the inferior limb passes below the flocculus
and the nerves that pass to the jugular foramen. The supe-
rior and inferior limbs meet laterally at the apex located
at the anterior end of the petrosal fissure that divides the
petrosal surface of the cerebellum into superior and infe-
rior parts. The fourth ventricle is located behind the pons
and medulla. The midbrain and pons are separated by the
pontomesencephalic sulcus and the pons and medulla by
the pontomedullary sulcus. The trigeminal nerves arise
from the midpons. The abducens nerve arises in the
medial part of the pontomedullary sulcus, rostral to the
medullary pyramids. The facial and vestibulocochlear
nerves arise at the lateral end of the pontomedullary
sulcus immediately rostral to the foramen of Luschka.
The hypoglossal nerves arise anterior to the olives, and the
glossopharyngeal and vagus nerves arise posterior to the
olives. The flocculus and choroid plexus protrude from
the foramen of Luschka behind to the glossopharyngeal
and vagus nerves. The foramen of Luschka opens into the
cerebellopontine angle below the junction of the facial
and vestibulocochlear nerves with the lateral end of the
pontomedullary sulcus. Choroid plexus protrudes from
the lateral recess and foramen of Luschka behind the
glossopharyngeal, vagus, and accessory nerves. (b)
Enlarged view of the right cerebellopontine angle. The
cerebellopontine fissure, which is most commonly
referred to as the cerebellopontine angle, is a V-shaped
fissure formed where the cerebellum wraps around the
pons and middle cerebellar peduncle. The flocculus and
choroid plexus extend laterally from the foramen of
Magendie above the lower limb of the fissure. The facial
and vestibulocochlear nerves join the brain stem 2 or
3 mm rostral to the glossopharyngeal nerve along a line
drawn dorsal to the olive along the origin of the rootlets
of the glossopharyngeal, vagus, and accessory rootlets.
(c) Cerebellopontine angle seen through a right
retrosigmoid approach. The petrosal surface of the
cerebellum faces forward toward the posterior surface of
the temporal bone. It is the surface that is elevated to
reach the cerebellopontine angle. The cerebellopontine
fissure, the V-shaped fissure formed by the cerebellum
wrapping around the pons and middle cerebellar pedun-
cle, has superior and inferior limbs that define the mar-
gins of the cerebellopontine angle. Cranial nerves V–XI
arise in or near the cerebellopontine angle. The superior
limb extends above the trigeminal nerve, and the inferior
limb passes below the flocculus and the cranial nerves IX,
X, and XI that pass to the jugular foramen. The flocculus
protrudes from the foramen of Luschka behind cranial
nerves VII and VIII. A large superior petrosal vein with
multiple tributaries passes behind the trigeminal nerve.
The trochlear nerve courses below the superior cerebellar
artery. The anteroinferior cerebellar artery passes between
the facial and vestibulocochlear nerves and turns back-
ward to course along the middle cerebellar peduncle and
cerebellopontine fissure. (d) The cerebellum has been
removed in this specimen to expose the dura covering the
part of the posterior fossa that faces the cerebellopontine
angle and is exposed in the retrosigmoid approach. The
fourth ventricle sits on the posterior surface of the pons
and medulla. The flocculus projects laterally into the cer-
ebellopontine angle. The glossopharyngeal, vagus, and
accessory nerves enter the jugular foramen. The superior
cerebellar artery is at the upper margin of the exposure.
The posteroinferior cerebellar artery courses around the
glossopharyngeal, vagus, and accessory nerves. The
endolymphatic sac sits beneath the dura inferolateral to
the internal acoustic meatus. (e) The supra- and infraten-
torial areas have been exposed while preserving the
bone at the site of the sutures. The asterion, located at the
junction of the lambdoid, occipitomastoid, and pariet-
omastoid sutures, usually overlies the lower half of the
junction of the transverse and sigmoid sinuses. The verti-
cal lateral suboccipital incision for the retrosigmoid
approach usually crosses the asterion. The burr hole for
elevating a suboccipital bone flap is usually placed at the
lower edge of the asterion. The junction of the supramas-
toid crest and the squamosal suture is located at the pos-
terior edge of the middle fossa and slightly anterior and
above the junction of the transverse and sigmoid sinuses.
The supramastoid crest is an inferior continuation of the
superior temporal line (From Rhoton [33])
The Cerebellopontine Angle
c d
Fig. 2.1 (continued)
rhomboid lip, and the choroid plexus protruding
from the foramen of Luschka.
Optimizing operative approaches to the cer-
ebellopontine angle requires an understanding of
the relationships of the cerebellar arteries, cranial
nerves, brain stem, cerebellar peduncles, fissures
between the cerebellum and brain stem, and the
cerebellar surfaces. When examining these relation-
ships, three neurovascular complexes are defined:
an upper complex related to the superior cerebellar
artery (SCA), a middle complex related to the antero-
inferior cerebellar artery (AICA), and a lower com-
plex related to the posteroinferior cerebellar artery
(PICA). Other structures, in addition to the three
cerebellar arteries, occurring in sets of three in the
posterior fossa that bear a consistent relationship to
the SCA, AICA, and PICA are the parts of the brain
stem (midbrain, pons, and medulla); the cerebel-
lar peduncles (superior, middle, and inferior); the
fissures between the brain stem and the cerebellum
(cerebellomesencephalic, cerebellopontine, and
cerebellomedullary); and the surfaces of the cere-
bellum (tentorial, petrosal, and suboccipital). Each
neurovascular complex includes one of the three
11
parts of the brain stem, one of the three surfaces of
the cerebellum, one of the three cerebellar pedun-
cles, and one of the three major fissures between
the cerebellum and the brain stem. In addition, each
neurovascular complex contains a group of cranial
nerves. The upper complex includes the oculo-
motor, trochlear, and trigeminal nerves that are
related to the SCA. The middle complex includes
the abducens, facial, and vestibulocochlear nerves
that are related to the AICA. The lower complex
includes the glossopharyngeal, vagus, accessory,
and hypoglossal nerves that are related to the PICA.
The structures in each complex are reviewed next.
Cerebellar Surfaces
The cerebellar surfaces are divided on the basis of
the structures they face, or along which they may
be exposed. The first surface, the tentorial surface,
faces the tentorium and is retracted in a supracer-
ebellar approach; the second surface, the suboc-
cipital surface, is located below and between the
lateral and sigmoid sinuses and is exposed in
12 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a b
Fig. 2.2 (a) Upper part of the cerebellopontine angle.
A large superior petrosal vein with multiple tributaries,
including the pontotrigeminal and transverse pontine
veins and the vein of the cerebellopontine fissure, passes
behind the trigeminal nerve. The trochlear nerve courses
below the superior cerebellar artery. The anteroinferior
cerebellar artery passes between the facial and vestibulo-
cochlear nerves and turns medially to course along the
middle cerebellar peduncle and cerebellopontine fissure.
(b) The flocculus and choroid plexus, which protrude
from the foramen of Luschka, have been elevated to
expose the junction of the facial and vestibulocochlear
nerves with the brain stem. The facial nerve is exposed
below the vestibulocochlear nerve. A branch of the
anteroinferior cerebellar artery gives rise to both the sub-
arcuate and labyrinthine arteries. The subarcuate artery
enters the dura and bone superolateral to the meatus. The
junction of the facial nerve with the brain stem is easier
to expose from below rather than above the flocculus and
vestibulocochlear nerve in an operation for hemifacial
spasm. This approach for decompressing the facial nerve
in hemifacial spasm is referred to as an “infrafloccular
approach.” A large posteroinferior cerebellar artery loops
upward behind the vestibulocochlear nerve. (c) The dura
lining the posterior wall of the internal acoustic meatus
has been removed, and the posterior meatal wall opened
to expose the dura lining the meatus. The subarcuate
artery usually has to be obliterated and divided prior to
removing the posterior meatal wall. Two bundles from
the nervus intermedius are exposed above the vestibulo-
cochlear nerve. Care is taken to avoid entering the semi-
circular canals and vestibule during drilling of the
posterior wall of the meatus if hearing is to be preserved.
(d) The meatal dura has been opened, the facial nerve
has been elevated, and the vestibulocochlear nerve
depressed to expose the facial nerve coursing in the
anterosuperior quadrant of the meatus. The nervus inter-
medius, which arises along the anterior surface of the
vestibulocochlear nerve and passes laterally to join the
facial nerve, is composed of several rootlets, as is com-
mon. The superior vestibular nerve passes posterior to
the facial nerve, and the cochlear nerve is hidden anterior
to the inferior vestibular nerve. (e) The cleavage plane
between the superior and inferior vestibular nerves has
been developed. The superior vestibular and facial nerves
pass above and the inferior vestibular and cochlear
nerves pass below the transverse crest. The facial nerve
courses anterior to the superior vestibular nerve, and the
cochlear nerve is located anterior to the inferior vestibu-
lar nerve. The vertical crest separates the superior ves-
tibular and facial nerves at the fundus of the meatus
(From Rhoton [33])
The Cerebellopontine Angle
d e
Fig. 2.2 (continued)
a b
Fig. 2.3 Retrosigmoid exposure of the nerves in the
right cerebellopontine angle. (a) The vestibulocochlear
nerve enters the internal acoustic meatus with a labyrin-
thine branch of the AICA. The PICA courses around
the glossopharyngeal, vagus, and accessory nerves. The
abducens nerve ascends in front of the pons. A subarcu-
ate artery enters the subarcuate fossa superolateral to
the porus of the meatus. Choroid plexus protrudes into
the cerebellopontine angle behind the glossopharyngeal
and vagus nerves. (b) The posterior wall of the internal
acoustic meatus has been removed. The cleavage plane
between the upper bundle, formed by the superior ves-
tibular nerve, and the lower bundle, formed by the infe-
rior vestibular and cochlear nerves, was begun laterally
where the nerves have separated near the meatal fundus
and extended medially. The nervus intermedius arises
13
on the anterior surface of the vestibulocochlear nerve,
has a free segment in the cistern and/or meatus, and
joins the facial nerve distally. The facial nerve is located
anterior to the superior vestibular nerve and the cochlear
nerve is anterior to the inferior vestibular nerve. (c) The
cleavage plane between the cochlear and inferior
vestibular nerves, which is well developed in the lateral
end of the internal acoustic meatus, has been extended
medially. Within the cerebellopontine angle, the supe-
rior vestibular nerve is posterior and superior, the facial
nerve anterior and superior, the inferior vestibular nerve
posterior and inferior, and the cochlear nerve anterior
and inferior. (d) The superior and inferior vestibular
nerves have been divided to expose the facial and
cochlear nerves. A labyrinthine branch of the PICA
enters the internal meatus (From Rhoton [40])
14 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

c d

Fig. 2.3 (continued)

a suboccipital craniectomy; and the third surface,
the petrosal surface, faces forward toward the pos-
terior surface of the petrous bone and is elevated to
expose the cerebellopontine angle. Each of the
surfaces has the vermis in the midline and the
hemispheres laterally (Figs. 2.4, 2.5, and 2.6).
Cerebellar Brain Stem Fissures
The three fissures formed by the embryological
folding of the cerebellum around the brain stem
are the cerebellomesencephalic fissure, which
extends inferiorly between the cerebellum and

a b

Fig. 2.4 SCA. (a) Superior view. The SCA arises at the
level of the midbrain and dips caudally along the pon-
tomesencephalic junction to enter the cerebellomedullary
fissure and supply the tentorial surface of the cerebellum.
(b) Lateral view of the right pontomesencephalic junction
showing the SCA dipping down toward the trigeminal
nerve and dividing into a rostral and caudal trunk. (c)
Anterior view. The SCA arises anterior to the midbrain
and loops downward to the level of the junction of the
trigeminal nerve with the midpons. (d) Posterolateral
view. The SCA bifurcates early and both the rostral and
caudal trunks dip into the cerebellomesencephalic fissure.
(e) The superior lip of the cerebellomesencephalic
fissure has been removed to expose the SCA within the fissure
where it gives branches that continue down the superior
cerebellar peduncle to the dentate nucleus. (f) Superior
view showing the cortical branches of the SCA supplying
the tentorial surface of the cerebellum
The Cerebellopontine Angle 15

c d

e f

Fig. 2.4 (continued)

a b

Fig. 2.5 AICA. (a) Anterior view. The AICA arises at
the pontine level and passes below CN VI, above the
flocculus, and between CNs VII and VIII to run on
the middle cerebellar peduncle, which forms the floor of the
cerebellopontine angle. (b) Enlarged view. The cerebel-
lopontine angle is formed by the cerebellum folding
around the pons to form an angular cleft with the flocculus
and CNs VII and VIII in the center. The fissure has upper
and lower limbs that meet at a lateral apex. (c) View
through a retrosigmoid craniotomy. The AICA passes
between CNs VII and VIII, above the flocculus on the
surface of the middle cerebellar peduncle, and toward the
apex of the cerebellopontine fissure. Its cortical branches
supply the petrosal surface of the cerebellum. (d) Posterior
view with the posterior meatal lip removed to show the
AICA looping into the porus of the meatus. (e) Right ante-
rolateral view of the AICA passing along the cerebello-
pontine fissure and supplying the petrosal cerebellar
surface. (f) Anterolateral view of another brain stem and
cerebellum showing the AICA passing above CNs VII
and VIII and along the cerebellopontine fissure to supply
the petrosal surface
16 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
c d
e f
Fig. 2.5 (continued)
mesencephalon and is intimately related to the
superior half of the roof of the fourth ventricle;
the cerebellopontine fissure, which is formed by
the folding of the cerebellum around the lateral
sides of the pons and is intimately related to the
lateral recesses of the fourth ventricle; and the
cerebellomedullary fissure, which extends supe-
riorly between the cerebellum and the medulla
and is intimately related to the inferior half of the
roof (Figs. 2.4, 2.5, and 2.6). Each fissure com-
municates with the adjacent fissure. The cerebel-
lopontine fissures are continuous around the
rostral surface of the middle cerebellar peduncles
with the caudal edges of the cerebellomesenceph-
alic fissure and around the caudal margin of the
middle cerebellar peduncles with the rostral lim-
its of the cerebellomedullary fissure. The external
or cisternal surfaces of the structures forming the
roof of the fourth ventricle are intimately related
to the fissures between the cerebellum and brain
stem. One of the three cerebellar arteries courses
in each fissure. The SCA courses within the cer-
ebellomesencephalic fissure, the AICA is related
to the cerebellopontine fissure (also called the
cerebellopontine angle), and the PICA is inti-
mately related to the cerebellomedullary fissure.
Cerebellar Arteries
The AICA is the cerebellar artery most intimately
related to the cerebellopontine angle, but a segment
of the SCA and PICA commonly enters the angle
especially if they have a tortuous course [39].
The SCA is intimately related to the cerebellom-
esencephalic fissure, the superior half of the fourth
ventricular roof, the superior cerebellar peduncle,
and the tentorial surface (Fig. 2.4). The SCA arises
in front of the midbrain, usually from the basilar
artery near the apex, and passes below the oculomo-
tor nerve, but may infrequently arise from the proxi-
mal PCA and pass above the oculomotor nerve.
The Cerebellopontine Angle
a b
c
e
Fig. 2.6 PICA. (a) Left posterolateral view. The cerebel-
lum was removed by dividing the cerebellar peduncles.
The PICA arises anterior to the inferior olive and courses
between CNs X and XI and along the posterolateral
medulla and inferior cerebellar peduncle. It forms a cau-
dal loop below the tonsil. (b) Posterior view. The AICA
courses below CN X and along the posterolateral medulla
and inferior cerebellar peduncle. (c) Superior view of an
axial section through the medulla. The PICA arises along
the anterolateral margin of the medulla near and distorting
It dips caudally and encircles the brain stem near the
pontomesencephalic junction, passing below the
trochlear nerve and above the trigeminal nerve. Its
17
d
f
the rootlets of the hypoglossal nerve. (d) Posterior view.
The PICA arises anterior to the midportion of the olive,
passes posteriorly by the rootlets of CN XII, and courses
along the posterolateral medulla. (e) The right half of the
cerebellum has been removed to show the PICA looping
around the tonsil to form a cranial loop and through the
cerebellomedullary fissure to supply the suboccipital
surface. (f) Posterior view of the suboccipital cerebellar
surface. The PICA courses along the caudal margin of the
tonsil and supplies the cortical surface
proximal portion courses medial to the free edge of
the tentorium cerebelli, and its distal part passes
below the tentorium. After passing above the trigeminal
18 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
nerve, it enters the cerebellomesencephalic fissure,
where its branches make several sharp turns and give
rise to the precerebellar arteries, which pass to the
deep cerebellar white matter and the dentate nucleus.
On leaving the cerebellomesencephalic fissure
where its branches are again medial to the tentorial
edge, its branches pass posteriorly under the tento-
rial edge and are distributed to the tentorial cerebel-
lar surface. It usually arises as a single trunk, but
may also arise as a double (or duplicate) trunk. The
SCAs arising as a single trunk bifurcate into a rostral
and a caudal trunk. The SCA gives off perforating
branches to the brain stem and cerebellar peduncles.
Precerebellar branches that pass along the superior
cerebellar peduncle to the dentate nucleus arise
within the cerebellomesencephalic fissure. The ros-
tral trunk supplies the vermian and paravermian
area, and the caudal trunk supplies the hemisphere
on the suboccipital surface.
The AICA courses through the central part of the
cerebellopontine angle near the facial and vestibulo-
cochlear nerve and is intimately related to the pons,
lateral recess, foramen of Luschka, cerebellopontine
fissure, middle cerebellar peduncle, and petrosal
cerebellar surface (Fig. 2.5). The AICA originates
from the basilar artery, usually as a single trunk, and
encircles the pons near the abducens, facial, and ves-
tibulocochlear nerves. After coursing near and send-
ing branches to the nerves entering the acoustic
meatus and to the choroid plexus protruding from
the foramen of Luschka, it passes around the
flocculus on the middle cerebellar peduncle to sup-
ply the lips of the cerebellopontine fissure and the
petrosal cerebellar surface. It commonly bifurcates
near the facial-vestibulocochlear nerve complex to
form a rostral and a caudal trunk. The rostral trunk
sends its branches laterally along the middle cere-
bellar peduncle to the superior lip of the cerebello-
pontine fissure and the adjoining part of the petrosal
surface, and the caudal trunk supplies the inferior
part of the petrosal surface, including a part of the
flocculus and the choroid plexus. The AICA gives
rise to perforating arteries to the brain stem, choroi-
dal branches to the tela and choroid plexus, and the
nerve-related arteries, including the labyrinthine,
recurrent perforating, and subarcuate arteries.
The PICA is intimately related to the cerebel-
lomedullary fissure, the inferior half of the fourth
ventricular roof, the inferior cerebellar peduncle,
and the suboccipital surface (Fig. 2.6). The PICA,
by definition, arises from the vertebral artery near
the inferior olive and passes posteriorly around the
medulla. At the anterolateral margin of the medulla,
it passes rostral or caudal to or between the rootlets
of the hypoglossal nerve, and at the posterolateral
margin of the medulla, it courses rostral to or
between the fila of the glossopharyngeal, vagus,
and accessory nerves. After passing the latter
nerves, it courses around the cerebellar tonsil,
enters the cerebellomedullary fissure, and passes
posterior to the lower half of the roof of the fourth
ventricle. On exiting the cerebellomedullary fissure,
its branches are distributed to the vermis and hemi-
sphere of the suboccipital surface. Most PICAs
bifurcate into a medial and a lateral trunk. The
medial trunk supplies the vermis and adjacent part
of the hemisphere, and the lateral trunk supplies the
cortical surface of the tonsil and the hemisphere.
The PICA gives off perforating, choroidal, and cor-
tical arteries. The cortical arteries are divided into
vermian, tonsillar, and hemispheric groups.
The Fourth Ventricle
The fourth ventricle is a broad, tent-shaped
midline cavity located ventral to the cerebellum,
dorsal to the pons and medulla, and medial to the
cerebellar peduncles. It is connected rostrally
through the aqueduct with the third ventricle, cau-
dally through the foramen of Magendie with the
cisterna magna, and laterally through the foram-
ina of Luschka with the cerebellopontine angles.
Most of the cranial nerves arise near its floor. It
has a roof, a floor, and two lateral recesses.
The ventricular roof is tent-shaped. The roof
expands laterally and posteriorly from its narrow
rostral end just below the aqueduct to the level of
the fastigium and lateral recess, the site of its
greatest height and width, and from there, it
tapers to a narrow caudal apex at the level of the
foramen of Magendie. The apex of the roof, the
fastigium, divides the roof into superior and infe-
rior parts. The superior part is distinctly different
from the inferior part in that the inferior part is
formed largely by thin membranous layers and
Upper Neurovascular Complex
the superior part is formed by thicker neural
structures.
The lateral recesses of the fourth ventricle are
narrow, curved pouches formed by the union of
the roof and the floor that extends laterally below
the cerebellar peduncles to open through the
foramina of Luschka into the cerebellopontine
angles. The ventral wall of each lateral recess is
formed by the junctional part of the ventricular
floor and the rhomboid lip, a sheetlike layer of
neural tissue that extends laterally from the floor
and unites with the tela choroidea to form a pouch
at the outer extremity of the lateral recess. The
rostral wall of each lateral recess is formed by
the caudal margin of the cerebellar peduncles.
The inferior cerebellar peduncle courses upward
in the floor ventral to the lateral recess and turns
posteriorly at the lower part of the pons, to form
the ventricular surface of the rostral wall of the
recess. The peduncle of the flocculus intercon-
necting the inferior medullary velum and the
flocculus of the vermis crosses in the dorsal mar-
gin of the lateral recess. The caudal wall is formed
by the tela choroidea that stretches from the lat-
eral edge of the ventricular floor to the peduncle
of the flocculus. The flocculus is superior to the
outer extremity of the lateral recess. The rootlets
of the glossopharyngeal and vagus nerves arise
ventral to and the facial nerve arises rostral to the
lateral recess. The dorsal and ventral cochlear
nuclei, the site of placement of the auditory brain
stem implant and fibers related to the vestibulo-
cochlear nerve, are in the floor of the recess.
Three Neurovascular Complexes
In summary, the upper complex includes the
SCA, midbrain, cerebellomesencephalic fissure,
superior cerebellar peduncle, tentorial surface of
the cerebellum, and the oculomotor, trochlear,
and trigeminal nerves (Fig. 2.7). The SCA arises
in front of the midbrain and passes below the
oculomotor and trochlear nerves and above the
trigeminal nerve to reach the cerebellomesen-
cephalic fissure, where it runs on the superior
cerebellar peduncle and terminates by supplying
the tentorial surface of the cerebellum.
19
The middle complex includes the AICA, pons,
middle cerebellar peduncle, cerebellopontine
fissure, petrosal surface of the cerebellum, and
the abducens, facial, and vestibulocochlear
nerves. The AICA arises at the pontine level and
courses in relationship to the abducens, facial,
and vestibulocochlear nerves to reach the surface
of the middle cerebellar peduncle, where it
courses along the cerebellopontine fissure and
terminates by supplying the petrosal surface of
the cerebellum.
The lower complex includes the PICA,
medulla, inferior cerebellar peduncle, cerebel-
lomedullary fissure, suboccipital surface of the
cerebellum, and the glossopharyngeal, vagus,
accessory, and hypoglossal nerves. The PICA
arises at the medullary level and encircles the
medulla, passing in relationship to the
glossopharyngeal, vagus, accessory, and hypo-
glossal nerves to reach the surface of the infe-
rior cerebellar peduncle, where it dips into the
cerebellomedullary fissure and terminates by
supplying the suboccipital surface of the
cerebellum.
The discussion of microsurgical anatomy is
divided in sections related to approaches to the
neurovascular complexes. The most common
operation directed to the upper neurovascular
complex is the exposure of the posterior root of
the trigeminal nerve for a vascular decompres-
sion procedure for trigeminal neuralgia.
Operations directed to the middle complex are
for the removal of acoustic neuromas and other
tumors and for the relief of hemifacial spasm.
Our most common operations directed to the
lower complex are for glossopharyngeal neural-
gia and tumors centered near the jugular tubercle
and foramen [40]. Tumors involving multiple
complexes are also reviewed.
Upper Neurovascular Complex
The most common operation directed to the
upper neurovascular complex is the exposure of
the posterior root of the trigeminal nerve. The
posterior trigeminal root joins the brain stem
about halfway between the lower and upper
20 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
Upper complex
SCA
Midbrain
CN III, IV, V
Cerebellomesencephalic Fiss.
Superior Cerebellar Ped.
Tentorial Surface
Lower complex
PICA
Medulla
CN IX, X, XI, XII
Cerebellomedullary Fiss.
Inferior Cerebellar Ped.
Suboccipital Surface
Fig. 2.7 Each of the three neurovascular complexes in the
posterior fossa includes one of the three cerebellar arter-
ies, one of the three parts of the brain stem, one of the
three cerebellar peduncles, one of the three cerebellar sur-
faces, one of the three fissures between the brain stem and
the cerebellum, and one of the three groups of cranial
nerves. The upper complex is related to the SCA, the mid-
dle complex is related to the AICA, and the lower com-
plex is related to the PICA. The upper complex includes
the SCA, midbrain, superior cerebellar peduncle, cerebel-
lomesencephalic fissure, tentorial cerebellar surface, and
the oculomotor, trochlear, and trigeminal nerves. The mid-
dle complex includes the PICA, pons, middle cerebellar
peduncle, cerebellopontine fissure, petrosal surface, and
the abducens, facial, and vestibulocochlear nerves. The
lower complex includes the PICA, medulla, inferior
borders of the pons (Figs. 2.1, 2.2, 2.3, 2.5, and
2.8). Frequently, the lip of cerebellum projects
forward and obscures the course of the posterior
root through the middle cerebellar peduncle into
the pons. In its intradural course, the trigeminal
nerve uniformly runs obliquely upward from the
lateral part of the pons toward the petrous apex.
It exits the posterior fossa to enter the middle
cranial fossa by passing forward beneath the
tentorial attachment to enter Meckel’s cave,
which sits in the trigeminal impression on the
upper surface of the petrous part of the temporal
bone.
Middle complex
AICA
Pons
CN VI, VII, VIII
Middle Cerebellar Ped.
Cerebellopontine Fiss.,
Petrosal Surface
cerebellar peduncle, cerebellomedullary fissure, suboc-
cipital surface, and the glossopharyngeal, vagus, acces-
sory, and hypoglossal nerves. The SCA is divided into
four segments: anterior pontomesencephalic (green), lat-
eral pontomesencephalic (orange), cerebellomesenceph-
alic (blue), and cortical (red). Each segment may be
composed of one or more trunks, depending on the level
of bifurcation of the main trunk. The AICA is divided into
four segments: anterior pontine (green), lateral pontomed-
ullary (orange), flocculonodular (blue), and cortical (red).
The PICA is divided into five segments: anterior medul-
lary (green), lateral medullary (orange), tonsillomedullary
(blue), telovelotonsillar (yellow), and cortical (red). AICA
anteroinferior cerebellar artery, CN cranial nerve, Fiss.
fissure, Ped. peduncle, PICA posteroinferior cerebellar
artery, SCA superior cerebellar artery (From Rhoton [39])
Trigeminal Root Anatomy
The fibers from the third division remain in a cau-
dolateral position in the posterior root throughout
the interval from the ganglion to the pons, the first
division rostromedial, with second-division fibers
in an intermediate position (Figs. 2.8, 2.9, and
2.10) [3, 6]. There are anastomoses between the
fibers from each division in the area posterior to
the ganglion (Fig. 2.8). Results of selective rhizo-
tomy of the posterior root indicate that somato-
topic localization with the third division
inferolaterally and the ophthalmic division
Upper Neurovascular Complex 21

a b

c d

Fig. 2.8 Lateral views, right trigeminal nerve. (a) Meckel’s
cave, the cistern which extends forward from the posterior
fossa along the posterior trigeminal root to the level of the
midportion of the ganglion, has been exposed by removing
the lateral dural wall of the cave. The motor root arises
rostral to the sensory root and passes through Meckel’s
cave on the medial side of the posterior sensory root and
ganglion. (b) The dura has been removed to expose the
posterior root and ganglion and the three trigeminal divi-
sions. There is diffuse anastomosis between the rootlets
posterior to the ganglion. (c) Enlarged view of the diffuse
anastomosis in the region of Meckel’s cave. (d) Four motor
rootlets, which arise around the rostral margin of the
sensory root, have been elevated to expose the anastomoses
between the motor and sensory roots. The cerebellar lip
projects forward and may hide the junction of the sensory
root with the pons in the retrosigmoid approach. (e) A
cleavage plane has been started anteriorly and extended
backward to the level of the posterior root. The first-division
fibers are rostromedial within the posterior root and the
third-division fibers caudolateral with the second division
being in an intermediate location (From Rhoton [40])
22 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a b
A B
F
E D
Fig. 2.9 Diagrams of 12 trigeminal nerves showing the
relationship of the trigeminal sensory root, motor rootlets,
and aberrant sensory rootlets at the site of entry into the
pons. The central diagrams are for orientation and show
the elliptical cross section of the sensory root. The large
ovals (A–F) represent the sensory root and are oriented in
the same manner as the sensory root in the central dia-
gram. The sites of origin of the motor rootlets are black.
(a) Nerves on the right. Only 5 motor rootlets are present
in B, but 13 are seen in F. The aberrant sensory rootlets
are shown by the dark outline with clear center. None are
present in D and F. In C and E, some aberrant rootlets
arose farther from the sensory root than some of the motor
rootlets. (b) Nerves on the left. Only 4 motor rootlets are
present in A, but there are 10 in B and C. Aberrant sensory
dorsomedially is well maintained posterior to and
despite the prominent retrogasserian anastomoses
[3]. A cross section of the sensory root between
the pons and the petrous apex is elliptical. In most
nerves, the angle between the longest diameter of
this cross section and the long axis of the body is
40–50°; the angle, however, can vary from 10° to
80° (Fig. 2.10) [10]. An angle of 80° places the
third-division fibers almost directly lateral to those
of the first division, but an angle of 10° places the
third-division fibers almost directly caudal to
those of the first division. The variability in the
degree of rotation of the sensory root entering the
pons may explain some differences in the quantity
of sensation retained after partial section of the
nerve in the posterior cranial fossa. The most fre-
quent pattern is for the third-division fibers to be
caudolateral to the first-division fibers; some
nerves, however, are rotated so that third-division
fibers will be almost directly lateral to the first
A
B
F C
E D
rootlets are shown by the dark outline with clear center.
None are present in B and C. In A, D, E, and F, some aber-
rant rootlets arose farther from the sensory root than some
of the motor rootlets. Lines through the oval representing
the main sensory root show portions of the nerve from
each of the three divisions. In all diagrams, the rostrome-
dial portion was from the first division, the caudolateral
portion was from the third division, and the second divi-
sion was in an intermediate position. In all these nerves,
except A and B in the left nerve, the second-division fibers
made up a greater portion of the medial than the lateral
portion of the sensory root. Small arteries or veins cours-
ing between the rootlets at the level of entry into the pons
are shown in all diagrams of both nerves except D in the
right nerve (a) (From Gudmundsson et al. [10])
division; others are rotated nearly 70° away from
this so that the third-division fibers will be directly
caudal to those of the first division. Cutting into
the nerve partially from a caudolateral direction
would give a significantly different pattern of sen-
sory loss if the nerve is rotated with the third divi-
sion lateral to the first, than if the third division is
almost directly caudal to the first division.
At the junction of the nerve with the pons, as
many as 15 separate nerve rootlets may be spread
around the rostral half of the site where the main
sensory cone enters the pons [10]. These rootlets
are either motor or aberrant sensory rootlets. The
aberrant sensory fibers are small rootlets that
penetrate the pons outside the main sensory root
(Figs. 2.9 and 2.11). The aberrant rootlets arise
around the rostral two-thirds of the nerve and
usually join the root a short distance from the
brain stem. There may be as many as eight
aberrant roots. Those arising rostral to the sensory
Upper Neurovascular Complex
V1
V1
V2 V2
V3
V1
V3
V2
ο ο
80 V3 10
ο
45
A C
B of 4–14 separate rootlets, each having a separate
Fig. 2.10 Variability of the longest axis of the elliptical
cross section of the trigeminal nerve at the pons (broken
line) to the longitudinal axis of the body (solid line). The
long axis of most nerves makes a 40–50° angle with the
longitudinal axis of the body (A); however, this can vary
from 10° (C) to 80° (B). In B, the third division is almost
directly lateral to the first division, and in C, it is almost
directly caudal (From Gudmundsson et al. [10])
Fig. 2.11 Lateral view of the junction of the left trigemi-
nal nerve with the pons. A nerve hook is between the large
aberrant sensory rootlet and the main sensory root. The
aberrant rootlet arises from the pons directly lateral to the
sensory root and joins the sensory root about 1 cm from
the brain stem. Four motor rootlets are seen above the sen-
sory root (From Gudmundsson et al. [10])
23
root most frequently enter the first division and
those arising more caudally enter the second or
third division. No aberrant rootlets originate
around the caudal third of the sensory root. Of 66
aberrant rootlets found in our study of 50 trigemi-
nal nerves, 49 went into the first division, 10 into
the second division, and 7 into the third division
[10]. The findings that aberrant rootlets are most
commonly related to the first division agree with
Dandy’s conclusion that when the accessory
fibers are spared, sensation in the first division
tends to be spared [3]. The aberrant rootlets
appear to be nonspecific sensory fibers separated
from the root by transverse pontine fibers [10, 48].
Aberrant roots contribute mainly to the first divi-
sion and probably do not convey a specific
sensory modality from all three divisions.
Motor rootlets also arise around the rostral part
of the nerve; however, they tend to arise further
from the main sensory cone than do the accessory
sensory rootlets. The motor root may be composed
exit from the pons (Figs. 2.8, 2.9, 2.11, and 2.12).
The aberrant sensory fibers usually arise closer to
the main sensory root than to the motor fibers.
Some aberrant sensory fibers, however, will arise
further from the main sensory root than does the
origin of some motor filaments; for this reason, it
is easy to confuse aberrant sensory fibers and
motor filaments at the nerve/pons junction.
Anastomoses between the motor and sensory
roots are present in most nerves (Fig. 2.8). Those
sensory fibers associated with the motor root
from the pons to just proximal to the ganglion,
where they anastomose with the sensory root,
would be spared with a rhizotomy in the posterior
fossa. Horsley et al. [13] suspected that there
were sensory fibers in the trigeminal motor root
suggesting that the motor root be sectioned if
trigeminal neuralgia recurred after the complete
section of the sensory root. Our studies offer two
explanations for the accidental preservation of
sensation after posterior rhizotomy: (1) sparing
of the aberrant sensory rootlets and (2) sparing of
the anastomotic sensory fibers that run with the
motor root at the level of the rhizotomy [10].
Anastomosis is a more likely explanation for the
accidental sensory preservation and recurrence of
trigeminal neuralgia after the section of the
24 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a b
Fig. 2.12 (a) Lateral view of the right trigeminal nerve
near its junction with the pons. The arrow points to the
intermediate group of fibers between the motor rootlet and
the sensory root. (b) The same trigeminal nerve. The
arrow points to the intermediate group of fibers that
posterior root because anastomotic rootlets are
present throughout the interval from the pons to
the ganglion. Aberrant sensory roots are present
in only one-half of the nerves. They provide
another explanation for the preservation of
sensation following the section of the main
sensory root.
Anatomy of Vascular Compression
in the Upper Neurovascular Complex
In 1934, Dandy postulated that arterial compres-
sion and distortion of the trigeminal nerve might
be the cause of trigeminal neuralgia [4]. He
described the SCA as affecting the nerve in
30.7 % of his 215 cases of trigeminal neuralgia.
The vascular compression theory failed to gain
acceptance at the time, but it awaited the better
demonstration of these pathological changes at
surgery by Jannetta [15, 18] using magnification
provided by the operating microscope.
This upper neurovascular complex, for a
vascular decompression operation, is approached
utilizing a vertical scalp incision crossing the
asterion, which usually overlies the junction of
the lower half of the transverse and sigmoid
sinuses (Fig. 2.13). The bone opening, a small
proved to be a motor rootlet when traced distally. This
illustrates the difficulty in telling whether an intermediate
group of fibers is motor or sensory unless the nerve
bundles can be separated and examined individually
(From Gudmundsson et al. [10])
craniotomy, located behind the upper half of the
sigmoid sinus exposes the edge of the junction of
the transverse and sigmoid sinuses in its supero-
lateral margin. The cerebellum is relaxed by
opening the arachnoid and removing cerebrospi-
nal fluid, a maneuver made safer by the use of the
operating microscope. A narrow brain spatula,
commonly 3 mm at the tip, is introduced parallel
and just below the superior petrosal sinus to ele-
vate the superolateral margin of the cerebellum
[30]. The use of a wider spatula or a lower entry
point along the lateral cerebellum risks damaging
the vestibulocochlear nerve. A bridging petrosal
vein, which commonly blocks access to the
trigeminal nerve, is coagulated with gentle bipo-
lar coagulation and divided nearer its junction
with the brain than to the superior petrosal sinus.
Unexpected bleeding encountered as the supero-
lateral margin of the cerebellum is elevated, if
venous in appearance, is usually related to stretch-
ing and tearing of the petrosal veins that pass
from the superior surface of the cerebellum to the
venous sinus in the tentorium or, if arterial in
appearance, to tearing of the subarcuate branch
of the AICA behind the internal auditory canal at
its site of penetration of the dura covering the
subarcuate fossa. The trochlear nerve is identified
before opening the arachnoid behind the
Upper Neurovascular Complex
VIII
VII
V Pons
Sup. Pet. Sinus
Fig. 2.13 Retrosigmoid approach to the trigeminal nerve
for a microvascular decompression operation. (a) (Upper
left) The patient is positioned in the three-quarter prone
position. The surgeon is seated at the head of the table.
The table is tilted so that the feet are lower than the heart.
(b) The vertical paramedian incision crosses the asterion.
The superolateral margin of the craniotomy is positioned
at the junction of the transverse and sigmoid sinuses. (c)
The superolateral margin of the cerebellum is gently ele-
vated using a brain spatula tapered from 10 mm at the base
25
Sig. Sinus
Trans. Sinus
S.C.A
IV
to 3 or 5 mm at the tip to expose the site at which the
trigeminal nerve enters the pons. The brain spatula is
advanced and aligned parallel to the superior petrosal
sinus. The trochlear nerve is at the superior margin of the
exposure, and the facial and vestibulocochlear nerves are
at the lower margin. The dura is tacked up to the adjacent
muscles to maximize the exposure along the superolateral
margin of the cerebellum. The main trunk of the SCA
loops down into the axilla of the trigeminal nerve (From
Rhoton [29])
26 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

a b

c d

Fig. 2.14 Trigeminal nerve and SCA relationships.
(a) The trigeminal posterior root, ganglion, and three divi-
sions have been exposed by removing the dura from the
lateral wall of Meckel’s cave and the cavernous sinus. The
posterior root enters the midpons below the SCA and is
intertwined with the branches of the superior petrosal
vein. (b) The SCA loops downward and, at the junction of
the rostral and caudal trunks, contacts the posterior
trigeminal root at the pontine junction. The cerebellar lip
projects forward and may block access to the junction of
the trigeminal nerve and pons in the retrosigmoid
approach. (c) SCA with an early bifurcation. The rostral
trunk loops downward and indents the upper surface of
the trigeminal nerve. (d) Another SCA passes around the
pons and bifurcates into its rostral and caudal trunks above
the trigeminal root entry zone (From Rhoton [40])

trigeminal nerve because it may be difficult to see
the nerve after the arachnoid has been opened
and shrinks into thick white clumps that may hide
the nerve. Usually, the trochlear nerve is several
millimeters above the trigeminal nerve; it may be
carried downward, however, if it is adherent to a
segment of the SCA that has looped into the axilla
of the trigeminal nerve. The overhanging lip of
the cerebellomesencephalic fissure must be
retracted gently to expose the junction of the
nerve with the pons.
Arterial Relationships
The most common finding at a vascular decom-
pression operation for trigeminal neuralgia is a
segment of the SCA compressing the trigeminal
nerve [12]. Normally, the SCA encircles the brain
stem well above the trigeminal nerve. In adults,
the SCA commonly makes a shallow, caudal loop
and courses inferiorly for a variable distance on
the lateral side of the pons (Figs. 2.13, 2.14, 2.15,
and 2.16). In those cases with the most prominent
caudally projecting loop, contact between the
artery and the trigeminal nerve occurs. The point
of contact with the SCA is usually on the superior
or supermedial aspect of the nerve, and often, a
few fascicles of the nerve are distorted by an SCA
that has looped down into the axilla between the
medial side of the nerve and the pons. An arterial
loop in the axilla may not be visible from the ret-
rosigmoid view behind the trigeminal nerve if the
SCA courses around the brain stem directly in
front of the nerve. The loop of the SCA also may
be difficult to see if the artery passes over the
Upper Neurovascular Complex
b Ro.Tr
Main Tr.
Ca.Tr
a V
Bas.A.
f S.C.A.
A.I.C.A.
Fig. 2.15 Sites of arterial compression of the trigeminal
nerve. Orientation as shown in the central diagram. (a)
Central diagram. The right trigeminal nerve is com-
pressed by a tortuous basilar artery, and the left trigemi-
nal nerve is compressed by the main trunk of the SCA.
(b) The SCA bifurcates into rostral and caudal trunks
prior to reaching the trigeminal nerve. The nerve is com-
pressed by the caudal trunk. (c) The SCA bifurcates
rostral aspect of the nerve very close to the brain
stem, where it may be hidden by the overhanging
lip of the cerebellomesencephalic fissure. The
loop of the SCA may be seen dangling below the
lower margin of the nerve, even though it is not
27
d
Ro.Tr
Ca.Tr
V
S.C.A
e
Pontine A.
distally to the nerve. The nerve is compressed by the
main trunk. (d) The SCA bifurcates prior to reaching the
nerve. The nerve is compressed by both the rostral and
caudal trunks. (e) The nerve is compressed by a large
pontine artery. (f) The nerve is compressed by an AICA
that has a high origin and loops upward into the medial
surface of the nerve. The SCA passes around the brain
stem above the nerve (From Rhoton [29])
visible above the nerve. These loops of the SCA,
however, always pass rostrally along the medial
and superior surfaces of the nerve to reach the cer-
ebellomesencephalic fissure. The medial axilla of
the nerve must be carefully explored before
28 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

Main Tr.

Ra.Tr.
Ca Tr.
c Ca.Tr.

IV

Ro Tr.
b Ca Tr. Sup.
d
Petrosal Sinus
V

S.C.V
Pons
VII
VIII

a Pontine A.

Bas.A.

e
A.I.C.A

Fig. 2.16 Sites of arterial compression of the trigeminal
nerve as seen through a suboccipital craniotomy. (a) Central
diagram. The site of the skin incision (solid line) and the
craniotomy (interrupted line) are shown in the insert. The
superolateral margin of the cerebellum is gently retracted to
expose the trigeminal nerve and the SCA. The brain spatula
is advanced parallel to the superior petrosal sinus. The tro-
chlear nerve is at the superior margin of the exposure and the
facial and vestibulocochlear nerves are at the lower margin.
The trigeminal nerve is compressed by a loop of the SCA
that dangles down into the axilla of the nerve. The site of
compression on the artery is at the junction of the main trunk
with the rostral and caudal trunks. (b) The nerve is com-
pressed by the caudal trunk. (c) The nerve is compressed by
the main trunk. (d) Compression by both the rostral and cau-
dal trunks. (e) Compression by a pontine branch of the basi-
lar artery. (f) Compression by the AICA. (g) Compression
by a tortuous basilar artery (From Rhoton [29])
Upper Neurovascular Complex
concluding that there is no arterial loop in the
axilla of the nerve. It is important to remember
that the trunks do not pass directly from the side
of the brain stem to the superior surface of the cer-
ebellum, but, rather, that they dip into the deep
fissure between the cerebellum and midbrain at
the posterior margin of the trigeminal nerve. The
SCA gives off perforating arteries that may limit
the degree of repositioning of the artery achiev-
able in a microvascular decompression operation.
The most common site of compression of the
trigeminal nerve on the SCA is at the junction of
the main trunk with the origin of the rostral and
caudal trunks [29]. However, other sites of com-
pression are seen depending on how far distal the
artery bifurcates in relation to the trigeminal
nerve. If the SCA bifurcates near the basilar
artery or if there is a duplicate configuration in
which the rostral and caudal trunks arise directly
from the basilar artery, both trunks may loop
down into the axilla and compress the nerve.
Alternatively, if the artery bifurcates before
reaching the nerve, the caudal trunk may com-
press the nerve and the rostral trunk may course
well above the nerve. If the artery bifurcates dis-
tal to the nerve, only the main trunk will be
involved in the compression. The point of bifur-
cation of the SCA does affect the caliber of the
vessel that makes contact with the nerve. The
contacting vessel will be of a smaller caliber if
the SCA bifurcates before reaching the trigemi-
nal nerve.
A less frequent source of compression of the
trigeminal nerve is by the AICA (Figs. 2.15 and
2.16). Normally, the AICA passes around the
pons below the trigeminal nerve with the facial
and vestibulocochlear nerves. The AICA, how-
ever, may have a high origin and loop upward to
indent the medial or lower surface of the trigemi-
nal nerve prior to passing downward to course
with the facial and vestibulocochlear nerves.
A serpentine basilar artery also may wander lat-
erally and compress the medial side of the trigem-
inal nerve [48]. This type of basilar artery often is
elongated and has a fusiform configuration. More
than one artery may compress the nerve. In a few
cases, the SCA will compress the rostral surface
of the nerve and the AICA will compress the
29
caudal surface. Infrequently, the PICA may reach
and groove the undersurface of the trigeminal
nerve. The trigeminal nerve also may be com-
pressed by a large pontine branch of the basilar
artery (Figs. 2.15 and 2.16). Normally, these
pontine branches pass around and penetrate the
pons before reaching the trigeminal nerve. A large
pontine artery, however, may indent the medial
surface of the trigeminal nerve and then course
rostral or caudal to the nerve to supply the pons
behind the nerve.
Venous Relationships
Compression and distortion of the trigeminal
nerve by the surrounding veins, although less fre-
quent than arterial compression, also is found in
trigeminal neuralgia (Figs. 2.17 and 2.18) [24]. It
is the superior petrosal veins, which empty into
the superior petrosal sinus, that are most fre-
quently encountered in operative approaches to
the trigeminal nerve and that most commonly
compress the trigeminal nerve.
The superior petrosal veins are among the
largest and most frequently encountered veins in
the posterior fossa. The superior petrosal veins
may be formed by the terminal segment of a sin-
gle vein or by the common stem formed by the
union of several veins. The most common tribu-
taries of the superior petrosal veins are the trans-
verse pontine and pontotrigeminal veins, the
veins of the cerebellopontine fissure and the mid-
dle cerebellar peduncle, and the common stem of
the veins draining the lateral part of the cerebellar
hemisphere. The transverse pontine veins, which
pass near the trigeminal nerve to reach the bridg-
ing veins entering the superior petrosal sinus, are
the most frequent veins to compress the trigemi-
nal nerve. They may course medially in the axilla
of the nerve or they also may pass above, below,
or lateral to the nerve and may indent any of its
surfaces. The vein of the middle cerebellar
peduncle may compress the lateral or medial sur-
face of the trigeminal nerve before joining the
petrosal veins as it ascends in the pons. The vein
of the cerebellopontine fissure may indent the lat-
eral margin of the trigeminal nerve as it ascends
30 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

b d

f e

Fig. 2.17 Sites of venous compression of the trigeminal
nerve. (a) Central diagram. Anterior view. The veins that
commonly compress the trigeminal nerve are tributaries
of the superior petrosal vein. The tributaries that converge
on and may compress the nerve are the transverse pontine
and pontotrigeminal veins and the veins of the cerebello-
pontine fissure and middle cerebellar peduncle. The trans-
verse pontine veins course transversely across the pons.
The vein of the middle cerebellar peduncle arises in the
region of the facial and vestibulocochlear nerves and
ascends on the pons. The vein of the cerebellopontine
fissure arises along the cleft between the pons and the cer-
ebellum and ascends behind the trigeminal nerve. The
pontotrigeminal vein arises on the upper pons and passes
above the trigeminal nerve. (b) A transverse pontine vein
compresses the lateral side of the nerve and joins the veins
of the middle cerebellar peduncle and cerebellopontine
fissure to empty into a superior petrosal vein. (c) The
medial side of the nerve is compressed by a tortuous trans-
verse pontine vein. (d) The lateral side of the nerve is
compressed by the junction of the transverse pontine vein
with the veins of the middle cerebellar peduncle and the
cerebellopontine fissure. (e) The nerve is compressed on
the medial side by the vein of the middle cerebellar
peduncle and on the lateral side by the vein of the cerebel-
lopontine fissure. (f) The lateral side of the nerve is com-
pressed by the vein of the cerebellopontine fissure (From
Rhoton [29])
Upper Neurovascular Complex
b
Fig. 2.18 Sites of venous compression of the trigeminal
nerve as seen through a retrosigmoid craniotomy.
(a) The insert shows the site of the scalp incision (solid
line) and the craniotomy (interrupted line). The cerebel-
lum has been elevated to expose the junction of the
trigeminal nerve with the pons. The superior petrosal
veins empty into the superior petrosal sinus. The tro-
chlear nerve is at the superior margin and the facial and
vestibulocochlear nerves are at the lower margin of the
exposure. The craniotomy exposes the junction of the
sigmoid and transverse sinuses. The trigeminal nerve is
compressed by the junction of a transverse pontine vein
and the vein of the middle cerebellar peduncle with the
superior petrosal vein. The vein of the cerebellopontine
fissure ascends behind the nerve, and the pontotrigeminal
31
d
vein passes above the nerve. (b) The trigeminal nerve is
compressed on its medial side by a transverse pontine
vein and on its lateral side by the vein of the middle
cerebellar peduncle. (c) The lateral side of the nerve is
compressed by a transverse pontine vein. (d) The medial
side of the nerve is compressed by the junction of a
transverse pontine vein with the veins of the middle
cerebellar peduncle and cerebellopontine fissure. (e) The
lateral side of the nerve is compressed by the junction of
the transverse pontine vein with the veins of the middle
cerebellar peduncle and cerebellopontine fissure. (f) The
medial side of the nerve is compressed by the vein of
the middle cerebellar peduncle. (g) The lateral side of the
nerve is compressed by the vein of the cerebellopontine
fissure (From Rhoton [29])
32 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
toward the superior petrosal sinus, and the
pontotrigeminal vein may indent the upper mar-
gin of the nerve.
The junction of these veins, which converge
and form a single trunk prior to entering the supe-
rior petrosal sinus, usually is lateral to the trigem-
inal nerve. This junction, however, may be located
medial to the trigeminal nerve, in which case the
common trunk must pass around the trigeminal
nerve prior to reaching the superior petrosal
sinus. These common trunks also may compress
the trigeminal nerve.
Suprameatal Extension
of the Retrosigmoid Approach
The part of the posterior surface of the temporal
bone that forms the superior lip of the porus of
the internal acoustic meatus is the site of a promi-
nence, the suprameatal tubercle, which blocks
access to the lateral margin of the trigeminal
nerve and the prepontine cistern medial to the
trigeminal nerve (Fig. 2.19). Removal of the supra-
meatal tubercle increases access to the region of
the upper neurovascular complex around the
trigeminal nerve and may possibly avoid the need
for a supratentorial craniotomy in exposing
tumors that are located predominantly in the cer-
ebellopontine angle but also extend into the pos-
terior part of the middle fossa in the region of
Meckel’s cave [47]. The tubercle, the most prom-
inent bony elevation around the circumference of
the internal acoustic meatus, is defined below by
the internal acoustic meatus, above by the petrous
ridge, laterally by a vertical line crossing the pos-
terior edge of the porus of the internal acoustic
meatus, and medially by a vertical line crossing
the medial edge of the trigeminal notch, a depres-
sion in the petrous ridge located below the porus
of Meckel’s cave. Above and medial to the
suprameatal tubercle, the posteromedial part of
the floor of the middle cranial fossa is the site of the
depression underlying Meckel’s cave in which
the posterior trigeminal root sits. The most prom-
inent posterior projection of the tubercle is
located above the lateral half of the porus of the
internal acoustic meatus.
The neural structures that surround and limit
access to the suprameatal tubercle are the cer-
ebellum posteriorly, the facial and vestibuloco-
chlear nerves below, the trigeminal nerve above
and medial, and the abducens nerve medially.
The cerebellopontine cistern opens through the
porus into Meckel’s cave. Meckel’s segment of
the trigeminal nerve, which begins at the porus
and extends to the trigeminal ganglion, is dif-
ferentiated from the cavernous segment in the
wall of the cavernous sinus. Meckel’s segment
is narrower adjacent to the porus and fans out
as it approaches the posterior edge of the gas-
serian ganglion, which is embedded in the dura
just anterior to the anterior edge of Meckel’s
cave.
The intraosseous structures, which limit the
extent of the drilling if they are to be preserved,
are the posterior part of the superior semicircular
canal, the upper part of the posterior semicircular
canal, and the common crus of the two canals.
After removal of the suprameatal tubercle, the
drilling can be extended below Meckel’s cave to
the edge of the petroclival fissure just in front of
the inferior petrosal sinus and immediately lateral
to the abducens nerve.
Removing the bone in this area provides
access, on average, to the posterior 10.3 mm
(range, 8.0–13.0 mm) of Meckel’s cave and the
enclosed portion of the trigeminal nerve and
opens a 180° window around the lateral and lower
surface of the posterior trigeminal root, which
may be used for accessing the posterior part of
the middle fossa (Fig. 2.19).
The size of the area created by removing the
suprameatal tubercle and adjacent part of the
petrous apex by the retrosigmoid route is lim-
ited superiorly by the superior petrosal sinus
and the dura covering the upper surface of the
petrous bone. The superior petrosal sinus can be
divided, and Meckel’s cave and the tentorium
lateral to the porus of Meckel’s cave opened to
provide intradural access to the posteromedial
part of the middle fossa, but cannot be extended
forward to the horizontal portion of the petrous
carotid.
The suprameatal extension of the retrosig-
moid approach may permit removal of some
Upper Neurovascular Complex 33

a b

c d

Fig. 2.19 Suprameatal variant of the retrosigmoid approach.
(a) The cerebellum has been elevated to expose the nerves in
the cerebellopontine angle. A large petrosal vein blocks
access to the suprameatal area. (b) The superior petrosal
vein has been divided to expose the suprameatal tubercle
located above the porus of the internal acoustic meatus and
lateral to the trigeminal nerve. (c) The dura over the
suprameatal tubercle has been removed in preparation for
drilling. (d) Removing the suprameatal bone, including the
tubercle, extends the exposure along the posterior trigeminal
root by approximately 1 cm and increases access to the front
of the brain stem and clivus (From Rhoton [40])
34 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
tumors that are located mainly in the posterior
fossa but that extend into the middle fossa in the
region of Meckel’s cave. The space created after
drilling the suprameatal tubercle and the bone
medial to the internal auditory canal and below
the trigeminal nerve was enough to extend the
retrosigmoid approach as far as 13.0 mm (aver-
age, 10.3 mm) anterior to what could be achieved
by using the retrosigmoid approach alone [47].
The extent to which the bone in the region of the
suprameatal tubercle could be removed using the
retrosigmoid approach is defined and limited by
the neural and bony structures in the region. The
cerebellum, with gentle retraction, limits the
angle at which the suprameatal tubercle can be
drilled, although when combined with evacua-
tion of cerebrospinal fluid, it provides a space to
adequately visualize and remove lesions medial
to the suprameatal tubercle. A portion of this
bone is commonly removed in approaching
tumors extending into the internal acoustic
meatus. It is possible to remove approximately
270° of the circumference of the wall of the inter-
nal acoustic meatus when using the retrosigmoid
approach; however, with the approach described
herein, only the bone in the region of the
suprameatal tubercle is removed. The drilling on
the lateral side of the tubercle should avoid the
posterior semicircular canal and common crus of
the posterior and superior canals if hearing is to
be preserved, but on the medial side, it can extend
through the petrous apex into the side of the
clivus.
Often, the subarcuate branch of the AICA
must be obliterated to access the suprameatal
tubercle. Accessing the suprameatal tubercle
often requires that the superior petrosal veins be
obliterated and divided. This allows the drilling
to be directed medially along the lateral and lower
margin of the porus of Meckel’s cave. The dura
along the lower and lateral margin of the porus of
Meckel’s cave and the tentorium lateral to the
porus trigeminus can then be opened to expose
the trigeminal nerve in the posterior part of
Meckel’s cave and the middle cranial fossa. Care
is taken to protect the trochlear nerve if the tento-
rial incision is to be extended through the free
edge.
Middle Neurovascular Complex
The middle complex includes the AICA, pons,
middle cerebellar peduncle, cerebellopontine
fissure, petrosal surface of the cerebellum, and the
abducens, facial, and vestibulocochlear nerves
(Figs. 2.1, 2.2, 2.3, 2.5, 2.7, 2.20, and 2.21). The
AICA arises at the pontine level and courses in
relationship to the abducens, facial, and vestibulo-
cochlear nerves to reach the surface of the middle
cerebellar peduncle, where it courses along the cer-
ebellopontine fissure and terminates by supplying
the petrosal surface of the cerebellum. Operations
directed to the middle complex are for the removal
of acoustic neuromas and other tumors and for the
relief of hemifacial spasm. The considerations
related to acoustic neuromas will be dealt with
first.
Anatomy of Acoustic Neuromas
Acoustic neuromas, as they expand, may involve
a majority of the cranial nerves, cerebellar
arteries, and parts of the brain stem. On the lat-
eral side, in the meatus, they commonly expand
by enlarging the meatus but infrequently erode
into the vestibule and cochlea. On the medial
side, they compress the pons, medulla, and cere-
bellum. An understanding of microsurgical anat-
omy is especially important in preserving the
facial and adjacent cranial nerves, which are the
neural structures at greatest risk during acoustic
neuroma removal. A widely accepted operative
precept is that a nerve involved by a tumor should
be identified proximal or distal to the tumor,
where its displacement and distortion is the least,
before the tumor is removed from the involved
segment of the nerve. Considerable attention has
been directed to the early identification of the
facial nerve distal to the tumor at the lateral part
of the internal acoustic canal. Less attention has
been directed to identification at the brain stem
on the medial side of the tumor. These anatomic
considerations are divided into sections dealing
with the relationships at the lateral end of the
tumor in the meatus and those on the medial end
of the tumor at the brain stem [31, 37, 45].
Middle Neurovascular Complex 35

a b

c d

Fig. 2.20 Left retrosigmoid exposure. (a) The cerebel-
lum has been elevated. A large AICA loops into the porus
of the internal meatus. The junction of the facial nerve
with the brain stem is located below and slightly in front
of the vestibulocochlear nerve. (b) The vestibulocochlear
nerve has been elevated to provide additional exposure of
the facial nerve. (c) Choroid plexus protrudes from the
foramen of Luschka into the cerebellopontine angle
behind the glossopharyngeal and vagus nerves. A nerve
hook has been placed inside the rhomboid lip, a pouch of
neural tissue attached along the anterior margin of the lat-
eral recess and extending laterally behind the glossopha-
ryngeal and vagus nerves. (d) Enlarged view of the
rhomboid lip (From Rhoton [40])
36 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

a b

c d

e f

Fig. 2.21 Left cerebellopontine angle. (a) The AICA
passes between the facial and vestibulocochlear nerves. A
dural septum separates the glossopharyngeal and vagus
nerves at the jugular foramen. (b) The vestibulocochlear
nerve and the flocculus have been elevated to expose the
junction of the facial nerve with the brain stem. In the ret-
rosigmoid approach, the facial nerve junction with the
brain stem can be exposed below the vestibulocochlear
nerve. (c) The posterior wall of the internal acoustic
meatus has been removed. The cleavage plane between
the superior and inferior vestibular nerves is especially
prominent. (d) The dura lining the internal acoustic
meatus has been opened. The transverse crest separates
the superior vestibular and facial nerves above from the
inferior vestibular and cochlear nerves below. (e) Enlarged
view of the nerves within the meatus. The cochlear nerve
is partially hidden anterior to the inferior vestibular nerve.
(f) The cleavage plane between the superior and inferior
vestibular and cochlear nerves has been started laterally
and extended medially to expose the individual nerve
bundles (From Rhoton [40])

Meatal Relationships
The nerves in the lateral part of the internal acous-
tic meatus are the facial, cochlear, and inferior
and superior vestibular nerves (Figs. 2.2, 2.3,
2.21, and 2.22) [28, 32, 35, 36]. The position of
the nerves is most constant in the lateral portion
of the meatus, which is divided into a superior and
an inferior portion by a horizontal ridge, called
either the transverse or falciform crest. The facial
Middle Neurovascular Complex
a Superior vestibular nerve
Facial nerve
Transverse
crest
Cochlear nerve
Inferior vestibular nerve
c Facial nerve
Fig. 2.22 View of right internal acoustic meatus with the
posterior lip removed to show variable direction of facial
nerve displacement by acoustic neuroma. (a) Normal neu-
ral relationships with the eighth nerve dividing into its
three parts in the lateral meatus. The facial and superior
vestibular nerves are above the transverse crest and the
cochlear and inferior vestibular nerves are below. The facial
nerve occupies the anterosuperior quadrant of the lateral
and the superior vestibular nerves are superior to
the crest. The facial nerve is anterior to the supe-
rior vestibular nerve and is separated from it at the
lateral end of the meatus by a vertical ridge of
bone, called the vertical crest. The vertical crest is
also called “Bill’s bar” in recognition of William
House’s role in focusing on the importance of this
crest in identifying the facial nerve in the lateral
end of the canal [14]. The cochlear and inferior
vestibular nerves run below the transverse crest
with the cochlear nerve located anteriorly. Thus,
the lateral meatus can be considered to be divided
into four portions, with the facial nerve being
anterosuperior, the cochlear nerve anteroinferior,
the superior vestibular nerve posterosuperior, and
the inferior vestibular nerve posteroinferior.
37
Superior vestibular nerve
b Facial nerve
anterior to tumor
Transverse
crest
Cochlear nerve
Inferior vestibular nerve
d
Facial nerve
meatus. (b) The facial nerve is displaced directly anteri-
orly. This is a frequent direction of displacement with
acoustic neuroma. (c) Another frequent direction of dis-
placement of the facial nerve is anterior and superior. (d)
The facial nerve is displaced anteriorly and inferiorly by
tumor, which erodes the superior wall of the meatus above
the nerves and grows into the area above the nerves, dis-
placing them inferiorly (From Rhoton [35])
Retrosigmoid Approach
The retrosigmoid approach to the meatus is
directed through a vertical scalp incision that
crosses the asterion. A burr hole is placed below
the asterion, and a craniotomy is performed expos-
ing the lower margin of the transverse sinus supe-
riorly, the posterior margin of the sigmoid sinus
laterally, and the inferior portion of the squamous
part of the occipital bone inferiorly. The intradural
exposure is directed down the plane between the
posterior face of the temporal bone and the petro-
sal cerebellar surface (Figs. 2.2, 2.3, 2.20, and
2.21) [30, 31, 45]. The petrosal cerebellar surface
usually relaxes away from the temporal bone after
opening the arachnoid membrane over the cisterna
38 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
magna and allowing cerebrospinal fluid to escape.
When removing the posterior meatal wall, it often
is necessary to sacrifice the subarcuate artery
because it passes through the dura on the posterior
meatal wall to reach the subarcuate fossa
(Figs. 2.2, 2.3, and 2.21) [22]. This artery usually
has a sufficiently long stem that its obliteration
does not risk damage to the AICA from which it
arises. In a few cases, however, the subarcuate
a Subarc.A CN VII
CN VIII
Labyr.A
AICA
c d
Fig. 2.23 Artist’s renderings showing posterior view of
the left IAM. (a) Subarcuate artery penetrates the dura of
the subarcuate fossa near the IAM. The labyrinthine artery
enters the meatus with the vestibulocochlear and the facial
nerves. (b) Laterally convex loop of the AICA is embed-
ded in the dura covering the subarcuate fossa, where it
gives off the subarcuate artery. (c) AICA loop is embedded
in the dura and bone (arrow) surrounding the subarcuate
artery and the segment of the AICA from which it
arises will be incorporated into the dura covering
the subarcuate fossa [49, 50]. In this case, the dura
and the artery will have to be separated together
from the posterior meatal lip wall in preparation
for opening the meatus (Fig. 2.23). In some of the
cases, the artery will be surrounded by bone that
has to be drilled to free the artery and access the
meatus.
b
fossa. (d) Dura over the subarcuate fossa has been incised,
and the dura with the adherent loop is dissected free from
the subarcuate fossa in preparation for opening the IAM.
(e) Dura over the subarcuate fossa has been incised and
remains attached to the artery. The bone surrounding the
embedded AICA loop is removed with a 2-mm diamond
drill to displace the artery medially for exposure of the
IAM (From Tanriover and Rhoton [50])
Middle Neurovascular Complex
The posterior semicircular canal and its com-
mon crus with the superior canal, both of which
are situated just lateral to the posterior meatal lip,
should be preserved when removing the posterior
meatal wall if there is the possibility of preserv-
ing hearing, since hearing may be lost if damage
occurs (Fig. 2.24). Care also is required to avoid
injury to the vestibular aqueduct, which is situ-
ated inferolateral to the meatal lip, and the endo-
lymphatic sac, which expands under the dura on
the posterior surface of the temporal bone infero-
lateral to the meatal porus (Fig. 2.24). The
endolymphatic sac may be entered in removing
the dura from the posterior meatal lip. There is
little danger of encountering the cochlear canali-
culus, which has a more medial course below the
internal auditory canal. An unusually high pro-
jection of the jugular bulb into the posterior wall
of the meatus presents an anomaly that may block
access to the posterior meatal lip. Mastoid air
cells commonly are encountered in the posterior
meatal lip.
After removing the posterior wall of the
meatus, the dura lining the meatus is opened to
expose its contents (Figs. 2.3 and 2.21). The
facial nerve is identified near the origin of
the facial canal at the anterosuperior quadrant of
the meatus rather than in a more medial location
where the direction of displacement is variable. If
the tumor extends into the vestibule, the latter can
easily be exposed by drilling removing the poste-
rior wall of the vestibule lateral to the meatal fun-
dus. The strokes of the fine dissecting instruments
along the vestibulocochlear nerve should be
directed from medial to lateral rather than from
lateral to medial, because traction medially may
tear the tiny filaments of the cochlear nerve at the
site where these filaments penetrate the lateral
end of the meatus to enter the modiolus of the
cochlea (Fig. 2.24).
Brain Stem Relationships
A consistent set of neural, arterial, and venous
relationships at the brain stem facilitates the
identification of the nerves on the medial side of
an acoustic neuroma (Figs. 2.25, 2.26, and 2.27)
[31, 32, 37].
39
Neural Relationships
The landmarks on the medial or brain stem side
of structures that are helpful in guiding the sur-
geon to the junction of the facial nerve with the
brain stem are the pontomedullary sulcus; the
junction of the glossopharyngeal, vagus, and spi-
nal accessory nerves with the medulla; the fora-
men of Luschka and its choroid plexus; and the
flocculus. The facial nerve arises from the brain
stem near the lateral end of the pontomedullary
sulcus 1–2 mm anterior to the point at which the
vestibulocochlear nerve joins the brain stem at
the lateral end of the sulcus. The interval between
the vestibulocochlear and facial nerves is greatest
at the level of the pontomedullary sulcus and
decreases as these nerves approach the meatus.
The facial nerve enjoys a consistent relation-
ship to the junction of the glossopharyngeal,
vagus, and spinal accessory nerves with the
medulla (Figs. 2.25, 2.26, and 2.27). The facial
nerve arises 2–3 mm above the most rostral root-
let contributing to these nerves. A helpful way of
visualizing the point where the facial nerve will
exit from the brain stem, even when displaced by
a tumor, is to project an imaginary line along the
medullary junction of the rootlets forming the
glossopharyngeal, vagus, and spinal accessory
nerves upward through the pontomedullary junc-
tion. This line, at a point 2–3 mm above the junc-
tion of the glossopharyngeal nerve with the
medulla, will pass through the pontomedullary
junction at the site where the facial nerve exits
from the brain stem. The filaments of the nervus
intermedius also are stretched around an acoustic
neuroma.
The structures related to the lateral recess of
the fourth ventricle that have a consistent rela-
tionship to the facial and vestibulocochlear nerves
are the foramen of Luschka and its choroid plexus
and the flocculus (Figs. 2.25, 2.26, and 2.27)
[8, 23]. The foramen of Luschka is situated at the
lateral margin of the pontomedullary sulcus, just
behind the junction of the glossopharyngeal
nerve with the brain stem, and immediately pos-
teroinferior to the junction of the facial and ves-
tibulocochlear nerves with the brain stem. The
foramen of Luschka is infrequently well visual-
ized. A consistently identifiable tuft of choroid
plexus, however, hangs out of the foramen of
40 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

a b

c d

e f
Middle Neurovascular Complex
Fig. 2.24 (a) Posterior surface of a right temporal bone.
The internal meatus is located near the center and the jugu-
lar foramen at the lower edge of the posterior surface. The
sigmoid sinus descends along the posterior surface of the mas-
toid part of the temporal bone and turns forward on the
occipital bone to pass through the sigmoid part of the jugu-
lar foramen. The inferior petrosal sinus descends along the
petroclival fissure and passes through the petrosal part of
the jugular foramen. The glossopharyngeal, vagus, and
accessory nerves pass through the intrajugular part of the
foramen between the sigmoid and petrosal part. The subar-
cuate fossa is located superolateral and the ostium for the
endolymphatic duct is positioned lateral to the internal
acoustic meatus. The trigeminal impression is a shallow
trough on the upper surface of the petrous part behind the
foramen ovale. The arcuate eminence overlies the superior
semicircular canal. (b) View of the fundus of the right inter-
nal acoustic meatus. The transverse crest divides the meatal
fundus into superior and inferior parts. Above the transverse
crest, the facial canal is anterior and the superior vestibular
area is posterior. Below the transverse crest, the cochlear
area is anterior and the inferior vestibular area is posterior.
The singular foramen, through which the singular branch of
the inferior vestibular nerve passes to innervate the posterior
canal ampullae, is located posterior to the inferior vestibular
area. The inferior vestibular nerve also has a saccular and,
occasionally, an utricular branch. The cochlear nerve splits
into tiny filaments as its fibers pass through the cochlear
area. These filaments are easily torn, with a resulting loss of
hearing, with medially directed retraction of the cerebellum
and nerve. (c) The facial and vestibulocochlear nerves enter
the internal acoustic meatus, and the glossopharyngeal,
vagus, and accessory nerves enter the jugular foramen. The
trigeminal nerve passes above the petrous apex and through
the porus of Meckel’s cave. The posterior and superior
semicircular canals have been exposed. The superior semi-
circular canal is positioned below the medial edge of the
arcuate eminence. The upper end of the posterior canal and
the posterior end of the superior canal join to form a com-
mon channel, the common crus, which opens into the vesti-
bule. The endolymphatic duct extends downward from the
vestibule and opens into the endolymphatic sac located
beneath the dura inferolateral to the internal acoustic
meatus. The endolymphatic ridge, the bridge of bone form-
ing the upper lip of the endolymphatic duct, has been pre-
served. The jugular bulb can be seen through the thin bone
below the internal meatus. Entering the posterior canal,
common crus, posterior portion of the superior canal, or the
41
vestibule during drilling of the posterior meatal wall may
result in a loss of hearing. (d) Enlarged view of the fundus
of the meatus after removal of the posterior wall. The upper
edge of the porus has been preserved. The facial nerve and
nervus intermedius are exposed medial to the porus of the
meatus. The subarcuate artery enters the subarcuate fossa.
The inferior vestibular nerve gives rise to the singular
branch to the posterior ampullae, plus utricular and saccular
branches. The superior vestibular nerve innervates the
ampullae of the superior and lateral semicircular canals and
commonly gives rise to a utricular branch. Care is taken to
preserve the superior and posterior canals and the common
crus, plus the endolymphatic sac in those cases in which
there is the opportunity to preserve hearing when drilling
the posterior wall of the meatus. (e) The petrous apex medial
to the internal acoustic meatus has been partially removed
to expose the petrous segment of the internal carotid artery.
The lateral genu of the petrous carotid, located at the junc-
tion of the vertical and horizontal segments, is situated
below and medial to the cochlea. The jugular bulb extends
upward, adjacent the posterior meatal wall, toward the ves-
tibule and semicircular canals. The inferior petrosal sinus
courses along the petroclival fissure and enters the petrosal
part of the jugular foramen. The sigmoid sinus descends in
the sigmoid sulcus and enters the sigmoid part of the fora-
men. The glossopharyngeal, vagus, and accessory nerves
pass through the central or intrajugular part of the jugular
foramen located between the sigmoid and petrosal parts. (f)
Bone has been removed along the anterior margin of the
meatal fundus to open the cochlea and along the posterior
margin to expose the vestibule. The cochlear nerve pene-
trates the modiolus of the cochlea where its fibers are dis-
tributed to the turns of the cochlear duct. The basal turn of
the cochlea communicates below the modiolus with the ves-
tibule. The stapes has been removed from the oval window.
The promontory in the medial wall of the tympanic cavity is
located lateral to the basal turn of the cochlea. A silver fiber
has been introduced into the superior semicircular canal, a
red fiber into the lateral canal, and a blue fiber into the pos-
terior canal. The ampullated ends of the canals are located
at the bulbous ends of the three fibers. The common crus of
the superior and posterior canals is located where the tips of
the blue and silver fibers cross. The superior vestibular
nerve passes to the ampullae of the superior and lateral
canals. The singular branch of the inferior vestibular nerve
innervates the posterior ampullae. A small black fiber has
been introduced into the opening of the endolymphatic duct
into the vestibule (From Rhoton [33])
42 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a b
Fig. 2.25 Neurovascular relationships on the brain stem
side of an acoustic neuroma. Anterolateral view of the
right cerebellopontine angle. (a) Neural relationships.
The facial and vestibulocochlear nerves arise from the
brain stem near the lateral end of the pontomedullary sul-
cus, anterosuperior to the choroid plexus protruding from
the foramen of Luschka, anterior to the flocculus, rostral
to a line drawn along the junction of the rootlets of the
glossopharyngeal, vagus, and accessory nerves with
the brain stem, and slightly posterior to the rostral pole of
the inferior olive. The cerebellopontine fissure formed by
the cerebellum wrapping around the lateral side of the
pons and middle cerebellar peduncle has a superior limb
that passes above the trigeminal nerve and an inferior limb
that extends below the foramen of Luschka. The cerebel-
lomedullary fissure, which extends superiorly between the
medulla and cerebellum, communicates in the region of
the foramen of Luschka with the cerebellopontine fissure.
(b) Arterial relationships. The AICA arises from the basi-
lar artery and divides into rostral and caudal trunks. The
rostral trunk, which is usually the larger of the two trunks,
courses below the facial and vestibulocochlear nerves and
then above the flocculus to reach the surface of the middle
cerebellar peduncle. The PICA arises from the vertebral
artery and passes first between the hypoglossal rootlets
and then between the vagus and accessory nerves on its
way to the cerebellar hemisphere. The SCA passes above
the trigeminal nerve. The cerebellar arteries give rise to
hemispheric branches. (c) Venous relationships. The veins
that converge on the junction of the facial and vestibuloco-
chlear nerves with the brain stem are the veins of the pon-
tomedullary sulcus, cerebellomedullary fissure, middle
cerebellar peduncle, and the retro-olivary and lateral med-
ullary veins. The vein of the cerebellopontine fissure,
which passes above the flocculus on the middle cerebellar
peduncle, is formed by the anterior hemispheric veins that
arise on the cerebellum. Transverse pontine and transverse
medullary veins cross the pons and medulla. The median
anterior medullary and median anterior pontomesenceph-
alic veins ascend on the anterior surface of the medulla
and pons. The veins of the middle cerebellar peduncle and
the cerebellopontine fissure and a transverse pontine vein
join to form a superior petrosal vein, which empties into
the superior petrosal sinus. A bridging vein passes below
the vagal rootlets toward the jugular foramen. (d)
Neurovascular relationships of an acoustic neuroma. The
tumor arises from the vestibulocochlear nerve and dis-
places the facial nerve anteriorly, the trigeminal nerve
superiorly, and the vagus and glossopharyngeal nerves
inferiorly. The facial nerve, even though displaced by the
tumor, enters the brain stem along the lateral margin of the
pontomedullary sulcus, rostral to the glossopharyngeal
and vagus nerves, anterior to the flocculus, and rostral to
the choroid plexus protruding from the foramen of
Luschka. The rostral trunk of the AICA, after passing
below the tumor, returns to the surface of the middle cer-
ebellar peduncle above the flocculus. The veins displaced
around the medial side of the tumor are the veins of the
middle cerebellar peduncle, cerebellomedullary fissure,
cerebellopontine fissure and pontomedullary sulcus, and
the retro-olivary and lateral medullary veins (From
Rhoton [37])
Middle Neurovascular Complex
c d
Fig. 2. 25 (continued)
Luschka and sits on the posterior surface of the
glossopharyngeal and vagus nerves just inferior
to the junction of the facial and vestibulocochlear
nerves with the brain stem. Another structure
related to the lateral recess, the flocculus, proj-
ects from the margin of the lateral recess and
foramen of Luschka into the cerebellopontine
angle, just posterior to where the facial and ves-
tibulocochlear nerves join the pontomedullary
sulcus.
Arterial Relationships
The arteries crossing the cerebellopontine angle,
especially the AICA, enjoy a consistent relation-
ship to the facial and vestibulocochlear nerves,
the foramen of Luschka, and the flocculus as
described elsewhere in this volume [11, 12, 21,
22]. In a prior study, the author’s group found
that the AICA formed a loop that reached the
porus or protruded into the canal in 54 % of
the cases [22]. When opening the meatus by the
43
middle fossa, translabyrinthine, or posterior
approaches, care is required to avoid injury to
the AICA if it is located at or protrudes through
the porus.
In most cases, the AICA passes below the
facial and vestibulocochlear nerves as it encircles
the brain stem, but it also may pass above or
between these nerves in its course around the
brain stem (Fig. 2.28). In the most common case,
in which the artery passes below the nerves, the
tumor would displace the artery inferiorly. If the
artery courses between the facial and vestibulo-
cochlear nerves, a tumor arising in the latter nerve
will displace the artery forward. Tumor growth
would displace the artery superiorly if it passes
above the nerves. Atkinson pointed out that those
cases of acoustic neuroma appearing at necropsy
after operation frequently had occlusion of the
AICA [1]. In three cases presented by Atkinson,
an arterial branch coursing over the tumor cap-
sule was ligated with resulting lateral pontine,
44 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a b
Fig. 2.26 Relationship of the foramen of Luschka and
the lateral recess of the fourth ventricle to the junction of
the facial and vestibulocochlear nerves with the brain-
stem, as seen through a suboccipital craniotomy. (a) The
orientation, skin incision (solid line), and craniotomy
(interrupted line) are shown in the insert. The foramen of
Luschka opens into the cerebellopontine angle behind the
glossopharyngeal and vagus nerves. The choroid plexus
protrudes from the foramen of Luschka, slightly below
and behind the facial and vestibulocochlear nerves, and
behind to the glossopharyngeal and vagus nerves. The
flocculus protrudes into the cerebellopontine angle above
the foramen of Luschka. The accessory nerve arises below
the vagus nerve. The hypoglossal rootlets arise ventral
to the olive. The trigeminal nerve crosses in the upper part
of the exposure. (b) The right cerebellar tonsil has been
removed by dividing the tonsillar peduncle to show the
relationship of the lateral recess to the facial and vestibu-
locochlear nerves. The flocculus and choroid plexus pro-
trude in the cerebellopontine angle behind the junction of
the facial and vestibulocochlear nerves with the brain-
stem. The inferior medullary velum stretches from the
lateral side of the vermis to the flocculus and is all that
remains of the connection between the flocculus and the
nodulus, which form the flocculonodular lobe of the cer-
ebellum. The inferior medullary velum stretches laterally
to form the peduncle of the flocculus. The tela choroidea
forms the caudal part of the roof of the fourth ventricle
and has the choroid plexus attached to its inner surface.
The facial and vestibulocochlear nerves enter the brain-
stem at the lateral end of the pontomedullary sulcus.
(c) The tela choroidea has been opened, but the choroid
plexus, which arises on the inner surface of the tela in the
fourth ventricle, has been preserved. The fringelike chor-
oid plexus extends through the foramen of Luschka
slightly below and behind the junction of the facial and
vestibulocochlear nerves with the brainstem. The inferior
cerebellar peduncle ascends on the dorsolateral margin of
the medulla. (d) Relationships of an acoustic neuroma.
The facial nerve is displaced anteriorly and superiorly in
the cerebellopontine angle and enters the brainstem at the
lateral end of the pontomedullary sulcus, anterosuperior
to the choroid plexus protruding from the foramen of
Luschka, and near where the flocculus is attached along
the margin of the lateral recess. The tumor displaces the
trigeminal nerve upward and the glossopharyngeal and
vagus nerves downward. The AICA gives rise to a subar-
cuate artery, which enters the subarcuate fossa in the pos-
terior wall of the internal acoustic meatus and bifurcates
into a rostral and a caudal trunk. The rostral trunk courses
above the flocculus to reach the surface of the middle cer-
ebellar peduncle. [37] (From, Rhoton AL Jr: Microsurgical
anatomy of the brainstem surface facing an acoustic neu-
roma. Surg Neurol 25:326–339, 1986.)
Middle Neurovascular Complex
c d
Fig. 2. 26 (continued)
tegmental, and medullary infarction in the area
supplied by the AICA and death. He noted that
the blood pressure rose at or near the time of
occlusion of the artery, although the hypertension
often subsided by the end of the operation. These
tumors may also displace the PICA and insinuate
themselves between the basilar artery and the
pons, stretching the perforating branches of the
basilar artery. The labyrinthine, recurrent perfo-
rating, and subarcuate branches arise from the
AICA near the facial and vestibulocochlear
nerves and are frequently stretched around a cer-
ebellopontine angle tumor.
Venous Relationships
The veins on the side of the brain stem that have
a predictable relationship to the facial and
vestibulocochlear nerves are the vein of the
pontomedullary sulcus, the veins of the cerebel-
lomedullary fissure, middle cerebellar pedun-
cle, and cerebellopontine fissure (Figs. 2.25,
2.26, and 2.27) [37, 40–42]. The identification
of any of these veins during the removal of the
tumor makes it easier to identify the site of the
45
junction of the facial and vestibulocochlear
nerves with the brain stem. These veins, which
cross the cerebellopontine angle to reach the
superior petrosal sinus, are the ones most
frequently occluded in the course of operations
in the cerebellopontine angle. Bridging veins
are exposed more frequently and sacrificed in
the rostral part of the cerebellopontine angle
during operations near the trigeminal nerve
than during operations near the nerves entering
the internal acoustic meatus. The exposure of
an acoustic neuroma in the central part of the
cerebellopontine angle near the lateral recess
usually can be completed without sacrificing a
bridging vein. If a vein is obliterated during
acoustic tumor removal, it is usually one of the
superior petrosal veins, which is sacrificed near
the superior pole of the tumor during the later
stages of the removal of a large tumor. Small
acoustic neuromas usually are removed without
sacrificing a petrosal vein. The largest vein
encountered around the superior pole of an
acoustic neuroma is the vein of the cerebello-
pontine fissure.
46 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
Summary: Anatomy of Acoustic
Neuromas
Because acoustic neuromas most frequently arise
in the posteriorly placed vestibular nerves, they
usually displace the facial and cochlear nerves
anteriorly (Figs. 2.25 and 2.29). The facial nerve
a b
Fig. 2.27 Neurovascular relationships on the brainstem
side of an acoustic neuroma. Anterosuperior views.
(a) Neural relationships. The cerebrum and tentorium cere-
belli have been removed, and the trigeminal, trochlear, and
oculomotor nerves have been divided to allow the brainstem
to be displaced posteriorly to expose the cerebellopontine
angle from above. The facial and vestibulocochlear nerves
arise at the lateral end of the pontomedullary sulcus anterior
to the flocculus, rostral to the glossopharyngeal, vagus, and
accessory nerves, and anterosuperior to the choroid plexus
protruding from the foramen of Luschka. The cerebellopon-
tine fissure, formed where the cerebellum wraps around the
lateral side of the pons and middle cerebellar peduncle, has
superior and inferior limbs. The foramen of Luschka opens
into the inferior limb near the facial and vestibulocochlear
nerves. (b) Arterial relationships. The AICA arises from the
basilar artery, passes below the facial and vestibulocochlear
nerves, gives rise to the subarcuate artery, and divides into a
rostral and a caudal trunk. The rostral trunk passes above the
flocculus to course on the middle cerebellar peduncle, and
the caudal trunk supplies the area below the flocculus.
(c) Venous relationships. The veins converging on the junc-
tion of the facial nerve with the brainstem are the lateral
medullary and retro-olivary veins, and the veins of the pon-
tomedullary sulcus, cerebellomedullary fissure, and middle
cerebellar peduncle. The median anterior pontomesenceph-
alic vein ascends on the anterior surface of the brainstem,
is stretched around the anterior half of the tumor
capsule. Variability in the direction of the growth
of the tumor arising from the vestibular nerves
may result in the facial nerve being displaced, not
only directly anteriorly but also anterosuperiorly
or anteroinferiorly. The nerve infrequently is
found on the posterior surface of the tumor.
and the transverse pontine and transverse medullary veins
cross the pons and medulla. The vein of the cerebellopontine
fissure passes above the flocculus. The transverse pontine
vein and the veins of the middle cerebellar peduncle and
cerebellopontine fissure join to form one of the superior pet-
rosal veins that empty into the superior petrosal sinus. A
bridging vein passes from the side of the brainstem to the
jugular foramen. The anterolateral marginal vein crosses the
anterolateral margin of the cerebellum. The vein of the pon-
tomesencephalic sulcus courses in the pontomesencephalic
sulcus below the oculomotor nerve. (d) Neurovascular rela-
tionships of an acoustic neuroma. The tumor arises from the
vestibulocochlear nerve and displaces the facial nerve ante-
riorly, the trigeminal nerve superiorly, and the glossopharyn-
geal and vagus nerves inferiorly. The vestibulocochlear
nerve disappears into the tumor. The facial nerve enters the
brainstem along the lateral margin of the pontomedullary
sulcus, rostral to the glossopharyngeal nerve, anterior to the
flocculus, and rostral to the choroid plexus protruding from
the foramen of Luschka. The AICA is usually displaced
around the lower margin of the tumor. The veins displaced
around the medial side of the tumor are the veins of the pon-
tomedullary sulcus, middle cerebellar peduncle, and cere-
bellomedullary fissure, and the lateral medullary and
retro-olivary veins. [37] (From, Rhoton AL Jr: Microsurgical
anatomy of the brainstem surface facing an acoustic neu-
roma. Surg Neurol 25:326–339, 1986.)
Middle Neurovascular Complex
c d
Fig. 2. 27 (continued)
Because the facial nerve always enters the facial
canal at the anterosuperior quadrant of the meatal
fundus, it usually is easiest to locate it here rather
than at a more medial location where the degree
of displacement of the nerve is more variable.
The cochlear nerve also lies anterior to the ves-
tibular nerve and is stretched most frequently
around the anterior half of the tumor. The strokes
of the fine dissecting instruments used in remov-
ing the tumor should be directed along the ves-
tibulocochlear nerve from medial to lateral rather
than from lateral to medial, because traction
medially may tear the tiny filaments of the
cochlear nerve at the site where these filaments
penetrate the lateral end of the meatus to enter the
cochlea (Figs. 2.24 and 2.30).
The operation for a cerebellopontine angle
tumor should be planned so that the tumor sur-
face is allowed to settle away from the neural tis-
sue rather than the neural structures being
retracted away from the tumor (Fig. 2.30). No
attempt is made to see the whole tumor upon
initial exposure. The surface of the tumor then
is opened, and the intracapsular contents are
removed. As the intracapsular contents are
evacuated, the tumor shifts laterally, allowing
more of the tumor to be removed through the
small exposure. The most common reason for the
47
tumor appearing to be tightly adherent to the neu-
ral structures is not adhesions between the cap-
sule and surrounding tissue but, rather, the
residual tumor within the capsule wedging the
tumor into position. As the intracapsular contents
are removed, the tumor capsule folds laterally,
revealing the structures on the brain stem side of
the tumor.
The landmarks that are helpful in identify-
ing the facial and vestibulocochlear nerves at
the brain stem on the medial side of the tumor
have been reviewed (Figs. 2.25, 2.26, 2.27, and
2.29) [37]. These nerves, although distorted
by the tumor, usually can be identified on the
brain stem side of the tumor at the lateral end
of the pontomedullary sulcus just rostral to the
glossopharyngeal nerve and just anterosuperior
to the foramen of Luschka, the flocculus, and
the choroid plexus protruding from the foramen
of Luschka. After the facial and vestibuloco-
chlear nerves are identified on the medial and
lateral sides of the tumor, the final remnants
of the tumor are separated from the interven-
ing segment of the nerves using fine dissect-
ing instruments (Fig. 2.30). It is especially
important to preserve the segment of the cer-
ebellar arteries adherent to the tumor capsule
because a major cause of operative mortality
48 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

S.C.A
Pons Dura
Tent.
Cerebellum
V
Tumor VII
R.P.A VIII S.V.
VIII Co.
Trans.crest
Flocculus
Ch.Pl. VIII I.V.
S.A.
Post.mea.seg.
Pre.mea.seg.
IX

P.I.C.A XI X
S.C.A.
Post.mea.seg.
Mea.seg.
V I.A.A.
VIII S.V.
VII

S.C.A. VIII I.V.

VIII co.
Pre.mea.seg.
R.P.A. IX
R.P.A.
V X
Mea.seg.
Post.mea.seg. P.I.C.A.

VII
VIII S.V. I.A.A.
VIII I.V. VIII Co.
Pre.mea.seg. IX
Medulla X
P.I.C.A.
XI

Fig. 2.28 Posterior views of the direction of displacement
of the AICA around an acoustic neuroma. Top left: The
insert shows the direction of view. Both the premeatal and
the postmeatal segments are in their most common loca-
tions around the lower margin of the tumor. The premeatal
segment approaches the meatus from anteroinferior, and
the postmeatal segment passes posteroinferior to the
tumor. The SCA and the trigeminal nerve are above the
tumor, and the PICA and the glossopharyngeal, vagus, and
spinal accessory nerves are below the tumor. The choroid
plexus protrudes into the cerebellopontine angle medial to
the tumor. The posterior wall of the internal acoustic canal
has been removed to expose the transverse crest and the
superior vestibular and inferior vestibular nerves. The
vestibular nerves disappear into the tumor; however, the
cochlear and facial nerves are displaced around the ante-
rior margin of the tumor. A subarcuate artery arises from
the premeatal segment, and a recurrent perforating artery
arises from the postmeatal segment. Center right: In a less
common pattern of displacement of the AICA, the pre-
meatal and postmeatal segments are above the tumor. The
internal auditory arteries arise from the meatal segment.
Bottom left: Both the premeatal and the postmeatal seg-
ments are displaced anteriorly to the tumor. This occurs if
the AICA courses between the vestibulocochlear and
facial nerves. The tumor arises in the vestibular nerves,
and tumor growth displaces both the premeatal and the
postmeatal segments anteriorly (From Martin et al. [22])
Anatomy of Vascular Compression in the Middle Neurovascular Complex
and morbidity is the loss of perforating arteries
and branches of the cerebellar arteries that may
be adherent to and displaced by the tumor. Any
vessel that stands above or is stretched around
the tumor capsule should be dealt with initially
as if it were an artery that runs over the tumor
surface to supply the brain. After the tumor
has been removed from within the capsule, an
attempt should be made to displace the vessel
off the tumor capsule. When dissected free of
the capsule, vessels that initially appeared to be
adherent to the capsule often prove to be neural
vessels. The number of veins sacrificed should
be kept to a minimum because of the undesir-
able consequences of their loss. Obliteration of
the petrosal veins, which pass from the surface
of the cerebellum and the brain stem to the
superior petrosal sinus, is inescapable when
reaching and removing some cerebellopontine
angle tumors. Occlusion of these veins, which
drain much of the cerebellum and the brain
stem, infrequently may cause hemorrhagic
edema of the cerebellum and the brain stem.
Some of these veins may need to be sacrificed if
the tumor extends into the area above the inter-
nal acoustic meatus. Small acoustic neuromas
and other tumors in the lower part of the cer-
ebellopontine angle, however, frequently may
be removed without sacrificing a petrosal vein.
In removing the posterior meatal lip, a commu-
nication may be established between the suba-
rachnoid space and the mastoid air cells that
will require careful closure to prevent a cere-
brospinal fluid leak. Laying a small pledget
of crushed fat over the drilled meatal area has
been successful in minimizing this complica-
tion. The retrosigmoid approach is used by this
author for most acoustic neuromas because it is
suitable for the removal of both small and large
tumors. Unlike the translabyrinthine approach,
described in the section on the temporal bone,
which is directed through the vestibule and
semicircular canals, the retrosigmoid approach
is not necessarily associated with hearing loss.
The retrosigmoid approach provides a broader
exposure of the small tumor than does the mid-
dle fossa approach. Also, once the nerves are
identified lateral to the tumor, there are advan-
49
tages to being able to separate the tumor cap-
sule off the nerves beginning medially for this
more often results in preservation of hearing
than dissection starting laterally. Compared to
the middle fossa approach, it has the advantage
that the facial nerve is usually deep to the tumor
and often is protected by a thin veil of vestibu-
locochlear nerve, thus increasing the opportu-
nity for facial nerve preservation. In the middle
fossa approach, the facial nerve is often in the
upper part of the exposure stretched over the
upper half of the tumor and much of the dis-
section is directly on the surface of the nerve,
which increases the risk of facial dysfunction
after surgery.
Anatomy of Vascular Compression
in the Middle Neurovascular Complex
Compression of the facial and vestibulocochlear
nerves by tortuous arteries is postulated to cause
dysfunction of these nerves, and cases in which
surgical liberation of the vessels from these nerves
has relieved the symptoms provide support for a
vascular compressive etiology (Figs. 2.31 and
2.32) [9, 16, 32]. Ectasia and elongation of the
arteries are important in forcing the arteries into
the nerves. Gardner was the first to treat hemifa-
cial spasm by removing a compressive arterial
loop from the facial nerve [9]. Jannetta et al.,
using the suboccipital approach to the cerebello-
pontine angle, found mechanical compression
and distortion of the root exit zone of the facial
nerve in all of 47 patients with hemifacial spasm
[16]. The distorting vessel not only was the AICA
and its branches, but in some cases was found to
be the PICA, the vertebral or basilar artery, veins,
or an arteriovenous malformation (Figs. 2.31 and
2.32) [16]. It is expected that the AICA would be
the compressing vessel in most cases because the
facial nerve is located in the middle neurovascu-
lar complex. However, a tortuous PICA is an
equally frequent offending vessel in hemifacial
spasm followed in order by the vertebral artery,
basilar artery, veins, and a combination of these
vessels (Figs. 2.31 and 2.32). The proximal part
of the PICA usually passes around the brain stem
50 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

d
Anatomy of Vascular Compression in the Middle Neurovascular Complex
Fig. 2.29 Neurovascular relationships on the brain stem
side of an acoustic neuroma. Posterior view through a ret-
rosigmoid craniotomy. (a) Neural relationships. The ori-
entation, skin incision (solid line), and craniotomy site
(interrupted line) are shown in the insert. The retractor is
on the petrosal surface of the cerebellum. The facial and
vestibulocochlear nerves arise at the lateral end of the
pontomedullary sulcus, anterior to the flocculus, rostral to
the glossopharyngeal, vagus, and accessory nerves, and
anterosuperior to the choroid plexus protruding from the
foramen of Luschka. The cerebellopontine fissure, formed
where the cerebellum wraps around the lateral side of the
pons and middle cerebellar peduncle, has superior and
inferior limbs. (b) Arterial relationships. The AICA arises
from the basilar artery and divides into a rostral trunk,
which passes above the flocculus to reach the surface of
the middle cerebellar peduncle, and a caudal trunk, which
supplies the area below the flocculus. The PICA arises
from the vertebral artery and passes dorsally between the
vagus and accessory nerves. The SCA courses above the
trigeminal nerve. (c) Venous relationships. The veins that
join near the junction of the facial and vestibulocochlear
nerves with the brain stem are the lateral medullary veins
and the veins of the cerebellomedullary fissure, pon-
tomedullary sulcus, and middle cerebellar peduncle. The
below the facial and vestibulocochlear nerves. In
some cerebellopontine angles, however, the prox-
imal part of the PICA, after coursing posteriorly
to the level of the hypoglossal rootlets, will loop
superiorly toward the facial and vestibulocochlear
nerves before descending to pass between the
glossopharyngeal, vagus, and spinal accessory
nerves.
The offending arterial loop may be located
on either the superior or the inferior aspect of
the facial nerve at its exit from the brain stem.
In the most common type of hemifacial spasm,
that beginning in the orbicularis oculi muscle
and gradually spreading downward to involve
the lower face, the anteroinferior aspect of the
nerve root exit zone will commonly be com-
pressed. Atypical hemifacial spasm, a much
less common entity, beginning in the lower or
midface and spreading upward to involve the
frontalis muscle, will be caused by the compres-
sion of the posterosuperior aspect of the facial
nerve at the brain stem. Jannetta and others
thought that the arteries frequently seen cours-
ing around or between the facial and vestibulo-
51
vein of the cerebellopontine fissure passes above the
flocculus along the superior limb of the cerebellopontine
fissure and joins the vein of the middle cerebellar pedun-
cle and a transverse pontine vein to form a superior petro-
sal vein, which empties into the superior petrosal sinus.
A bridging vein passes behind the vagus nerve. The lateral
anterior pontomesencephalic vein ascends on the pons.
(d) Neurovascular relationships of an acoustic neuroma.
The tumor arises from the vestibulocochlear nerve and
displaces the facial nerve anteriorly, the trigeminal nerve
superiorly, and the glossopharyngeal and vagus nerves
inferiorly. The vestibulocochlear nerve disappears into the
tumor. The facial nerve enters the brain stem at the lateral
margin of the pontomedullary sulcus anterior to the
flocculus and rostral to the choroid plexus protruding from
the foramen of Luschka. The rostral trunk of the AICA
courses below the tumor and above the flocculus to reach
the surface of the middle cerebellar peduncle. The veins
displaced around the medial side of the tumor are the lat-
eral medullary veins and the veins of the middle cerebellar
peduncle, cerebellomedullary fissure, and pontomedul-
lary sulcus. The vein of the cerebellopontine fissure passes
above the tumor. A recurrent perforating branch of the
AICA passes across the tumor and supplies the brain stem
(From Rhoton [37])
cochlear nerves in the interval between the brain
stem and porus acusticus, as found by Gardner
[9], were not the cause of hemifacial spasm, but
that cross compression of the facial nerve by the
same arteries coursing at right angles to the
nerve at the root exit zone was the essential ele-
ment [16]. The craniotomy for hemifacial spasm
is positioned behind the lower half of the
sigmoid sinus.
The operation for hemifacial spasm is directed
along the inferolateral margin of the cerebellum
(Figs. 2.33 and 2.34). The craniotomy is located
medial to the lower half of the sigmoid sinus. It is
not necessary to extend the bone opening down-
ward to the foramen magnum or upward to the
transverse sinus. The inferolateral margin of the
cerebellum is elevated with a small brain spatula,
and the arachnoid behind the glossopharyngeal
and vagus nerves is opened.
This will expose the tuft of choroid plexus
protruding from the foramen of Luschka, which
sits on the posterior surface of the glossopharyn-
geal and vagus nerves. Commonly, the flocculus
is seen protruding behind the nerves and blocks
52 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

b c

d e
Anatomy of Vascular Compression in the Middle Neurovascular Complex
Fig. 2.30 Retrosigmoid approach for removal of small-
or medium-sized acoustic neuromas. (a) The patient is
positioned in the three-quarter prone position with the
surgeon behind the head. The insert (right) shows the site
of the scalp incision (continuous line) and the bony open-
ing (interrupted line). (b) The posterior wall of the inter-
nal auditory canal is removed using an irrigating drill.
The AICA courses around the lower margin of the tumor.
(c) The intracapsular contents of the tumor have been
removed. The capsule of the tumor is being separated
from the pons and the posterior surface of the part of the
facial and vestibulocochlear nerves adjacent to the brain
stem. The superior and inferior vestibular nerves are seen
their visualization at the junction with the brain
stem. It also may be difficult to see the facial
nerve that is hidden in front of the vestibuloco-
chlear nerve. At this time, it is important to recall
that the facial nerve root exits the brain stem
2–3 mm rostral to the point at which the
glossopharyngeal nerve enters the brain stem. To
expose the nerve’s exit zone, it may be necessary
to gently separate the choroid plexus from the
posterior margin of the glossopharyngeal nerve
so that its junction with the brain stem can be
seen. The brain spatula is advanced upward to
elevate the choroid plexus away from the poste-
rior margin of the glossopharyngeal nerve. The
exposure is then directed several millimeters
above the glossopharyngeal nerve to where the
facial nerve will be seen joining the brain stem
below and in front of the vestibulocochlear nerve.
The spatula often needs to be positioned so that it
elevates the lower margin of the flocculus, which
results in the approach being referred to as an
infrafloccular approach. Care must be taken to
avoid damage to the vestibulocochlear nerve,
which may be adherent to the flocculus. In my
experience, the most common offending artery is
a PICA that loops upward prior to passing
between the glossopharyngeal, vagus, and spinal
accessory nerves. After looping into the facial
nerve exit zone, the PICA then passes distally
between the rootlets of the lower cranial nerves.
The compressing artery may also be the pre-
meatal or postmeatal segments of the AICA or a
tortuous vertebral or basilar artery. Care is taken
to explore the interval between the facial and ves-
53
at the lateral end of the internal auditory canal. The
trigeminal nerve and SCA are above the tumor, and the
glossopharyngeal and vagus nerves and the PICA are
below the tumor. (d) The dissection along the eighth
nerve is done in a medial to lateral direction (arrows) in
order to avoid tearing the tiny filaments of the cochlear
nerve in the lateral end of the canal where they pass
through the lamina cribrosa. The transverse crest sepa-
rates the superior and inferior vestibular nerves in the lat-
eral end of the canal. (e) Cerebellopontine angle and
internal auditory canal after tumor removal. The facial
and vestibulocochlear nerves have been preserved (From
Rhoton [31])
tibulocochlear nerves because it would be easy to
miss a vessel compressing the facial nerve in this
location.
Venous compression is less commonly encoun-
tered. The most common venous compression is
by the vein of the pontomedullary sulcus, the
retro-olivary vein, or the vein of the middle cere-
bral peduncle [24]. The vein of the pontomedul-
lary sulcus and the retro-olivary vein commonly
join in the region of the facial nerve to form the
vein of the middle cerebellar peduncle, which
ascends on the middle cerebral peduncle toward
the superior petrosal sinus. The vein of the middle
cerebral peduncle commonly passes between the
facial and vestibulocochlear nerves. It is not
uncommon to encounter a bridging vein that
passes from the lateral side of the medulla to the
jugular bulb. At the time of elevating the cerebel-
lum, it is best to obliterate this vein with gentle
bipolar coagulation.
Cochlear and Vestibular Nerve
Compression Syndromes
Vascular compression has been reported as a
cause of cochlear and vestibular nerve dysfunction
manifested by tinnitus, hearing loss, disequilib-
rium, and disabling positional vertigo [18, 25,
27]. The site of the compressive lesion with ves-
tibulocochlear nerve dysfunction has been
reported to be more peripheral along the nerve
rather than at the junction with the brain stem, as
commonly seen in trigeminal neuralgia and
54 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a III
IV
Pre.Mea.Seg.
Post.Mea.Seg.
VIII
Mea.Seg.
VII
Ch.Pl.
Pons
P.IC.A
VI
V.A Medulla
b III
P.C.A
V V
A.I.C.A.
VII
VIII
V.A.
X IX
Fig. 2.31 Sites of arterial compression of the facial nerve
in hemifacial spasm. (a) Anterosuperior view. The facial
and vestibulocochlear nerves are distorted at their junc-
tion with the brain stem by the right premeatal and the left
postmeatal segments of the AICAs. (b) Anterior view.
hemifacial spasm. Jannetta and others have
restricted the use of the operation for vestibulo-
cochlear nerve symptoms to those patients who
are disabled and have documented unilateral dis-
ease on neuro-otologic testing. Jannetta et al. [18]
and Gardner [9] have postulated that vascular
compression of a cranial nerve is more likely to
be symptomatic when it is located on the nerve
proximal to the Obersteiner–Redlich zone where
the axons are insulated by central myelin pro-
duced by oligodendroglia. It is proximal to this
glial-neurilemmal junction that the compression
causes transaxonal excitement between the affer-
ent and efferent fibers. This glial-neurilemmal
junction on the facial and trigeminal nerves is
situated at the nerve root junction with the brain
P.C.A
S.C.A
Post.Mea.Seg.
Flocculus
VIII
VII
Pre.Mea.Seg.
IX
X
XI
V.A
S.C.A
A.I.C.A.
VII
VIII
P.I.C.A.
Flocculus
VI
The junction of the right facial and vestibulocochlear
nerves with the brain stem is compressed by a tortuous
vertebral artery. The nerves on the left side are compressed
by the PICA (From Martin et al. [22])
stem, but the entire intracranial portion of the
vestibulocochlear nerve is sensitive to compres-
sion because the glial-neurilemmal junction is
located at or in the internal acoustic meatus [18].
Compression by veins is less common around
the facial and vestibulocochlear nerves than in
the region of the trigeminal nerve because the
veins around the facial and vestibulocochlear
nerves tend to be smaller. Because no large bridg-
ing veins cross the subarachnoid space around
the facial and vestibulocochlear nerves, as are
seen frequently around the trigeminal nerve, any
vascular cross compression of facial and vestibu-
locochlear nerves peripheral to the brain stem is
likely to be caused by arteries that loop through
the cerebellopontine angle and even into the
Anatomy of Vascular Compression in the Middle Neurovascular Complex
Fig. 2.32 (a) The PICA arises from the vertebral artery,
passes between the rootlets of the hypoglossal nerve, and
loops superiorly under the glossopharyngeal and vagus
nerves before passing posteroinferiorly between the root-
lets of the vagus and spinal accessory nerves. The verte-
bral artery stretches the rootlets of the hypoglossal nerve
posteriorly. The AICA loops posterior to the facial and
vestibulocochlear nerves. (b) A tortuous PICA arises from
the vertebral artery and passes rostrally toward the ves-
tibulocochlear and facial nerves. At the level of the ves-
tibulocochlear nerve, it loops inferiorly and descends
anterior to the glossopharyngeal and vagus nerves, and
55
passes between the vagus and spinal accessory nerves.
The PICA compresses the medulla anterior to the origin
of the glossopharyngeal and vagus nerves. The choroid
plexus protrudes from the foramen of Luschka posterior
to the glossopharyngeal nerve. The cerebellar peduncles
are above the lateral recess of the fourth ventricle. (c) The
vertebral artery displaces and stretches the hypoglossal
rootlets so far posteriorly that they intermingle with the
rootlets of the spinal accessory nerve. The PICA descends
between the rootlets of the spinal accessory nerve (From
Lister [21])
56 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
meatus. The veins at the level of the junction of
the facial and vestibulocochlear nerves with the
brain stem tightly hug the pontomedullary junc-
tion where they are adhere to the pial membrane
as described in the section on hemifacial spasm.
Geniculate Neuralgia
Sectioning the nervus intermedius for genicu-
late neuralgia requires an understanding of the
a b
Fig. 2.33 Infrafloccular exposure of the facial nerve in
hemifacial spasm. (a) The insert shows the approach
along the inferolateral margin of the cerebellum and below
the flocculus. The cerebellum has been elevated to expose
the right cerebellopontine angle. The facial nerve exit
zone from the brain stem is seen along the lower margin of
the vestibulocochlear nerve. The AICA passes between the
facial and vestibulocochlear nerve. A large tortuous PICA
loops upward anterior to the facial and vestibulocochlear
nerves and behind the trigeminal nerve, before turning
downward to reach the medulla. The flocculus and the
choroid plexus protruding from the foramen of Luschka
often hide the junction of the facial and vestibulocochlear
nerves with brain stem. In this case, the flocculus has been
gently elevated to expose the junction of these nerves with
complex anatomy of this small nerve which is
hidden between the vestibulocochlear and facial
nerves (Figs. 2.2, 2.3, and 2.35) [44]. The ner-
vus intermedius usually is described as a com-
ponent of the facial nerve. Relatively little note
has been taken of the fact that it may be closely
bound to the vestibulocochlear nerve for a vari-
able distance before it enters the brain stem,
and that in the cerebellopontine angle, it may
consist of as many as four rootlets. The nervus
intermedius is found divisible into three parts: a
the brain stem. (b) Enlarged view. Exposing the facial
nerve exit zone from the brain stem is facilitated by direct-
ing the exposure along the inferolateral margin of the cer-
ebellum in the area above the glossopharyngeal nerve and
below the lower edge of the flocculus. (c) The vestibuloco-
chlear nerve has been depressed. This exposes the distal
segment of the facial nerve, but does not provide access to
the junction of the facial nerve with the brain stem, which
should be visualized in dealing with hemifacial spasm.
(d) The vestibulocochlear nerve has been gently elevated.
This exposes both the rostral and caudal margins of the
facial nerve at the brain stem. A rootlet of the nervus inter-
medius is also exposed. The vein of the middle cerebellar
peduncle passes between the facial and vestibulocochlear
nerve (From Rhoton [40])
Lower Neurovascular Complex
c d
Fig. 2. 33 (continued)
medial segment that adheres closely to the ves-
tibulocochlear nerve, an intermediate segment
that lies freely between the acoustic nerve and
the motor root of the facial nerve, and a distal
segment that joins the motor root to form the
facial nerve [44]. Twenty-two percent of the
nerves were adherent to the acoustic nerve for
14 mm or more (the entire course of the nerve in
the posterior cranial fossa) and could be found
as a separate structure only after opening the
internal acoustic meatus. In most instances, the
nerve was a single trunk, but in some cases, it
was composed of as many as four rootlets. A
single large root most frequently arises at the
brain stem anterior to the superior vestibular
nerve and, in the meatus, lies anterior to the
superior vestibular nerve. When multiple root-
lets are present, they may arise along the whole
anterior surface of the vestibulocochlear nerve;
however, they usually converge immediately
proximal to the junction with the facial motor
root to form a single bundle that lies anterior to
the superior vestibular nerve.
57
It is the free segment between the facial and
vestibulocochlear nerve that is divided in genicu-
late neuralgia. This segment, where the nervus
intermedius is free of both the facial and vestibu-
locochlear nerves, may be located in the cerebel-
lopontine angle or in the meatus if the nervus
intermedius is composed of a single rootlet. If the
nervus intermedius is composed of more than one
rootlet, however, there may be free segments both
in the cerebellopontine angle and in the meatus.
Geniculate neuralgia with or without vestibulo-
cochlear dysfunction also has been postulated to
be caused by vascular compression of the nervus
intermedius or vestibulocochlear nerve [18, 27].
Lower Neurovascular Complex
The lower complex, which is related to the PICA,
includes the medulla, inferior cerebellar pedun-
cle, cerebellomedullary fissure, suboccipital sur-
face of the cerebellum, and the glossopharyngeal,
vagus, spinal accessory, and hypoglossal nerves
58 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

a b Mea.seg.
Subarc.A.
VIII,VII

Pons Pre.,mea.seg.

Post.mea.seg. IX
VII,VIII

IX X
Flocculus
A.I.C.A.
X
XI
XI
P.I.C.A.
Vert.A. Chor.Plex.

Medulla

P.I.C.A. Vert.A.

c d
Subarc.A. Mea.seg. Labyrin.A.

Pre.mea.seg. VIII Pre.mea.seg.

VII
IX IX
Post.mea.seg.
Post.mea.seg.
X X
VIII
XI XI
VII

P.I.C.A.
Chor.Plex.
P.I.C.A.

Medulla Chor.plex.

Fig. 2.34 Arterial compression of the facial nerve in
hemifacial spasm as viewed through a retrosigmoid cran-
iotomy performed with the patient in the three-quarter
prone position. (a) The upper drawing shows the site of
the incision (straight line) and the location of the cran-
iotomy for the infrafloccular approach to the facial nerve
(broken line). The lower drawing shows the surgical expo-
sure obtained with this approach. The AICA and the facial
and vestibulocochlear nerves are in the midportion of the
exposure. The vertebral artery, PICA, and the glossopha-
ryngeal, vagus, and spinal accessory nerves are below. (b)
The cerebellum is elevated to expose the facial and ves-
tibulocochlear nerves and the premeatal, meatal, and post-
meatal segments of the AICA. The flocculus and the
choroid plexus block the view of the junction of the facial
and vestibulocochlear nerves with the brain stem. (c) The
flocculus and the choroid plexus have been elevated in
the infrafloccular approach to the root entry/exit zone of
the facial and vestibulocochlear nerves. The premeatal
segment compresses the nerves at the junction with the
pons and the medulla. (d) The nerve root entry/exit zone
is compressed by the postmeatal segment. (e) A tortuous
PICA loops upward to compress the nerves at their junc-
tion with the brain stem before turning inferiorly to pass
between the glossopharyngeal and vagus nerves. (f) A tor-
tuous vertebral artery compresses the nerve root entry/exit
zone (From Rhoton [40])
Lower Neurovascular Complex
e f
VIII
A.I.C.A. VII
Pons IX
X X
P.I.C.A.
XI
P.I.C.A.
Vert.A.
Fig. 2. 34 (continued)
(Figs. 2.1, 2.6, 2.7, 2.19, and 2.32). The PICA
arises at the medullary level and encircles the
medulla, passing in relationship to the glossopha-
ryngeal, vagus, spinal accessory, and hypoglossal
nerves to reach the surface of the inferior cerebellar
peduncle, where it dips into the cerebellomedul-
lary fissure and terminates by supplying the sub-
occipital surface of the cerebellum.
Neural Relationships
The glossopharyngeal, vagus, spinal accessory,
and hypoglossal nerves arise from the medulla
along the margin of the inferior olive. The
glossopharyngeal, vagus, and spinal accessory
nerves arise as a line of rootlets that exit the brain
stem along the posterior edge of the olive in the
postolivary sulcus, a shallow groove between the
olive and posterolateral surface of the medulla
(Figs. 2.1, 2.20, and 2.32). The hypoglossal nerve
arises as a line of rootlets that exit the brain stem
along the anterior margin of the lower two-thirds
of the olive in the preolivary sulcus, a groove
between the olive and the medullary pyramid.
The glossopharyngeal and vagus nerves arise at the
59
A.I.C.A.
IX
VIII
VII
XI
Vert.A.
P.I.C.A.
Vert.A.
level of the superior one-third of the olive. The
spinal accessory rootlets arise along the posterior
margin of the inferior two-thirds of the olive and
from the lower medulla and the upper segments
of the cervical spinal cord. The glossopharyngeal
and vagus nerves arise rostral to the level of ori-
gin of the hypoglossal rootlets.
The glossopharyngeal nerve arises as one or
rarely two rootlets from the upper medulla, pos-
terior to the olive, just caudal to the origin of the
facial nerve. It courses ventral to the choroid
plexus protruding from the foramen of Luschka
on its way to the jugular foramen. Frequently, a
larger dorsal and a smaller ventral component
can be seen at the junction with the brain stem
(Fig. 2.36) [19, 43]. The smaller ventral rootlets
have been demonstrated to be motor and the
larger main bundle to be sensory [5, 51].
The larger dorsal component usually arises from
the medulla as one root except in a few cases in
which it will originate as two rootlets. The two
rootlets may remain separate throughout their
course to the dura (Fig. 2.34). The vagus nerve
arises below the glossopharyngeal nerve as a line
of tightly packed rootlets along a line 2–5.5 mm
in length posterior to the superior one-third of
60 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a b
N.I.
VII
VIII
VII
VIII
Fig. 2.35 View of the cerebellopontine angle from above
to show the relationship of the nervus intermedius to the
facial and vestibulocochlear nerves. (a) Most common
relationship. The nervus intermedius is joined to the ven-
tral surface of the vestibulocochlear nerve for a few mil-
limeters adjacent to the brain stem, then has a free segment
in the cerebellopontine angle as it courses to join the facial
motor root. (b) Pattern present in 20 % of the nerves
the olive (Figs. 2.1, 2.30, and 2.34). The most
rostral vagal fibers arise adjacent to the glossopha-
ryngeal origin, from which they are sometimes
separated by as much as 2 mm. The vagus is
composed of multiple combinations of large and
small rootlets that pass ventral to the choroid
plexus protruding from the foramen of Luschka
on its way to the jugular foramen. Occasionally,
N.I.
VII
VIII
studied. The free segment is entirely in the meatus. (c)
The nervus intermedius consists of three free segments:
two are the angle and one is in the meatus. The nervus
intermedius in a could be exposed in the angle without
drilling off the posterior lip of the meatus. In b, the free
segment could not be found in the angle but only in the
meatus (From Rhoton and Tedeschi [45])
several small rootlets are found originating ven-
tral to the majority of the vagal rootlets
(Fig. 2.36). These small ventral rootlets are con-
sidered to be motor [5].
The accessory nerve arises as a widely sepa-
rated series of rootlets that originated from the
medulla at the level of the lower two-thirds of
the olive and from the upper cervical cord. The
Lower Neurovascular Complex
cranial rootlets of the accessory nerve arise as a
line of rootlets ranging in diameter from 0.1 to
1 mm just caudal to the vagal fibers (Figs. 2.32
and 2.37). The cranial rootlets of the accessory
nerve are more properly regarded as inferior
vagal rootlets since they arise from vagal nuclei
[19, 43]. It may be difficult to separate the lower
vagal fibers from the upper accessory rootlets
because the vagal and cranial accessory fibers
usually enter the vagal meatus as a single
bundle.
The upper rootlets of the spinal portion of the
accessory nerve originate several millimeters
caudal to the lowest cranial accessory fibers and
either course to join the cranial accessory bundle
or enter the lower border of the vagal meatus
separate from the cranial accessory rootlets. The
spinal accessory fibers pass superolateral from
their origin to reach the jugular foramen. Although
the cranial and spinal portion of the accessory
nerve most frequently entered the vagal meatus
together, they may infrequently be separated by a
dural septum.
The rootlets forming the hypoglossal nerve
arise from the medulla along a line that is
continuous inferiorly with the line along which
the ventral spinal roots arise (Figs. 2.1, 2.7, and
2.32). These rootlets arise in a nucleus, whose
rostral part sits deep to the hypoglossal triangle in
the floor of the fourth ventricle, and exit the
medulla along the anterior margin of the caudal
two-thirds of the olive. The hypoglossal rootlets
course anterolateral through the subarachnoid
space and pass behind the vertebral artery to
reach the hypoglossal canal. If the vertebral artery
is short and straight, it may not contact or distort
the hypoglossal rootlets, but if the artery is tortu-
ous, it may stretch the hypoglossal rootlets poste-
riorly over its dorsal surface [32]. Infrequently,
the vertebral artery passes between the rootlets of
the hypoglossal nerve [21]. Prior to entering the
hypoglossal canal, the rootlets collect into two
bundles, and in some cases, the canal is divided
by a bony septum that separates the two bundles.
After passing through the canal, the bundles unite
and the nerve lies medial to the internal jugular
vein, and the glossopharyngeal, vagus, and acces-
sory nerves.
61
Anatomy of Glossopharyngeal
Neuralgia
Dandy [2] described endocranial sectioning of
the glossopharyngeal nerve for neuralgia, but
because this alone did not adequately control the
neuralgia, he later advocated the additional sec-
tioning of “perhaps 1/8 to 1/6 of the vagus”
(Figs. 2.20, 2.32, and 2.37). Tarlov [51, 52] sec-
tioned the cephalic third of the vagal–spinal
accessory group and produced analgesia of the
epiglottis but only hypalgesia over the mucosa of
the lower pharynx and larynx. In his second case,
he sectioned the cephalic half of the vagal–spinal
accessory complex; this caused both analgesia
and transient paralysis of the ipsilateral soft pal-
ate, pharynx, and larynx. In our study, the struc-
ture of the vagus nerve was variable, being
composed of all large or all small rootlets or any
combination of the two. It is suggested that fewer
of the rostral rootlets be cut if the diameters of the
upper rootlets are large rather than small;
the diameter of the largest rootlet is 1.5 mm and
the smallest is 0.1 mm [43].
A large glossopharyngeal nerve diameter
might be associated with a small diameter of the
upper rootlets of the vagus nerve or a large vagus
nerve might be associated with a small glossopha-
ryngeal nerve because the two nerves arise from
the same nuclei and have a similar function [5].
This idea that more fibers might be distributed to
one nerve, leaving the other smaller, was not
confirmed in our studies [43]. When the diameter
of the dorsal root of the glossopharyngeal nerve
is compared to the mean of the upper rootlets of
the vagus nerve, no significant correlation is
found [43]. A smaller diameter of the glossopha-
ryngeal nerve is not commonly associated with a
large mean diameter of the upper rootlets of the
vagus nor is a large glossopharyngeal nerve
diameter associated with a small diameter of the
vagal rootlets.
The only location where the glossopharyngeal
nerve can consistently be distinguished from the
vagus is just proximal to the dural meatus where
a dural septum separates the glossopharyngeal
and vagus nerves. This septum varies in width
from 0.5 to 4.9 mm and serves to differentiate
62 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
a b
Fig. 2.36 (a) Lateral view of the left side of the brain
stem as outlined by the broken line. (b) Note the ventral
and dorsal rootlets of the glossopharyngeal and vagus
the glossopharyngeal nerve from the vagus
nerve [43]. The close medullary origin of the
glossopharyngeal and vagus nerves and the fre-
quent arachnoid adhesions between the two make
separation difficult in their course through the
subarachnoid space or adjacent to the brain stem
except in the few cases in which there will be a
1–2-mm separation between their origins at the
medulla.
The superior glossopharyngeal and vagal gan-
glia may be visible intracranially [19]. In
glossopharyngeal neuralgia, the glossopharyn-
geal nerve should be sectioned proximal to the
superior ganglion. The superior ganglion was
nerves. One ventral glossopharyngeal and two ventral
vagal rootlets (arrows) are seen (From Rhoton and Buza
[43])
intracranial in 32 % of 50 jugular foramina that
we examined and within or extracranial to the
foramen in 68 % [43]. The superior ganglion of
the vagus could be seen intracranially in only
14 % of the cases.
Vascular Relationships
The vertebral artery courses anterior to the nerves
in the lower neurovascular complex. The hypo-
glossal rootlets usually pass behind the vertebral
artery; however, some hypoglossal rootlets infre-
quently pass anterior to the artery (Fig. 2.6). If
Lower Neurovascular Complex
a c
Left
b d
Fig. 2.37 The broken line on the drawing of the lateral
surface of the brain stem outlines the area shown in each
diagram, demonstrating the brain stem origin and varia-
tions of the rootlet size of the glossopharyngeal, vagus,
and spinal accessory nerves. The large ovoid structure is
the inferior olive. The broken-line circles outline the ori-
gin of the facial and vestibulocochlear nerves. The most
cephalad, shaded circles indicate glossopharyngeal root-
let origins; intermediate, open circles indicate vagal
rootlet origins; and caudal, black circles outline spinal
accessory rootlet origins. The glossopharyngeal nerve
usually originates as one large rootlet, the vagus as a series
of large and small rootlets, and the spinal accessory as a
the vertebral artery is elongated or tortuous and
courses laterally to the olive, it will stretch the
hypoglossal rootlets over its posterior surface.
Some tortuous vertebral arteries will stretch the
hypoglossal rootlets so far posterior that they
intermingle with the glossopharyngeal, vagus,
and spinal accessory nerves. The PICA has a
much more complex relationship to these nerves
(Figs. 2.6 and 2.32). The proximal part of the
PICA passes around or between and often
stretches or distorts the rootlets of the nerves in
the lower complex. At the anterolateral medulla,
the PICA passes around or between the rootlets
of the hypoglossal nerve. At the posterolateral
margin of the medulla, it passes between the fila
of the glossopharyngeal, vagus, and spinal
accessory nerves. The PICA may be ascending,
descending, or passing laterally or medially or
may be involved in a complex loop that stretches
63
a c
Right
b d
series of small rootlets. Left: Note the small ventral root-
lets of the glossopharyngeal nerve in (a, b), and (c) and
the small ventral rootlet between the glossopharyngeal
and vagus nerves in (a). The glossopharyngeal rootlet is
larger than the rostral rootlet of the vagus nerve in all
except (d), in which the rostral vagal rootlet is larger than
the glossopharyngeal nerve. Right: Note the wide separa-
tion of the origin of the glossopharyngeal and vagus
nerves in (c), the small ventral rootlet of the glossopha-
ryngeal nerve in (c), and the small ventral rootlets of the
glossopharyngeal and vagus nerves in (a). The glossopha-
ryngeal nerve is smaller than the upper vagal rootlet in (a)
and (d) (From Rhoton and Buza [43])
and distorts these nerves as it passes between
them (Fig. 2.30). The relationships of the PICA
and vertebral artery to these nerves are reviewed
in greater detail in the section on the PICA.
Vascular Compression in the Lower
Neurovascular Complex
The close relationship of the PICA and the verte-
bral artery to the glossopharyngeal and vagus
nerves makes it logical to explore these relation-
ships in glossopharyngeal neuralgia [20]. Both
the glossopharyngeal and vagus nerves have been
found to be compressed at their junction with the
brain stem by the PICA and/or the vertebral artery,
with relief following separation of the arteries and
nerves [20]. The adverse cardiovascular effects of
mobilization of these nerves and the risk to
64 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
causing swallowing and vocal cord defects have
led some to conclude that rhizotomy of the
glossopharyngeal nerve and upper vagal rootlets
is a reasonable alternative to vascular mobiliza-
tion along the lateral medulla [16–18, 27, 46].
Jannetta has proposed that compression of the
left side of the medulla by the PICA or the verte-
bral artery may be a cause of hypertension and
that diabetes mellitus may result from right lat-
eral medullary compression as a result of vagal
effects on pancreatic islet cell secretion [7, 17].
The fact that hypertension is a component of the
Cushing response to lateral medullary
compression has been well established and that
hypertension has been relieved following decom-
pression of the left side of the medulla supports
this concept. The relationship of vascular com-
pression to diabetes mellitus awaits further
elucidation.
Tumors Involving Multiple
Neurovascular Complexes
Tumors in the cerebellopontine angle commonly
involve more than one of the neurovascular com-
plexes (Fig. 2.38). An especially difficult challenge
is exposing and removing the tumors that are situ-
ated medial to the nerves. In this case, the opera-
tion must be directed through the interval between
the neurovascular complexes because these tumors
often will widen these intervals. Lesions in the
upper cerebellopontine angle may be exposed
through the interval between the lower margin of
the tentorium and the upper edge of the trigeminal
nerve. Care is needed to protect the trochlear nerve
and the SCA in this area. Further inferiorly, the
medially placed tumor may be approached through
the interval between the trigeminal nerve above
and the facial and vestibulocochlear nerves below.
If the tumor has an even lower attachment near the
jugular foramen, it can be approached through the
interval between the lower margin of the nerves
entering the internal meatus and the upper margin
of the glossopharyngeal nerve, or through the
interval between the lower rootlets of the vagus
nerve and the upper rootlets of the spinal acces-
sory nerve. The intervals between the glossopha-
ryngeal and vagus nerves and between the
individual vagal rootlets usually are too narrow to
work through unless they have been opened by the
tumor; however, the interval between the lower of
the cranial accessory rootlets may provide access
to lesions in the area.
Telovelar Approach to Fourth
Ventricle
Lesions of the fourth ventricle, commonly
approached through the cerebellomedullary or
cerebellopontine fissures, posed a special chal-
lenge to neurosurgeons because of the severe
deficits which may follow injury to the struc-
tures in the ventricular walls and floor. In the
past, operative access to the fourth ventricle was
obtained by splitting the cerebellar vermis or
removing part of a cerebellar hemisphere
[26, 34, 38]. In examining the clefts and walls of
the cerebellomedullary fissure, we have found
that opening the tela alone will provide adequate
ventricular exposure in most cases without split-
ting the vermis (Fig. 2.39). The inferior medul-
lary velum, another paper-thin layer, can also be
opened if opening the tela does not provide
adequate exposure. Opening the tela alone pro-
vides access to the full length of the floor and all
of the ventricular cavity except, possibly, the
fastigium, superolateral recess, and the superior
half of the roof. Opening the inferior medullary
velum accesses the latter areas and the superior
half of the roof. Extending the telar opening lat-
erally toward the foramen of Magendie opens
the lateral recess and exposes the peduncular
surfaces bordering the recess. Tumors in the
fourth ventricle may stretch and thin these two
semitranslucent membranes to a degree that one
may not be aware that they are being opened in
exposing a fourth ventricular tumor. There are
no reports of deficits following isolate opening
of the tela and velum. However, other structures
exposed in the ventricle walls and at risk for
producing the deficits described above include
the dentate nuclei, cerebellar peduncles, floor of
the fourth ventricle, and the PICA. During an
operation on the caudal part of the roof, one
Telovelar Approach to Fourth Ventricle
should remember that the dentate nuclei are
located just rostral to the superior pole of the
tonsils underlying the dentate tubercles in the
posterolateral part of the roof where they are
wrapped around the superolateral recesses near
the lateral edges of the inferior medullary velum
(Fig. 2.36). All the cerebellar peduncles con-
verge on the lateral wall and roof where they
may be damaged. The superior cerebellar
peduncle is more likely to be injured during
VII,VIII
V X
IV
Flocculus
Fig. 2.38 Tumors involving multiple neurovascular com-
plexes. (a) Routes that can be taken between the cranial
nerves to expose and remove a tumor situated medial to
and involving multiple cranial nerves. The patient is posi-
tioned in the three-quarter prone position. The insert
(upper left) shows the site of the vertical scalp incision
and craniotomy. The approach to pathology located medial
to the nerves can be directed (arrows) between the
trochlear nerve above and trigeminal nerve below,
between the trigeminal nerve above and the facial and vesti-
bulocochlear nerves below, between the facial and
vestibulocochlear nerves above and the glossopharyngeal
nerve below, between the glossopharyngeal and vagus
nerves, between the vagus nerve and accessory rootlets,
and between the widely separated rootlets of the acces-
sory nerve. Tumor located medial to the nerves will often
widen the intervals between the nerves, depending on the
site of origin of the tumor. Choroid plexus protrudes from
the foramen of Luschka. (b) Meningioma attached lateral
to the trigeminal nerve in the region of the superior petro-
sal sinus. The trochlear nerve is elevated, the trigeminal
nerve is pushed medially, and the facial and vestibuloco-
65
operations on lesions involving the superior part
of the roof above the level of the dentate tuber-
cles; the inferior peduncles are most susceptible
to damage in exposing lesions within the lateral
recess; and the middle cerebellar peduncle is
susceptible to injury in procedures near the
external wall of the superior half of the roof
such as those in the cerebellopontine angle
because the middle peduncle forms a major part
of the cisternal surface of the ventricular wall.
IX
XI
Ch.plex
chlear nerves are stretched below the tumor. (c) The tumor
has been removed. The thin distorted nerves have been
preserved, and the remaining dural attachment is removed
or cauterized with bipolar coagulation. The basilar artery
and abducens nerve are exposed. (d) Large meningioma
arising from the clivus in the region of the inferior petro-
sal sinus with involvement of the fourth through the elev-
enth nerves. The nerves are displaced laterally around the
tumor. The tumor is removed by working through the
intervals between the nerves. (e) The meningioma has
been removed. The dural attachment has been partially
removed and the base is being cauterized. (f) Meningioma
arising medial to the jugular bulb in the region of the jugu-
lar tubercle and involving the lower cranial nerves. (g)
The tumor was removed by operating through the inter-
vals between the facial and vestibulocochlear nerves
above and the glossopharyngeal nerve below and between
the glossopharyngeal and vagus nerves (round insert). (h)
Large epidermoid tumor being removed by working
through the intervals between the nerves. (i) Distorted
nerves after the removal of the epidermoid tumor (From
Rhoton [40])
66 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

Sup.pet.sinus
VII,VIII

Tent. A.I.C.A.
Sig.sinus
Tumor
IX ,X
XI
IV
S.C.A. Vert.A.

Flocculus
XII

P.I.C.A.
VII,VIII
Ch.Plex

A.I.C.A.

VII,VIII
IX ,X

VI

Tent.
S.C.A. V

IV
Vert.A.

Midbrain

Medulla

Bas.A.
P.I.C.A.
Pons

Fig. 2. 38 (continued)
Telovelar Approach to Fourth Ventricle 67

VII,VIII
d

Sig.sinus

Sup.pet.sinus IX,X

XI,XI
V
S.C.A.
Tumor Vert.A.
IV

A.I.C.A.

P.I.C.A.
Ch.Plex.

e VII,VIII IX
Dural Edge
X

V XI

IV XII

S.C.A.
Vert.A.
Tent.

A.I.C.A. P.I.C.A.
Ch.plex.

Fig. 2. 38 (continued)
68 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

f
VI VII,VIII
V

Tumor
S.C.A.
Sig. sinus
IV IX,X,XI

Tent. Vert.A.
Medulla

Pons
P.I.C.A.
A.I.C.A.

IX
g
VII,VIII

VII,VIII VI

A.I.C.A. A.I.C.A. XI
IV

V
IX XII
X

X
XI
S.C.A.
P.I.C.A.
Tent.

IV

Bas.A.
Vert.A.

P.I.C.A.

Fig. 2. 38 (continued)
Telovelar Approach to Fourth Ventricle 69

h VII,VIII Tumor Sig. sinus

Sup.pet.sinus XII
V XI

IV
Tent. Vert.A.
S.C.S.

X
A.I.C.A.

P.I.C.A.
IX

i IV VII,VIII IX

X
XII
V

XI

Tent.

IV Vert.A.

S.C.A.

Pons
P.I.C.A.
Ch.plex.
A.I.C.A.

Fig. 2. 38 (continued)
70 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach

a b

c d

e f

Fig. 2.39 Telovelar approach to the fourth ventricle and
lateral recess. (a) The suboccipital cerebellar surface is
located below and between the sigmoid and lateral sinuses
and is the surface that is exposed in a suboccipital craniec-
tomy. The vermis sits in a depression, the posterior cerebel-
larincisura,betweenthehemispheres.Thecerebellomedullary
fissure extends superiorly between the cerebellum and
medulla along the inferior half of the ventricular roof and
lateral recess. The vallecula extends upward between the
tonsils and communicates through the foramen of Magendie
with the fourth ventricle. The posteroinferior cerebellar
arteries loop above the tonsil and exit the fissure to supply
the suboccipital surface. (b) Both tonsils have been retracted
laterally to expose the inferior medullary velum and tela
choroidea that form the lower half of the ventricular roof.
The nodule of the vermis, on which the inferior medullary
arises, is hidden deep to the uvula. (c) The uvula has been
retracted to the right and the tonsil to the left to expose the
inferior medullary velum and the tela choroidea forming the
lower half of the roof of the ventricle. The tela choroidea has
been opened extending from the foramen of Magendie to
the junction with the inferior medullary velum to expose the
ventricular floor and aqueduct. (d) The left half of the infe-
rior medullary velum has been divided to expose the supero-
lateral recess and the ventricular surface formed by the
superior and inferior peduncles. (e) The cerebellar tonsil has
been elevated to expose the tela forming the lower part of
the roof of the lateral recess. (f) The tela has been opened to
expose the lateral recess. The opening extends laterally to
the foramen of Luschka. The choroid plexus and flocculus
are exposed in the cerebellopontine angle behind the
glossopharyngeal nerve (From Rhoton [34])
References 71

References 17. Jannetta PJ, Gendell HM (1979) Clinical observations

on etiology of essential hypertension. Surg Forum
30:431–432
1. Atkinson WJ (1949) The anterior inferior cerebellar
18. Jannetta PJ, Moller MB, Moller AR, Sekhar LN
artery; its variations, pontine distribution, and significance
(1986) Neurosurgical treatment of vertigo by micro-
in the surgery of cerebello-pontine angle tumours. J
vascular decompression of the eighth cranial nerve.
Neurol Neurosurg Psychiatry 12(2):137–151
Clin Neurosurg 33:645–665
2. Dandy WE (1927) Glossopharyngeal neuralgia (tic
19. Katsuta T, Rhoton AL Jr, Matsushima T (1997) The
douloureux): its diagnosis and treatment. Arch Surg
jugular foramen: microsurgical anatomy and operative
15:198–214
approaches. Neurosurgery 41(1):149–201; discussion
3. Dandy WE (1929) An operation for the cure of tic
201–202
douloureux: partial section of the sensory root at the
20. Laha RK, Jannetta PJ (1977) Glossopharyngeal
Pons. Arch Surg 18:687–734
neuralgia. J Neurosurg 47(3):316–320. doi:10.3171/
4. Dandy WE (1934) Concerning the cause of trigeminal
jns.1977.47.3.0316
neuralgia. Am J Surg 24:447–455
21. Lister JR, Rhoton AL Jr, Matsushima T, Peace DA
5. DuBois FSFJ, Foley JO (1936) Experimental studies
(1982) Microsurgical anatomy of the posterior infe-
on the vagus and spinal accessory nerves in the cat.
rior cerebellar artery. Neurosurgery 10(2):170–199
Anat Rec 64:285–307
22. Martin RG, Grant JL, Peace D, Theiss C, Rhoton AL
6. Emmons WF, Rhoton AL Jr (1971) Subdivision of the
Jr (1980) Microsurgical relationships of the anterior
trigeminal sensory root. Experimental study in the
inferior cerebellar artery and the facial-vestibuloco-
monkey. J Neurosurg 35(5):585–591. doi:10.3171/
chlear nerve complex. Neurosurgery 6(5):483–507
jns.1971.35.5.0585
23. Matsushima T, Rhoton AL Jr, Lenkey C. (1982).
7. Fein JM, Frishman W (1980) Neurogenic hyperten-
Microsurgery of the fourth ventricle I: Microsurgical
sion related to vascular compression of the lateral
anatomy. Neurosurgery, 11, 631–667
medulla. Neurosurgery 6(6):615–622
24. Matsushima T, Rhoton AL Jr, de Oliveira E, Peace D
8. Fujii K, Lenkey C, Rhoton AL Jr (1980) Microsurgical
(1983) Microsurgical anatomy of the veins of the
anatomy of the choroidal arteries. Fourth ventricle
posterior fossa. J Neurosurg 59(1):63–105.
and cerebellopontine angles. J Neurosurg 52(4):
doi:10.3171/jns.1983.59.1.0063
504–524. doi:10.3171/jns.1980.52.4.0504
25. Moller MB, Moller AR, Jannetta PJ, Sekhar L (1986)
9. Gardner WJ (1962) Concerning the mechanism of
Diagnosis and surgical treatment of disabling posi-
trigeminal neuralgia and hemifacial spasm. J Neurosurg
tional vertigo. J Neurosurg 64(1):21–28. doi:10.3171/
19:947–958. doi:10.3171/jns.1962.19.11.0947
jns.1986.64.1.0021
10. Gudmundsson K, Rhoton AL Jr, Rushton JG (1971)
26. Mussi AC, Rhoton AL Jr (2000) Telovelar approach to
Detailed anatomy of the intracranial portion of the
the fourth ventricle: microsurgical anatomy. J Neurosurg
trigeminal nerve. J Neurosurg 35(5):592–600.
92(5):812–823. doi:10.3171/jns.2000.92.5.0812
doi:10.3171/jns.1971.35.5.0592
27. Ouaknine GE, Robert F, Molina-Negro P, Hardy J
11. Hardy DG, Peace DA, Rhoton AL Jr (1980)
(1980) Geniculate neuralgia and audio-vestibular dis-
Microsurgical anatomy of the superior cerebellar
turbances due to compression of the intermediate and
artery. Neurosurgery 6(1):10–28
eighth nerves by the postero-inferior cerebellar artery.
12. Hardy DG, Rhoton AL Jr (1978) Microsurgical
Surg Neurol 13(2):147–150
relationships of the superior cerebellar artery and the
28. Pait TG, Harris FS, Paullus WS, Rhoton AL Jr (1977)
trigeminal nerve. J Neurosurg 49(5):669–678.
Microsurgical anatomy and dissection of the temporal
doi:10.3171/jns.1978.49.5.0669
bone. Surg Neurol 8(5):363–391
13. Horsley V, Taylor J, Coleman WS (1981) Remarks on
29. Rhoton AL Jr (1990) Microsurgical anatomy of
the various surgical procedures devised for the relief
decompression operations on the trigeminal nerve.
or cure of trigeminal neuralgia (tic douloureux). Brit
In: Rovit RLMR, Murali R, Jannetta PJ (eds)
Med J 2:1139–1143, 1191–1193, 1249–1252
Trigeminal neuralgia. Williams and Wilkins,
14. House WF (1979) Translabyrinthine approach. In:
Baltimore, pp 165–200
House WFLC, Luetje CM (eds) Acoustic tumors, II
30. Rhoton AL Jr (1993) Instrumentation. In: Apuzzo MLJ
management. University Park Press, Baltimore, pp
(ed) Brain surgery: complication, avoidance and man-
43–87
agement. Churchill-Livingstone, New York, pp
15. Jannetta PJ (1967) Arterial compression of the trigem-
1647–1670
inal nerve at the pons in patients with trigeminal neu-
31. Rhoton AL Jr (1993) Microsurgical anatomy of
ralgia. J Neurosurg 26(1 Suppl):159–162. doi:10.3171/
acoustic neuromas. In: Sekhar LN, Janecka IP (eds)
jns.1967.26.1part2.0159
Surgery of cranial base tumors. Raven Press, Ltd,
16. Jannetta PJ, Abbasy M, Maroon JC, Ramos FM, Albin
New York, pp 687–713
MS (1977) Etiology and definitive microsurgical
32. Rhoton AL Jr (1993) Microsurgical anatomy of
treatment of hemifacial spasm. Operative techniques
posterior fossa cranial nerves. In: Barrow DL (ed)
and results in 47 patients. J Neurosurg 47(3):321–328.
Surgery of the cranial nerves of the posterior fossa:
doi:10.3171/jns.1977.47.3.0321
72 2 Microsurgical Anatomy of the Cerebellopontine Angle by the Retrosigmoid Approach
neurosurgical topics. American Association of
Neurological Surgeons, Chicago, pp 1–103
33. Rhoton AL Jr (2007) Cerebellopontine angle and the
retrosigmoid approach. Neurosurgery 61(S4):175–192
34. Rhoton AL Jr (2007) Telovelar approach to the fourth
ventricle. Neurosurgery 61(S4):193–210
35. Rhoton AL Jr (1974) Microsurgery of the internal
acoustic meatus. Surg Neurol 2(5):311–318
36. Rhoton AL Jr (1976) Microsurgical removal of acous-
tic neuromas. Surg Neurol 6(4):211–219
37. Rhoton AL Jr (1986) Microsurgical anatomy of the
brainstem surface facing an acoustic neuroma. Surg
Neurol 25(4):326–339
38. Rhoton AL Jr (2000) Cerebellum and fourth ventricle.
Neurosurgery 47(3 Suppl):S7–S27
39. Rhoton AL Jr (2000) The cerebellar arteries.
Neurosurgery 47(3 Suppl):S29–S68
40. Rhoton AL Jr (2000) The cerebellopontine angle and
posterior fossa cranial nerves by the retrosigmoid
approach. Neurosurgery 47(3 Suppl):S93–S129
41. Rhoton AL Jr (2000) The posterior fossa cisterns.
Neurosurgery 47(3 Suppl):S287–S297
42. Rhoton AL Jr (2000) The posterior fossa veins.
Neurosurgery 47(3 Suppl):S69–S92
43. Rhoton AL Jr, Buza R (1975) Microsurgical anatomy
of the jugular foramen. J Neurosurg 42(5):541–550.
doi:10.3171/jns.1975.42.5.0541
44. Rhoton AL Jr, Kobayashi S, Hollinshead WH (1968)
Nervus intermedius. J Neurosurg 29(6):609–618.
doi:10.3171/jns.1968.29.6.0609
45. Rhoton AL Jr, Tedeschi H (1992) Microsurgical anat-
omy of acoustic neuroma. Otolaryngol Clin North
Am 25(2):257–294
46. Segal R, Gendell HM, Canfield D, Dujovny M,
Jannetta PJ (1979) Cardiovascular response to pulsa-
tile pressure applied to ventrolateral medulla. Surg
Forum 30:433–435
47. Seoane E, Rhoton AL Jr (1999) Suprameatal exten-
sion of the retrosigmoid approach: microsurgical
anatomy. Neurosurgery 44(3):553–560
48. Sunderland S (1948) Neurovascular relations and
anomalies at the base of the brain. J Neurol Neurosurg
Psychiatry 11(4):243–257
49. Tanriover N, Abe H, Rhoton AL Jr, Kawashima M,
Sanus GZ, Akar Z (2007) Microsurgical anatomy of the
superior petrosal venous complex: new classifications
and implications for subtemporal transtentorial and ret-
rosigmoid suprameatal approaches. J Neurosurg
106(6):1041–1050. doi:10.3171/jns.2007.106.6.1041
50. Tanriover N, Rhoton AL Jr (2005) The anteroinferior
cerebellar artery embedded in the subarcuate fossa: a
rare anomaly and its clinical significance. Neurosurgery
57:314–319
51. Tarlov I (1937) Structure of the nerve root. II: differ-
entiation of sensory from motor roots; observations
on identification of function in roots of mixed cranial
nerves. Arch Neurol Psychiatry 37:1338–1355
52. Tarlov I (1942) Section of the cephalic third of the
vagus-spinal accessory complex: clinical and histo-
logic results. Arch Neurol Psychiatry 47:141–148
 Neuropathology
of the Cerebellopontine Angle
and Its Surroundings
Introductory Remarks
In this chapter, the properties of the most typical
and relevant tumors and other pathologies of the
cerebellopontine angle (CPA) and its surround-
ings are dealt with from a neuropathological
point of view in order to present a quick orienta-
tion for clinicians interested in macroscopic and
microscopic pathomorphology. It is a main inten-
tion to provide a broad scale of pathomorphologi-
cal images enabling clinicians to get impressions
on the relevant macropathology of the CPA on
the one hand and to better interpret the value and
the limitations of histopathological findings on
the other hand.
For practical reasons, I have chosen to divide
the chapter in the following subchapters, all of
them introduced by an overview of the described
entities:
• Pathology of the peripheral nervous system
• Pathology of the autonomous nervous
system
• Pathology of the meninges
• Pathology of the osseous skull base
• Pathology of the sellar region
• Pathology of regional glial tumors
• Pathology of the choroid plexus
• Pathology of the hematopoietic system
• Pathology of germ cells
• Pathology of blood vessels and circulation
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_3, © Springer-Verlag Berlin Heidelberg 2013
3
For tumors as the most important pathological
entities with regard to neurosurgical interven-
tions, the relevant international reference for
classification is the series of classification books
edited by the World Health Organization (WHO).
For skull base tumors, three of the books are of
special interest, the WHO Classification of
Tumours of the Central Nervous System [25], the
WHO Classification of Head and Neck Tumours
[4], and the WHO Classification of Tumours of
Soft Tissue and Bone [12].
For non-tumorous pathological entities, the stan-
dard handbook Greenfield’s Neuropathology is rec-
ommended for further reading [26]. The description
of non-tumorous pathological entities in the CPA is
restricted to some of those entities, which might be
encountered more commonly by the neurosurgeon.
Pathology of the Peripheral Nervous
System
Schwannoma
Cellular schwannoma
Plexiform schwannoma
Melanotic schwannoma
Malignant peripheral nerve sheath tumor (MPNST)
Epithelioid MPNST
MPNST with mesenchymal differentiation
Malignant Triton tumor
Glandular MPNST
73
74 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings
Schwannoma
Age distribution: All ages with a peak in the
fourth to sixth decades.
Sex distribution: Male to female ratio of 1:2.
Epidemiology: 8 % of intracranial tumors;
85 % of cerebellopontine angle tumors. Ninety
percent solitary and sporadic, 5 % multiple and
associated with NF2, 5 % multiple and unassoci-
ated with NF2.
Grading: WHO grade I (non-melanotic);
about 10 % of melanotic schwannomas follow a
malignant course.
Schwannomas derive from the Schwann cells
which form the myelin sheath in peripheral
nerves. With the exception of the olfactory and
optic nerves being entirely invested by central
myelin formed by oligodendrocytes, the periph-
eral portion of the other cranial nerves is invested
by peripheral myelin formed by Schwann cells.
The vast majority of schwannomas of the skull
base occur in the cerebellopontine angle arising
from the eighth cranial nerve, the vestibular
schwannomas. Nonvestibular schwannomas of
the trigeminal nerve are rare; all other locations
are extremely rare. In German-speaking coun-
tries, the synonym acoustic neurinoma is com-
monly used; the synonym neurilemoma is almost
everywhere abandoned.
a b
Fig. 3.1 (a) Small vestibular schwannoma in an early
stage extending from the internal auditory canal (arrow).
(b) Large vestibular schwannoma protruding into the cer-
ebellopontine angle (arrow). (c) Transition between the
central oligodendroglial portion (turquoise) and the
peripheral Schwann cell portion (dark violet) of the acous-
tic nerve. Luxol fast blue–PAS staining. (d) Incipient
Vestibular schwannomas arise from the supe-
rior or the inferior branches of the vestibular and
not from the cochlear portion of the vestibuloco-
chlear nerve. The tumor growth regularly com-
mences in the internal auditory canal, later
occupying the cerebellopontine angle and reaching
a diameter of a few centimeters, thereby producing
a deformation of the brainstem and the adjacent
cerebellum as well as stretching and compressing
the neighboring cranial nerves.
Histologically, schwannomas are usually
encapsulated and composed of elongated cells in
interlacing fascicles (Antoni A-areas) often inter-
mingled with extracellular clear areas of fatty
degeneration and more variable cell shapes (Antoni
B-areas). The arrangement of tumor nuclei in pali-
sades is a common feature. Verocay bodies in form
of parallel nuclei surrounding zones with few
nuclei can infrequently be found. So-called ancient
schwannomas show a distinct nuclear polymor-
phism as well as hyalinized vessels indicating
regressive changes but not malignancy. Also the
variant of cellular schwannoma with a high cellu-
larity remains benign (Figs. 3.1 and 3.2).
Bilateral vestibular schwannomas are pathog-
nomonic for neurofibromatosis 2 (NF2), an
autosomal dominant disorder additionally char-
acterized by multiple peripheral schwannomas
including plexiform cutaneous schwannomas,
trigeminal micro-schwannoma expanding within the
nerve. Luxol fast blue. (e) Vestibular schwannoma with
dense fascicular Antoni A-areas and clear Antoni B-areas
by fatty degeneration (arrows). (f) Encapsulated schwan-
noma compressing the adjacent eighth nerve; note the
compressed red myelin sheaths. Masson trichrome
Pathology of the Peripheral Nervous System 75

c d

e f

Fig. 3.1 (continued)

a b

c d

Fig. 3.2 (a) Loose fascicular pattern of elongated tumor polymorphism but less cell density than in cellular
cells with spindle-shaped monomorphous nuclei (Antoni schwannoma. HE. (d) Cellular schwannoma forming a
A). HE. (b) Typical palisading of tumor cell nuclei. HE. fascicular pattern with high cell density. HE
(c) Ancient schwannoma with prominent nuclear
76 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

Fig. 3.3 (a) Bilateral vestibular schwannoma in NF2 note the decompression of the pons after unilateral surgical
before surgical removal of the smaller one. After surgical tumor removal. (c) Plexiform schwannoma with lobular
tumor removal, the patient died of pulmonary embolism. pattern in NF2. HE. (d) Within the lobules, palisading of
(b) Remaining larger vestibular schwannoma in autopsy; the nuclei can be observed. HE

dysplastic lesions of Schwann cells (schwan-
nosis), meningiomas, gliomas (astrocytomas,
ependymomas), and glial microhamartomas as
well as cerebral calcifications and posterior lens
opacities. NF2 vestibular schwannomas may
entrap nerve fibers of the facial nerve (Fig. 3.3).
Melanotic schwannomas occur in non-psam-
momatous and psammomatous varieties grossly
appearing pigmented, because the Schwann cells
contain melanosomes immunohistochemically
reactive for melanoma markers. About 50 % of
psammomatous melanotic schwannomas are
accompanied by the autosomal dominant Carney
complex which is characterized by lentiginous
facial pigmentation, cardiac myxoma, and endo-
crine disorders including Cushing’s syndrome and/
or acromegaly. Melanotic non-psammomatous
trigeminal schwannoma as the first manifestation
of Carney complex is reported postulating that the
absence of psammoma bodies or cranial localiza-
tion does not exclude this diagnosis [8]. Melanotic
schwannomas may rarely affect cranial nerves such
as the acoustic nerve [31]. However, there is a dis-
cussion if melanocytic tumors of the cerebellopon-
tine angle mimicking vestibular schwannomas
might actually be malignant melanomas [37].
Malignant Peripheral Nerve Sheath
Tumor (MPNST)
Age distribution: Primarily adults in the third to
sixth decades; in NF1-associated cases in the
third to fourth decades.
Sex distribution: No sex predilection; in NF1-
associated cases slightly more common in males.
Epidemiology: No accurate incidence rates for
tumors arising from intracranial nerves.
Grading: WHO grade II, III, or IV; applied
following the criteria of sarcoma grading [13].
MPNSTs of the vestibular nerve are rare
[21, 29, 32]. Extremely rare variants with
Pathology of the Autonomous Nervous System
a b
c d
Fig. 3.4 (a) Glomus jugulare tumor presenting as
circumscribed vascularized soft mass. (b) Jugulotympanic
paraganglioma with “Zellballen” arrangement of chief
cells. HE. (c) Strong positivity for synaptophysin within
a potentially malignant course comprise the
malignant Triton tumor with rhabdomyoblas-
tic cells [5, 14] reminiscent of the Triton tumor
observed in neurofibromatosis 1 (NF1).
Pathology of the Autonomous
Nervous System
Jugulotympanic paraganglioma (chemodectoma)
Glomus jugulare tumor
Glomus tympanicum tumor
Jugulotympanic Paraganglioma
Age distribution: More common in Caucasians
with a peak in the fifth and sixth decades.
Sex distribution: Strong female predilection;
the familial type with gene mutation on chromo-
some 11 occurs predominantly in men.
77
the chief cells of the “Zellballen.” Synaptophysin. (d)
Ultrastructure of a chief cell with cytoplasmic dark round
granules containing noradrenaline (arrows). Transmission
electron microscopy
Epidemiology: No accurate incidence rates
available.
Grading: WHO grade I.
Paragangliomas derive from sympathetic
or parasympathetic autonomous nervous cell
complexes producing catecholamines. In
the literature, frequently also the synonym
chemodectoma is used. Intracranial paragan-
gliomas are rare. At the skull base, they may
be situated either in relation to the jugular
bulb (glomus jugulare tumor) or under the
mucosa of the middle ear (glomus tympani-
cum tumor). Though the jugular variant may
infiltrate the petrous bone, the course normally
remains benign. Histologically, paragan-
gliomas are composed of epithelioid uniform
chief cells and sustentacular cells character-
istically forming clusters or “Zellballen.”
Immunohistochemically, the epithelioid chief
cells express synaptophysin and chromogra-
nin (Fig. 3.4).
78 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

Pathology of the Meninges 10 %, anaplastic meningiomas approximately

1–3 % of all meningiomas.
Meningioma Grading: WHO grade I, II, or III (see text
Meningothelial
below).
Fibrous (fibroblastic)
The histogenesis of meningiomas is both neu-
Transitional (mixed)
roectodermal and mesenchymal. They derive on
Psammomatous
the one hand from neuroectodermal cells of the
Angiomatous
Microcystic
arachnothelial layer, the arachnoidal cap cells,
Secretory and on the other hand from mesenchymal cells of
Lymphoplasmacyte-rich subarachnoidal blood vessels and from fibroblasts
Metaplastic of the subarachnoidal connective tissue. The
Chordoid changing composition of these histological ele-
Clear cell ments is responsible for the different subtypes of
Atypical meningiomas. Thus, meningiomas are tumors
Papillary with both neuroectodermal and mesenchymal
Rhabdoid histogenesis. Since the first description by
Anaplastic (malignant) Cushing and Eisenhardt, the correlation between
Hemangiopericytoma pregnancy and rapid increase in neurological
Anaplastic symptoms is known.
Hemangioblastoma At the skull base, the typical nodular spherical
Leptomeningeal melanocytic lesions
or dumbbell-shaped growth type of meningiomas
Diffuse melanocytosis
can be seen but also an “en plaque”-growth type
Melanomatosis
expanding in a diffuse carpet-like fashion.
Melanocytoma
Especially meningiomas en plaque tend to per-
Malignant melanoma
Metastatic meningiosis and solid metastastic tumors
meate adjacent bony structures causing hyperos-
Meningiosis carcinomatosa tosis. All types of the histologically manifold
Meningiosis sarcomatosa meningiomas can occur.
Meningiosis lymphomatosa WHO grade I subtypes comprise the following:
Meningitis Meningothelial meningiomas form syncytial
Bacterial meningitis cell clusters. Few whorls or psamomma bodies
Acute bacterial sive purulent meningitis may be encountered. The oval nuclei contain little
Tuberculous meningitis chromatin and are characteristically pale. Fibrous
Sarcoidosis meningiomas show a conspicuous formation of
Parasitic meningitis fibroblasts between elongated strands of menin-
Cysticercosis gothelial tissue. Transitional meningiomas are
Fungal meningitis characterized by a pronounced tendency to form
Aspergillosis whorls. Psammomatous meningiomas contain
Cryptococcosis
large amounts of calcified round psammoma bod-
ies. Angiomatous meningiomas exhibit many
Meningioma large and small blood vessels, often with a fibrous
hyaline thickening of the vessel wall. Microcystic
Age distribution: All ages with a peak in the fifth meningiomas form microcysts containing pale-
to seventh decades. eosinophilic mucinous fluid. Secretory menin-
Sex distribution: Female patients predominate giomas contain small round PAS-positive hyaline
in benign meningiomas, whereas no sexual predi- inclusions, the pseudopsammoma bodies.
lection in malignant variants. Lymphoplasmacyte-rich meningiomas show lym-
Epidemiology: 25–30 % of primary intracra- phocytic infiltrates of chronic inflammatory char-
nial tumors; atypical meningiomas approximately acter. Metaplastic meningiomas exhibit focal
Pathology of the Meninges 79

a b

c d

e f

Fig. 3.5 (a) Dura (blue collagenous fibers) with incipient
meningioma. Masson trichrome. (b) Meningothelial men-
ingioma with syncytially arranged tumor cells. Masson
trichrome. (c) Fibrous meningioma characterized by a
strong proliferation of collagenous fibers (blue) in between
the meningothelial tumor cells (violet). Masson trichrome.
(d) Transitional meningioma with abundant whorl forma-
tion. Masson trichrome. (e) Psammomatous meningioma
with abundant calcified psammoma bodies. Masson
trichrome. (f) Angiomatous meningioma characterized by
its predominant blood vessels. Masson trichrome

mesenchymal components such as xanthomatous,
lipomatous, osseous, or cartilaginous areas, sin-
gly or in combination (Figs. 3.5, 3.6, and 3.7).
WHO grade II subtypes comprise the
following:
Chordoid meningiomas resemble the histologi-
cal features of chordoma. In some cases, chordoid
meningioma is associated with Castleman’s dis-
ease, a benign local or generalized angiofollicular
hyperplasia of lymph nodes [20]. Clear cell
meningiomas show a patternless histology with
rather uniform glycogen-rich cells which, in con-
ventional histology, appear “clear” when the gly-
cogen is washed out. Interestingly, clear cell
meningiomas have a certain predilection for the
cerebellopontine angle (Fig. 3.6d). Atypical men-
ingiomas combine increased mitotic activity of
four or more mitoses per ten high-power fields
80 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

Fig. 3.6 (a) Microcystic meningioma. Masson trichrome. ingioma. Note the lymphocytic infiltration (arrow) Masson
(b) Secretory meningioma with pseudopsammoma bodies trichrome. (d) Clear cell meningioma with glycogen-rich
rich in glycogen. PAS. (c) Lymphoplasmacyte-rich men- cytoplasm; the glycogen is washed out by fixation. HE

a b

c d

Fig. 3.7 (a) Xanthomatous metaplasia with foci of xan- Osteoblastic metaplasia with bone formation. Masson
thoma cells. Masson trichrome. (b) Lipoblastic metapla- trichrome. (d) Chondroblastic metaplasia with foci of car-
sia with scattered lipocytes. Masson trichrome. (c) tilage. Masson trichrome
Pathology of the Meninges
a b
c d
Fig. 3.8 (a) Chordoid meningioma with strands of tumor
cells in a mucous-rich matrix. HE. (b) Atypical menin-
gioma with increased mitotic rate. HE. (c) Papillary men-
with increased cellularity and other signs of his-
tological pleomorphism. Chordoid, clear cell,
and atypical meningiomas are classified with
WHO grade II because of a much higher rate of
recurrence than WHO grade I meningiomas
(Fig. 3.8a, b).
WHO grade III subtypes comprise the
following:
Papillary meningioma is a variant with a pre-
dominant perivascular pseudopapillary pattern.
The young patients, including children, suffer
from local invasion in the brain, recurrences, and
even metastases mostly to the lung. The rare
rhabdoid meningioma is characterized by plump
rhabdoid cells with eccentric nuclei and often
undergo an aggressive clinical course. The ana-
plastic (malignant) meningioma exhibits malig-
nant histological features and a markedly elevated
mitotic activity of 20 or more mitoses per 10
high-power fields (Fig. 3.8c, d).
81
ingioma with focal necrosis. HE. (d) Anaplastic
(malignant) meningioma characterized by polymorphous
cells with increased mitotic rate. HE
Hemangiopericytoma
Age distribution: Relatively rare, mainly occur-
ring in younger adults.
Sex distribution: Slightly more often in men
than in women.
Epidemiology: 0.4 % of primary CNS tumors.
Grading: WHO grade II; anaplastic hemangi-
opericytoma WHO grade III.
The meningeal hemangiopericytoma is a
highly cellular and vascularized mesenchymal
tumor almost always attached to the dura.
Histologically, the hemangiopericytoma is com-
posed of closely packed tumor cells with a rich
network of reticulin fibers and with numerous
slit-like vascular channels and branching stag-
horn-type vessels (Fig. 3.9a, b).
The anaplastic hemangiopericytoma has a
high tendency to recur and to metastasize outside
the central nervous system. Histologically, it
82 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

e f

Fig. 3.9 (a) Hemangiopericytoma with high cellularity
and jumbled arrangement of spindle cells. Masson
trichrome. (b) Hemangiopericytoma with abundant reti-
culin fibers and staghorn-type vessel (arrow). Gomori
reticulin impregnation. (c) Hemangioblastoma extending
into the fourth ventricle and appearing red yellow owing
to its rich lipid content and abundant capillaries.
(d) Hemangioblastoma forming a typically large central
cyst (arrow). Nissl. (e) Hemangioblastoma with large
vacuolated stromal cells and vascular cells. Masson
trichrome. (f) Hemangioblastoma with accumulation of
lipid droplets (red) within stromal cells. Oil red

shows nuclear atypia, high mitotic activity, necro- Grading: WHO grade I.
ses, and hemorrhage. The hemangioblastoma is a benign, relatively
rare, highly vascular tumor of angio-mesenchy-
mal origin [40] localized in the cerebellum,
Hemangioblastoma brain stem, or spinal cord. Histologically, the
hemangioblastoma is characterized by two
Age distribution: Adults (sporadic), young adults, components, namely, large, vacuolated stromal
and juveniles (VHL). cells containing lipid droplets and abundant
Sex distribution: No sex predilection. thin-walled small vessels. Therefore, it is also
Epidemiology: No accurate incidence rates referred to as capillary hemangioblastoma
available. (Fig. 3.9c–f).
Pathology of the Meninges
In association with von Hippel–Lindau syn-
drome (VHL), one or multiple hemangioblastomas
occur in significantly younger patients. VHL is an
autosomal dominant disorder caused by germline
mutations of the VHL gene on chromosome 3p.
Different mutations of the VHL gene are correlated
with different phenotypes. In VHL, next to heman-
gioblastomas of the central nervous system and the
retina, extraneural manifestations such as clear cell
renal carcinoma, pheochromocytoma and paragan-
gliomas, epididymal cystadenoma, neuroendocrine
tumors and microcystic adenomas of the pancreas,
and endolymphatic sac tumors can be observed.
Morbidity and mortality for VHL patients is high.
Yearly lifetime screening with familial screening
and counseling is recommended.
Leptomeningeal Melanocytic Lesions
Age distribution: Children (melanocytosis and
melanomatosis linked with neurocutaneous mel-
anosis); all ages with a peak in the fifth decade
(melanocytoma and malignant melanoma).
Sex distribution: No sex predilection (melano-
cytosis and melanomatosis); male to female ratio
of 1:1.5 (melanocytoma).
Epidemiology: 0.1 % of brain tumors; one per
ten million population per year (melanocytomas);
0.5 per ten million population per year (primary
CNS melanoma).
Grading: Benign (melanocytosis, melanocy-
toma); malignant (melanomatosis, malignant
melanoma).
Primary melanocytic lesions of the meninges are
rare. They arise from leptomeningeal melanocytes.
Diffuse and circumscribed, benign and malignant,
and melanotic and amelanotic variants occur.
The diffuse melanocytosis involves the
leptomeninges including those of the skull base
by a proliferation of leptomeningeal melanocytes
within the Virchow–Robin spaces but without
invasion of the central nervous system. It may be
associated with the congenital nevus of Ota [2].
The malignant change to melanomatosis is char-
acterized by CNS parenchymal invasion. Both
diffuse leptomeningeal melanocytosis and mela-
nomatosis are strongly linked with neurocutane-
ous melanosis, a phakomatosis of childhood.
83
The melanocytoma is a solitary circumscribed
tumor deriving from leptomeningeal melano-
cytes. It does not invade surrounding structures.
The primary leptomeningeal malignant mela-
noma has to be distinguished from metastatic
malignant melanoma. It is a highly aggressive
tumor and may metastasize along the CSF path-
ways and to remote organs.
Histologically, leptomeningeal melanocytic
lesions are composed of spindle cells or epithe-
lioid cells similar to melanocytic tumors arising
in other sites (Fig. 3.10a, b). It is important to
distinguish melanocytic lesions from histogeneti-
cally different tumor entities undergoing
melanization such as schwannoma, medulloblas-
toma, or paraganglioma [6].
Meningiosis and Solid Metastatic
Tumors
Age distribution: Depending from the primary
tumor.
Sex distribution: Depending from the primary
tumor.
Epidemiology: Depending from the primary
tumor.
Grading: Malignant.
Metastatic tumor cells may diffusely spread
into the arachnoid forming meningiosis carcino-
matosa, sarcomatosa, or lymphomatosa. A men-
ingeal metastatic spread of malignant melanoma
is possible, but the differential diagnosis of a dif-
fuse benign meningeal melanocytosis or a malig-
nant meningeal melanomatosis has to be kept in
mind. Rarely a carcinosis of the dura with meta-
static islands can be observed (Fig. 3.10c, d).
Of course, all kinds of metastatic tumors
spreading from carcinomas, sarcomas, melano-
mas, or lymphomas can be encountered in all
parts of the central nervous system including
the cerebellopontine angle and the adjacent
osseous skull base. Mainly circumscribed solid
metastases with more or less distinct borders
toward the surrounding brain tissue occur. But
also a continuous tumor growth of different
primary tumors of the surrounding tissues
toward the cerebellopontine angle is possible
(Fig. 3.10e, f).
84 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

d
c

Fig. 3.10 (a) Melanocytoma with loose nests of slightly
pigmented spindle cells. HE. (b) Epithelioid malignant
melanoma with polymorphous tumor cells heavily laden
with melanin pigment. HE. (c) Thickened and infiltrated
arachnoid in meningiosis lymphomatosa. (d) Carcinosis
of the dura with small islands of carcinoma cells sur-
rounded by fibrous dural tissue. Masson trichrome. (e)
Pons and cerebellum with metastasis of bronchial carci-
noma. (f) Continuous growth of a squamous epithelium
carcinoma of the paranasal sinus toward the skull base

Acute Bacterial Sive Purulent sub-Saharan Africa; pneumococcal meningitis at

Meningitis
Age distribution: Age relation of various bacteria,
for example, menigococcal meningitis in chil-
dren in Europe and North America and adoles-
cents or young adults in the “meningitis belt” of
the extremes of age.
Sex distribution: More prevalent in males than
females.
Epidemiology: 1–5 per 100,000 population
per year in Europe and North America; endemic
in the “meningitis belt” of sub-Saharan Africa. H.
Pathology of the Meninges
influenzae meningitis due to vaccination virtually
eliminated in developed countries. 0.5–6 % post-
operative meningitis in neurosurgical patients.
Pathogens: Streptococcus pneumoniae
(Pneumococcus), Neisseria meningitidis
(Meningococcus), and Haemophilus influenzae
cause approximately 80 % of cases, worldwide.
Bacterial meningitis may complicate neurosur-
gical procedures such as craniotomy for the
resection of tumor and shunting; the most com-
mon bacteria causing postoperative meningitis
include Escherichia coli, Klebsiella pneumo-
niae, Pseudomonas aeruginosa, Acinetobacter
spp., and Staphylococcus aureus.
Morphological changes are indistinguishable,
irrespective of the bacterial cause, thus
unspecific. In hyperacute cases, the Waterhouse–
Friderichsen syndrome characterized by bilat-
eral adrenal hemorrhage, centrilobular necroses
of the liver, and disseminated intravascular
coagulation may cause death within 24 h. Up to
15 % of patients with meningococcal sepsis
develop a Waterhouse–Friderichsen syndrome.
In the acute stage, after 2 days or longer, the
migration of polymorphonuclear neutrophilic
leukocytes (PMNs) into the leptomeninges
becomes visible in form of a layer of pus cover-
ing the vertex and/or the base of the brain. In the
subacute stage, PMNs become less numerous in
the exudates, and lymphocytes and a few plasma
cells appear. Lumbar puncture for CSF cytology
a b
Fig. 3.11 (a) Bilateral adrenal hemorrhage in neonatal
Waterhouse–Friderichsen syndrome. (b) Hyperacute stage
of neonatal bacterial meningitis with excessive numbers
of Streptococcus pneumoniae bacteria in CSF cytology.
May–Grünwald–Giemsa. (c) Acute stage of bacterial
meningitis with pus layer covering the brain surface. (d)
Massive infiltration of the leptomeninges, discrete
85
allows a reliable morphological diagnosis by
pleiocytosis up to 10,000 and more cells/mm3
and predominance of PMNs (Fig. 3.11).
Tuberculous Meningitis
Age distribution: All ages, more indolent course
in adults than in children.
Sex distribution: More prevalent in males than
females.
Epidemiology: Rare in developed countries
but still important in developing countries; more
frequent in people with impaired immunity (alco-
holism, HIV-AIDS, intravenous drug abuse, long-
term steroid or immunosuppressive therapy) and
in residents in closed facilities (nursing homes,
chronic care facilities, prisons, refugee camps).
Pathogens: Mycobacterium tuberculosis,
Mycobacterium bovis (rare infection acquired by
ingestion of contaminated milk).
Tuberculous meningitis is the most common
form of tuberculosis of the central nervous sys-
tem. Macrosopically, a thick pale-green exudate
fills the basal cisterns, covers the cerebellopon-
tine angle and the front of the pons, and enters the
Sylvian fissures. Histologically, specific granulo-
mas, the tubercles, consist of central caseous
necrosis surrounded by epithelioid cells, among
them multinucleated Langhans’ giant cells, and a
peripheral ring of lymphocytes. In CSF cytology,
perivascular infiltration in a Virchow–Robin space (arrow)
by PMNs, no infiltration of the brain cortex. Masson
trichrome. (e) Acute stage of bacterial meningitis with
massive pleocytosis of PMNs in CSF cytology. May–
Grünwald–Giemsa. (f) Subacute stage of bacterial menin-
gitis with less numerous PMNs, lymphocytes, and plasma
cells (arrows) in CSF cytology. May–Grünwald–Giemsa
86 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

c d

e f

Fig. 3.11 (continued)

pleocytosis usually below 500 cells/mm3 and
predominance of transformed lymphocytes are
observed. The identification of acid-fast bacilli is
diagnostic (Fig. 3.12a–e).
Sarcoidosis
Age distribution: Young adults with a peak in the
third decade, a second peak in women over 50
years of age.
Sex distribution: Slightly prevalent in females
than males.
Epidemiology: CNS affected in about 5 % of
cases.
Pathogen: Unknown etiology.
Sarcoidosis (synonym Besnier–Boeck–
Schaumann disease) is a granulomatous multi-
system disorder with worldwide distribution.
Sarcoidosis may be oligosymptomatic and com-
monly improves or clears up spontaneously.
About 50 % of the patients have relapses, about
10 % of the patients develop serious disability.
Sarcoid granulomas most often affect the
lungs, but virtually any organ can be affected
including the CNS. The most frequent manifesta-
tion of neurosarcoidosis is characterized by sar-
coid granulomas in the meninges, in individual
cases also without an evidence of systemic dis-
ease. The posterior fossa, the cerebellopontine
angle, and the suprasellar region are preferentially
involved. Histologically, the sarcoid granulomas
have a specific pattern and consist of a central
mass of epithelioid cells and few multinucleated
giant cells surrounded by a ring of lymphocytes.
Central caseous necrosis as seen in tuberculous
meningitis does not occur (Fig. 3.12f).
Aspergillosis
Age distribution: All ages.
Sex distribution: No sex predilection.
Epidemiology: People with impaired immu-
nity, cerebral infection in 10–20 % of cases with
invasive aspergillosis.
Pathology of the Meninges 87

a b

c d

e f

Fig. 3.12 (a) Tuberculous meningitis with pale-greenish
pus layer covering the basal meninges including the cere-
bellopontine angles. (b) Tuberculous granuloma of the
basal meninges with central caseous necrosis surrounded
by a wall of inflammatory cells (arrow). Elastica-van
Gieson. (c) Detail of a tuberculous granuloma with central
caseous necrosis and the wall of epithelioid cells and
lymphocytes as well as Langhans’ giant cells (arrows).
HE. (d) Langhans’ giant cell in CSF cytology. May–
Grünwald–Giemsa. (e) Acid-fast bacilli of Mycobacterium
tuberculosis. Ziehl–Neelsen. (f) Meningeal sarcoid granu-
loma with a central mass of epithelioid cells surrounded
by lymphocytes. There is no caseous necrosis. Masson
trichrome

Pathogens: Aspergillus fumigatus (majority of
cases), Aspergillus flavus, Aspergillus terreus,
Aspergillus niger.
The fungus Aspergillus ssp. is commonly
found growing on decaying vegetation such as
dead leaves, stored grain or compost piles.
Although most people are frequently exposed to
Aspergillus ssp., infections caused by the fungus
rarely occur in people with a competent immune
system.
Aspergillosis is a typical example of a mold-
like fungal infection. Brain infection usually
manifests in form of granulomatous fungal
abscesses surrounded by fungal hyphae
88 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

e f

Fig. 3.13 (a) Aspergilloma with central necrosis and
radiated fungal hyphae arranged in a starburst pattern. HE.
(b) Close-up of septate aspergillus hyphae within necrosis
as example of typical mold like mycosis. PAS. (c)
Meningeal cryptococcus granuloma (arrows). HE. (d)
Cryptococcus yeasts with clear capsule as example of
typical yeastlike mycosis. HE. (e) Multiple cysticercosis
cysts at the brain base including the cerebellopontine
angle (arrows). (f) Multiple proto-scolices in a hydatid
cyst in echinococcosis. Masson trichrome

arranged in a starburst pattern. In rare cases, a
subdural manifestation may be observed
(Fig. 3.13a, b).
Cryptococcosis
Age distribution: All ages.
Sex distribution: No sex predilection.
Epidemiology: People with impaired immu-
nity; 5–10 % of HIV-AIDS patients in developed
countries, and 15–45 % of HIV-AIDS patients in
sub-Saharan Africa have cryptococcal CNS
infection.
Pathogens: Cryptococcus neoformans (majority
of cases), Cryptococcus gattii, Cryptococcus
grubii.
The fungus Cryptococcus ssp. is commonly
found in the soil and the feces of birds.
Cryptococcosis (synonym torulosis) is a typi-
cal example for a yeastlike fungal infection.
CNS infection manifests as meningitis or
Pathology of the Osseous Skull Base
meningoencephalitis. Macroscopically, the lep-
tomeninges appear as granular milky texture.
An infiltration of the brain parenchyma along
the Virchow–Robin spaces is possible.
Histologically, the fungus forms specific
budding, round or oval yeasts surrounded by a
broad mucoid polysaccharide capsule
(Fig. 3.13c, d).
Cysticercosis
Age distribution: All ages.
Sex distribution: No sex predilection.
Epidemiology: Sporadic in Europe, more com-
mon in the tropics, the highest rates being in Latin
America, prevalence of neurocysticercosis up to
3–4 %. In Mexico, Brazil, and India, the most
common space-occupying brain lesion.
Pathogen: Cysticerci are the larvae of the pig
tapeworm Taenia solium.
During the past 30 years, neurocysticercosis
has been increasingly recognized as a major
cause of neurologic disease worldwide and an
important problem among immigrant populations
in the United States.
Cysticercosis is the most common of all para-
sitic infections to cause CNS disease. The human
normally is the definitive host in which the pig
tapeworm develops to maturity producing eggs
which are passed out in the feces. Excreted eggs
of the tapeworm can therefore be found in fecally
contaminated water or food as well as on the
hands of the infected person. In humans as inter-
mediate hosts, autoinfection occurs most fre-
quently by hand-to-mouth infection with the
person’s own feces. Excreted eggs are ingested
by humans and hatch in the gut into oncospheres.
The oncospheres burrow into the gut wall and are
disseminated by blood vessel to any organ includ-
ing brain and meninges. There they develop into
cystic larvae, the cysticerci.
Macroscopically, the cysticerci appear as
fluid-filled bladders of 0.5–2 cm in diameter;
their number can vary from a few to hundreds
(Fig. 3.13e). Cysts may degenerate and resolve
into calcified nodules. Histologically, the cysts
contain one proto-scolex, the head of the future
tapeworm.
89
Echinococcosis
Age distribution: All ages.
Sex distribution: No sex predilection.
Epidemiology: Echinococcus granulosus
endemic worldwide, more common in the Middle
East and Eastern Africa; Echinococcus multiloc-
ularis restricted to Central Europe, Russia,
Turkey, China, and North America; Echinococcus
vogeli and Echinococcus oligarthus are rare spe-
cies limited to Central and South America.
Pathogens: Dog tapeworm Echinococcus
granulosus (classic hydatid disease), fox tape-
worm Echinococcus multilocularis (alveolar
hydatid disease), Echinococcus vogeli,
Echinococcus oligarthus (the two latter causing
polycystic echinococcosis).
Dogs and other canines are definitive hosts of
Echinococcus granulosus. Humans as intermedi-
ate hosts acquire Echinococcus granulosus clas-
sic hydatid disease by ingesting food contaminated
by canine feces containing eggs of the tapeworm.
The eggs hatch in the duodenum into oncospheres
that burrow into the gut wall and reach the liver.
The oncospheres grow into the hydatid cysts that
may spread to other organs including the brain.
Macroscopically, cysts in the cerebrum or cere-
bellum can reach a diameter of up to 10 cm, con-
sisting of one cyst or several cysts. Histologically,
within the cysts, 200 mm-long proto-scolices with
four spherical suckers and two rows of hooklets
can be observed (Fig. 3.13f).
The fox is the definitive host of Echinococcus
multilocularis. Cerebral infections are rare and
present as multiseptate, partly solid, partly cystic
space-occupying lesion mimicking a cerebral
tumor because the cyst wall penetrates into the
surrounding brain parenchyma.
Pathology of the Osseous Skull Base
Fibrous dysplasia
Aneurysmal bone cyst
Osteochondroma
Chondroblastoma
Chondrosarcoma
Chordoma
Endolymphatic sac tumor
90 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

Fig. 3.14 (a) Fibrous dysplasia with C-shaped bony
spicules (red) within a hypocellular fibrous stroma.
Masson trichrome. (b) Aneurysmal bone cyst with cystic
spaces separated by septa of connective tissue, reactive
woven bone (turquois), and multinucleated osteoclast-
type giant cells (dark violet). Goldner. (c) Chondroblas-
toma with well-defined cytoplasmic borders of the tumor
cells. Some nuclei display indentations (arrows). HE.
(d) Chondrosarcoma with ill-defined cytoplasmic borders
of the tumor cells. HE

Fibrous Dysplasia one bone or polyostotic (PFD) involving several

Age distribution: Children and young adults.
Sex distribution: There is no general sex predi-
lection in monostotic fibrous dysplasia (MFD);
however, with regard to the skull base manifesta-
tion, there is a predilection for males. To the con-
trary for polyostotic fibrous dysplasia (PFD) male
to female ratio of 1:3.
Epidemiology: one with FD per 15,000–30,000
population per year. MFD six times more common
than PFD. About 45 % of FD arise in the craniofa-
cial region (skull, mandible, facial bones).
Grading: Benign; malignant transformation
may occur in McCune–Albright syndrome, but
rarely.
Fibrous dysplasia is a medullary fibro-osseous
lesion and may be monostotic (MFD) involving
bones. The tympanic, mastoid, squamous, or
petrous temporal bones may be involved.
Unusual sites include the internal auditory canal,
the lateral semicircular canal, and the ossicles.
PFD may manifest in infants in the setting of
McCune–Albright syndrome caused by muta-
tions in the GNAS1 gene and associated with
café-au-lait spots and hyperfunctioning
endocrinopathies.
The bone shows local grayish and firm
expansions. Histologically, the bone marrow is
replaced by an intramedullary proliferation of
trabecular woven bone admixed with fibrous
tissue, and typical C-shaped bony spicules
within the fibrous matrix. The lesion arises
abruptly from a bland spindle cell stroma
(Fig. 3.14a).
Pathology of the Osseous Skull Base
Aneurysmal Bone Cyst
Age distribution: All age groups, most common
in the first two decades with a median age of
approximately 13 years.
Sex distribution: No sex predilection.
Epidemiology: 0.15 per million population per
year.
Grading: Benign, potentially locally recurrent.
Though aneurysmal bone cysts usually arise
in the metaphysis of long bones, any bone includ-
ing the skull base can be affected. The well-cir-
cumscribed lesion is composed of blood-filled
cystic spaces separated by septa of connective
tissue, reactive woven bone, and multinucleated
osteoclast-type giant cells (Fig. 3.14b).
Approximately 70 % of the cystic lesions arise
de novo, thus forming primary aneurysmal bone
cysts. Secondary aneurysmal bone cysts develop
most commonly in association with benign bone
tumors, for example, giant cell tumor, osteoblas-
toma, chondroblastoma, and fibrous dysplasia,
but may also complicate malignant bone tumors
that have undergone hemorrhagic cystic change,
especially osteosarcoma.
Osteochondroma
Age distribution: Juvenile and young adults.
Sex distribution: No sex predilection.
Epidemiology: Osteochondromas of the skull
base are rare [15, 36, 39].
Grading: Benign.
Osteochondroma is a cartilage-capped bony
projection arising on the external surface of bone
containing a marrow cavity that is continuous
with that of the underlying bone. Usually osteo-
chondromas are well demarcated. Histologically,
an outer layer of fibrous perichondrium underly-
ing cartilage and bone can be differentiated.
Chondroblastoma
Age distribution: Patients with skull and temporal
bone involvement present at 40–50 years of age.
Sex distribution: Male predominance.
Epidemiology: Less than 1 % of bone tumors.
91
Grading: Benign.
Within the craniofacial region, the temporal
bone is most frequently affected. Macroscopically,
a whitish, partly cystic tumor tissue is seen.
Histologically, sheets of uniform round to polyg-
onal chondroblasts with well-defined cytoplasmic
borders are characteristic. The round
nuclei frequently display grooves and indenta-
tions (Fig. 3.14c).
Chondrosarcoma
Age distribution: Adults with a peak in the fifth to
seventh decades.
Sex distribution: Slight male predominance.
Epidemiology: Extremely rare in craniofacial
bones.
Grading: Malignant; grade 1, few cells with
no variation in size and shape; grade 2, hypercel-
lularity with variation in size and shape; grade 3,
high cellularity with prominent pleomorphic
appearance, atypia, and mitoses.
Chondrosarcomas display a pure hyaline car-
tilage differentiation. Macroscopically, they have
a translucent, blue-gray or white color and often
a lobular growth pattern. Histologically, the carti-
lagineous tumor cells with ill-defined borders
permeate between preexisting bony trabeculae.
Myxoid changes, calcification, or ossification
may be present (Fig. 3.14d). Secondary chondro-
sarcoma may arise in a benign precursor, for
example, an osteochondroma.
Chordoma
Age distribution: Adults with a peak in the sixth
decade.
Sex distribution: Male to female ratio of 1.8:1.
Epidemiology: 1–4 % of primary malignant
bone tumors.
Grading: Intermediate.
Small remnants of the embryonic notochord
may be found in form of the ecchordosis physali-
phora. Chordomas are bone tumors that recapitu-
late notochord. With regard to the skull base, they
are located in the spheno-occipital region, typi-
cally beginning in the clivus. A small proportion
92 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

e f

Fig. 3.15 (a) Ecchordosis physaliphora (arrow). (b) from the clivus. (e) Chordoma lobule with cords of physa-
Ecchordosis physaliphora. Masson trichrome. (c) liphorous tumor cells in a myxoid matrix. HE. (f)
Chordoma masses protruding in the skull base. (d) Chordoma exhibiting a strong positivity of cytokeratin
Expansively growing chordoma of the skull base arising within the tumor cells. Pancytokeratin AE1/3

may involve the nasopharynx and/or paranasal
sinuses. They extend into the surrounding struc-
tures and may often form large tumor masses.
Metastases may occur. Histologically, chordomas
are lobulated tumors with tumor cells arranged in
sheets or cords within an abundant myxoid
stroma. The classic physaliphorous tumor cells
have a pale vacuolated cytoplasm (Fig. 3.15).
The chondroid chordoma with areas mimick-
ing cartilage and the “dedifferentiated” sarcoma-
toid chondroma with cell-dense sarcomatoid
areas are rare variants.
Endolymphatic Sac Tumor
Age distribution: Adults with a peak at 50 years
of age without von Hippel–Lindau disease
(VHL), and with a peak of 30 years of age with
VHL.
Pathology of the Sellar Region
Sex distribution: No sex predilection without
VHL; male to female ratio of 1:2 with VHL.
Epidemiology: Extremely rare in general pop-
ulation; prevalence in VHL 1–10 %, VHL occurs
at a rate of about 1:40 000 population.
Grading: Intermediate; slow growing, locally
invasive but not metastasizing; a differential
grading system was proposed by Bambakidis
et al. [3].
Endolymphatic sac tumors (synonyms: cere-
bellopontine angle ceruminoma, Heffner tumor,
aggressive papillary middle ear adenoma, and
low-grade adenocarcinoma of endolymphatic sac
origin) are destructive, papillary hypervascular
lesions that arise from the temporal bone retro-
labyrinthine region. At an early stage, the tumor
is located within the endolymphatic sac, at a later
stage, it destroys much of the petrous bone and
involves the cerebellopontine angle. Most endo-
lymphatic sac tumors occur sporadically, rarely
bilaterally. In association with von Hippel–Lindau
disease (VHL), the tumors are more frequently
bilateral and less advanced as opposed to the non-
VHL patients [24]. Mutations in the VHL gene
have been demonstrated in endolymphatic sac
tumor. For more information on VHL, see above
in the paragraph on hemangioblastoma.
Histologically, endolymphatic sac tumors
show a papillary adenomatous architecture, the
papillary processes infiltrating the surrounding
connective tissue and bone. The tumor may con-
tain areas of hemorrhage, hemosiderin, and cho-
lesterol clefts with scattered inflammatory giant
cell reactions. Dilated glands may contain secre-
tion, which resembles colloid.
Pathology of the Sellar Region
Pituitary adenoma
Pituitary carcinoma
Spindle cell oncocytoma of the adenohypophysis
Pituicytoma
Granular cell tumor of the neurohypophysis
Craniopharyngioma
Adamantinomatous craniopharyngioma
Papillary craniopharyngioma (Xanthogranuloma of
the sellar region)
93
Pituitary Adenoma
Age distribution: Adults; growth hormone-con-
taining tumors causing gigantism in children.
Sex distribution: No sex predilection; ACTH-
containing adenomas causing Cushing’s disease
more often in women than men with a male to
female ratio of 1:2.3.
Epidemiology: 15–25 % of intracranial tumors;
1 per 1,000 adult population per year.
Grading: Benign.
Pituitary adenomas derive from adenohypo-
physeal cells and are the most common tumors of
the adenohypophysis. Their prevalence increases
with advancing age. Although classified as
benign, these lesions can be highly invasive and
may cause major complications due to infiltration
into functionally delicate neighboring structures.
Also metabolic effects of hormone excess syn-
dromes can occur. A minority of patients with
large tumors may have acute bleeding into the
tumor (pituitary apoplexy), leading to an abrupt
increase in size and causing the sudden onset of
headache, visual loss, double vision, and/or pitu-
itary failure. Multiple endocrine neoplasia type 1
(MEN 1) is a rare condition characterized by
simultaneous tumors of the pituitary, pancreas,
and parathyroid glands. Pituitary adenomas
develop in 25 % of patients with MEN 1.
In rare cases, ectopic pituitary adenomas can
arise in embryonic remnants of Rathke’s cleft,
that is, the rare ectopic pharyngeal pituitary gland
(Fig. 3.16a, b). They may be found in the parapha-
ryngeal area, sphenoid sinus, middle nasal
meatus, petrous temporal bone, clivus, hypothal-
amus, and third ventricle.
Whereas microadenomas (less than 1 cm in
diameter) are still growing within the intra-
sellar adenohypohysis, macroadenomas (larger
than 1 cm in diameter) grow upward, the tumor
can elevate and compress the optic chiasm
(Fig. 3.16c, d).
Histologically, the traditional classification of
chromophobic, acidophilic, and basophilic ade-
nomas has largely been abandoned or at least has
to be completed by an immunohistochemical
classification based on hormone content. For a
reliable morphological determination of the
94 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings
a b
c d
Fig. 3.16 (a) Ectopic pharyngeal pituitary gland (arrow)
HE. (b) Detail of the ectopic pharyngeal pituitary gland.
HE. (c) Pituitary macroadenoma with infra- and suprasellar
hormone excretion, transmission electron
microscopy is indispensable. The ultrastructure
reveals densely granulated adenomas, thus, ade-
nomas with a high number of hormone granules
within the cytoplasm, and after enhanced extru-
sion of hormone granules, sparsely granulated
adenomas (Fig. 3.17), the latter often negative in
immunohistochemical staining.
The most effective way is the use of a clinico-
pathological classification in functioning and
nonfunctioning adenomas [11] using both mor-
phological and clinical features (Fig. 3.18).
Functioning adenomas with growth hormone
(GH) excess comprise densely or sparsely
granulated somatotroph adenomas and mammoso-
matotroph adenomas, adenomas causing hyperpro-
lactinemia (PRL) comprise lactotroph adenomas
and acidophilic stem cell adenomas, adenomas
growth. (d) Circumscribed pituitary microadenoma
surrounded by non-tumorous pituitary tissue contained to
the sella turcica. Elastica-van Gieson
causing thyroid-stimulating hormone (TSH) excess
are thyrotroph adenomas, adenomas causing adre-
nocorticotroph hormone (ACTH) excess comprise
densely or sparsely granulated corticotroph ade-
nomas, and finally adenomas causing gonadotropin
(FSH/LH) excess are gonadotroph adenomas.
Plurihormonal adenomas, especially GH- and
PRL-containing or GH-, PRL-, and TSH-containing
adenomas, may be observed. PRL-containing ade-
nomas (prolactinomas) are the most common type
followed by GH- and ACTH-containing adenomas.
Thyrotroph and gonadotroph adenomas are rare.
Nonfunctioning adenomas account for approx-
imately one-third of all pituitary adenomas. They
may be extremely sparsely granulated silent soma-
totroph, lactotroph, thyrotroph, corticotroph
(except a subtype of silent but densely granulated
corticotroph adenoma), or gonadotroph adenomas
Pathology of the Sellar Region 95

a b

c d

e f

Fig. 3.17 (a) Non-tumorous pituitary gland. Masson sparsely granulated (arrow) somatotroph adenoma.
trichrome. (b) Chromophobic adenoma. Masson trichrome. Transmission electron microscopy. (f) Nonfunctioning
(c) Eosinophilic adenoma. Masson trichrome. (d) extremely sparsely granulated “silent” somatotroph ade-
Basophilic adenoma. Masson trichrome. (e) Functioning noma. Transmission electron microscopy

on the one hand or true null cell adenomas on the
other hand. Oncocytomas represent silent gonado-
troph adenomas with extensive oncocytic change.
Pituitary Carcinoma
Age distribution: Adults.
Sex distribution: No sex predilection.
Epidemiology: 0.2 % of pituitary tumors.
Grading: Malignant.
Pituitary carcinomas are rare and character-
ized by marked polymorphism and mitoses. The
majority of carcinomas are secretory, usually
arising from corticotroph tumors or prolactino-
mas, but all histological types and secretory pat-
terns are represented [19]. They have to be
differentiated from polymorphous, in some cases
96 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

Fig. 3.18 (a) Border of a chromophobic adenoma, to the
left compressed preexistent non-tumorous pituitary tissue
with light-blue proliferated connective tissue fibers. Masson
trichrome. (b) The same case as in a with no hormone
expression within the adenoma (null cell adenoma) and
growth hormone expression within the scattered preexistent
eosinophilic cells. Immunohistochemistry with antibodies
to growth hormone. (c) Border of an eosinophilic adenoma,
to the left preexistent non-tumorous pituitary tissue. Masson
trichrome. (d) The same case as in c with growth hormone
expression within the adenoma (somatotroph adenoma)
and growth hormone expression within the scattered preex-
istent eosinophilic cells. Immunohistochemistry with anti-
bodies to growth hormone

calcified benign adenomas with regressive
changes. Pituitary carcinomas can locally invade
surrounding tissue or spread through the venous
system and the cerebrospinal fluid pathways
forming distant metastases (Fig. 3.19a, b).
adenomas. Histologically, the tumor is composed
of interlacing fascicles of eosinophilic epithelioid
cells with oncocytic cytoplasm.
Pituicytoma

Spindle Cell Oncocytoma Age distribution: Adults in the fifth to seventh

of the Adenohypophysis
Age distribution: Adults (mean 56 years).
Sex distribution: No sex predilection.
Epidemiology: No accurate incidence rates
available; 0.4 % of sellar tumors.
Grading: WHO grade I.
The spindle cell oncocytoma of the adenohy-
pophysis is a rare non-endocrine tumor that mac-
roscopically is indistinguishable from pituitary
decades.
Sex distribution: Male to female ratio of 1.6:1.
Epidemiology: Less than 30 cases described.
Grading: WHO grade I.
The pituicytoma is an extremely rare, circum-
scribed, and solid tumor that originates in the neu-
rohypophysis or infundibulum [7]. Histologically,
the glial tumor consists of elongate spindle cells
arranged in interlacing fascicles or in a storiform
pattern.
Pathology of the Sellar Region
a b
c d
Fig. 3.19 (a) Regressive pituitary adenoma with focal
calcifications (arrow). HE. (b) Pituitary carcinoma with
extensive cellular polymorphism. HE. (c) Nodular
arrangement of a granular cell tumor of the
Granular Cell Tumor
of the Neurohypophysis
Age distribution: Adults; exceptionally in
children.
Sex distribution: Male to female ratio of 1:2.
Epidemiology: No accurate incidence rates
available.
Grading: WHO grade I.
The granular cell tumor is a rare tumor that
arises from the neurohypophysis or infundibu-
lum. In the literature, synonyms such as
Abrikossoff tumor, choristoma, granular cell
myoblastoma, and granular cell neuroma can be
found. The usually lobulated and well-circum-
scribed tumor occurs in a sellar and suprasellar
anatomic location. Histologically, the tumor cells
are arranged in a nodular pattern and are charac-
terized by an abundant granular eosinophilic
cytoplasm (Fig. 3.19c, d).
97
neurohypophysis. HE. (d) Granular cell tumor of the
neurohypophysis showing the granular eosinophilic
cytoplasm. HE
Craniopharyngioma
Age distribution: Adults above 40 years of age; a
second peak in children (adamantinomatous
craniopharyngioma).
Sex distribution: No sex predilection.
Epidemiology: 1–5 % of intracranial tumors;
in children 5–10 % of intracranial tumors. 0.5–
2.5 per million population per year;
more frequent in Japanese children and in
Nigerian.
Grading: WHO grade I.
Craniopharyngiomas presumably derive
from remnants of Rathke’s pouch epithelium
and the craniopharyngeal duct. They have supra-
sellar localization with a minor intrasellar
component.
The adamantinomatous craniopharyngioma
is characterized by strands and lobules of
squamous epithelium bordered by tumor cell
98 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

e f

Fig. 3.20 (a) Well-circumscribed cystic craniopharyn-
gioma expanding into the third ventricle. (b) Poorly
demarcated craniophyaryngioma invading the third ven-
tricle and the basal ganglia. (c) Adamantinous craniophar-
yngioma with squamous epithelium bordered by cell
palisades. Masson trichrome. (d) Nodules of “wet kera-
tin” representing remnants of pale nuclei embedded within
a keratinous mass. Masson trichrome. (e) Calcified xan-
thogranuloma of the sellar region. (f) Xanthogranuloma
with hemosiderin deposits (brownish green) and choles-
terol clefts surrounded by some multinucleated giant cells.
Masson trichrome

palisades. Nodules of “wet keratin” are typical.
Cystic degeneration and calcifications may be
encountered. The papillary craniopharyngioma
is composed of papillae of squamous epithelium
lacking surface maturation. Calcifications are
absent (Fig. 3.20a–d).
Xanthogranuloma of the Sellar Region
Age distribution: All ages.
Sex distribution: No sex predilection.
Epidemiology: No accurate incidence rates
available.
Pathology of Regional Glial Tumors
Grading: Benign.
The xanthogranuloma of the sellar region is a
rare tumor, not yet fully defined as a separate
entity [18]. Transitional cases to craniopharyn-
gioma occur. Histologically, xanthogranulomas
show xanthoma cells, hemosiderin deposits, cho-
lesterol clefts occasionally surrounded by multi-
nucleated giant cells, and lymphocytic infiltrates
(Fig. 3.20e, f).
Pathology of Regional Glial Tumors
Pilocytic astrocytoma
Pilomyxoid astrocytoma
Ependymoma
Papillary
Clear cell
Tanycytic
Cellular
Anaplastic
Pilocytic Astrocytoma
Age distribution: First two decades; rare in
adults.
Sex distribution: No sex predilection.
Epidemiology: 5–6 % of gliomas; 0.8 per
100,000 population per year.
Grading: WHO grade I.
a b
Fig. 3.21 (a) Pilocytic astrocytoma of the optic chiasm.
(b) Pilocytic astrocytoma with bipolar hairlike piloid cells
showing numerous red Rosenthal fibers. Masson
trichrome. (c) Large pilomyxoid astrocytoma with
99
The pilocytic astrocytoma is a relatively cir-
cumscribed, often cystic tumor occurring in
children and young adults. Preferred sites at the
skull base include the optic nerve and the chias-
matic and hypothalamic region as well as brain
stem and cerebellum. There is no sex predilec-
tion. Histologically, the tumor exhibits an often
biphasic pattern with proportions of bipolar cells
with Rosenthal fibers on the one hand and loose-
textured multipolar cells with microcysts and
eosinophilic granular bodies on the other hand
(Fig. 3.21a, b).
Pilomyxoid Astrocytoma
Age distribution: Very young children (mean 10
months); rarely in older children.
Sex distribution: No sex predilection.
Epidemiology: No accurate incidence rates
available.
Grading: WHO grade II.
The pilomyxoid astrocytoma is closely related
to pilocytic astrocytoma. The chiasmatic and
hypothalamic region is the most common loca-
tion. Histologically, the tumor is characterized
by a markedly myxoid matrix. The bipolar tumor
cells show a predominantly angiocentric arrange-
ment around blood vessels (Fig. 3.21c, d). Due to
the slow tumor growth, regressive changes can
be seen.
prominent myxoid matrix. (d) Pilomyxoid astrocytoma
with tumor cells in a myxoid matrix. Masson trichrome.
(e) Ependymoma of the fourth ventricle. (f) Ependymoma
with typical perivascular pseudorosettes. HE
100 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings
c d
e f
Fig. 3.21 (continued)
Ependymoma
Age distribution: Infratentorial ependymomas in
children with a mean age of 6.4 years.
Sex distribution: No sex predilection.
Epidemiology: 6–12 % of intracranial tumors
in children; up to 30 % of intracranial tumors in
children younger than 3 years of age; 0.2–0.3 per
100,000 population per year.
Grading: WHO grade I; cellular ependymoma
WHO grade II; anaplastic ependymoma WHO
grade III.
Ependymomas originate from ependymal
cells and are well demarcated. Histologically, the
momomorphic tumor cells have round to oval
nuclei. Typical features are perivascular pseudor-
osettes with tumor cells arranged radially around
blood vessels with perivascular anuclear zones,
and ependymal rosettes (Fig. 3.21e, f). Variants
comprise the papillary ependymoma forming
pseudopapillae, the clear cell ependymoma with
an oligodendroglia-like appearance, and the
tanycytic ependymoma with tumor cells arranged
in fascicles, the latter variant is most common in
the spinal cord.
Variants with a semibenign or semimalignant
behavior comprise the cellular ependymoma with
a conspicuous increased cellularity, and the ana-
plastic ependymoma, which is relatively frequent
in children, particularly in the posterior fossa
location.
Pathology of the Choroid Plexus
of the Fourth Ventricle
Choroid plexus papilloma
Atypical choroid plexus papilloma
Choroid plexus carcinoma
Choroid Plexus Papilloma
Age distribution: Children and young adults
(mean 22.5 years).
Sex distribution: Male to female ratio of 1.5:1
(fourth ventricle).
Pathology of the Hematopoietic System 101

a b

c d

Fig. 3.22 (a) Circumscribed cauliflower-like plexus papil- trichrome. (c) Plexus carcinoma of the cerebellopontine
loma of the cerebellopontine angle. (b) Papillomatous pattern angle with necrotic areas. (d) Papillomatous pattern of poly-
of monomorphous cuboidal tumor cells cover a connective morphous tumor cells with distinct nuclear polymorphism
tissue matrix (blue) with blood vessels (red). Masson cover a connective tissue matrix with blood vessels. HE

Epidemiology: 10–20 % of brain tumors in Choroid Plexus Carcinoma

children in the first year of life, 2–4 % of brain
tumors in children under 15 years. 0.3 per one Age distribution: Children and young adults.
million population per year. Sex distribution: Male to female ratio of 1.5:1
Grading: WHO grade I; WHO grade II (atypi- (fourth ventricle).
cal choroid plexus papilloma, about 15 % of Epidemiology: Less than 20 % of choroid
choroid plexus papillomas). plexus tumors.
Relevant for the cerebellopontine angle are the Grading: WHO grade III.
choroid plexus papillomas of the fourth ventricle, Choroid plexus carcinomas exhibit all signs of
which comprise about 40 % of this entity (50 % malignancy such as frequent mitoses of five or
lateral ventricles, 5 % third ventricle, 5 % more more per HPF, increased cellular density, nuclear
than one ventricle). However, a primary manifesta- polymorphism, necrotic areas, and also often a
tion in the cerebellopontine angle near the foramina diffuse invasion into the neighboring brain tissue
of the fourth ventricle is uncommon. Circumscribed (Fig. 3.22c, d).
cauliflower-like tumors are well delineated from
brain tissue. Histologically, a single layer of cuboi-
dal cells cover a connective tissue matrix. Mitotic Pathology of the Hematopoietic
activity is extremely low (Fig. 3.22a, b). System
Atypical choroid plexus papillomas show an
increased mitotic activity of two or more mitoses Primary CNS lymphomas
Langerhans cell histiocytosis
per high-power field (HPF), increased cellularity,
Non-Langerhans cell histiocytosis
and nuclear polymorphism.
102 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings
Primary CNS Lymphomas
Age distribution: All ages with a peak in immu-
nocompetent patients in the sixth and seventh
decades of life. In cases of immunodeficiency,
young patients may be involved.
Sex distribution: Male to female ratio of 3:2.
Epidemiology: 1–7 % of primary intracranial
tumors; incidence highly increased in AIDS
patients who develop in 2–12 % primary CNS
lymphomas (PCNSL).
Grading: Malignant.
Lymphoma is most often encountered in the
central nervous system as secondary spread from
another site. Extra-nodal malignant lymphomas
arising primarily in the central nervous system
also affecting the skull base or occurring as pri-
mary leptomeningeal lymphomas must be differ-
entiated from secondary involvement. However,
given the absence of lymphoid tissue in the CNS,
it remains possible that the PCNSL is rather a
systemic lymphoma with a restricted tropism for
the CNS. Approximately 13 % of PCNSL involve
the posterior fossa. A secondary leptomeningeal
spread in form of meningiosis lymphomatosa is
observed in 30–40 % of PCNSL. In many cases,
a diagnosis by means of CSF cytology is possible
(Fig. 3.23a, b). Surgery should be restricted to
stereotactic biopsy.
Histologically as in other anatomic locations,
malignant lymphomas are classified according to
a b
Fig. 3.23 (a) CSF cytology of a malignant non-Hodgkin dif-
fuse large B-cell lymphoma. Note the large nuclei and the
numerous mitoses. May–Grünwald–Giemsa. (b) CSF cytol-
ogy of a diffuse large B-cell lymphoma with immunocy-
tochemically positive B-cell marker (red). CD20, APAAP.
the WHO classification respecting the Revised
European–American Lymphoma (REAL)
classification [16]. B-cell non-Hodgkin lympho-
mas constitute up to 98 % of PCNSL, most of
them being diffuse large B-cell lymphomas. A
primary CNS presentation of Hodgkin’s disease
is rare [10].
Langerhans Cell Histiocytosis
Age distribution: Children under 15 years of age
(mean 12 years).
Sex distribution: No sex predilection.
Epidemiology: 0.5 per 100,000 children per
year.
Grading: Benign (unifocal); intermediate to
malignant (multifocal).
In 1997, the WHO Committee on Histiocytic/
Reticulum Cell Proliferations and the
Reclassification Working Group of the
Histiocyte Society proposed a new classification.
With regard to skull base manifestations
Langerhans cell histiocytosis (LCH) was previ-
ously referred to as histiocytosis X embracing
skull and brain manifestations such as eosino-
philic granuloma and Hand–Schüller–Christian
disease.
The most common form is a solitary unifocal
(monostotic) osteolytic lesion of the skull in form
of an eosinophilic granuloma. The cerebral LCH
(c) Eosinophilic granuloma of the skull as unifocal LCH
manifestation. (d) Hypothalamic LCH manifestation com-
pressing the optic chiasm. (e) LCH composed of plasma
cells, lymphocytic, eosinophilic, and Langerhans histiocytic
tumor cells. HE. (f) Multinucleated Touton giant cell. HE
Pathology of the Hematopoietic System
c d
e f
Fig. 3.23 (continued)
with principal involvement of the hypothalamus
and posterior pituitary leads to diabetes insipidus
often associated with signs of hypothalamic dys-
function (obesity, hypogonadism, growth retar-
dation), optic atrophy with visual field defects,
and raised intracranial pressure. Multifocal (poly-
ostotic) lesions of the bone with hypothalamic
involvement have been referred to as Hand–
Schüller–Christian disease. While unifocal LCH
surgically can be removed without recurrence or
even spontaneously recover, multifocal manifes-
tations have a poor outcome with a mortality rate
up to 20 %.
Histologically, tumor cell infiltrates are com-
posed of Langerhans cells, macrophages, lym-
phocytes, and plasma cells. A variable fraction of
eosinophils and Touton giant cells may occur
(Fig. 3.23c–d).
Non-Langerhans Cell Histiocytosis
Age distribution: Children under 15 years of age.
Sex distribution: No sex predilection.
103
Epidemiology: one per one million children
per year.
Grading: Benign to malignant.
Non-LCH variants such as Rosai–Dorfman
disease, Erdheim–Chester disease, and juve-
nile xanthogranuloma are characterized by
macrophage differentiation. Histologically,
emperipolesis is typical, that is, well-preserved
lymphocytes and plasma cells taken in the cyto-
plasm of macrophages.
Intracranial Rosai–Dorfman disease in adults
shows dural-based solitary or multiple masses.
Intrasellar lesions and intracranial extension from
an orbital mass or from the nasal cavity and para-
nasal sinuses may occur.
Intracranial Erdheim–Chester disease in adults
may involve cerebellum, cerebellopontine angle,
choroid plexus, pituitary, meninges, and orbit.
An extracerebral subdural manifestation of
Erdheim–Chester disease associated with a giant
adenoma of the pituitary was reported [42].
Intracranial juvenile xanthogranuloma in
young children may arise in the brain or the
meninges with or without cutaneous lesions.
104 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings
Pathology of Germ Cells
Germ cell tumors
Germinoma
Embryonal carcinoma
Yolk sac tumor
Choriocarcinoma
Teratoma
Mature teratoma
Immature teratoma
Teratoma with malignant transformation
Mixed germ cell tumor
Tumorlike dysontogenetic cysts
Epidermoid cyst
Dermoid cyst
Germ Cell Tumors
Age distribution: Children and young adults with
a peak in the second decade.
Sex distribution: Strong male predilection,
especially in the pineal region; certain female
predilection in the suprasellar region.
Epidemiology: In Far East Asia 2–3 % of
intracranial tumors, 8–15 % of intracranial
tumors in children, 0.17 per 100,000 population
per year; in Western countries 0.3–0.6 % of
intracranial tumors; 0.09 per 100,000 population
per year.
Grading: Primordial, embryonal, extraembry-
onal, and mixed tumors malignant; mature
a b
Fig. 3.24 (a) Germinoma with biphasic appearance of
large tumor cells and small lymphocytes due to a typical
inflammatory reaction. HE. (b) Germinoma mixed with
placenta-derived syncytiotrophoblastic giant cells. HE. (c)
Embryonal carcinoma with cohesive group of tumor cells.
teratomas benign, immature teratomas intermedi-
ate, teratomas with malignant transformation
malignant.
In human embryos, the primordial germ cells
are produced in the fetal yolk sac and migrate
along the midline to the gonadal folds, thereby
passing the central nervous, mediastinal, and ret-
roperitoneal regions [44]. Along this way, scat-
tered primordial germ cells in different stages of
differentiation can lead to dysontogenetic tumors
or tumorlike lesions.
In principle, the same undifferentiated or
poorly differentiated primary germ cell tumors
can be encountered in the brain as, for example,
in the gonads. The primordial germ cells them-
selves give rise to germinoma (identical with
seminomas of the testicles and dysgerminomas
of the ovaries). Cells with early embryonic dif-
ferentiation give rise to embryonal carcinoma,
whereas extraembryonal cells deriving from the
yolk sac or the placenta may develop to yolk sac
tumor (syn. endodermal sinus tumor) or chorio-
carcinoma, respectively. Mixed tumors, for
example, germinoma with placenta-derived syn-
cytiophoblastic giant cells, may be attributed to a
differentiation of the single totipotential germ
cell into different cell lines. All these tumors are
highly malignant. They are rare but may be
encountered along the midline of the brain and
the pineal region and also extend to the skull base
(Fig. 3.24).
HE. (d) Choriocarcinoma with predominantly placenta-
derived cytotrophoblastic cells. HE. (e) Yolk sac tumor with
reticular pattern in form of an allantois-like meshwork. HE.
(f) Yolk sac tumor with endodermal sinus pattern (Schiller–
Duval body) forming a distinctive cell wreath (arrow). HE
Pathology of Blood Vessels and Circulation
c d
e f
Fig. 3.24 (continued)
Teratomas and rare teratoma-like tumor exam-
ples deriving from all three germ layers or only
one or two ones comprise a varying tissue mixture
of all kind of immature and mature tissue, even
small well-structured bones or teeth (Fig. 3.25).
Although the vast majority of these tumors are
benign, teratomas with malignant transformation
of one of the tissue components can occur.
Tumorlike Dysontogenetic Cysts
Age distribution: All ages.
Sex distribution: No sex predilection.
Epidemiology: 0.2–2 % of intracranial tumors;
dermoid cysts rarer than epidermoid cysts.
Grading: Benign.
The epidermoid cyst is the main representative
of the differentiated dysontogenetic cysts. It
derives from differentiated cells of the ectoder-
mal germ layer forming cysts with squamous
epithelial-lined wall. In the first description of an
epidermoid cyst by Cruveilhier [9], he painted a
poetic picture of the outer surface of “metallic
105
sheen like silver or like a pearl of the finest water,”
therefore also the synonym of pearly tumor. The
cerebellopontine angle is a common location.
The dermoid cyst usually occurs in the poste-
rior fossa and exhibits cysts with squamous epi-
thelial-lined wall and derivatives from skin
appendices such as hair follicles with hairs and
sebaceous glands (Fig. 3.26).
Pathology of Blood Vessels
and Circulation
Arteriovenous malformation
Saccular aneurysm
Ischemic brain infarct
Hypertensive angiopathy and brain hemorrhage
Vasculitis
Arteriovenous Malformation
Age distribution: All ages.
Sex distribution: No sex predilection.
106 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

e f

Fig. 3.25 (a) Teratoid lipoma including a small mature
bone (arrow). Note the dysraphic malformation of the
cerebellum. (b) Detail of the mature bone with compacta,
bone marrow, and cartilage within the lipoma. Masson
trichrome. (c) Mature teratoma with mixture of squamous
epithelium, glands, bone, and connective tissue. HE.
(d) Tooth-anlage in a mature teratoma within the brain.
HE. (e) Numerous small but mature teeth within a brain
teratoma. (f) Detail showing mature molars and an
incisor

Epidemiology: In a consecutive series of 4,530 well as treatment options and recommendations

autopsies, 4.3 % AVMs were found, among them
only 0.5 % became symptomatic [28]. In 30–80 %
of the patients, the possibly life-threatening hem-
orrhage is the leading clinical manifestation [27]
with a lethality of 10–15 % and a morbidity of up
to 50 % [30, 38, 43]. The occurrence of AVM-
related hemorrhage counts for about 1 % among
all strokes [13].
Epidemiological data, current concepts on
diagnostic procedures, and grading systems, as
for the clinical management of arteriovenous
malformations (AVMs), were formulated by the
AVM Study Group [41] (AMSG) and a special
writing group of the Stroke Council of the
American Stroke Association [34].
Morphologically, AVMs are a complex tangle
of abnormal arteries and veins linked by one or
often more fistulae. Connecting capillaries are
missing. It is supposed that AVMs are caused by
a prenatal disorder of primordial vascular
Pathology of Blood Vessels and Circulation
a b
c d
Fig. 3.26 (a) Surface of an epidermoid cyst shining like
a cluster of pearls. (b) Epidermoid cyst of the CPA filled
with densely packed white keratin lamellae. (c) Epidermoid
cyst with squamous epithelial-lined wall filled with kera-
channels when the normal development to
mature arteries, capillaries, and veins is inhib-
ited [33, 35]. Alternatively, since juvenile and
adult AVMs show some different features, also a
possible postnatal development was proposed
[22, 23]. For their tumorlike appearance, in
German literature AVMs are often described as
arteriovenous hemangiomas, differentiating
macroangiomas with a diameter of several cen-
timeters and microangiomas with a diameter of
a few millimeters up to one centimeter
(Fig. 3.27a–d). However, AVMs may increase
their volume, remain static, or may even sponta-
neously shrink [1].
Superficial cortical AVMs lie in a sulcus or in
the cortex. They may extend subcortically and
are then wedge-shaped with the base in the cor-
tex. The apex may reach the ventricular wall
(corticoventricular or transcerebral AVMs). They
are fed by cortical branches of major cerebral
arteries and drained by superficial veins, in tran-
scerebral AVMs often also by means of the deep
107
tin slivers. Masson trichrome. (d) Dermoid cyst with
squamous epithelial-lined wall, hair follicle (arrow), and
sebaceous glands (asterisk). Masson trichrome
venous system. Deep noncortical AVMs that lie
in the white matter are supplied by both surface
and deep arteries, those in the basal ganglia, thal-
amus, and brain stem by the small perforating
arteries, mainly lenticulostriate and thalamoper-
forant arteries. Drainage is by means of the deep
veins. Deep AVMs count for 15 % of all AVMs.
Choroid plexus AVMs are fed by choroidal arter-
ies and subependymal branches of the arteries at
the base of the brain. They are drained exclu-
sively by means of the deep veins.
Saccular Aneurysm
Age distribution: Adults with a peak in the sixth
decade; rare in children (less than 5 % under the
age of 20 years).
Sex distribution: Male to female ratio of 2:3;
males predominate in the younger age groups.
Epidemiology: 2–5 % prevalence in the gen-
eral population.
108 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

a b

c d

e f

Fig. 3.27 (a) Ruptured arteriovenous malformation
(AVM) of the cerebellum with massive bleeding involving
the cerebellopontine angle. (b) Rupture of a larger vessel
within an AVM with an atomic mushroom-like bleeding.
Masson trichrome. (c) AVM characterized by a convolute
of larger and smaller vessels of varying thickness of the
vessel wall. (d) AVM with convolute of vessels. Elastica-
van Gieson. (e) Saccular (berry) aneurysm of the basilar
artery. (f) Note the collagenous wall of the aneurysm
(arrow) without internal elastic lamina (red) and muscular
media compared to the adjacent normal vessel. Masson
trichrome

Almost all saccular or berry aneurysms arise
at the bifurcations of the main intracranial arter-
ies and have a propensity to spontaneous rupture
leading to primary nontraumatic subarachnoid
hemorrhage. Only about 5–10 % of saccular
aneurysms involve the posterior cerebral arteries
and the vertebrobasilar arteries, thus directly
involving the CPA area. Aneurysms with a diam-
eter over 25 mm are called giant aneurysms;
interestingly 30–40 % of aneurysms of children
are giant aneurysms. Histologically, the wall of
the aneurysm consists only of fibrous connective
tissue and is characterized by the absence of both
the muscular tunica media and the internal elastic
lamina, both ending abruptly at the neck of the
aneurysm (Fig. 3.27e, f).
Different syndromes with gene mutations are
linked with saccular aneurysms such as Marfan’s
syndrome, Ehlers–Danlos syndrome type IV, auto-
somal dominant polycystic kidney disease, and
Pathology of Blood Vessels and Circulation
hereditary hemorrhagic telangiectasia. In about
12–15 % of patients, the aneurysms are familial.
Familial aneurysms were more often multiple and
have a tendency to rupture at a smaller size.
Ischemic Brain Infarct
Age distribution: Age dependent, increasing with
higher age, in children under 15 years of age 1
per 100,000 population, in adults 85 years and
older 1 per 33 population.
Sex distribution: Males predominate.
Epidemiology: Because of higher proportion
of elderly people, high incidence rates in Western
countries and Japan; white people have lower
rates than nonwhite people.
The cause of ischemia of brain areas is mani-
fold. However, most cases are related to athero-
sclerosis of brain arteries often in combination
with thrombosis at sites of atherosclerotic
endothelial ulcerations (Fig. 3.28a). Another
important cause is emboli that break loose from
thrombi of extracranial atherosclerotic arteries or
the heart. In rare cases, tumor emboli or fat embo-
lism may cause ischemia. Vasculitides (see
below) may lead to stenosis of arteries. Cerebral
vasospasm after severe head injury and subarach-
noidal hemorrhage is correlated with ischemic
damage and may complicate neurosurgical inter-
ventions in such cases. When the ischemia is
a b
Fig. 3.28 (a) Severe atherosclerosis of all arteries of
Willis’ circle. Note the fusiform atherosclerotic aneurysm
of the basilar artery. (b) Ischemic infarct stage I of the
cerebellum with loss of tissue staining (arrow). Nissl. (c)
Ischemic infarct stage II of the pons with loss of myelin
sheaths. Heidenhain–Woelcke. (d) Ischemic infarct stage
109
severe enough and lasts for long enough, perma-
nent nerve cell damage ensues, clinically appear-
ing as stroke. Stroke is defined as an abrupt onset
of focal or global neurological symptoms caused
by ischemia or hemorrhage.
After 4–12 h in stage I of ischemic brain
infarct, the tissue macroscopically turns pale, and
histologically Nissl bodies and nuclei disappear
(Fig. 3.28b); after 24 h, neutrophil leukocyte
infiltration begins. After 48 h in stage II, mac-
rophages which histologically appear as foam
cells invade the infarct and start with phagocytiz-
ing the damaged tissue (Fig. 3.28c–e). After 5
days, the neutrophil leukocyte infiltration ceases.
In larger infarcts, the phagocytosis may last for
months. Finally in stage III, the finished phago-
cytosis results in lacunae in small infarcts or in
extended cysts in larger infarcts, both surrounded
by a layer of proliferated astrocytes. When, for
instance, the precentral brain cortex is irrevers-
ibly destroyed by an infarct, not only the area of
infarction is destroyed but also the entire pyrami-
dal tract will become atrophic (Fig. 3.28f).
Hypertensive Angiopathy and Brain
Hemorrhage
Age distribution: Age dependent, increasing with
higher age.
Sex distribution: Males predominate.
II with macrophages (arrow) phagocytizing lipids within
the infarction. Nissl. (e) Macrophages (foam cells) phago-
cytizing lipids within the infarct. Nissl. (f) Secondary uni-
lateral degeneration of the pyramidal tract after ischemic
infarct stage III in the precentral cortex
110 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings

c d

e f

Fig. 3.28 (continued)

Epidemiology: 10–30 per 100,000 population; Vasculitis

higher frequency in black people and Far Eastern
populations due to higher prevalence of hyper-
tension; hypertensive brain hemorrhage 50 % of
brain hemorrhages; 13 % of hypertensive brain
hemorrhages in the cerebellum, 7 % in the pons.
Chronic hypertension on the one hand aggra-
vates atherosclerotic changes, on the other hand
induces a hypertensive angiopathy characterized
by reduplication of the basal lamina, and fibrosis
of the arterial vessel wall where fibrotic connec-
tive tissue replaces smooth muscle cells. In later
stages, arteries with homogenous fibrinoid necro-
sis of the vessel wall are prone to rupture. The
subsequent arterial bleeding, the hypertensive
brain hemorrhage, may result in a considerable
size of the blood clot which displaces the sur-
rounding brain tissue (Fig. 3.29a, b).
Other etiologies of nontraumatic brain hemor-
rhage comprise cerebral amyloid angiopathy, arterio-
venous malformations, aneurysms, tumor bleedings,
and any cause of impaired blood coagulation, for
example, by anticoagulants and other drugs.
Age distribution: Adults over 55 years of age
(GCA); young adults (Takayasu); all ages with a
peak in the fifth decade (PAN).
Sex distribution: No sex predilection (GCA);
female predilection (Takayasu); male to female
ratio of 2:1 (PAN).
Epidemiology: 20 per 100,000 population over
50 years of age (GCA); 1 per 3,000 population in
Japan, rare outside Japan (Takayasu); CNS
involvement in 10–50 % of generalized PAN;
CNS involvement in 50–75 % of generalized
SLE.
Inflammatory diseases of CNS blood vessels
are caused by infections or immunological pro-
cesses, respectively, and may lead to ischemic
tissue damage. Histologically, inflammatory
infiltrates in the vessel wall in various cell com-
positions are diagnostic. The inflammatory
changes are often focal (focal granulomas) which
means that a negative histological result in a
biopsy cannot completely rule out the possibility
Pathology of Blood Vessels and Circulation 111

a b

c d

e f

Fig. 3.29 (a) Hypertensive angiopathy characterized by
fibrinoid necrosis (red) destroying the smooth muscle
cells of the vessel wall. Masson trichrome. (b) Hypertensive
hemorrhage of the pons. (c) Arteritis temporalis Horton
characterized by numerous multinucleated giant cells
within the inflammatory infiltrate of the vessel wall.
Masson trichrome. (d) Eosinophilic (allergic) arteritis
Churg–Strauss characterized by predominance of eosino-
phils (red) within the inflammatory infiltrate of the vessel
wall. Masson trichrome. (e) Panarteritis nodosa character-
ized by the lymphocytic inflammatory infiltrate and focal
fibrinoid necrosis (red). Masson trichrome. (f) Panarteritis
nodosa with extended fibrinoid necrosis of intracerebral
vessels. HE

of a vascultitis, for example, giant cell arteritis of
the temporal artery. Therefore, if possible, a
biopsy of 3–5 cm length is recommended.
Direct microbial infections with bacterial
(e.g., due to streptococci, mycobacteria, spiro-
chetes), viral (e.g., due to herpes zoster), fungal,
or protozoal etiologies have different age and sex
distributions as well as epidemiology depending
of the pathogen in question. For examples of
microbial infections, see the paragraph on the
different forms of meningitis above.
For vasculitides caused by immunological
processes, a classification of three groups based
on pathogenetic mechanisms includes cell-medi-
ated, immune-complex-mediated, and antineu-
trophil cytoplasmic antibody (ANCA)-mediated
112 3 Neuropathology of the Cerebellopontine Angle and Its Surroundings
inflammations [17]. Cell-mediated inflammations
comprise giant cell arteritis (GCA, synonym
temporal arteritis Horner) which is the most com-
mon form in Europe (Fig. 3.29c), Takayasu’s
arteritis which is very common in Japan, primary
angiitis of the central nervous system, and
Kawasaki’s disease. Immune-complex-mediated
inflammations comprise panarteritis nodosa
(PAN) manifesting as systemic or limited form
(Fig. 3.29e, f), systemic lupus erythematosus
(SLE), Behçet syndrome, and some infection- or
drug-induced, or malignancy-related vasculiti-
des. ANCA-mediated inflammations comprise
Churg–Strauss syndrome, which is also called
eosinophilic or allergic angiitis (Fig. 3.29d),
Wegener’s granulomatosis, and some drug-
induced vasculitides. A mixed immunological
etiology is observed in Sjögren’s syndrome.
References
1. Abdulrauf SI, Malik GM, Awad IA (1999) Spontaneous
angiographic obliteration of cerebral arteriovenous
malformations. Neurosurgery 44(2):280–287; discus-
sion 287–288
2. Balmaceda CM, Fetell MR, O’Brien JL, Housepian
EH (1993) Nevus of Ota and leptomeningeal melano-
cytic lesions. Neurology 43(2):381–386
3. Bambakidis NC, Megerian CA, Ratcheson RA (2004)
Differential grading of endolymphatic sac tumor
extension by virtue of von Hippel-Lindau disease sta-
tus. Otol Neurotol 25(5):773–781
4. Barnes L, Eveson JW, Reichart P, Sidransky D (eds)
(2005) World Health Organization classification of
tumours. Pathology and genetics of head and neck
tumours. IARC Press, Lyon
5. Best PV (1987) Malignant triton tumour in the cere-
bellopontine angle. Report of a case. Acta Neuropathol
74(1):92–96
6. Brat DJ, Giannini C, Scheithauer BW, Burger PC
(1999) Primary melanocytic neoplasms of the central
nervous systems. Am J Surg Pathol 23(7):745–754
7. Brat DJ, Scheithauer BW, Staugaitis SM, Holtzman
RN, Morgello S, Burger PC (2000) Pituicytoma: a
distinctive low-grade glioma of the neurohypophysis.
Am J Surg Pathol 24(3):362–368
8. Carrasco CA, Rojas-Salazar D, Chiorino R, Venega
JC, Wohllk N (2006) Melanotic nonpsammomatous
trigeminal schwannoma as the first manifestation
of Carney complex: case report. Neurosurgery
59(6):E1334–E1335; discussion E1335. 00006123-
200612000-00022
9. Cruveilhier L (1829) Anatomie pathologique du corps
humain, vol tome 1, livre II. Baillière, Paris
10. Deckert-Schluter M, Marek J, Setlik M, Markova J,
Pakos E, Fischer R, Wiestler OD (1998) Primary
manifestation of Hodgkin’s disease in the central ner-
vous system. Virchows Arch 432(5):477–481
11. Ellison DW, Perry A, Rosenblum M, Asa S, Reid R,
Louis DN (2008) Tumours: non-neuroepithelial
tumours and secondary effects. In: Love S, Louis DN,
Ellison DW (eds) Greenfield’s neuropathology, 8th
edn. Edward Arnold, London, pp 2088–2106
12. Fletcher DM, Unni KK, Meterns F (eds) (2002) World
Health Organization classification of tumours.
Pathology and genetics of tumours of soft tissue and
bone. IARC Press, Lyon
13. Furlan AJ, Whisnant JP, Elveback LR (1979) The
decreasing incidence of primary intracerebral hem-
orrhage: a population study. Ann Neurol 5(4):
367–373
14. Han DH, Kim DG, Chi JG, Park SH, Jung HW, Kim
YG (1992) Malignant triton tumor of the acoustic
nerve. Case report. J Neurosurg 76(5):874–877.
doi:10.3171/jns.1992.76.5.0874
15. Ito U, Hashimoto K, Inaba Y (1974) Osteochondroma
of the posterior clinoid process. Report of a case with
special reference to its histogenesis. Acta Neuropathol
27(4):329–335
16. Jaffe ES, Harris NL, Stein H, Vardiman JW (2001)
World Health Organization classification of tumours.
Pathology and genetics of tumours of haematopoietic
and lymphoid tissues. IARC Press, Lyon
17. Jennette JC, Falk RJ, Milling DM (1994) Pathogenesis
of vasculitis. Semin Neurol 14(4):291–299
18. Jung CS, Schanzer A, Hattingen E, Plate KH, Seifert
V (2006) Xanthogranuloma of the sellar region. Acta
Neurochir (Wien) 148(4):473–477
19. Kaltsas GA, Nomikos P, Kontogeorgos G, Buchfelder
M, Grossman AB (2005) Clinical review: diagnosis
and management of pituitary carcinomas. J Clin
Endocrinol Metab 90(5):3089–3099
20. Kepes JJ, Chen WY, Connors MH, Vogel FS (1988)
“Chordoid” meningeal tumors in young individuals
with peritumoral lymphoplasmacellular infiltrates
causing systemic manifestations of the Castleman syn-
drome. A report of seven cases. Cancer 62(2):391–406
21. Kudo M, Matsumoto M, Terao H (1983) Malignant
nerve sheath tumor of acoustic nerve. Arch Pathol
Lab Med 107(6):293–297
22. Lasjaunias P (1997) Vascular diseases in neonates,
infants and children: interventional neuroradiology
management. Springer, Berlin/Heidelberg/New York
23. Lasjaunias PTBK (1996) Vascular diseases in chil-
dren. Springer, Berlin/Heidelberg/New York
24. Lonser RR, Kim HJ, Butman JA, Vortmeyer AO,
Choo DI, Oldfield EH (2004) Tumors of the endolym-
phatic sac in von Hippel-Lindau disease. N Engl J
Med 350(24):2481–2486
25. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK
(eds) (2007) WHO classification of tumours of the
central nervous system. IARC Press, Lyon
26. Love S, Louis DN, Ellison DW (eds) (2008)
Greenfield’s neuropathology, vol 1 and 2, 8th edn.
Hodder Arnold, London
References
27. Mast H, Mohr JP, Osipov A, Pile-Spellman J, Marshall
RS, Lazar RM, Stein BM, Young WL (1995) ‘Steal’ is
an unestablished mechanism for the clinical presenta-
tion of cerebral arteriovenous malformations. Stroke
26(7):1215–1220
28. McCormick W (1978) Classification, pathology, and
natural history of angiomas of the central nervous sys-
tem. Weekly update. Neurol Neurosurg 14:2–7
29. McLean CA, Laidlaw JD, Brownbill DS, Gonzales MF
(1990) Recurrence of acoustic neurilemoma as a malig-
nant spindle-cell neoplasm. Case report. J Neurosurg
73(6):946–950
30. Michelsen WJ (1979) Natural history and pathophysi-
ology of arteriovenous malformations. Clin Neurosurg
26:307–313
31. Miller RT, Sarikaya H, Sos A (1986) Melanotic
schwannoma of the acoustic nerve. Arch Pathol Lab
Med 110(2):153–154
32. Mrak RE, Flanigan S, Collins CL (1994) Malignant
acoustic schwannoma. Arch Pathol Lab Med
118(5):557–561
33. Nelson MD Jr, Gonzalez-Gomez I, Gilles FH (1991)
Dyke award. The search for human telencephalic ven-
triculofugal arteries. AJNR Am J Neuroradiol 12(2):
215–222
34. Ogilvy CS, Stieg PE, Awad I, Brown RD Jr,
Kondziolka D, Rosenwasser R, Young WL,
Hademenos G (2001) AHA scientific statement: rec-
ommendations for the management of intracranial
arteriovenous malformations: a statement for health-
care professionals from a special writing group of the
Stroke Council, American Stroke Association. Stroke
32(6):1458–1471
113
35. Padget DH (1956) The cranial venous system in man
in reference to development, adult configuration, and
relation to the arteries. Am J Anat 98(3):307–355
36. Padhya TA, Athavale SM, Kathju S, Sarkar S, Mehta
AR (2007) Osteochondroma of the skull base.
Otolaryngol Head Neck Surg 137(1):166–168
37. Piedra MP, Scheithauer BW, Driscoll CL, Link MJ
(2006) Primary melanocytic tumor of the cerebellopon-
tine angle mimicking a vestibular schwannoma: case
report. Neurosurgery 59(1):E206; discussion E206
38. Samson DSBH (1991) Preoperative evaluation of the
risk/benefit ratio for arteriovenous malformations of the
brain. In: Williams RH, Rengachary SS (eds) Neurosurgery
update II: vascular, spinal pediatric, and functional neuro-
surgery. MacGraw-Hill, New York, pp 129–133
39. Sato K, Kodera T, Kitai R, Kubota T (1996)
Osteochondroma of the skull base: MRI and histo-
logical correlation. Neuroradiology 38(1):41–43
40. Stein AA, Schilp AO, Whitfield RD (1960) The histo-
genesis of hemangioblastoma of the brain. A review
of twenty-one cases. J Neurosurg 17:751–761
41. The AMSG (1999) Arteriovenous malformations of
the brain in adults. N Engl J Med 340:1812–1818
42. Thorns V, Zajaczek J, Becker H, Walter GF, Hori A
(2003) Extracerebral subdural manifestation of Chester-
Erdheim disease associated with a giant adenoma of
the pituitary. Clin Neuropathol 22(5):246–251
43. Wilkins RH (1985) Natural history of intracranial vas-
cular malformations: a review. Neurosurgery 16(3):
421–430
44. Witschi E (1948) Migration of germ cells of human
embryos from the yolk sac to the primitive gonadal
folds. Contrib Embryol 32:67–80
 Approaches to the Cerebellopontine
Angle
In this chapter, the three main operative
approaches to the internal auditory canal and CP
angle—the retrosigmoid, the translabyrinthine,
and the middle fossa approach—are discussed.
An overview of the other approaches and tech-
niques to the CP angle is presented in the chapter
on the “anatomy of the CP angle.”
Middle Fossa Approach
The MF approach to the IAC was described by
Parry in 1904 who used it to section the auditory
nerve in a patient with vertigo and tinnitus [67]. It
was W. F. House who popularized the approach
as a reliable technique initially for the treatment
of otosclerosis and then for removing of small
VSs [60]. Currently the approach is utilized in
case of small tumors, in which there is an oppor-
tunity to preserve hearing, especially in those
located mainly laterally within the IAC [9, 46,
90, 93]. It is used rarely in other tumors, such as
small meningiomas located at the lip of the IAC.
The MF approach provides a direct exposure
of the IAC including its fundus from a superior
trajectory [7, 95, 104]. The facial nerve can be
exposed from its cisternal to its tympanic seg-
ment. Moreover, the facial and the cochlear
nerves can be identified early in the fundus region,
which facilitates safe tumor dissection. Many
authors assert that the location of the posterior
semicircular canal and of the vestibule may limit
the lateral exposure if the RS approach is used
and hearing is to be preserved [95]. The main
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_4, © Springer-Verlag Berlin Heidelberg 2013
4
advantage of the MF approach, according to these
authors, is that the lateral end of the IAC is
exposed adequately, which ensures removal of
the entire tumor [4, 11, 104]. With the MF
approach, however, the transverse crest may hin-
der the visualization of the inferior half of the
fundus—of the so-called inferior recesses [18,
24]. The approach has several additional
drawbacks: limited exposure of the CP angle,
especially of its lower portion, which restricts the
control of possible bleeding, and the potential
major morbidity related to the retraction of the
temporal lobe or to damage to the vein of Labbé
[7, 45, 46]. Furthermore, it is related to a risk of
injuring the facial nerve during opening of the
meatal dura and tumor removal because the nerve
is located in the anterosuperior part of the IAC,
sometimes immediately beneath the dura. Thus,
the surgeon must work around the nerve to
remove the tumor, increasing the risk of its dam-
age during the surgical maneuvers.
Standard MF Approach
The patient is placed supine on the operating
table, and the head is rotated so that the plane of
the temporal bone becomes parallel to the floor.
Alternatively, obese patients or those with comor-
bidities can be placed in lateral or modified
park-bench position. A lumbar drainage may be
inserted to relax the brain and enable safe tempo-
ral lobe elevation. The skin incision begins just in
front of the ear and is vertical, curved, inverted
115
116
“U,” or question mark shaped. Then, the temporal
bone is exposed, and a craniotomy is performed,
located generally one-third posterior and two-
thirds anterior to the external auditory meatus
[20]. Importantly, sufficient bone has to be
removed basally in order to acquire a flat plane of
view across the middle cranial fossa floor and
avoid excessive retraction of the temporal lobe.
Next, the dura is dissected and elevated from
the floor of middle cranial fossa in a posterior-
to- anterior direction until the arcuate eminence
and the greater petrosal nerve are identified
[97]. Thus, inadvertent injury of the greater
superficial petrosal nerve (GSPN), which lies in
the major petrosal groove and is covered by a
thin layer of periosteum, is avoided. Injury to
this nerve leads to “dry eye” syndrome due to
impaired lacrimation. In rare cases, though, the
nerve has to be sacrificed in order to prevent
traction injury to the geniculate ganglion [103].
The middle meningeal artery at the foramen
spinosum, the greater superficial petrosal nerve,
and the arcuate eminence (overlying the superior
semicircular canal) are identified. Meticulous
care should be taken to maintain the integrity of
the dura in order to avoid CSF leak or iatrogenic
temporal lobe injury. This might be more
difficult in the elderly who have a thin dura that
may be easily torn. A possible dehiscent genicu-
late ganglion has to be recognized early [7].
According to the findings from one study, in
15 % of the examined cases, all or part of the
geniculate ganglion and the genu of the facial
nerve were exposed in the floor of middle cra-
nial fossa [72]. Importantly, the petrous segment
of the carotid artery may lie in the floor of the
middle fossa deep to the greater petrosal nerve
without bony covering [73, 92]. The medial
limit of dural elevation is the trigeminal impres-
sion. An important step is to identify reliably
the superior petrous ridge, along which the
superior petrosal sinus lies.
The identification of IAC is traditionally based
on anatomical relationships of the middle cranial
fossa structures. With the technique described by
House [45], the GSPN is identified and followed
up to the facial hiatus and to the geniculate gan-
glion by progressively drilling the middle fossa.
4 Approaches to the Cerebellopontine Angle
The labyrinthine segment of the facial nerve is then
followed to the lateral end of IAC. This direct
approach to the facial nerve may be used to access
facial nerve tumors or for reconstruction of the
facial nerve [7, 11]. The technique of U. Fisch is
based on the relation of the superior semicircular
canal to the IAC [30]. A line is drawn over the long
axis of the arcuate eminence (lying over the supe-
rior semicircular canal), and a second line is drawn
60° to the first one. The IAC is located along the
second line. This technique is still applied in case
of complex temporal bone fractures. Garcia-Ibanez
[32] introduced the method of direct identification
of the IAC and applied it initially to perform ves-
tibular neurectomy. The angle between GSPN and
arcuate eminence is 120°, and the bisecting line
of the angle determines the position of IAC.
Advantages of this method are the lower risk of
facial nerve injury because the technique requires
less dissection along the nerve; secondly, the early
and more extensive drilling to locate the IAC
enhances the subsequent exposure of the tumor.
Whatever method is utilized, however, the surgeon
should account for the individual anatomical vari-
ability. Currently the neuronavigatory guidance, in
particular the fusion of bone-window CT and MRI
datasets, allows for direct demonstration of these
structures with high precision. Its usefulness is
especially high in case of absent normal anatomy,
for example, in reoperations or in case of extensive
bone destruction (Fig. 4.1).
The IAC, which lies 3–4 mm under the middle
cranial fossa floor, is unroofed using progres-
sively smaller diamond burrs, and its dura is
exposed from the porus to the fundus. The verti-
cal crest (Bill’s bar) and the entrance of the facial
nerve into the fallopian canal are the lateral limit
of the dissection. Drilling in the angle between
the GSPN and the IAC should be avoided because
of risk of injury to the underlying cochlea [103].
Brackmann [7] proposed to decompress the prox-
imal labyrinthine portion of the facial nerve for
approximately 3 mm to compensate for any
swelling that may be caused during dissection.
The dura is incised along the long axis of the
IAC and the cranial nerves are identified. The
VS, especially if it originates from the inferior
vestibular nerve, tends to displace the facial
Middle Fossa Approach
a b
c d
Fig. 4.1 (a, b) Navigation screenshots of the initial expo-
sure of the MCF base and of the IAC; (c, d) respective
intraoperative images, demonstrating the foramen
nerve superiorly. It lies frequently immediately
underneath the dura, between the tumor and the
surgeon. Tumor removal follows the general
principles: an initial debulking has to be done
in larger VSs, followed by dissection of the
capsule from the cranial nerves. Smaller VSs
are removed piecemeal or—more rarely—as
a single mass. The removal of the most lateral
tumor portion, especially if it extends inferior
the transverse crest, may require blind dis-
section with an angled curette [24]. Of utmost
importance is to preserve the blood supply to the
cochlea, which usually runs between the facial
and cochlear nerves. Some authors state that
117
spinosum, foramen ovale, trigeminal nerve (V3), and mid-
dle meningeal artery
all non-involved structures, such as the supe-
rior and inferior vestibular nerves, should not
be cut: their preservation may help to maintain
the cochlear blood supply [7]. The importance
of the medial-to-lateral dissection technique for
hearing preservation has been frequently high-
lighted. It avoids the risk of avulsion of the very
fragile fibers that enter the modiolus. Further, the
blood vessels when retracted laterally have some
elasticity from their medial origins but not from
their foraminal terminations.
The opened IAC has to be sealed off—either
with muscle or fat pieces—in order to prevent
CSF leak. The major surgical complications with
118
the MF approach are related to damage of the
temporal lobe (edema, ischemia, or hemorrhage
that may lead to functional deficit or seizures),
injury to the vein of Labbé, and sub/epidural
hematomas.
Extended MF Approach
The classical MF approach was modified by two
teams—in Germany and in Japan—and applied
successfully in case of VSs with a substantial
component in the CP angle [91, 109]. The major
difference to the standard MF approach is the
amount of petrous bone removal: with the classi-
cal technique, the IAC is just unroofed, while the
extended MF approach provides additional expo-
sure by extensive extradural removal from the
petrous apex anteriorly to the superior semicircu-
lar canal posteriorly [92, 107, 108]. With the
technique described by Wigand, the inner ear
structures are preserved [107]. The author
reported on his experience with this approach in
a series of 735 patients with VS growing more
than 2 cm into the CP angle [35]. Twenty-two
percent of the tumors were intracanalicular, 42 %
had less than 1-cm extracanalicular extension,
29 % had 1–1.9-cm extracanalicular extension,
and 7 % had larger than 2-cm extracanalicular
extension; complete VS removal was achieved in
97.1 %. Depending on the tumor size, normal and
near-normal facial nerve function ranged from 83
to 99 %. Hearing preservation rate at or near the
preoperative level was preserved in 60.2, 48.2,
23.9, and 17.6 %, respectively.
The extensive bone removal is key to expose
the tumor portion protruding into the CP angle.
At the porus, the exposure should be approxi-
mately three-quarters of the circumference of the
canal. It is not possible to achieve this degree of
exposure in the lateral portion of the IAC: the
bone removal is limited posteriorly by the supe-
rior semicircular canal, while the anteromedial
edge of the exposure is limited by the cochlea [70,
71]. Therefore, at the fundus the IAC is exposed
only 90° to avoid injury to these structures.
The extended MF approach is currently used
in VSs with less than 1-cm extension into the
4 Approaches to the Cerebellopontine Angle
CP angle. The upper size limit of the extracanali-
cular tumor portion for the extended MF approach
is stated to be even 1.6–2 cm. Its application in
larger tumors is rather controversial despite the
fact that some good results have been reported.
The temporal lobe has to be retracted for a longer
period, and the facial nerve may be more difficult
to dissect due to its location in front of the tumor.
Importantly, the access and the possibility to con-
trol the inferior aspect of the CP angle are also
limited [84].
Satar et al. [84] examined the issue, which
VSs may profit from the extended MF approach.
These authors evaluated the facial nerve func-
tion and hearing outcome in case of intracanali-
cular VSs, VSs extending 1–9 mm into the CP
angle extension, and VSs with 10–18-mm CP
angle extension. They found that the functional
outcome was similar in the first two groups:
functional hearing preservation rate was 62.2 and
63.1 %, respectively, and good FN outcome had
93.7 % vs. 97.6 %. For tumors of 10–18-mm CP
angle extension, however, the hearing preserva-
tion rate was lower (34 %). Patients with >10-
mm tumors should be advised that choosing the
MF approach for hearing preservation carries a
somewhat higher risk of persistent FN dysfunction,
concluded the authors.
The MF approach has been modified in order
to access lesions located in the anterior CP angle,
in the petroclival area and anterior brain stem [3,
22, 44, 48–50]. The initial operative steps are
similar to the described above. The superior pet-
rosal sinus, however, has to be ligated and cut.
Once this is performed and the key structures—
GSPN, foramen ovale, mandibular nerve, IAC,
and vestibulocochlear nerves—are identified, the
medial petrous bone lying within the Kawase
rhomboid is removed [49, 50]. It should be
remembered that the cochlea is located at the
posterolateral corner of this rhomboid. The limits
of the extended MF approach are the inferior pet-
rosal sinus inferiorly, the middle ear ossicles lat-
erally, the gasserian ganglion anteriorly, and the
superior semicircular canal posteriorly [103].
The Kawase approach is similar to the extended
MF approach with the same extent of medial
petrous bone removal. The main difference is the
Translabyrinthine Approach and Its Variations
additional transverse incision of the tentorium to
the incisura. Elevating the divided tentorial edges
provides wide exposure of the petroclival region
down to the midclivus level. Thus, the basilar
artery, the posterior cerebral artery, the superior
cerebellar artery, and the anterior inferior cere-
bellar artery, as well as the cranial nerves III to
VIII, are well visualized.
Translabyrinthine Approach
and Its Variations
The translabyrinthine approach (TL approach)
was refined and popularized by WF House and
WE Hitselberger in the early 1960s (see Chap. 1).
The approach is performed usually by a team of
neurotologist (translabyrinthine temporal bone
dissection) and neurosurgeon (microsurgical
tumor removal).
The TL approach is used widely for removal
of VSs when hearing preservation is not an issue.
In combination with other approaches, it is used
in various cranial base tumors [8, 10, 21, 56, 74].
Candidates for this approach are VSs of any size
with poor hearing, but it is most frequently
applied for those >2–2.5 cm [5, 83, 102].
Assuming that in tumors larger than 2.5 cm the
possibility of hearing preservation is “extremely
unlikely,” some authors state that this consider-
ation should not influence the choice of the
surgical approach [21, 111]. They recommend
the TL approach in patients with such tumors
even if they have good preoperative hearing—a
concept that is rather disputable, as discussed in
the section on large VSs operated via the retrosig-
moid route.
Advantages of the TL approach are shorter
distance between the surface and the neoplasm,
allowing the surgeon to work significantly closer
to the tumor; early identification of facial nerve
using a constant bony landmark (Bill’s bar); min-
imal, if any, cerebellar and brain stem retraction;
ready ability to perform a direct facial nerve
reconstruction, if necessary; low incidence of
postoperative headaches; and easy access to the
tumor bed in case of postoperative complications
[17, 29, 56, 106].
119
The size of the VSs is not a contraindication
for selecting this approach, and even tumors mea-
suring 4 cm or more can be exposed and removed
successfully [2, 21, 53]. As pointed by Day et al.
[21], when the bone removal is adequate at
superficial levels, the corridor of access is more
than sufficient regardless of tumor size. Although
some anatomical variations, such as a low-lying
tegmen, anterior sigmoid sinus, or high jugular
bulb may complicate the removal of large tumors,
various techniques to overcome these obstacles
have been described [31].
Operative Technique
Patients are usually positioned supine with the
head turned opposite the side of the lesion. Rarely,
the lateral position is preferred. A retroauricular
curved skin incision is made, extending approxi-
mately 1 cm behind the body of the mastoid. The
incision starts above the pinna and extends inferi-
orly to the mastoid tip. The temporal fascia and
pericranium are incised and elevated as a flap.
Mastoidectomy
After the mastoid is exposed, the spine of Henle,
the external auditory meatus, the mastoid ridge,
the supramastoid crest, and the mastoid tip are
identified [71, 100]. The cortical bone of the mas-
toid is drilled off and the underlying air cells are
exposed. The mastoid antrum lies approximately
15 mm deep to the surface below the spine of
Henle. Once the antrum is opened, the bony
semicircular canals are identified. Sufficient bone
at the margins of the mastoidectomy has to be
removed not to restrict the exposure of the deep
structures [21, 53].
Then, the sigmoid sinus, the middle fossa dura,
and the upper surface of the jugular bulb are skel-
etonized. The facial nerve must be identified early
in the procedure: it lies below the lateral semicir-
cular canal. The inferior limit of the approach is
the jugular bulb. Anteriorly only a thin layer of
bone should be left at the posterior aspect of the
external auditory canal. This bone layer and the
120
mastoid segment of the facial nerve form the
anterior limit of the exposure. Posteriorly the
bone overlying the sigmoid sinus is completely
exposed. In order to enhance the exposure of large
tumors, the bone removal should go beyond the
sinus, allowing its slight retraction [17].
Labyrinthectomy
The labyrinthectomy is then performed, which
includes removing of the semicircular canals and
vestibule to expose the dura of the IAC [41]. The
posterior, superior, and inferior walls of the IAC
(two-thirds of its circumference) must be
removed. The canal is opened widely until the
transverse and vertical crests (Bill’s bar) are
exposed to help in locating the facial nerve later-
ally. The cochlear aqueduct is the medial
landmark of the inferior bone removal. In order
to obtain adequate inferior exposure in case of
high jugular bulb, the bulb should be skeleton-
ized and the entire inferior lip of the IAC removed.
Inferior exposure is obtained by skeletonizing the
posterior aspect of the jugular bulb [53, 71].
As outlined by A. Rhoton [71], the two main
goals are exposure of the dura of Trautman’s tri-
angle on the posterior surface of the temporal
bone facing the CP angle and removal of enough
bone to identify the nerves lateral to the tumor in
the fundus area. The dura lining the IAC and the
dura of the Trautman’s triangle are opened, con-
sidering the possibility to come upon the subarcu-
ate artery or the AICA in the dura of the triangle.
The superior vestibular nerve is the first structure
encountered when the IAC is opened due to its
location posterosuperiorly within the canal, which
decreases the risk of injury to the facial nerve.
As with other approaches, once the critical
structures are identified, tumor debulking is per-
formed; then, its capsule is dissected from the
neural structures in the arachnoid plane. In case
the anatomical integrity of facial nerve is lost
during surgery, the exposed proximal and distal
segments facilitate its repair: either with a direct
anastomosis or with a cable graft. Following
tumor removal and hemostasis, the dura is closed
and fat is packed into the mastoid. Packing with
muscle, fat, or Tabotamp® (Johnson&Johnson
4 Approaches to the Cerebellopontine Angle
Medical) to occlude the Eustachian tube and
antrum decreases the risk of CSF leaks.
Surgical Outcome
The most frequent surgical complication related
to the TL approach is CSF leak. Its rate ranges
between 1.4 and 20 % and is higher than with the
alternative approaches [14, 15, 58, 75, 82]. The
risk is related to tumor size: in larger tumors it is
more than 15 % [53, 56]. The reported rates of
meningitis range from 1.6 to 4 % [53, 56, 75].
Other complications include arterial injuries (of
the AICA or of the internal auditory artery), injury
to veins or sinuses, postoperative hemorrhages,
cerebellar edema, and pneumocephalus. The rate
of complete tumor removal in experienced hands
is >95 %, and the recurrence rate is very low: less
than 2 % [53, 56, 83, 89]. The rate of facial nerve
preservation with the TL approach is 88–94 %
[14, 41, 53, 96, 99, 101]. In intracanalicular
tumors, the rate is 97–100 %; in VS <2 cm, 92.5–
95 %; and in VS >2 cm, 63 % [10, 13, 53].
The refinement of the TL technique allows
achieving very good results even in large VSs.
Briggs et al. [14] presented the outcome of treat-
ment of 167 patients with VS >4 cm: complete
removal was achieved in 95 %, and the mortality
rate was 0 %. The rate of anatomical facial nerve
preservation was 91 %. With an approximate 2
years follow-up, 42 % of the patients had HB
grade I–II function. The authors stressed the
significance of wide bone exposure in case of
large tumors. Thus, removal of the part of the tem-
poral squama allows extending the rostro-caudal
working angle. The complete jugular bulb skele-
tonization increases the access to the caudal tumor
part. Lanman et al. [53] evaluated the outcome of
TL removal of such VSs in the same clinic in a
later period. Increased experience allowed increas-
ing the rates of complete tumor removal to 96.3 %
and of anatomical facial nerve preservation to
93.7 %. The facial nerve functional outcome was
also improved: 1 year after discharge, excellent
function (House–Brackmann grade I or II) was
present in 52.6 % of the patients. This outcome
was possible with a relatively low morbidity rate:
CSF leakage rate was 14 % but required surgical
Combined Approaches
repair only in 1.1 %; eight patients had ninth cra-
nial nerve deficit, seven had tenth cranial nerve
deficit, and seven showed diplopia secondary to
either fourth or sixth cranial nerve palsies. In the
series there were no mortalities.
The main disadvantages of the approach are
that it sacrifices hearing, restricts the exposure, in
particular the access to the caudal tumor portion
and the tumor/brain stem interface, and is related
to a higher CSF leak rate [12, 17]. Further, the
facial nerve is at risk of injury during the exten-
sive petrosal resection. The venous sinuses may
be injured during bone drilling. The wide expo-
sure and manipulation of these sinuses may cause
late thrombosis. Roche et al. [74] pointed out
that the rate of complications related to sinuses
injury has never been specifically addressed
for this approach. In one study, however, 143
patients operated via the presigmoid transpetro-
sal approach were evaluated [64]: seven patients
(5 %) had sigmoid sinus occlusion due to direct
sinus injury or compression by bone particles.
Retrolabyrinthine Approach
Compared to the TL, this approach requires less
bone removal, provides a possibility to preserve
hearing, and is related to a lower risk of injury to
the facial nerve. The approach provides good
exposure of the IAC and of the posterior CP angle
but a limited one to the anterior CP angle and to
the petroclival area [19]. The initial part—mas-
toidectomy—is performed as described in the TL
approach section. The three semicircular canals
are skeletonized but preserved. In case the sigmoid
sinus is located more anteriorly or is larger than
normal, the access to the CP angle may be restricted
[103]. Bento et al. [6] pointed that the approach
allows for high rate of facial nerve preservation. In
case the exposure is insufficient, it can be extended
to a complete translabyrinthine approach [27].
Partial Translabyrinthine Approach
This approach increases the exposure to CP angle
and petroclival area, if compared to the retrolaby-
rinthine approach. Hearing can be preserved in
121
most instances [103]. The approach includes a
meticulous drilling of either the superior or the
posterior semicircular canal, depending on the
tumor location. The transmastoid transpetrous
approach with partial labyrinthectomy is used in
small VSs with functional hearing preoperatively
[37, 87].
Transcochlear Approach
The transcochlear approach is primarily an anter-
omedial extension of the translabyrinthine
approach [8, 42, 43, 71, 103]. It requires division
of the GSPN, transposition of the facial nerve,
and resection of the cochlea. The initial part of
the dissection is the same as for the translabyrin-
thine approach, except that the external auditory
canal is closed and the tympanic membrane and
ossicles are removed [71].
The facial nerve is skeletonized completely
from its geniculate genu to the stylomastoid fora-
men. The GSPN is transected, and the facial
nerve is transposed posteriorly, which allows for
further bone drilling through the facial canal and
the cochlea to the petrous apex. The bone removal
extends to the edge of the clivus medially, expos-
ing the dura between the inferior and superior
petrosal sinuses. The wide bony opening creates
direct access to the anterior CP angle, the middle
third of clivus, and the anterior brain stem and
allows visualizing the abducens nerve, the lower
margin of the trigeminal nerve, the nerves enter-
ing the jugular foramen, as well as a part of the
basilar artery and of the AICA [71].
Combined Approaches
Combined Translabyrinthine–
Retrosigmoid Approach
The TL approach may also be combined with
a retrosigmoid or with a supra- and infratento-
rial presigmoid approach. Glasscock et al. [36]
used four different approaches in 616 cases,
selecting each one on the basis of tumor size
and hearing level. They recommended the com-
bined translabyrinthine–suboccipital approach
122
for tumors larger than 3 cm. H. E. Maddox [55]
used a lateral approach, which is a combination
of the translabyrinthine and retrosigmoid routes
in a series of 106 consecutive cases with facial
nerve preservation rate of 88 %. Anderson et al.
[1] recommend the use of a combined translab-
yrinthine–retrosigmoid approach in very large
tumors. The translabyrinthine exposure allowed
for early identification of the facial nerve distally,
while the exposure posterior to the sigmoid sinus
was found to be especially beneficial in case of
abnormally high jugular bulb that restricts the
vertical exposure.
Translabyrinthine–Transtentorial
Approach
This technique was introduced by Morrison and
King in 1973 [62] and has the advantage of a bet-
ter rostral tumor exposure, which is achieved by
ligating the superior petrosal sinus and incision
of the tentorium. The authors achieved complete
VS removal in 91.7 % and preservation of the
anatomical integrity of the facial nerve in 80.8 %.
The approach-related morbidity, however, was
unacceptably high and included aphasia, epilep-
tic seizures, and CSF leaks [51]. Sluyter et al.
[94] used this combined approach in a series of
120 patients with VS <2 cm and achieved com-
plete tumor removal in 91.7 % and anatomical
facial nerve preservation in 80.8 % of the cases.
They could reduce the morbidity rate to accept-
able levels, with the exception of the relatively
high rate of patients with postoperative epilepsy
that requires medical management—3.3 %. Tator
and Nedzelski [99] found that the combined
middle fossa translabyrinthine–transtentorial
approach provides very clear visualization of the
CP angle and is especially suitable for large
tumors with anterior extensions.
The transpetrosal retrosigmoid approach
is used in tumors larger than 4 cm that extend
superiorly, well into the tentorial notch [37, 81,
87]. The wide exposure affords an anterolateral
access to the lateral brain stem surface from
the jugular foramen to the trochlear nerve and
multiple working angles. The transmastoid
4 Approaches to the Cerebellopontine Angle
transpetrosal partial labyrinthectomy approach
is another combined technique, promoted by
Sekhar et al. [87].
The use of two different approaches, however,
does not only provide operative advantages; the
risks of approach-related morbidity are also
essentially higher, and such combined techniques
are rarely necessary.
Retrosigmoid Approach
Benefits of the Approach
The retrosigmoid approach (RS approach) is a
safe, elegant, and relatively simple technique.
With small modifications, described in the corre-
sponding chapters of this book, it provides access
to all four levels of the CP angle and their related
structures:
1. Petroclival area, Meckel’s cave, and part of the
middle cranial fossa
2. Internal auditory canal
3. Posterior part of the petrous bone
4. Jugular foramen
5. Craniocervical junction
The technique is related to very low procedure-
related morbidity rate and therefore is among the
most frequently utilized operative routes in neu-
rosurgery [26, 65, 70, 110]. It is a hearing-pre-
serving technique that offers an excellent
panoramic visualization to the whole CP angle
and a wide exposure of the tumor, whatever its
size and pathological type. The dissection at all
stages is performed under direct visual control,
and the cranial nerves can be identified early both
in their proximal and distal ends, thus increasing
the chances for their preservation. If the RS
approach is combined with an intradural resection
of the petrous apex with/without incision of the
tentorium, petroclival lesions can be adequately
exposed [78]. This retrosigmoid suprameatal
approach (RSMA), which was developed and
introduced by the senior author in 1982, provides
access to meningiomas of the Meckel’s cave, the
petroclival area, and even the posterior cavernous
sinus [16, 80]. It evades the risks related to alterna-
tive approaches, such as extensive petrous bone
Anesthetic Considerations
resection or retraction of the temporal lobe with
the associated risks of damage to neural and vas-
cular structures. Wide access to the IAC—if
needed—can be gained by drilling of its poste-
rior wall. Lesions involving the inframeatal area
of the petrous bone—inferior and/or anterome-
dial to the IAC—may be accessed via the so-
called retrosigmoid inframeatal approach. This
technique was elaborated by the senior author
and utilized for the first time in 2004. The
approach provides access to the area inferior and
anteromedial to the internal auditory canal, the
infralabyrinthine region, and the petrous apex
and has been used successfully for removal of
chondrosarcoma, cholesterol granuloma, and
chordoma (see Chap. 11, section “Temporal Bone
and Petrous Apex Lesions”). The RS approach
provides excellent access to the jugular foramen
area: lesions extending into the canal can be
accessed by removing its dorsal wall (see the
corresponding chapter). Tumors extending to the
foramen magnum level can be removed via a ret-
rosigmoid craniotomy and a C1 hemilaminectomy/
laminectomy (see the section “Foramen Magnum
Meningiomas”) [79].
Patient Positioning and Anesthesia
Considerations
The position of the patient—semi-sitting, prone,
three-quarter lateral (or park bench), lateral-
oblique, supine-oblique, concorde, or supine with
the head rotated 90° to the contralateral side—is
a matter of surgeons’ experience and institutional
tradition. With the supine position, the head is
turned to the contralateral side and a pillow
(sandbag or bolster) is placed under the ipsilat-
eral shoulder to rotate the shoulders and chest
into a semilateral position. The advantages of
this position are that it is simple, fast, and not
related to major complications. Besides the risks
to the cervical spine, however, the rotation of the
head can lead to venous outflow impediment with
resultant increased intracranial pressure and
parenchymal bleeding. Alternatively, the patient
can be positioned in the three-quarter lateral posi-
tion, which does not endanger the cervical spine
123
or venous drainage. Compression or traction
injury to the peripheral nerves should be
avoided.
Having experience with all patient positions,
we favor the semi-sitting one (Fig. 4.2). Its major
advantages are:
• The spontaneous outflow of CSF, blood, and
fluids used for irrigation is facilitated by
gravity.
• There is no need of constant suction to keep
the operative field clear, and the surgeon can
work bimanually.
• The continuous irrigation of the operative field
performed by the assistant obviates the need
for frequent coagulation during tumor
removal.
• The surgeon can easily change his line of
sight, for example, to the brain stem or to the
IAC, by slightly changing his position without
the need of rotation of the table from side to
side.
• Importantly, there is less engorgement of the
cerebellum, the venous outflow is promoted,
and the venous bleeding is less.
Anesthetic Considerations
In all patients operated in the semi-sitting posi-
tion, central venous line is required to monitor
the central venous pressure, as well as to apply
catecholamines or hyperosmolar fluids, if needed.
A slightly elevated central venous pressure with
normo- or slight hypervolemia is aimed. Almost
all surgeries in the semi-sitting position are per-
formed with a positive PEEP. Important fact to
consider, however, is that the PEEP does not
decrease the risk of venous air embolism. The
incidence of air embolism is similar in patients
operated with a PEEP of 10 cm H20 and with
PEEP of 3 cm H20 [33]. The tip of the central
venous line has to be positioned exactly at the
transition between the superior caval vein and the
right atrium, which allows aspiration of air if
venous air embolism occurs. Arterial line is
required to measure the blood pressure invasively
and for blood gas analysis. Patients are monitored
throughout the surgery with precordial Doppler.
124 4 Approaches to the Cerebellopontine Angle

a b

c d

Fig. 4.2 The semi-sitting position: the patients’ head is extended, flexed, and slightly rotated to the contralateral side.
The right leg of the first patient (e) is prepared for sural nerve harvesting
Anesthetic Considerations 125

e f

i
j

Fig. 4.2 (continued)

126
Transesophageal echocardiography is not applied
routinely in our department.
The Semi-sitting Position
The semi-sitting position differs from the sitting
one by the smaller angle of flexion of the operat-
ing table (Fig. 4.2). The operating table is flexed,
and the legs of the patient are elevated to the level
of the right atrium of the heart, which elevates the
venous pressure and decreases the risk of venous
air embolism. The head is immobilized with a
three-point head fixation frame. Under the con-
tinuous feedback control from the SSEP mea-
surement, the head is slightly extended, flexed,
and rotated to the involved side (Fig. 4.2). The
degree of rotation depends on the location of
the tumor. In case of pure intracanalicular VS,
the rotation is approximately 30° because the tra-
jectory is from medial to lateral; in tumors with
larger extracanalicular portion, it should be >30°
to allow adequate visualization of the brain stem.
Hyperflexion that can lead to occlusion of the
jugular venous outflow, airway obstruction, or
complications related to the cervical spine should
be avoided: the distance between the manubrium
and the chin should be at least two fingerbreadths.
In case of deterioration of the SSEP waveform,
the head should be repositioned and the degree of
its flexion should be reduced. In the literature
some cases of postoperative quadriplegy or quad-
riparesis in patients operated in the semi-sitting
position have been reported [61]. In order to
avoid such outcome, we perform routinely in all
patients cervical spine X-ray in flexion and exten-
sion before surgery to detect potential instability
and/or severe degenerative spine disease. In
patients with NF-2 cervical spine, MRI should
also be done to exclude the presence of tumors,
compressing the spinal cord. If these examina-
tions prove that a patient is at risk, the head
flexion should be less.
An acute drop in systolic blood pressure of
more than 20 mmHg may occur after position-
ing but is easy to manage by increasing the rate
of intravenous infusion or by the administra-
tion of 1-mg adrenalin or noradrenalin [112].
4 Approaches to the Cerebellopontine Angle
Rarely, in some elderly or compromised patients,
systemic hypotension may be more difficult
to control. Care is taken to protect all pressure
points in order to avoid peripheral nerve pal-
sies. Antithrombotic stockings or an intermittent
pneumatic compression device is used to prevent
deep vein thrombosis.
Some authors defined certain contraindications
for the semi-sitting position, such as age, arterial
hypertension, obstructive lung disease, and diag-
nosed persistent foramen ovale [69]. The issue if
these conditions should be regarded as a relative
or absolute contraindication is related to the expe-
rience of the team—the anesthesiological and the
surgical ones. An assessment of the benefit–risk
ratio should be made in each individual case.
The risks related to the semi-sitting position are
venous air embolism, paradoxical air embolism,
tension pneumocephalus, circulatory instability,
and cervical spine injury, which may cause even
quadriplegia. In the literature the sitting and semi-
sitting positions are frequently discussed together.
Many of the risks related to the sitting position,
however, are avoided in the semi-sitting one.
Venous air embolism occurs when an open
venous structure draws in air due to the negative
venous pressure gradient between the operative
site and the right atrium. It has been reported to
occur in 10–45 %, even in 100 %, of the patients
depending on the sensitivity of the monitor-
ing device [57, 66]. Venous air embolism can
lead to severe cardiopulmonary complications.
However, if the anesthetic and operative prin-
ciples are followed strictly, such complications
can be avoided. In experienced hands the venous
air embolism is not related to any lasting mor-
bidity. Transesophageal echocardiography is the
most specific and sensitive method in detection
of such embolism. An alternative method is the
continuous monitoring of the pulmonary artery
pressure. Our experience has shown that the
combined monitoring of end-tidal carbon dioxide
and precordial Doppler echocardiography yields
similar results, and we rely exclusively on the
combined use of these two techniques. An accu-
rately placed precordial Doppler is so sensitive
that it can detect the clinically irrelevant volume
of 0.015 ml air/kg/min [34].
Anesthetic Considerations
In patients with persistent foramen ovale,
there is certain risk of paradoxical embolism in
the arterial system that could cause cerebral and/
or heart ischemia [28]. The condition is diag-
nosed in 25–30 % of the normal population [39,
59]. Persistent foramen ovale is demonstrated
directly using transthoracic echocardiography or
Color Doppler study, which shows the flow across
the atrial septum. Transesophageal echocardiog-
raphy is preferred in case the atrial septum is
inadequately visualized by transthoracic echocar-
diography. A strong relationship between
persistent foramen ovale and clinically significant
paradoxical air embolism, however, has not been
established [28]. In the experience of the senior
author with approximately 10,000 cases, oper-
ated in the semi-sitting position, major lasting
complications related to paradoxical air embo-
lism have never been observed. Preoperative
examinations for persistent foramen ovale are
therefore warranted only in children or in patients
with suspicious clinical history. In case an opened
foramen is diagnosed, the decision how to per-
form the surgery should be made considering
carefully the potential benefits and risks of the
semi-sitting position and discussing the pros and
cons with the anesthesiological team. During the
last 10 years, we selected the supine position just
in one child with a significant right-to-left shunt,
while all other cases were operated in the semi-
sitting position. Lasting complications due to
venous air embolism have not been registered.
Other authors have also shown that the morbidity
due to venous air embolism is similar in both the
semi-sitting and supine positions [25]. Zeilstra
et al. [112] studied a group of 108 patients and
recorded 23 instances of venous air embolism in
20 patients. It did not lead, however, to any seri-
ous hemodynamic changes. Duke et al. [25]
reviewed 432 consecutive operations: 222 per-
formed with the patients in the sitting position
and 210 with the patients in the supine position.
The incidence of venous air embolism was 28 %
in the first group and 5 % in the second group.
Their analysis showed that although the inci-
dence of detected venous air embolism is higher
in sitting patients, the morbidity is not statisti-
cally greater.
127
Venous air embolism should be detected early,
and immediate measures should be taken to detect
and occlude its source/s. The most common
sources of major venous air embolism are the
transverse and sigmoid sinuses and the jugular
bulb, which—due to their dural attachments—do
not collapse if opened. Air entry may also occur
via emissary veins and diploic veins of the occip-
ital bone. Veins in the posterior cervical region
are generally small and collapse easily; still, they
can also be the source of air entry. The risk of air
embolism is not confined to the initial part of sur-
gery. It can develop even during tumor removal
and—besides the aforementioned causes—can
be caused by an undetected rupture of a bridging
vein.
If a change in the Doppler signal is noticed,
especially in case a concomitant decrease in end-
tidal carbon dioxide is observed, air embolism
should be suspected. A direct confirmation is the
aspiration of air bubbles through the central
venous catheter. Nonsurgical causes of air embo-
lism, such as untight connections or incidental air
infusion, should be excluded. Ventilation with a
FiO2 of 1.0 is recommended to prevent a poten-
tial hypoxemia. If nitrous oxide is used, it should
be stopped. Venous air embolism can cause acute
strain to the right heart and acute right heart fail-
ure. Later on, left heart failure may follow. Volume
therapy or catecholamines application may be
required to keep an adequate systemic and coro-
nary perfusion pressure. Aspiration of air via the
central venous catheter might be of help.
The surgeon has to be informed immediately at
first signs of venous air embolism and both jugu-
lar veins should be compressed manually. This
stops further air entry and helps the surgeon to
identify its entry point. If the patient is in the
semi-sitting position, a venous lesion, even a tear
of the sinus, may not cause obvious bleeding. The
assistant should continuously irrigate the surgical
field. In case a vein in the soft tissues is bleeding,
it can be coagulated; in case of bleeding from
emissary veins, bone wax is applied. If the origin
of bleeding is a tear in the sinuses, a small piece of
sponge, for example, Gelita (Johnson & Johnson
Medical), is used to seal it. In the rare case the
source remains unclear, the craniotomy area is
128
covered temporarily with multiple wet sponges,
and a wet gauze is laid. In case of continuous air
entry that causes hemodynamic problems, the
upper part of the surgical table has to be lowered
and the end-expiratory pressure increased. The
surgery is continued when the end-tidal carbon
dioxide stops to fall because the complete recov-
ery of this value is a slower process [28]. The
degree of blood gas abnormality dictates the need
of postoperative ventilator support.
Standard Instrument Sets Used
in All CP Angle Surgeries
The standard set of microinstruments used for the
intradural part of the retrosigmoid tumor removal
is presented on Fig. 4.3. During VS surgeries
there is seldom need of additional instruments. In
case the endoscope-assisted microsurgical tech-
nique is implemented, however, some other
instruments are required, for example, angled
forceps or suction tips.
Skin Incision and Craniotomy
Different skin incisions have been proposed: lin-
ear vertical incision, inverted J-shaped incision,
lazy S-shaped incision, and semi-curved or
C-shaped incision (Fig. 4.4). Principally, the inci-
sion should be designed so that it allows adequate
bone exposure and does not restrict the viewing
line and access to all parts of the CP angle.
Optimizing wound healing and obtaining good
cosmetic result are further considerations.
In case of CP angle tumors, we perform a
slightly curved skin incision, 2.5–3.5 cm behind
the mastoid (Fig. 4.4b). The inferior part of the
incision should be slightly curved laterally to
allow approach to the mastoid tip, which is
essential to allow for an unrestricted access by
the soft tissues to the cerebellomedullary cis-
tern and lower part of the CP angle (the lateral-
to-medial perspective). The length and position
of the incision in relation to the mastoid should
be adapted to the patient’s individual anatomic
4 Approaches to the Cerebellopontine Angle
characteristics. In patients with more bulky
subcutaneous soft tissue, the incision line
should be more medial to not obstruct the
mediolateral optical view of the surgeon to the
fundus of IAC. The superficial muscle layer
(composed of the trapezius and sternocleido-
mastoid muscles) and the intermediate muscle
layer (splenius capitis, longissimus capitis, and
semispinalis capitis muscles) of the lateral neck
are cut in line with the incision and carefully
separated from their attachments to the bone
with subperiosteal dissection. The suboccipital
muscles and the soft tissues between the occiput,
the posteromedial end of the mastoid, and the
posterior arch of the atlas should be dissected
carefully in order to avoid injury to the underly-
ing vertebral artery.
The asterion is the converging point of the
lambdoid, parietomastoid, and occipitomastoid
sutures. This landmark overlies the junction of
the transverse and sigmoid sinuses and tradition-
ally guided the placement of the initial burr hole.
It has been recommended to place the burr hole
behind the transverse–sigmoid junction at the
asterion or below it [52, 70]. Some recent stud-
ies, however, have shown that the asterion is not
absolutely a reliable anatomic landmark and is
variable both in the cranial–caudal plane and in
the anterior–posterior plane [23]. The superior
nuchal line has been recommended as a guide for
the location of the transverse sinus but is also
variable. The course of the sigmoid sinus is less
variable: it descends along an axis defined by the
mastoid tip and the squamosal–parietomastoid
suture junction or over the mastoid groove. We
prefer to place the burr hole 2–2.5 cm below the
superior nuchal line, two-thirds behind and one-
third in front of the occipitomastoid suture. In
case an emissary vein is seen in the area or sus-
pected on the bone-window CT images, the
placement of the burr hole should be modified
accordingly in order to avoid injury to the vein.
The surgeon should be aware that less venous
bleeding during the incision and craniotomy, as
well as a slack dura, could be related to a low
venous blood pressure, respectively, to a low
intracranial pressure, which increases the risk of
Anesthetic Considerations 129

a h

b k

c l

m
d

e
o

f p

q
g

Fig. 4.3 (a) Standard microinstrument set (Aeskulap sizes. (h) Microscissor. (i) Angled microhook, large size.
AG). (b–r) Close view of these microinstruments. Their (j) Angled microhook, small size. (k) Microdissectors.
use is described in the text. (b) Bayonet forceps. (c) (l) Platelet knives of various sizes. (p–r) Angled micro-
Bayonet forceps (sharp). (d–g) Tumor forceps of various hook of various sizes

venous air embolism. The anesthesiologist should
be informed and adequate volume therapy initi-
ated or the PEEP should be elevated.
The issue of whether to make a craniectomy
or craniotomy is a matter of debate. Our experi-
ence has shown that the craniotomy might be a
more dangerous procedure due to the related
high risk of injury to underlying sinuses and
tearing of the emissary vein or dura by the cran-
iotome. Therefore, we avoid making one-piece
130
a b
Fig. 4.4 (a) Lazy S skin incision (the initial operation
has been performed elsewhere): the access to the lateral
cerebellomedullary cistern and the lateral-to-medial
craniotomy in the RS approach, although we
performed it earlier. The bone is removed step-
wise with bone rongeurs and/or high-speed drill
after the underlying dura has been detached from
the bone.
The exposure of the sigmoid sinus may require
opening of mastoid air cells (Fig. 4.5).
Nevertheless, the bone removal should proceed
to the sinus edge. The opened air cells are sealed
off with fat pieces and fibrin glue at the end of
surgery.
The location of the emissary vein/veins should
be studied on the thin-sliced bone-window CT
(Fig. 4.6). Excessive traction to the vein during
the craniectomy could cause its rupture from the
sinus and sinus laceration. The vein should be
hence skeletonized with a diamond drill along
their length until it is free of any bony encase-
ment and can be safely coagulated close to their
entrance in the sinus.
The dura overlying the sigmoid sinus is fre-
quently very fragile; in order to preserve the last,
millimeters of overlying bone are removed with a
diamond drill. Extensive exposure of the trans-
verse and sigmoid sinuses is unnecessary and
might lead to sinus laceration or desiccation, with
the risk of subsequent thrombosis: just the edges
4 Approaches to the Cerebellopontine Angle
viewing angle is limited by the overlying soft tissues.
(b) C-shaped incision that we favor
of the sinuses, approximately 2–3 mm, have to be
exposed (Fig. 4.7).
The size of the tumor does not generally
dictate the size of craniotomy: the edges of the
sinuses should be exposed always, and the bone
opening has to extend caudally to the floor (the
horizontal plane) of the posterior fossa
(Fig. 4.8). This affords direct access to the lat-
eral cerebellomedullary cistern without the
need of excessive cerebellar retraction. Further,
the dural incision can be extended basally to
allow continuous egress of CSF throughout the
surgery. Small tumors of the petrous apex are
an exception. They are approached via a supe-
rolateral route (the cerebellum is retracted inf-
ero-medially) and, therefore, the extent of
inferior bone opening may be less (see section
“Petroclival Meningiomas”). The medial extent
of the bone opening should be large enough to
provide access to the lateral part of IAC (the
angle of access to the fundus) even in small
intracanalicular VSs. In case of pure CP angle
tumors, the foramen magnum should not be
exposed. In craniocervical tumors or CP angle
tumors with such caudal extension, however, it
might be necessary to open the foramen
magnum.
Anesthetic Considerations
a b
Fig. 4.5 Bone-window CTs, demonstrating the medial
extension of the mastoid air cell system. Still, the cells
have to be opened, and sufficient bone should be removed
to expose the sigmoid sinus. The extent of petrous bone
Dura Incision
The dura is opened under the operating micro-
scope. Once again, a large variety of dural inci-
sions have been proposed: vertical, curvilinear,
or stellate, with or without additional relaxing
incisions or division of the dural flap into triangles.
The curvilinear dural incision favored by us is
placed approximately 1.5–2 mm medial to the sig-
moid and inferior to the transverse sinus (Fig. 4.9a).
It allows for unrestricted access to the CP angle
and IAC and—importantly—for a primarily water-
tight closure. Auxiliary dural incisions are not nec-
essary. Thus, the need of dural reconstruction can
be avoided in almost all cases. If the dura is incised
131
pneumatization has been found to correlate with the risk
of postoperative CSF leak [96]. Therefore, the occlusion
of the opened air cells with fat pieces and fibrin glue in
such cases should be especially meticulous
too close to the sinuses, its suturing may be more
difficult. On the contrary, if it is incised far from
the sinuses, the abundant dura restricts the over-
view and the access to the CP angle. The medial
dural flap is left as a protective layer on the cere-
bellum, while the lateral dural part is retracted
with two stay sutures—one is placed in the area of
the sinus knee and another in the mid-sigmoid
sinus level (Fig. 4.9b). The slight traction of these
sutures and of the dura enlarges additionally the
CP angle exposure. Importantly, the traction does
not lead to venous stasis because the overlying
bone protects the sinuses. A wide brain spatula is
used to elevate slightly the lower lateral cerebel-
lum (Fig. 4.9c) and to visualize the lateral cerebel-
132
a b
c d
Fig. 4.6 Bone-window CT in four patients, demonstrat-
ing the emissary veins (arrow). The emissary veins are
highly variable in size, location, and number. Some small
lomedullary cistern. The arachnoid of the cistern is
opened as much as necessary to allow sufficient
amount of CSF to be drained until the cerebellum
relaxes away from the petrous bone. Although
some authors recommend inserting the tip of a
small catheter or cottonoid in the cistern in order to
ensure continuous CSF outflow throughout the
procedure [65], in the semi-sitting position, this is
not necessary. Care should be taken to recognize
bleeding from the supracerebellar bridging veins
that may tear during CSF draining, especially in
patients with slightly atrophic brain. A quadran-
gular flat cottonoid is then laid over the cerebel-
lum, and a self-retaining retractor is placed
that should support and protect the cerebellar
hemisphere, instead of compressing it. An
attempt should be made to preserve the major
4 Approaches to the Cerebellopontine Angle
veins are not visible on the CT scan but may be recog-
nized at surgery
bridging veins and prevent any venous outflow
disturbance.
Microsurgical dissection proceeds as the
lesion type and location dictate and is discussed
in the corresponding chapters of this book.
Hemostasis, dural closure and mastoid
occlusion
During tumor removal, brisk bleeding may occur;
the bleeding vessel should be exposed and
coagulated. In case of diffuse bleeding, however,
coagulation would not be helpful. Moreover,
uncontrolled coagulation must be avoided to
prevent thermal injury to the vulnerable neural
structures. The irrigation performed by the
Anesthetic Considerations
a b
Fig. 4.7 Exposure of the sigmoid (SS) and transverse
sinuses (TS). The preoperative bone-window CT is
presented on a (arrow: sigmoid sinus). (b) Intraoperative
image, showing the standard retrosigmoid bone opening
with exposure of 2–3 mm of the transverse (TS) and sig-
moid sinuses (SS). (c) The skull has been reconstructed
assistant clears the operative field and allows
continuing the dissection. Final hemostasis
should be done only following tumor removal
and reliable identification of all neural structures.
If the source of bleeding is a vessel along or too
close to the nerves or to the brain stem, coagula-
tion is prohibited. A piece of Tabotamp ® is put
onto the bleeding point and compressed gently
133
with methyl methacrylate (Palacos ®). (d) Postoperative
CT, demonstrating the extent of the bony removal (arrow-
heads), reconstructed with Palacos. The long arrow points
at the sigmoid sinus, located in a bony groove. Note that
only a small part of this bone covering has been removed
and the sinus remained protected throughout the procedure
with a cottonoid as long as necessary to control
the bleeding. If this is unsuccessful, the vessel
should be dissected and mobilized from the nerve
and only then coagulated at a very low current.
Blood clothes that have been accumulated in the
basal cisterns should be meticulously removed
after tumor removal because they may hide an
active bleeding point.
134
Fig. 4.8 Retrosigmoid craniotomy in a patient with a
small VS. The edges of the transverse and sigmoid sinuses
are exposed. Note the widely opened mastoid air cells
The lower intracranial pressure and lack of
venous stasis are beneficial factors for surgery
with the patient in the semi-sitting position.
However, they increase the risk of venous bleed-
ing when the patient resumes his normal posi-
tion in the bed. The source of such bleeding
typically is undetected ruptured and collapsed
bridging vein/s. The elevation of venous pres-
sure, most easily by performing jugular veins
compression, readily identifies such veins. The
jugular veins compression is performed while
the retractor is still in place and a second time
after its removal.
Two adapting dural sutures are placed and the
dura is closed with a continuous suture. In case
watertight closure is not possible or the dura is
extremely thinned, fat pieces and fibrin glue are
additionally placed.
The opened mastoid air cells have to be
occluded to prevent a CSF leak (if a defect in
the dura exists) and to prevent air collecting in the
4 Approaches to the Cerebellopontine Angle
epidural space. Use of bone wax is not advisable.
The wax may cause severe infectious complica-
tions and we avoid it, except to control bleeding
from the bone edges. We use multiple small fat
pieces harvested from the appropriate subcutane-
ous layer at the incision site. These pieces should
not simply cover the mastoid air cells—they
should be plugged in the bone openings and
sealed with fibrin glue.
Reconstruction of the Posterior
Skull Base
This stage is essential to avoid postoperative
complications, such as pseudomeningocele, head-
ache, or poor cosmetic result. A large piece of
Gelita sponge is placed over the dura, and the
posterior skull base is reconstructed with methyl
methacrylate. The still soft compound is poured
into the bone defect on the sponge, formed to the
desired shape, and then allowed to harden
(Fig. 4.7c). It should optimally overlap slightly
the adjacent bone edges (Fig. 4.10a). In case the
bone implant remains mobile, it can be fixed with
miniplates or with CranioFix® (Aesculap AG),
similarly to the supratentorial craniotomy flaps
(Fig. 4.10b). The wound is closed in layers.
Drawbacks of the RS Approach
When discussing the drawbacks of a given
operative technique, one should be cautious to
generalize his experience. Frequently, a given
approach is blamed for the bad outcome, while
the application of suboptimal operative technique
or lack of sufficient knowledge/experience is not
considered (Fig. 4.11).
Limited Exposure of the Fundus
The exposure of the most lateral part of the IAC
(its fundus) may be limited by the location of
semicircular canals and vestibule (Fig. 4.12). One
study has shown that in approximately 30 % of
the cases, the posterior semicircular canal, the
Drawbacks of the RS Approach
a b
Fig. 4.9 Intraoperative images. (a) The dura has been
incised along the transverse and the sigmoid sinuses, and
a wide spatula is used to elevate the cerebellum and expose
the lateral cerebellomedullary cistern. (b) Two stay sutures
crus communis, and even the vestibule are posi-
tioned along the visual axis between the surgeon
and the lateral third of the IAC [98]. In order to
avoid damage to the labyrinth in such cases, the
removal of the most lateral tumor part must be
accomplished blindly. An important fact to con-
sider, however, is that similar problem exists with
the MF approach: frequently the transverse crest
blocks the visualization of the inferior half of the
fundus and of the inferior recess [18]. This is
especially important in case of deep falciform
135
(asterisk) and the retractor blade have been placed, thus
exposing the tumor in the depth of the CP angle. (c) View
of the VS under higher magnification
crest in VS arising from the inferior vestibular
nerve. With the MF approach, the hearing is often
lost exactly during tumor dissection from the fun-
dus of the IAC [24, 38, 84].
This limitation of the RS approach, however,
can be overcome with the technical modifications
that we use (described in the Chap. 5). The very
low rate of incomplete VS removal and of recur-
rences, as well as the functional outcome in our
and in other large published series, is a proof that
this limitation is rather theoretical.
136
a b
Fig. 4.10 Postoperative bone-window CT scans, demonstrating the reconstruction of the occipital bone with methyl
methacrylate. The implant may be fixed with miniplates or with CranioFix® (Aeskulap AG), as shown on b
Need of Cerebellar Retraction
This is another frequently mentioned “drawback”
of the RS approach. Indeed, uncontrolled retrac-
tion of the cerebellar hemisphere can lead to
damage of the cerebellum or of the draining veins
and can cause lasting neurological dysfunction.
To avoid such retraction, the lateral cerebelom-
edullary cistern has to be opened immediately after
the dural incision, and sufficient CSF should be
allowed to egress. Some surgeons state that the RS
approach does not permit a direct access to the
arachnoid cisterns, thus restricting the CSF outflow,
especially in large tumors. In our experience, nei-
ther tumor size nor the presence of intracranial
hypertension has precluded the access to this cis-
tern, provided that sufficient amount of bone has
been removed along the lower one-third of the sig-
moid sinus. The self-retaining retractor should be
placed only when the cerebellar pressure is reduced
sufficiently: to protect rather than to compress the
hemisphere. If a stronger retraction is needed, then
the amount of CSF drained is still insufficient.
Excessive cerebellar retraction may also lead
to traction injury of the vulnerable cochlear
nerve and—more rarely—of the facial nerve.
The continuous monitoring of BAEP allows for
early recognition of any cochlear nerve conduc-
tive dysfunction. This feedback allows adapting
the tension on the retractor and the duration of
retraction.
4 Approaches to the Cerebellopontine Angle
Postoperative Headache
The RS approach is frequently blamed for caus-
ing postoperative occipital headache. Rates
between 2.8 and 83 % have been reported, but
most authors accept a rate of 3–10 % as more
realistic [18, 40, 47, 54]. Indeed some studies
demonstrated that this complaint occurs more fre-
quently and is typically more severe after RS
approach than after translabyrinthine or MFA [76,
86]. Staecker et al. [95] registered such headache
in 16 % of their patients operated via the middle
fossa corridor and in 46 % of those operated via
the RS approach. In most current series of ret-
rosigmoid tumor removal, however, the headache
rate is from 0 to 5 % [63, 65, 77, 88, 105].
The pathogenetic factors related to the occur-
rence of headache are not clearly identified.
Possible causes are adhesions between the richly
innervated dura to the nuchal muscles or to sub-
cutaneous tissues with subsequent traction during
neck motion, dural tension in cases of direct dural
closure, chemical meningitis from bone dust
contaminating the subarachnoid space during
intradural drilling, and use of fibrin glue that can
also lead to aseptic meningitis [40, 47, 85, 105].
Direct or indirect injury—during retraction—to
the greater and lesser occipital nerves has also
been proposed as a possible mechanism [68].
Probably the headache is provoked by a combi-
nation of these mechanisms.
Drawbacks of the RS Approach
a b
Fig. 4.11 MR images (a, b) of a patient with VS, oper-
ated in another neurosurgical facility via the “retrosigmoid
approach.” A paramedian suboccipital craniotomy had
been performed that did not reach the sigmoid sinus
Different techniques have been introduced to
prevent postoperative headache such as prevent-
ing the use of fibrin glue, limiting the extent of
drilling of the posterior aspect of the IAC or gen-
erous irrigation and suction of the debris during
intradural drilling, duraplasty instead of primary
dural closure, and replacement of the bone flap at
the end of surgery or cranioplasty [88, 105]. The
methyl methacrylate cranioplasty offers the pos-
sibility of achieving a better cosmetic result and
prevents scar tissue formation between the dura
137
(a). The tumor had been approached via a transcerebellar
route (b), and just a small part of the tumor has been
removed. The tumor was removed completely at our insti-
tute via the “preformed” route. (c) Postoperative CT
and neck muscles. In our experience this tech-
nique is related to optimal outcome: less than
1 % of the patients had persistent headaches.
Pneumocephalus and Tension
Pneumocephalus
Air entry in the subdural space, usually bifrontal
or more rarely intraventricular, subarachnoid, or
in the tumor bed area (Fig. 4.13) is a common
138 4 Approaches to the Cerebellopontine Angle

a b

Fig. 4.12 (a, b) The location of the semicircular canals prevents complete exposure of the fundus of the IAC
(a-arrow)

finding after surgery in the semi-sitting posi-
tion and is asymptomatic in most of the patients.
We have never observed any serious or last-
ing complications due to pneumocephalus. In
case of larger volume, the air can cause tran-
sient headache, nausea, and vomiting. Tension
pneumocephalus with prolonged emergence
from anesthesia or drowsiness is a rare finding.
Patients with symptomatic tension pneumoceph-
alus and radiological evidence of more severe
compression of the ventricles may require burr
hole trepanation and evacuation of the air collec-
tion. Such additional surgery had to be performed
very rarely in our series: in 0.15 % of all patients
operated in the semi-sitting position (Figs. 4.14
and 4.15).
Drawbacks of the RS Approach 139

a b

c d

Fig. 4.13 Various forms of pneumocephalus: typical condition, and only then a decision for the management
subdural bifrontal air collection (a, b), subarachnoid (c), should be taken. None of these patients required surgery
in the tumor bed area (d), and intraventricular (e, f). The for evacuation of the air
radiological findings have to be correlated to the patients’
140 4 Approaches to the Cerebellopontine Angle

e f

Fig. 4.13 (continued)

a b

Fig. 4.14 Massive intraventricular air collection after had strong headache and nausea. Hence, an EVD had to
removal of a very large CP angle meningioma, extending be inserted (b)
to the foramen magnum (a). The patient was drowsy and
References
a b
Fig. 4.15 Massive intraventricular, subarachnoid, and cisternal air collection in a patient, operated for a right-sided
epidermoid tumor. The patient was treated with an EVD for 4 days
References
1. Anderson DE, Leonetti J, Wind JJ, Cribari D, Fahey K
(2005) Resection of large vestibular schwannomas:
facial nerve preservation in the context of surgical
approach and patient-assessed outcome. J Neurosurg
102(4):643–649. doi:10.3171/jns.2005.102.4.0643
2. Angeli RD, Piccirillo E, Di Trapani G, Sequino G,
Taibah A, Sanna M (2011) Enlarged translabyrinthine
approach with transapical extension in the manage-
ment of giant vestibular schwannomas: personal
experience and review of literature. Otol Neurotol
32(1):125–131. doi:10.1097/MAO.0b013e3181ff7562
3. Arriaga MA, Brackmann DE, Hitselberger WE (1993)
Extended middle fossa resection of petroclival and
cavernous sinus neoplasms. Laryngoscope 103(6):
693–698. doi:10.1288/00005537-199306000-00020
4. Arts HA, Telian SA, El-Kashlan H, Thompson BG
(2006) Hearing preservation and facial nerve outcomes
in vestibular schwannoma surgery: results using the
middle cranial fossa approach. Otol Neurotol 27(2):
234–241. doi:10.1097/01.mao.0000185153.54457.16,
00129492-200602000-00016 [pii]
5. Bennett M, Haynes DS (2007) Surgical approaches and
complications in the removal of vestibular schwanno-
mas. Otolaryngol Clin North Am 40(3):589–609.
doi:10.1016/j.otc.2007.03.007, S0030-6665(07)00042-4
[pii], ix–x
141
6. Bento RF, De Brito RV, Sanchez TG, Miniti A (2002)
The transmastoid retrolabyrinthine approach in ves-
tibular schwannoma surgery. Otolaryngol Head Neck
Surg 127(5):437–441. doi:10.1067/mhn.2002.129824,
S0194599802002243 [pii]
7. Brackmann D (1979) Middle cranial fossa approach.
In: House WF, Luetje CM (eds) Acoustic tumors, vol
II, Management. University Park Edn., Baltimore,
pp 5–41
8. Brackmann D (1993) Translabyrinthine/transcochlear
approaches. In: Sekhar LN, Janecka IP (eds) Surgery
of cranial base tumors. Raven, New York, pp
351–365
9. Brackmann DE (1991) Acoustic neuroma: surgical
approaches and complications. Ann Acad Med
Singapore 20(5):674–679
10. Brackmann DE, Green JD Jr (2008) Translabyrinthine
approach for acoustic tumor removal. 1992. Neurosurg
Clin N Am 19(2):251–264. doi:10.1016/j.
nec.2008.02.007, S1042-3680(08)00008-9 [pii], vi
11. Brackmann DE, House JR 3rd, Hitselberger WE
(1994) Technical modifications to the middle fossa
craniotomy approach in removal of acoustic neuro-
mas. Am J Otol 15(5):614–619
12. Brennan JW, Rowed DW, Nedzelski JM, Chen JM
(2001) Cerebrospinal fluid leak after acoustic neuroma
surgery: influence of tumor size and surgical approach
on incidence and response to treatment. J Neurosurg
94(2):217–223. doi:10.3171/jns.2001.94.2.0217
142
13. Briggs RJ, Fabinyi G, Kaye AH (2000) Current man-
agement of acoustic neuromas: review of surgical
approaches and outcomes. J Clin Neurosci 7(6):
521–526. doi:10.1054/jocn.2000.0728, S0967-
5868(00)90728-8 [pii]
14. Briggs RJ, Luxford WM, Atkins JS Jr, Hitselberger
WE (1994) Translabyrinthine removal of large acous-
tic neuromas. Neurosurgery 34(5):785–790; discus-
sion 790–791
15. Bryce GE, Nedzelski JM, Rowed DW, Rappaport JM
(1991) Cerebrospinal fluid leaks and meningitis in
acoustic neuroma surgery. Otolaryngol Head Neck
Surg 104(1):81–87
16. Chanda A, Nanda A (2006) Retrosigmoid intradural
suprameatal approach: advantages and disadvantages
from an anatomical perspective. Neurosurgery 59(1
Suppl 1):ONS1–ONS6. doi:10.1227/01.NEU.
0000220673.79877.30, 00006123-200607001-00001
[pii]; discussion ONS1–ONS6
17. Chen TC, Giannotta SL, Brackmann DE (1993)
Acoustic neuromas. Translabyrinthine approach. In:
Apuzzo MLJ (ed) Brain surgery. Complication avoid-
ance and management. Churchill Livingstone,
New York, pp 1772–1800
18. Colletti V, Fiorino F (2003) Middle fossa versus ret-
rosigmoid-transmeatal approach in vestibular schwan-
noma surgery: a prospective study. Otol Neurotol
24(6):927–934
19. Darrouzet V, Guerin J, Aouad N, Dutkiewicz J,
Blayney AW, Bebear JP (1997) The widened retro-
labyrinthe approach: a new concept in acoustic neu-
roma surgery. J Neurosurg 86(5):812–821.
doi:10.3171/jns.1997.86.5.0812
20. Day JD (2011) The middle fossa approach and
extended middle fossa approach: technique and oper-
ative nuances. Neurosurgery. doi:10.1227/NEU.
0b013e31823583a1
21. Day JD, Chen DA, Arriaga M (2004) Translabyrinthine
approach for acoustic neuroma. Neurosurgery
54(2):391–395; discussion 395–396
22. Day JD, Fukushima T, Giannotta SL (1994)
Microanatomical study of the extradural middle fossa
approach to the petroclival and posterior cavernous
sinus region: description of the rhomboid construct.
Neurosurgery 34(6):1009–1016; discussion 1016
23. Day JD, Kellogg JX, Tschabitscher M, Fukushima T
(1996) Surface and superficial surgical anatomy of the
posterolateral cranial base: significance for surgical
planning and approach. Neurosurgery 38(6):1079–
1083; discussion 1083–1084
24. Driscoll CL, Jackler RK, Pitts LH, Banthia V (2000)
Is the entire fundus of the internal auditory canal vis-
ible during the middle fossa approach for acoustic
neuroma? Am J Otol 21(3):382–388
25. Duke DA, Lynch JJ, Harner SG, Faust RJ, Ebersold MJ
(1998) Venous air embolism in sitting and supine
patients undergoing vestibular schwannoma resection.
Neurosurgery 42(6):1282–1286; discussion 1286–1287
4 Approaches to the Cerebellopontine Angle
26. Ebersold MJ, Harner SG, Beatty CW, Harper CM Jr,
Quast LM (1992) Current results of the retrosigmoid
approach to acoustic neurinoma. J Neurosurg
76(6):901–909. doi:10.3171/jns.1992.76.6.0901
27. Enee V, Guerin J, Bebear JP, Darrouzet V (2003)
Acoustic neuroma surgery. Results and complications
in 348 cases. Rev Laryngol Otol Rhinol (Bord)
124(1):45–52
28. Engelhardt M, Folkers W, Brenke C, Scholz M,
Harders A, Fidorra H, Schmieder K (2006)
Neurosurgical operations with the patient in sitting
position: analysis of risk factors using transcranial
Doppler sonography. Br J Anaesth 96(4):467–472.
doi:10.1093/bja/ael015, ael015 [pii]
29. Fagan PA, Sheehy JP, Chang P, Doust BD, Coakley D,
Atlas MD (1998) The cerebellopontine angle:
does the translabyrinthine approach give adequate
access? Laryngoscope 108(5):679–682. doi:10.1097/
00005537-199805000-00010
30. Fisch U (1970) Transtemporal surgery of the internal
auditory canal. Report of 92 cases, technique, indica-
tions and results. Adv Otorhinolaryngol 17:203–240
31. Friedman RA, Brackmann DE, van Loveren HR,
Hitselberger WE (1997) Management of the con-
tracted mastoid in the translabyrinthine removal of
acoustic neuroma. Arch Otolaryngol Head Neck Surg
123(3):342–344
32. Garcia-Ibanez E, Garcia-Ibanez JL (1980) Middle
fossa vestibular neurectomy: a report of 373 cases.
Otolaryngol Head Neck Surg 88(4):486–490
33. Giebler R, Kollenberg B, Pohlen G, Peters J (1998)
Effect of positive end-expiratory pressure on the inci-
dence of venous air embolism and on the cardiovascu-
lar response to the sitting position during neurosurgery.
Br J Anaesth 80(1):30–35
34. Gildenberg PL, O’Brien RP, Britt WJ, Frost EA
(1981) The efficacy of Doppler monitoring for the
detection of venous air embolism. J Neurosurg
54(1):75–78. doi:10.3171/jns.1981.54.1.0075
35. Gjuric M, Wigand ME, Wolf SR (2001) Enlarged
middle fossa vestibular schwannoma surgery: experi-
ence with 735 cases. Otol Neurotol 22(2):223–230;
discussion 230–231
36. Glasscock ME 3rd, Kveton JF, Jackson CG, Levine
SC, McKennan KX (1986) A systematic approach to
the surgical management of acoustic neuroma.
Laryngoscope 96(10):1088–1094
37. Gormley WB, Sekhar LN, Wright DC, Kamerer D,
Schessel D (1997) Acoustic neuromas: results of cur-
rent surgical management. Neurosurgery 41(1):50–
58; discussion 58–60
38. Haberkamp TJ, Meyer GA, Fox M (1998) Surgical
exposure of the fundus of the internal auditory canal:
anatomic limits of the middle fossa versus the ret-
rosigmoid transcanal approach. Laryngoscope 108(8
Pt 1):1190–1194
39. Hagen PT, Scholz DG, Edwards WD (1984) Incidence
and size of patent foramen ovale during the first 10
References
decades of life: an autopsy study of 965 normal hearts.
Mayo Clin Proc 59(1):17–20
40. Harner SG, Beatty CW, Ebersold MJ (1993) Headache
after acoustic neuroma excision. Am J Otol
14(6):552–555
41. House W (1979) Translabyrinthine approach. In:
House WF, Luetje CM (eds) Acoustic tumors,
Management. University Park Press, Baltimore, pp
43–87
42. House WF, De la Cruz A, Hitselberger WE (1978)
Surgery of the skull base: transcochlear approach to
the petrous apex and clivus. Otolaryngology
86(5):ORL-770–ORL-779
43. House WF, Hitselberger WE (1976) The transcochlear
approach to the skull base. Arch Otolaryngol
102(6):334–342
44. House WF, Hitselberger WE, Horn KL (1986) The
middle fossa transpetrous approach to the anterior-
superior cerebellopontine angle. Am J Otol 7(1):1–4
45. House WF, Shelton C (1992) Middle fossa approach
for acoustic tumor removal. Otolaryngol Clin North
Am 25(2):347–359
46. House WF, Shelton C (2008) Middle fossa approach
for acoustic tumor removal. 1992. Neurosurg Clin N
Am 19(2):279–288, S1042-3680(08)00010-7 [pii], vi
47. Jackson CG, McGrew BM, Forest JA, Hampf CR,
Glasscock ME 3rd, Brandes JL, Hanson MB (2000)
Comparison of postoperative headache after retrosig-
moid approach: vestibular nerve section versus ves-
tibular schwannoma resection. Am J Otol
21(3):412–416
48. Kanzaki J, Shiobara R, Toya S (1991) Classification
of the extended middle cranial fossa approach. Acta
Otolaryngol Suppl 487:6–16
49. Kawase T, Shiobara R, Toya S (1991) Anterior transpet-
rosal-transtentorial approach for sphenopetroclival
meningiomas: surgical method and results in 10 patients.
Neurosurgery 28(6):869–875; discussion 875–876
50. Kawase T, Shiobara R, Toya S (1994) Middle fossa
transpetrosal-transtentorial approaches for petroclival
meningiomas. Selective pyramid resection and radi-
cality. Acta Neurochir (Wien) 129(3–4):113–120
51. King TT, Morrison AW (1980) Translabyrinthine and
transtentorial removal of acoustic nerve tumors.
Results in 150 cases. J Neurosurg 52(2):210–216.
doi:10.3171/jns.1980.52.2.0210
52. Lang J Jr, Samii A (1991) Retrosigmoidal approach to
the posterior cranial fossa. An anatomical study. Acta
Neurochir (Wien) 111(3–4):147–153
53. Lanman TH, Brackmann DE, Hitselberger WE, Subin
B (1999) Report of 190 consecutive cases of large
acoustic tumors (vestibular schwannoma) removed
via the translabyrinthine approach. J Neurosurg
90(4):617–623
54. Levo H, Blomstedt G, Hirvonen T, Pyykko I (2001)
Causes of persistent postoperative headache after sur-
gery for vestibular schwannoma. Clin Otolaryngol
Allied Sci 26(5):401–406, 492 [pii]
143
55. Maddox HE 3rd (1977) The lateral approach to acous-
tic tumors. Laryngoscope 87(9 Pt 1):1572–1578.
doi:10.1288/00005537-197709000-00017
56. Mamikoglu B, Wiet RJ, Esquivel CR (2002)
Translabyrinthine approach for the management of
large and giant vestibular schwannomas. Otol Neurotol
23(2):224–227
57. Mammoto T, Hayashi Y, Ohnishi Y, Kuro M (1998)
Incidence of venous and paradoxical air embolism in
neurosurgical patients in the sitting position: detection
by transesophageal echocardiography. Acta
Anaesthesiol Scand 42(6):643–647
58. Mangham CA (1988) Complications of translabyrin-
thine vs. suboccipital approach for acoustic tumor
surgery. Otolaryngol Head Neck Surg 99(4):396–400
59. Matjasko MJ, Hellman J, Mackenzie CF (1987)
Venous air embolism, hypotension, and end-tidal
nitrogen. Neurosurgery 21(3):378–382
60. Monfared A, Mudry A, Jackler R (2009) The history of
middle cranial fossa approach to the cerebellopontine angle.
Otol Neurotol. doi:10.1097/MAO.0b013e3181c0e98e
61. Morandi X, Riffaud L, Amlashi SF, Brassier G (2004)
Extensive spinal cord infarction after posterior fossa
surgery in the sitting position: case report. Neurosurgery
54(6):1512–1515; discussion 1515–1516
62. Morrison AW, King TT (1973) Experiences with a
translabyrinthine-transtentorial approach to the cere-
bellopontine angle. Technical note. J Neurosurg
38(3):382–390. doi:10.3171/jns.1973.38.3.0382
63. Mosek AC, Dodick DW, Ebersold MJ, Swanson JW
(1999) Headache after resection of acoustic neu-
roma. Headache 39(2):89–94. doi:10.1046/j.
1526-4610.1999.3902089.x, HEDhed3902089 [pii]
64. Ohata K, Haque M, Morino M, Nagai K, Nishio A,
Nishijima Y, Hakuba A (1998) Occlusion of the sig-
moid sinus after surgery via the presigmoidal-trans-
petrosal approach. J Neurosurg 89(4):575–584.
doi:10.3171/jns.1998.89.4.0575
65. Ojemann RG (2001) Retrosigmoid approach to acous-
tic neuroma (vestibular schwannoma). Neurosurgery
48(3):553–558
66. Papadopoulos G, Kuhly P, Brock M, Rudolph KH,
Link J, Eyrich K (1994) Venous and paradoxical air
embolism in the sitting position. A prospective study
with transoesophageal echocardiography. Acta
Neurochir (Wien) 126(2–4):140–143
67. Parry D (1904) A case of tinnitus treated by division of
the auditory nerve. J Laryngol Otol 19(19):402–406
68. Pedrosa CA, Ahern DK, McKenna MJ, Ojemann RG,
Acquadro MA (1994) Determinants and impact of
headache after acoustic neuroma surgery. Am J Otol
15(6):793–797
69. Porter JM, Pidgeon C, Cunningham AJ (1999) The
sitting position in neurosurgery: a critical appraisal.
Br J Anaesth 82(1):117–128
70. Rhoton AL Jr (2000) The cerebellopontine angle and
posterior fossa cranial nerves by the retrosigmoid
approach. Neurosurgery 47(3 Suppl):S93–S129
144
71. Rhoton AL Jr (2000) The temporal bone and tran-
stemporal approaches. Neurosurgery 47(3
Suppl):S211–S265
72. Rhoton AL Jr, Pulec JL, Hall GM, Boyd AS Jr (1968)
Absence of bone over the geniculate ganglion. J
Neurosurg 28(1):48–53. doi:10.3171/jns.1968.
28.1.0048
73. Rhoton AL Jr, Tedeschi H (2008) Microsurgical anat-
omy of acoustic neuroma. 2002. Neurosurg Clin N
Am 19(2):145–174. doi:10.1016/j.nec.2008.02.005,
S1042-3680(08)00006-5 [pii], v
74. Roche PH, Pellet W, Moriyama T, Thomassin JM
(2008) Translabyrinthine approach for vestibular
schwannomas: operative technique. Prog Neurol Surg
21:73–78. doi:10.1159/000156708, 156708 [pii]
75. Rodgers GK, Luxford WM (1993) Factors affecting
the development of cerebrospinal fluid leak and men-
ingitis after translabyrinthine acoustic tumor surgery.
Laryngoscope 103(9):959–962. doi:10.1288/
00005537-199309000-00003
76. Ryzenman JM, Pensak ML, Tew JM Jr (2004) Patient
perception of comorbid conditions after acoustic neu-
roma management: survey results from the acoustic
neuroma association. Laryngoscope 114(5):814–820.
doi:10.1097/00005537-200405000-00005, 00005537-
200405000-00005 [pii]
77. Samii M, Gerganov V, Samii A (2006) Improved pres-
ervation of hearing and facial nerve function in ves-
tibular schwannoma surgery via the retrosigmoid
approach in a series of 200 patients. J Neurosurg
105(4):527–535. doi:10.3171/jns.2006.105.4.527
78. Samii M, Gerganov VM (2008) Surgery of extra-axial
tumors of the cerebral base. Neurosurgery 62(6 Suppl
3):1153–1166. doi:10.1227/01.neu.0000333782.
19682.76, 00006123-200806001-00024 [pii]; discus-
sion 1166–1168
79. Samii M, Klekamp J, Carvalho G (1996) Surgical
results for meningiomas of the craniocervical junction.
Neurosurgery 39(6):1086–1094; discussion 1094–1095
80. Samii M, Tatagiba M, Carvalho GA (2000)
Retrosigmoid intradural suprameatal approach to
Meckel’s cave and the middle fossa: surgical tech-
nique and outcome. J Neurosurg 92(2):235–241.
doi:10.3171/jns.2000.92.2.0235
81. Sampath P, Holliday MJ, Brem H, Niparko JK, Long
DM (1997) Facial nerve injury in acoustic neuroma
(vestibular schwannoma) surgery: etiology and pre-
vention. J Neurosurg 87(1):60–66. doi:10.3171/
jns.1997.87.1.0060
82. Sanna M, Falcioni M, Rohit (2003) Cerebro-spinal
fluid leak after acoustic neuroma surgery. Otol
Neurotol 24(3):524
83. Sanna M, Russo A, Taibah A, Falcioni M, Agarwal M
(2004) Enlarged translabyrinthine approach for the
management of large and giant acoustic neuromas: a
report of 175 consecutive cases. Ann Otol Rhinol
Laryngol 113(4):319–328
84. Satar B, Jackler RK, Oghalai J, Pitts LH, Yates PD
(2002) Risk-benefit analysis of using the middle fossa
approach for acoustic neuromas with >10 mm
4 Approaches to the Cerebellopontine Angle
cerebellopontine angle component. Laryngoscope
112(8 Pt 1):1500–1506. doi:10.1097/00005537-
200208000-00031
85. Schaller B, Baumann A (2003) Headache after removal
of vestibular schwannoma via the retrosigmoid
approach: a long-term follow-up-study. Otolaryngol
Head Neck Surg 128(3):387–395. doi:10.1067/
mhn.2003.104, S0194599802233136 [pii]
86. Schessel DA, Rowed DW, Nedzelski JM, Feghali JG
(1993) Postoperative pain following excision of
acoustic neuroma by the suboccipital approach: obser-
vations on possible cause and potential amelioration.
Am J Otol 14(5):491–494
87. Sekhar LN, Schessel DA, Bucur SD, Raso JL, Wright
DC (1999) Partial labyrinthectomy petrous apicec-
tomy approach to neoplastic and vascular lesions of
the petroclival area. Neurosurgery 44(3):537–550;
discussion 550–552
88. Sepehrnia A, Knopp U (2001) Osteoplastic lateral
suboccipital approach for acoustic neuroma surgery.
Neurosurgery 48(1):229–230; discussion 230–231
89. Shelton C (1995) Unilateral acoustic tumors: how
often do they recur after translabyrinthine removal?
Laryngoscope 105(9 Pt 1):958–966. doi:10.1288/
00005537-199509000-00016
90. Shelton C, Brackmann DE, House WF, Hitselberger
WE (1989) Middle fossa acoustic tumor surgery:
results in 106 cases. Laryngoscope 99(4):405–408.
doi:10.1288/00005537-198904000-00009
91. Shiobara R (1980) A modified extended middle cra-
nial fossa approach for acoustic tumors (author’s
transl). Neurol Med Chir (Tokyo) 20(2):173–182
92. Shiobara R, Ohira T, Inoue Y, Kanzaki J, Kawase T
(2008) Extended middle cranial fossa approach for
vestibular schwannoma: technical note and surgical
results of 896 operations. Prog Neurol Surg 21:65–72.
doi:10.1159/000156595
93. Slattery WH 3rd, Brackmann DE, Hitselberger W
(1997) Middle fossa approach for hearing preserva-
tion with acoustic neuromas. Am J Otol
18(5):596–601
94. Sluyter S, Graamans K, Tulleken CA, Van Veelen CW
(2001) Analysis of the results obtained in 120 patients
with large acoustic neuromas surgically treated via the
translabyrinthine-transtentorial approach. J Neurosurg
94(1):61–66. doi:10.3171/jns.2001.94.1.0061
95. Staecker H, Nadol JB Jr, Ojeman R, Ronner S,
McKenna MJ (2000) Hearing preservation in acoustic
neuroma surgery: middle fossa versus retrosigmoid
approach. Am J Otol 21(3):399–404
96. Sterkers JM, Desgeorges M, Corlieu P, Sterkers O
(1984) Translabyrinthine ablation of voluminous
acoustic neuromas. Ann Otolaryngol Chir Cervicofac
101(1):9–14
97. Tanriover N, Sanus GZ, Ulu MO, Tanriverdi T, Akar
Z, Rubino PA, Rhoton AL Jr (2009) Middle fossa
approach: microsurgical anatomy and surgical tech-
nique from the neurosurgical perspective. Surg Neurol
71(5):586–596. doi:10.1016/j.surneu.2008.04.009,
S0090-3019(08)00408-4 [pii]; discussion 596
References
98. Tatagiba M, Samii M, Matthies C, el Azm M,
Schonmayr R (1992) The significance for postopera-
tive hearing of preserving the labyrinth in acoustic
neurinoma surgery. J Neurosurg 77(5):677–684.
doi:10.3171/jns.1992.77.5.0677
99. Tator CH, Nedzelski JM (1982) Facial nerve preser-
vation in patients with large acoustic neuromas
treated by a combined middle fossa transtentorial
translabyrinthine approach. J Neurosurg 57(1):1–7.
doi:10.3171/jns.1982.57.1.0001
100. Tedeschi H, Rhoton AL Jr (1994) Lateral approaches
to the petroclival region. Surg Neurol 41(3):180–
216, 0090-3019(94)90123-6 [pii]
101. Tos M, Thomsen J (1979) Translabyrinthine surgery
of acoustic neurinoma. Acta Otolaryngol Suppl
360:45–47
102. Tos M, Thomsen J, Harmsen A (1988) Results of
translabyrinthine removal of 300 acoustic neuromas
related to tumour size. Acta Otolaryngol Suppl
452:38–51
103. Tummala RP, Coscarella E, Morcos JJ (2005)
Transpetrosal approaches to the posterior fossa.
Neurosurg Focus 19(2):E6, 190206 [pii]
104. Wackym PA, Andrews JC (1995) Middle cranial
fossa approach. In: Cheatham M, Becker DP, Samii
M (eds) Atlas of cranial base surgery. W. B. Saunders,
Philadelphia, pp 26–31
145
105. Wazen JJ, Sisti M, Lam SM (2000) Cranioplasty in
acoustic neuroma surgery. Laryngoscope 110(8):1294–
1297. doi:10.1097/00005537-200008000-00013
106. Whittaker CK, Luetje CM (1985) Translabyrinthine
removal of large acoustic tumors. Am J Otol
6(Suppl):155–160
107. Wigand ME, Haid T, Berg M (1989) The enlarged
middle cranial fossa approach for surgery of the tem-
poral bone and of the cerebellopontine angle. Arch
Otorhinolaryngol 246(5):299–302
108. Wigand ME, Haid T, Berg M, Schuster B, Goertzen
W (1991) Extended middle cranial fossa approach
for acoustic neuroma surgery. Skull Base Surg
1(3):183–187
109. Wigand ME, Rettinger G, Haid T, Berg M (1985)
Removal of acoustic neuromas of the cerebellopon-
tile angle with transtemporal approach by the middle
cranial fossa. HNO 33(1):11–16
110. Yasargil M (1996) Acoustic neuromas, vol IVb,
Microneurosurgery. Thieme, New York
111. Yates PD, Jackler RK, Satar B, Pitts LH, Oghalai JS
(2003) Is it worthwhile to attempt hearing preserva-
tion in larger acoustic neuromas? Otol Neurotol
24(3):460–464
112. Zeilstra DJ, Groen RA (1999) Venous air embolism
in sitting and supine patients undergoing vestibular
schwannoma resection. Neurosurgery 44(2):426
 Vestibular Schwannomas
Epidemiology
VSs comprise 6–8 % of all intracranial tumors,
25–33 % of the posterior cranial fossa, and
80–94 % of the CP angle tumors [32, 58, 126,
246, 254]. Meningiomas are the second most
common CP angle tumor (3–10 %), followed by
epidermoids (2–4 %). Much more rare tumors are
the schwannomas of other cranial nerves: trigem-
inal nerve, facial nerve or lower cranial nerves,
paragangliomas, metastases, lipomas, osteogenic
sarcomas, osteomas, or tumors extending sec-
ondarily into the CP angle [13, 20].
The average patients’ age at diagnosis is 45–55
years [22, 32, 83, 145]. VSs are more frequent in
females, and the female-to-male relation ranges
from 1:0.8 to 1.5:1. Frohlich and Sutherland [70]
reported that the incidence is similar in both gen-
der but age differences exist: males with VS are
usually 30–39 years old, while in female the peak
is in the period 60–69 years. The incidence of
sporadic VSs in children is very low [3].
VSs can be divided into three groups: asymp-
tomatic tumors (occult or silent VS without
subjective or objective symptoms), symptomatic
but not diagnosed tumors, and diagnosed tumors
[246]. It is impossible to determine the actual
incidence of VSs because of the unknown inci-
dence of asymptomatic and symptomatic undiag-
nosed tumors in the population. The incidence of
VSs, according to pathological examination of
temporal bones, is between 0.57 and 2.7 % [85,
203, 222, 244]. Schuknecht [208] in a study of
1,400 pyramids found that the incidence of
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_5, © Springer-Verlag Berlin Heidelberg 2013
5
asymptomatic VSs is 570/100,000 (0.57 %).
A study of 240 temporal bones performed at
Johns Hopkins University Hospital showed an
incidence of 2.7 % [90]. These rates are, how-
ever, generally evaluated as too high. According
to recent MRI studies, the incidence of asymp-
tomatic VSs is 0.07 % or even 0.2 % [5, 251].
Most of these tumors never become symptom-
atic, probably due to arrested or extremely slow
growth [271].
The clinical incidence of VSs (or the incidence
of diagnosed VSs) has been estimated to range
from 0.2 to 5/100,000/year; most authorities
agree that it is between 1 and 2/100,000/year [67,
90, 94, 117, 242, 261]. The consensus statement
by the NIH in 1991 estimated that in the USA
2,000 to 3,000 VSs are diagnosed each year,
which corresponds to a symptomatic incidence of
1/100,000 [187]. The rate of detection of VSs was
found to correspond to the distribution of radio-
logical facilities and to the awareness of the doc-
tors: a gradual increase of the annual incidence of
diagnosed tumors from 3.1 per million in 1976 to
22.8 per million in 2004 in Denmark paralleled
the wider distribution of CT and MRI [225, 247].
In 2008 the incidence decreased to 19.4 per mil-
lion, while the mean tumor size decreased from
30 mm in 1979 to 10 mm in 2008.
VSs occur in all races but some authors believe
that racial differences exist [32]. The incidence
was found to be 1.7/100,000/year in the white
population and just 0.1/100,000/year in the black
population of South Africa [210]. Preston-Martin
et al. [177] found that the incidence among the
147
148
Afro-Americans from Los Angeles is approxi-
mately 0.2/100,000/year. It is hypothesized that
these differences may be due to the varying muta-
tion rate of the NF2 gene in different races.
Another plausible explanation is the lower rate of
diagnosing of the tumor due to differences in the
social status and accessibility of MRI devices.
Biology of VS
Nowadays the molecular cascade of events lead-
ing to VS formation is much better understood,
but this knowledge is still insufficient to allow
precise prediction of the individual tumor evolu-
tion. Correlation between the tumor’s biological
characteristics and its clinical behavior has yet
not been found. Detailed understanding of the
molecular biology and genetics of VSs should
ultimately allow developing a targeted molecular
therapy that will stop tumor progression or even
eradicate preexisting tumors.
Molecular Genetics
Both sporadic and NF-2-associated VSs are
caused by loss of or mutation of the NF2 tumor
suppressor gene on chromosome 22q12. The NF2
gene was independently identified by Troffater
et al. [251] and Rouleau et al. [188] in 1993 and
encodes for a cell membrane-related protein. The
majority of NF2 mutations are truncating muta-
tions that cause the synthesis of a smaller and
probably nonfunctional protein product. Mutations
within the NF2 gene have been identified in
50–60 % of spontaneous meningiomas and a small
proportion of spontaneous ependymomas [190].
The occurrence of any VS requires inactiva-
tion of both allelic NF2 genes (double hit model),
which is caused by mutation, loss of the long
arm, or loss of the whole chromosome 22. The
frequency, type, and distribution of these muta-
tions are different in the sporadic and in the
familial NF-2 tumors [260]. In NF-2 patients the
most common cause is point mutations, whereas
small deletions account for the majority of muta-
tions in sporadic VSs [101, 102]. In 20–40 % of
the sporadic tumors, the genes are inactivated by
5 Vestibular Schwannomas
methylation [116]. Other potential mechanisms
of gene inactivation are the posttranscriptional
alternative splicing and differential polyadenyla-
tion, as well as influence of a modifier gene [27].
The clinical response to different mutations is
heterogeneous. No clear correlation between the
genotype and phenotypic features of VSs, such as
tumor growth rate and cyst formation, could be
yet established [102, 165, 229, 260]. Even within
a given NF-2 family with the same mutation,
phenotypic variability has been seen. Patients
with missense mutations have statistically greater
survival than those with nonsense and frameshift
mutations [14]. Mutations causing truncation of
the NF-2 protein, as a result of insertions or dele-
tions that lead to disruption of the merlin open
reading frame (frameshift mutations) or of pre-
mature termination codons (nonsense mutations),
have been reported to cause more severe clinical
phenotype [103, 165, 236]. These findings, how-
ever, could not be confirmed in all studies.
Notably, not all VSs exhibit inactivation of the
NF2 gene, showing that this gene may not be the
only determinant of their origin [236]. Mutation
of the NF2 gene is found in 60 % of sporadic VSs
[183]. Tumorigenesis in the remaining cases
could be explained by the influence of other
mechanisms, for example, of epigenetic factors.
Using microarray technology, Caye -Thomasen
et al. [31] identified 78 genes that were
significantly up- or downregulated in tumor tis-
sue. Further studies are necessary to identify
which of them act together with the NF2 gene.
The NF2 Protein
The NF2 gene encodes a 69-kDa protein consist-
ing of 595 amino acids that was named merlin
(Moesin–ezrin–radixin-like protein) or schwan-
nomin (derived from “schwannoma”) [188, 251].
This protein is similar to the 4.1 superfamily of
proteins—the ERM family—which are involved
in cellular remodeling and link the actin cytoskel-
eton to the plasma membrane [251]. All proteins
of this family consist of a highly homologous
N-terminal globular domain (the FERM domain),
an a-helical stretch, and a charged C-terminus
[40]. The ERM proteins bind to actin in the
Pathogenesis of VS
cytoskeleton via their C-terminal domain and via
a second actin-binding site in the N-terminal
domain. The FERM region interacts with a num-
ber of molecules of the plasma membrane, includ-
ing membrane receptors. Interestingly, the merlin
molecule contains a unique 7-amino acid domain
that has been hypothesized to mediate its effects
[158]. Merlin is a part of two pathways, the pRb-
CDK pathway and the PI3K/Akt/mTOR pathway,
which have a crucial role in cell-cycle regulation.
Many hypotheses have been put forward to
explain the mechanism of tumorigenesis after
merlin loss. The molecules with which merlin
interacts are transmembrane proteins, scaffold-
ing/adapter proteins, signaling molecules/
kinases, cytoskeletal proteins, as well as differ-
ent other proteins of unknown function [89].
Merlin plays an important role not only in actin
cytoskeleton-mediated processes, which are
highly relevant in myelination, but also in regu-
lating cell proliferation, cell movement, and
communication [50, 236]. Pelton et al. [168]
found that schwannoma cells from NF-2 tumors
show major alterations in actin cytoskeleton and
display abnormalities in cell spreading reversible
on the reintroduction of merlin. Experiments
with NF2 knockout mouse and in vitro schwan-
noma preparations showed that loss of merlin
causes loss in contact inhibition, which conse-
quently leads to tumorigenesis [236].
Merlin suppresses cellular growth via a yet
unknown mechanism. Its overexpression in
mouse fibroblasts or rat schwannoma cells can
limit cell growth [137]. Furthermore, the cell
response to environmental growth regulatory fac-
tors is supposed to be mediated by merlin.
Consequently, its loss may lead to increased cell
growth and tumor formation [236].
Merlin is found primarily in actin-rich cellular
protrusions and in cell–cell and cell–matrix con-
tact sites [65, 123]. It may be involved also in cell
cycle-dependent nucleus shuttling [155]. Cells
lacking merlin show deregulated adhesion to
extracellular matrix [253]. Several studies demon-
strated that merlin binds to adhesion molecules
such as b1 integrin and layillin, as well as to
molecules, which are part of the focal adhesion
complexes, such as paxillin and focal adhesion
kinase [65, 105]. The impaired cell–cell contact in
149
case of deficient merlin is probably due to destabi-
lized adherent junctions [123]. Merlin’s role in
stabilizing these junctions might involve erbin—a
PDZ protein that binds indirectly to merlin [179].
As pointed by Hanemann and Evans [89],
there is accumulating evidence that merlin has an
important role in the coordination of two relevant
and interdependent processes: cellular adhesion
and growth factor receptor response. Although
merlin/schwannomin is expressed in all tissues
affected in NF-2, it is also expressed in unaf-
fected tissues. Obviously, other proteins compen-
sate for the lost function, or a differential
expression of important merlin/schwannomin
modifying molecules occurs in different tissues.
Nuclear proliferation markers have been stud-
ied for correlation to various clinical parameters.
Although some studies demonstrated a correla-
tion of tumor growth with an increase in Ki-67
index [160], the data so far are not conclusive.
There is no consensus on the role of cellular
factors on tumor growth in both sporadic VSs and
NF-2-related tumors. The influence of various
hormonal factors is also controversial. It is known
that pregnancy and delivery can accelerate VS
growth. Cushing was the first to report the accel-
eration of VS growth during pregnancy in 1917.
Some studies have shown an increased concentra-
tion of estrogen and progesterone receptors on the
tumor cell surface and suggested a correlation
between tumor behavior and expression levels of
hormone receptors [30]. A recent study of Patel
et al. [166] showed that these hormonal receptors
were upregulated in most VSs. Other authors
argue that the concentration of these receptors is
not higher in VS cells and is gender specific [54].
Pathogenesis of VS
Origin of VS
VSs originate from the vestibular nerve. The exact
site of origin along the nerve, however, is unknown,
and their predilection for this particular nerve is
still unexplained. Several theories have been pro-
posed, but neither of them elucidates satisfactory
all aspects of VS pathogenesis. Schwannomas
may arise anywhere from the glial–Schwann cell
150
junction to the termination of the nerve sheath at
the end organ. Most frequently, however, they
arise from the transition zone between the central
and peripheral myelin, also known as glial–
Schwann cell junction or Obersteiner–Redlich
zone [226]. This hypothesis explains the various
sites of tumor origin along the nerve because the
location of the junction is not constant. According
to the findings of one study, the distribution of
Schwann cells was nearly uniform among the
facial, cochlear, superior, and inferior vestibular
nerves [238]. It remains unclear why schwanno-
mas generally originate from the vestibular nerve
and spare the cochlear one. The amount of
Schwann cells was found to be higher along the
distal vestibular nerve—a finding that can explain
the higher incidence of tumors originating in that
region [219]. According to other studies, however,
their density along the distal portions of the ves-
tibular nerves or at the glial–Schwann cell junc-
tion was not increased [136, 226]. Another
hypothesis states that VSs originate in the area of
the vestibular (Scarpa’s) ganglion. The glial–
Schwann cell junction most often lies in the vicin-
ity of vestibular ganglion, implying that both
theories might be correct.
An interesting hypothesis—the embryologic
theory—has been formulated by Sterkers et al.
[226], who suppose that VSs arise from imma-
ture cells existing in the adult superior and inferior
vestibular ganglia.
Nerve of Origin
VSs originating from the superior vestibular nerve,
which innervates the lateral semicircular canal,
show much more frequently a reduced caloric
response. Origin from the inferior vestibular nerve
may be suspected by recording the rotatory nystag-
mus [148]. At a certain stage, however, the tumor
affects the function of both nerves, and the tests
become unreliable. The exact site and nerve of ori-
gin can be determined at surgery and is reliable only
in case of small VS. According to some authors,
70–91.4 % of VSs originate from the inferior ves-
tibular nerve [115, 120, 183]. Other authors point
out that the superior vestibular nerve is the more
common origin [141]. The currently dominating
opinion, however, is that the tumor originates from
both nerves in approximately similar rates [44, 45].
5 Vestibular Schwannomas
Site of Origin
Most VSs arise within the IAC: typically in its
medial portion and rarely in its lateral end. VSs
grow medially toward the CP cistern and to a lesser
degree laterally toward the fundus. Exceptionally,
the tumor can originate in the inner ear [66]. The
category of “medial VS” has been introduced to
designate those tumors that originate from the cis-
ternal part of the nerve in the CP angle cistern
(Fig. 5.1)—they arise just outside the porus and
show no extension into the IAC [120, 248]. The
incidence of such VSs is less than 2 % [96].
However, if those VSs that have minimal extension
into the IAC are also defined as “medial VS,” the
incidence rises to 12 % [230]. These less strict cri-
teria explain the observation of Strauss et al. [230]
that normal IAC had one-third of cases, porus
enlargement had 20 %, and a funnel-shaped canal
had almost 50 % of the patients with medial VS.
MG Yasargil hypothesized that VS originate
epiarachnoidally in the IAC and gradually push
the arachnoid membrane of the IAC medially
into the CP angle. At this stage a single arachnoid
layer covers the tumor [268]. In the CP angle the
tumor and the brain stem are covered by their
own arachnoid membrane. Therefore, a double
arachnoid layer surrounds VSs reaching the brain
stem. This theory has been questioned by Tarlov
[241], Ohata et al. [162], and Lescane et al. [130].
Still, it has a major practical implication: tumor
dissection should be performed within the arach-
noid space, leaving a layer of arachnoid on the
tumor and a layer on the pons.
Very rarely VS originate from the distal seg-
ment of the vestibular nerve and develop in the
area of the vestibule or semicircular canals
without extension into the IAC. These intralaby-
rinthine schwannomas are regarded as a separate
clinical entity from the more common VSs aris-
ing within the IAC and extending laterally to
involve the labyrinth.
Growth Pattern and Growth Rate
VSs are typically slow-growing tumors, but their
growth rate may be irregular over the time, even
in a single patient. Some tumors enlarge rapidly,
whereas others remain stable or even regress.
The natural evolution of VSs cannot be predicted.
At least five growth patterns of VSs have been
Pathogenesis of VS 151

a b

Fig. 5.1 MRI study of a patient with a “medial VS”: the IAC is completely free from tumor. (a, b) T1-weighted
sequences after gadolinium application. (c, d) CISS sequences

outlined: continuous growth (40 %), no measur-
able growth (18 %), no measurable growth
followed by continuous growth (18 %), negative
growth (8 %), and various types of positive tumor
growth or irregular growth (16 %) [34]. The
reported rates of VSs that grow further, do not
change, or even show involution or “negative
growth” vary widely. Progressive tumor growth
is observed in 54–91 % of the cases, no change of
tumor size in 30–40 %, and tumor involution in
0–18 % [17, 157, 187]. The spontaneous involu-
tion of some VSs may be explained by tumor
necrosis caused by intratumoral thrombosis
[135]. Longer follow-up studies showed that the
duration of follow-up correlates with a higher
rate of tumors that show progressive growth ten-
dency [221]. Resumption of growth even after a
period of quiescence can occur.
The annual growth rate of VSs is between 1
and 2.4 mm/year [12, 48, 83, 94, 260]. It is known
that cystic and the NF-2-related tumors have a
higher growth rate: up to 3.2 mm/year [34]. A sub-
group of VSs, however, which do not differ
macroscopically or radiologically from the rest,
have a propensity for more rapid growth. In the
series evaluated by Bakkouri et al. [12], the average
152
growth rate was 1.15 ± 2.4 mm/year. In 10.2 % of
all VSs, however, tumor growth was ³3 mm/year.
Tumors with extrameatal extension generally have
a higher growth rate [12, 87, 224], although not all
studies support this correlation [250]. Despite the
large number of studies on the VS growth, the
clinical applicability of the findings, as pointed by
Charabi et al. [36], remains limited.
Classification
Various methods of measurement of VS size are
still in use, confusing the comparison of the results
between different series. While some authors rely
on measurement of maximal tumor diameter in
the axial plane, others use the product of the cis-
ternal diameters, anteroposterior × mediolateral;
yet others recommend volumetric measurements.
Controversial is also the inclusion of the intrac-
analicular tumor part, which generally is between
8 and 12 mm. The definition of “large” or “giant”
VS also varies.
The classification of VS is based either on
their maximal diameter or on the degree of exten-
sion in the CP angle. There are several widely
accepted classification schemas. The classification
of W. T. Koos is based on the maximal diameter
of VS [120]. Classifications, frequently applied
by neurootologists, are the one of D. E. Brackmann
[21] and Tos and Thomsen [249]. The classification
proposed by L. Sekhar is similar [212]:
• 0 stage—intracanalicular VS
• I stage—<1 cm
• II stage—1–2.5 cm
• III stage—2.5–4 cm
• IV stage—>4 cm
The complexity is made even higher by the use
of different criteria in different publications of the
same authors. Gormley and Sekhar [83] defined
VS ³4 cm as large, while Sekhar and Tzortidis
[212] defined as large those between 2.5 and 4 cm.
Table 5.1 Consensus meeting on
systems for reporting results in vestibular
Grade 1
schwannoma classification [47, 110]
Grade 2
Grade 3
Grade 4
Grade 5
5 Vestibular Schwannomas
The outcome of treatment of two series of patients
with “large VS” operated in the same institution
was presented in 1994 Briggs et al. [26] and in
1999 [125]. In the first only VSs ³4 cm were
included, while in the second all VSs ³3 cm were
included. The reported outcome was respectively
better in the later series. The effect of the learning
curve on outcome, however, is also responsible to
a certain extent for the differences.
At the Consensus Meeting on Systems for
Reporting Results in Vestibular Schwannoma
held in Tokyo in 2001, an agreement was reached
that VS size should defined based on measure-
ment of the linear tumor dimensions and not on
its volumetric measurements [47, 110]. The size
of the intracanalicular and of the extracanalicular
parts should be reported separately; however,
only the extracanalicular diameter should be
applied for the determination of tumors’ size. At
the conference the following scale has been
accepted (Table 5.1).
The extent of tumor extension in CP angle is
the main factor determining the clinical presenta-
tions, the operative difficulty, and the outcome.
Therefore, we believe that any classification of VS
should consider this parameter. VSs of the same
absolute size may lead to different clinical picture.
Thus, in young patients with small subarachnoid
space, they might reach and compress the brain
stem, while in elderly with significant brain atro-
phy, VS of the same size may still be located
entirely in the CP cistern (Fig. 5.2). The Hannover
tumor extension system, introduced by the senior
author, is based on this criterion: tumor extension.
This classification system has been modified
recently: giant VSs are now considered as a sepa-
rate fifth category (Fig. 5.3). Previous radiosurgi-
cal treatment renders the surgical tumor removal
more difficult. In this set of patients, specific
problems tend to appear (see the corresponding
chapter). Hence, this has to be specified explicitly
(Table 5.2).
Intracanalicular tumor
Small 1–10 mm extrameatal
Medium 11–20 mm extrameatal
Moderately large 21–30 mm extrameatal
Large 31–40 mm extrameatal
Giant > 40 mm extrameatal
Classification 153

a b

Fig. 5.2 Examples of VSs with similar size but different not contact the brain stem. This issue—whether the tumor
relation to brain stem. In the first and second examples (a, contacts the brain stem or not—has a major clinical
b), the tumor reaches the brain stem, while in the third (c) significance: it determines the operative difficulty and,
the subarachnoid space is much larger and the tumor does respectively, the outcome

a b

Fig. 5.3 Axial and coronal MR images of VS of various application). Grade T1: (a, b). Grade T2: (c, d). Grade
extensions, according to the INI (Samii) classification of T3a: (e, f). Grade T3b: (g, h). Grade T4a: (i, j). Grade
VS extension (T1-weighted sequences after contrast T4b: (k, l). Grade T5: (m, n)
154 5 Vestibular Schwannomas

c d

e f

g h

Fig. 5.3 (continued)

Classification 155

i j

k l

m n

Fig. 5.3 (continued)

156
Table 5.2 INI (or Samii) Tumor extension
classification of VS extension grade
T1
T2
T3a
T3b
T4a
T4b
T5
In case the VS has been previously treated with radiosurgery, this is designated
with an additional “+R,” for example, stage “T4 a+R”
Neuroimaging
VSs are isodense on native CT imaging; follow-
ing contrast administration, they enhance strongly.
The bone-window high-resolution CT imaging is
highly sensitive in regard to the bony changes of
IAC. Furthermore, it demonstrates the normal
structures of the petrous bone, which are essen-
tial for surgery: emissary vein/s, jugular bulb,
position of the labyrinth, etc. MRI is the gold
standard for diagnosing VSs: at T1-weighted
MRI images, VSs are isointense to slightly
hypointense, and on T2-weighted images, they
are hyperintense [19]. They enhance intensely
after contrast administration. The three patterns
of contrast enhancement are homogeneous (in
50–60 %), heterogeneous (in 30–40 %), and cys-
tic (in 5–15 %) [38, 51]. Cystic VSs have to be
distinguished from CP angle epidermoids and
subarachnoid cysts, which are hypointense in T1
and hyperintense in T2 and do not enhance after
contrast administration [53].
In contrast to CP angle meningiomas, VSs are
centered at the widened IAC, cause typically ero-
sion and/or dilatation of the IAC (in 70–90 % of
the cases), extend almost always into the IAC,
form acute angle to the posterior surface of the
petrous bone, and show intratumoral calcifications
or hyperostotic bone changes extremely rarely.
Although VSs lack the typical for meningiomas
“dural tail sign,” in rare cases enhancement of the
adjacent meninges is possible [85].
Small intracanalicular VSs have to be differ-
entiated from some inflammatory or infectious
conditions, such as viral neuropathy or arach-
noiditis, following viral infection [94]: at
5 Vestibular Schwannomas
Tumor extension in the IAC and CP angle
Purely intracanalicular
Intra-/extrameatal
Filling the CPA cistern
Reaching the brain stem
Compressing the brain stem
Severe compression and dislocation of brain stem and fourth
ventricle; obstructive hydrocephalus
Giant tumors (maximal diameter >4 cm), extension over the
midline
T1-weighted MRI following gadolinium admin-
istration, they also appear as small areas of con-
trast enhancement. In contrast to VSs, however,
which are globular and more sharply delineated,
the inflammatory lesions show typically a more
linear or ill-defined enhancement [6]. Further
clue is the absence of T2 abnormality and/or the
size discrepancy between the lesions observed on
T1 and T2 sequences.
Some more advanced neuroimaging modali-
ties have been evaluated recently. Okamoto et al.
have demonstrated that a small focus of high sig-
nal T2 intensity may be observed in the dorsal
brain stem ipsilateral to the tumor. They hypoth-
esized that this signal results presumably from
the degeneration of the vestibular nucleus associ-
ated with VS [163]. The relative tumor blood vol-
ume (rCBV) is reported to be significantly lower
in VSs than in meningiomas [88]. The presence
of microhemorrhages on T2-weighted gradient-
echo sequences may be useful to differentiate VS
from CP angle meningiomas [243]. Using proton
MR spectroscopy, VSs could be differentiated
from meningiomas by the presence of prominent
myoinositol peak at 3.55 ppm; in contrast alanine
is found only in meningiomas [41].
Clinical Presentation
The typical CP angle syndromes have been well
recognized since the end of the nineteenth century.
Neurological dysfunction is a result of direct com-
pression of neurovascular structures, brain stem
compression, obstruction of CSF flow, and intrac-
ranial hypertension. The clinical presentation of
Clinical Presentation 157

patients with CP angle tumors is related to multi-

ple factors: type of the lesion, origin and extension
pattern, size, and growth rate.
The growth of VS follows a certain pattern
that traditionally has been divided into four
stages: intracanalicular (intrameatal), cisternal,
brain stem compressive, and hydrocephalic.
Each stage correlates closely with corresponding
clinical symptoms. VSs originate within the IAC
and gradually fill it; then, they expand into the
CP angle and occupy the CP angle cistern, dis-
placing the seventh and the eighth CNs, as well
as the AICA. During its further growth, the
tumor reaches, compresses, and then displaces
a
the brain stem and cerebellum. Depending on the
degree of tumors’ cranial and caudal extension,
the fifth and the lower cranial nerves become
involved. The hydrocephalic stage begins with
the obstruction of fourth ventricle and its outlets.
This four-stage concept, however, does not
reflect entirely the high variability of VS growth
and extension patterns; furthermore, the individ-
ual anatomical variability is also not considered
(Figs. 5.4 and 5.5).
The degree of bony changes of the IAC caused
by the growing VS differs: some VSs expand
along the direction of least resistance toward the
CP angle and lead to minimal or even no bone
changes; other VSs cause dilatation and even ero- b
sion of the IAC, which may be out of proportion
to overall tumor size (Figs. 5.6, 5.7, 5.8, and 5.9).
Yamakami et al. [265] showed that the extent of
IAC bony changes does not correlate with the
overall tumor size. In their material, widened
IAC had 46 % of the patients, extensively eroded
IAC, 17 %, and normal IAC, 36 %. Preoperative
hearing level was significantly more severely dis-
turbed in patients with extensive erosion of the
IAC compared to those with normal or only wid-
ened canal [265]. Badie et al. [9] demonstrated
that the pressure within the IAC is significantly
elevated in most patients and correlates to the
extent of tumor in the IAC and not to the total
tumor size. Their findings explain the frequently c
observed lack of correlation between the preop-
erative hearing function and tumor size. Thus, Fig. 5.4 MR images demonstrating the varying exten-
some patients with small intracanalicular VS sion pattern of the tumor in the CP angle. (a) Predominant
have profound auditory dysfunction, while others anteroposterior extension. (b) Predominant mediolateral
extension (the mediolateral tumor diameter is greater than
with large CP angle tumor extension have normal the anteroposterior one). (c) Round tumor shape
158 5 Vestibular Schwannomas

hearing. The enlargement of the IAC has proved

to be an unfavorable factor for postoperative
hearing preservation [45, 206]. The elevated pres-
sure on the cochlear nerve and on the labyrinthine
artery may increase their vulnerability during
tumor dissection.
The degree of filling of the IAC is also vari-
able (Fig. 5.10) and correlates with the probabil-
ity for hearing preservation [152, 222]. In an MRI
study of 106 patients, Mohr et al. [152] found
that the IAC was filled partially in 59 % (>1/2 in
21 % and >1/2 in 38 %) and completely in 41 %.
Incomplete filling of the IAC was an independent
a favorable factor in hearing preservation. Some
tumors even spare the IAC entirely—the so-
called medial VS—which were discussed earlier
in the text.
From a surgical point of view, the key issue is
not the absolute tumor size but the degree of brain
stem compression [145, 198].
Patients with intracanalicular VS present with
vestibulocochlear nerve dysfunction: hearing loss,
tinnitus, and/or vestibular dysfunction. Hearing
loss is usually of insidious onset and is the most
frequent symptom, observed in up to 95 % of
patients [145]. Audiograms reveal typically high-
frequency sensorineural hearing loss. Speech dis-
crimination is affected out of proportion to
b pure-tone hearing loss. Several theories have been
proposed to explain how VS causes hypacusis:
mechanical injury to the cochlear apparatus; bio-
chemical changes of inner ear fluid; ischemic
injury resulting from occlusion or spasm of the
labyrinthine artery; tumor compression, stretch-
ing, or infiltration of the cochlear nerve; or eleva-
tion of the intracanalicular pressure [122, 127,
207]. As Lapislava et al. [127] point, however, the
mechanism of auditory dysfunction is most likely

Fig. 5.5 MRI of VS with various growth patterns in

relation to the IAC. (a) The tumor is centered at the IAC
c and causes compression of both the brain stem and cere-
bellum. (b) The main tumor bulk is anterior to the IAC
and causes compression of the brain stem and middle
cerebellar peduncle (but not of cerebellum). (c) The main
tumor bulk is posterior to the IAC: the tumor compresses
mainly the middle cerebellar peduncle and the cerebellum
(minimal contact to brain stem)
Clinical Presentation 159

a b

c d

Fig. 5.6 Relatively small VS (grade 3a) causing severe dilatation of the IAC, which does not correspond to the overall
tumor size

multifactorial. The incidence of VS that presents
with sudden hearing loss ranges from 3 to 26 % in
various series [145, 207]. Interestingly, only
1–2 % of all patients presenting with sudden hear-
ing loss will be diagnosed to have a VS.
Tinnitus is the second most common present-
ing symptom. Its incidence is 48–74 % of the
patients and has an inverse correlation with tumor
extension. Vestibular symptoms (vertigo, dizziness,
and unsteadiness) frequently are not recognized by
the patients but are always discovered with special
testing. The caloric test, which was performed
widely before the wide distribution of MRI
facilities, detects even mild vestibular changes.
During the cisternal stage, hearing deteriorates
further and disequilibrium gradually replaces the
160
a
b
Fig. 5.7 VS grade T3b: the right IAC is not widened by
the tumor and is approximately similar to the contralateral
one
vertigo. The CP angle tumor portion is always
centered at the IAC; the structure and the resis-
tance of surrounding arachnoid membranes prob-
ably influence its shape. The shape may be oval
(if the shortest diameter, determined in the axial
plane of MRI, is less than 90 % of the longest
diameter), round (if this relation is equal or
greater than 90 %), or polycyclic [77]. Oval VS
with larger mediolateral diameter may compress
the brain stem and lead to corresponding neuro-
logical symptoms to a degree, which seems out of
proportion to their overall size. Oval tumors with
larger anteroposterior diameter may attain rela-
tively large size with minimal brain stem com-
pression [99]. Later on trigeminal symptoms,
headache, lower cranial nerve palsies, and ataxia
5 Vestibular Schwannomas
a
b
Fig. 5.8 VS grade T3a with moderate enlargement of the
IAC (left side)
appear. Obstructive hydrocephalus results from
occlusion of CSF outflow at the stage of severe
brain stem and fourth ventricle compression. Late
symptoms are contralateral long-tract signs,
severe gait disturbance, visual loss, and signs of
intracranial hypertension.
Symptom presentation is related to VS size.
In a study on the clinical presentation of 1,000
VSs, the senior author and C. Matthies [145]
showed that in small tumors the mean patients’
age was significantly higher; trigeminal hypes-
thesia, dizziness, and subjective deafness dura-
tion were shorter; while tinnitus duration was
longer. The most frequently involved CN was
the cochlear—90–97 % of the patient had some
Clinical Presentation
b c
Fig. 5.9 (a) The figure demonstrates a patient with NF-2
and bilateral VSs: although the right-sided tumor is larger
than the left-sided, its intrameatal part is smaller.
Respectively, the right IAC (b) is less dilated than the left
one (c)
hearing loss with an average duration of 3.7
years. Deaf before surgery were 3 %. Headaches
in patients with VS are typically in the occipital
region (12 % of patients in the senior authors’
series), unless related to an intracranial hyper-
tension syndrome. The patient’s subjective
awareness of trigeminal and facial nerve dys-
function is lower than their actual rates, assessed
objectively. Symptoms of trigeminal nerve dys-
function were reported by 8 %, whereas 19 %
had trigeminal signs at examination; facial nerve
weakness was noted by 6 % (in half of them due
to previous surgery) but objectively found in
17 %. Preoperative facial weakness in previ-
ously untreated cases is exceptional and
161
e
Fig. 5.10 Examples of VS with varying extension in the
IAC (coronal CISS MRI sequences). (a, b) The tumor
extends up to the fundus. (c–e) The most lateral part of the
IAC is free of tumor. The arrows in c–e point at the CSF
lateral to the VS
frequently indicates that the tumor actually is
schwannoma originating from the facial nerve.
Facial spasm and trigeminal neuralgia are very
unusual symptoms in patients with VS.
Symptoms of vestibular dysfunction had 61 %,
and vestibular signs had 45–59 % of all patients.
The lower cranial nerves were affected in 3 %
(glossopharyngeal paresis in 2 % and hypoglos-
sal paresis in 1 %) and the abducens nerve in
1.8 % of the patients.
162 5 Vestibular Schwannomas

Management of Patients with VS a

Conservative Management

The follow-up or “wait-and-scan” strategy has

become an acceptable management option due to
the constantly growing number of incidentally
found VSs. An increasing number of patients
with small and asymptomatic tumors are being
currently managed conservatively, and the ratio-
nale is the lack of progress of some VSs. Current
MRI facilities allow for earlier VS detection,
when they are still asymptomatic and small in
size. Furthermore, MRI is a reliable means for
control of their growth (Fig. 5.11). b
The decision on the individual management
plan should always be taken together with the
patient and his family, considering not only the
tumors’ features and patient condition but also his
expectations and professional and daily activities.
We believe that initial observation is an appropri-
ate option in elderly or somatic unstable patients
with small tumors and mild stable symptoms, in
patients with small tumors and complete hearing
loss, in patients with VS on the only hearing side,
or in those unwilling to undergo surgery.
Some surgeons are still not inclined to advice
surgery until the tumor extends into the CP angle
or neurological deficits develop. The goal of mod-
Fig. 5.11 MR images of VS that shows a certain growth
ern VS surgery is preservation of functions. Surgery tendency (a, b). The second image (b) is made approxi-
at an earlier stage, in patients with smaller tumors mately 18 months after the first one. The patient was ini-
with available useful hearing and good BAEP tially considered as good candidate for hearing
response, allows for its preservation in high num- preservation. Shortly before the second MRI, he experi-
enced sudden loss of hearing on the left side
bers (see section “Hearing”). Many studies have
demonstrated that a significant risk of loss of use-
ful hearing during observation exists even in stable percent of the patients had to be operated due to
nongrowing VS—between 50 and 67 % [34, 82, tumor enlargement, while 6 % died of brain stem
87, 256]. Hearing deterioration is not always slowly herniation induced by tumor compression.
progressive; sudden hearing loss may develop in a Interestingly, 28 patients were assessed initially
single or repeated episodes. Thus, a hearing- as candidates for hearing preservation surgery;
preserving surgery might become impossible. during the observation period, however, this
In a widely cited study performed by the group option became impossible in 75 % of them due to
of Charabi et al. [34], 123 patients (127 tumors) tumor growth and/or deterioration of hearing.
were followed with a “wait-and-scan” policy over The further follow-up of the patients in this series
a 20-year period. Tumor growth was observed in was presented in a later paper, and the results were
74 %; 18 % of the tumors did not grow, and nega- even more convincing: 89 % of the candidates
tive tumor growth occurred in 8 %. Twenty seven for hearing preservation surgery lost eligibility
Management of Patients with VS 163

because of tumor growth or deterioration of

hearing level, or both [37].
It is still impossible to predict the tumor
growth rate in an individual patient, but in
10–50 %, the conservative treatment fails and
active management is necessary [12, 82, 256,
257, 263]. As pointed by Sughrue et al. [233],
this wide range can be explained by the small to
modest size of the reported series, the varying
methods for growth measurement, the lack of sta-
tistical power, and the surgeon’s bias.
If the hearing is already lost, the decision to
follow the tumor further or to remove it depends
a
on the individual patients’ wish. Slight tumor
enlargement does not necessarily indicate that
immediate treatment is required. No reliable pre-
dictive factors of the tumor growth pattern that
could support the therapeutic decision-making
have been identified up to now [87]. Tumors with
extrameatal extension have a higher tendency
for future growth [180, 257, 263]. According to
other studies, however, the overall growth rate
did not differ significantly between intrameatal
and extrameatal tumors [12, 34, 67]. Younger
age is also not consistently associated with more
rapid growth [12, 82]. Most authors did not find
any correlation between tumor growth rate and b
patients’ sex or age, tumor size, and hearing sta-
tus at presentation [67, 180]. The only predic- Fig. 5.12 Example of a growing VS (T1-weighted MRI
tive factor of tumor growth is the delay between study). The interval between the two images is 2 years
the appearance of the first symptoms and time
of diagnosis: a shorter delay in diagnosis cor- Surgical Treatment of VS
relates with the need of active therapy at some
point during the observation [12, 34]. Cystic At present surgery of VSs should be safe for the
tumors may display sudden and dramatic growth patient. Major morbidity rate should be reduced to
and are not suitable candidates for observational very low levels. Preservation of normal facial
management [72]. nerve function should be the rule, and useful hear-
A correlation between VS growth in the first ing preservation should be the goal. Another issue
year or years of follow-up and the risk of later to consider when discussing the management
growth has been found in many studies options is the preservation and even improvement
(Fig. 5.12) [67, 224, 263]. The tumor may of the patients’ quality of life. The complete
enlarge, however, at varying intervals during removal of these benign tumors is curative and is
follow-up; a previously “inactive” tumor may to be strived in every case. Still, avoidance of
still exhibit tumor growth after many years. major deficits and preservation of facial nerve
Hence, the radiological control—even in non- function have highest priority. Exceptions to com-
growing tumors—should not be restricted only plete removal hence are all those cases in which
to the first years of observation. the surgeons—due to the characteristics of a given
164
tumor or due to less experience—feel that contin-
uation of surgery may be too dangerous for the
patient. In case that further tumor dissection would
result in anatomic discontinuity of the facial nerve,
a thin veil of tumor capsule attached to the most
adherent portion of the nerve might be left. Tumor
remnants are followed radiologically, and if a
growth tendency is shown, active treatment should
be considered: either with new surgery or with
gamma knife, depending on the experience of the
surgeon. The size of the remaining tumor is an
important predictor of the risk for its future growth:
a significant difference exists in case the tumor
removal is near total (>95 % resection), subtotal
(>90 %), or partial (<90 %) [18, 57, 213]. Other
exceptions to the rule of complete tumor removal
are patients with NF-2, such as those with bilateral
VSs and surgery on the only hearing side (see sec-
tion “Neurofibromatosis Type 2 and Other
Bilateral Cerebellopontine Angle Tumors” for
detailed discussion).
Planned subtotal tumor removal with follow-
up or immediate radiosurgery of the remnant was
promoted as an option to reduce postoperative
morbidity [111, 113, 133, 217]. Considering the
high probability of growth of the remnant, espe-
cially after longer follow-up and the much higher
morbidity rate and risks of damage to the facial
nerve at reoperations, the rationale of this concept
remains elusive. Exceptions are the rare cases of
lifesaving decompressive surgeries (10 cases =
>1 % in the series of the senior author) [197].
Recently, another concept has become popular:
to perform initially intracapsular decompression
of the VS and then to treat the remnant with radio-
surgery [71, 98]. The evaluation of the general and
functional outcome following such management,
however, shows that it is not superior to that after
radical microsurgical removal in one stage. The
patient is exposed to two procedures, each with its
specific risk of morbidity and only tumor “growth
control” is achieved. Moreover, the phenomenon
of radiosurgical failure should be considered: in
some patients the tumor grows further despite the
treatment (see section “Radiosurgery” of VS).
Different operative approaches and techniques,
reflecting individual surgeons’ experience and
5 Vestibular Schwannomas
preferences and institutional tradition, are
applied for removal of VS and are discussed in
the Chap. 4:
• Translabyrinthine approach and its
modifications: translabyrinthine–transtentorial
approach, extended translabyrinthine approach,
transmastoid transpetrosal partial labyrinthec-
tomy, retrolabyrinthine approach, combined
translabyrinthine–retrosigmoid approach
• Middle fossa approach and extended middle
fossa approach, which allows removing of
VSs extending up to 2 cm into the CP angle
• Retrosigmoid suboccipital approach
Some surgeons select the approach depend-
ing on tumor size, extension into the IAC, and
preoperative hearing level. We believe that the
results depend more on the individual surgeons’
experience rather than on the advantages or dis-
advantages of a particular approach. Excellent
results in terms of rate of complete removal and
functional preservation of the facial nerve func-
tion have been achieved in experienced hands
with each of these approaches. The reported rates
of complete tumor removal are 90–99 %, and the
recurrence rates are 0.5–5 % [7, 22, 80, 125, 194,
211, 244, 264]. The rates of anatomic and func-
tional preservation of the facial nerve also appear
to be similar and are related mainly to tumor size.
Preservation of the facial nerve anatomical integ-
rity is possible in 93–99 % of all patients, and
good facial nerve function is achieved in 52–93 %
of them. In regard to hearing preservation, how-
ever, a major difference exists between the dif-
ferent approaches: only the middle fossa and the
retrosigmoid approaches provide the opportu-
nity for hearing preservation. The retrosigmoid
approach is the only one that allows for hearing
preservation in case of large VSs.
The advantages and disadvantages of these
techniques are widely discussed in the literature.
Comparisons of the outcomes of surgery with
different approaches have been frequently made.
We believe, however, that they are unreliable and
have low scientific and practical value: optimal
results are achieved with that approach the sur-
geon is best familiar with. Notably, neurophysi-
ological monitoring should be an integral part of
Management of Patients with VS
each VS surgery, whatever operative route or
technique is preferred.
Staged removal of VS is a concept that at
present is considered obsolete. It has been intro-
duced in the early years of VS surgery—in
1925—by W. Dandy [49]. He performed the
operation in 2 stages and found that at the second
stage the residual tumor was softer, more necrotic,
and less vascular. Later on, similar technique has
been applied in case of tumors with large size
and major brain stem compression, particularly
those that are deeply embedded within the sub-
stance of the brain stem and cerebellum that were
difficult to remove completely during the initial
surgery: the tumor is removed in 2 or even in 3
stages [46, 167, 215]. Major disadvantages of
this strategy are the need of two separate surger-
ies with their cumulative risks and the develop-
ment of adhesions and scar tissue, which render
the second surgery much more difficult.
The philosophy of the senior author is that the
goal of VS surgery—complete tumor removal
with preservation of all neural functions—can be
best achieved with the retrosigmoid approach
with the patient in the semi-sitting position and
has been applied successfully in more than 4,000
such surgeries.
Preoperative Examinations
Neuroimaging
Neuroimaging (discussed in detail earlier),
besides establishing the diagnosis VS, provides
data important for the operative planning.
Audiometric and Audiological Examinations
The evaluation of hearing is important for the
decision-making process. Pure-tone audiometry
165
is the most important hearing functional test.
Speech discrimination is important in determin-
ing the usefulness of hearing.
Electrophysiological Studies
Electronystagmography assesses the superior
vestibular nerve, which innervates the horizon-
tal semicircular canal. Importantly, the vestibu-
lar nerve may function until almost all fibers are
destroyed. The vestibular evoked potential test
provides information on the function of infe-
rior vestibular nerve that innervates the saccule.
Brain stem auditory evoked potential (BAEP)
testing correlates with the chances of hearing
preservation.
Retrosigmoid Approach in VS
The general principles of the RS approach have
been outlined in the Chap. 4. Here we will
describe the steps specific for VS surgery.
Once the CP angle has been exposed, the sur-
geon should identify the main anatomical land-
marks that would be helpful for the precise
orientation. The size of the tumor dictates the
subsequent steps. In most VS the intrameatal
tumor part is approached initially in order to
identify the distal intracanalicular segments of
facial and cochlear nerves, which benefits their
subsequent dissection from the tumor.
Furthermore, the initial drilling of the posterior
wall of the IAC prevents spillage of bone dust
throughout the basal cisterns. Exceptions are the
very large VSs that not only fill the CP angle but
also “hang” on the posterior surface of the pyra-
mid. IAC in such cases can be opened only after
partial removal of the CP angle tumor part (see
Case Illustration 5.1).
166
Case Illustration 5.1
Large VS Removal with Primary Debulking
Forty-two-year-old male patient presenting
with a history of hearing loss and taste distur-
bance since several years and gait instability
since less than 1 year. During the last few
months, he complained of strong headache and
visual problems. His neurological examination
a b
c d
Preoperative MR scans of the patient demonstrating a
tumor in the right CP angle with growth pattern and
imaging characteristics, typical for a VS. (a)
T1-weighted contrast-enhanced sequence. (b) CISS
sequence. Intraoperative images illustrating the stages
of tumor removal. (c) Initial overview of the tumor and
the CP angle, demonstrating the relations to the tumor
to surrounding structures: the tumor is attached to the
posterior petrous bone, and the porus of the IAC is not
visible. Hence, debulking had to be performed initially
5 Vestibular Schwannomas
revealed trigeminal hypesthesia (V2, V3) and
severe hearing loss (more than 70-dB loss in
the main speech area) on the right side and
severe gait ataxia. MRI examination demon-
strated a large VS on the right side with a larg-
est diameter of 4.5 cm, severe compression of
the brain stem, and hydrocephalus (a, b).
details of surgery are shown on c-j
(d). Then, the posterior wall of the IAC could be
exposed. The overlying dura was excised in the stan-
dard technique (e), and the bone was drilled off (f).
The intracanalicular segment of the facial nerve was
exposed distal to the tumor (g) and could be dissected
medially to the area of the porus of the IAC (h).
(i) Dissection of the proximal segment of the facial
nerve from the tumor. (j) Complete tumor removal and
preservation of facial nerve (Tu tumor, 7 facial nerve,
BS brain stem)
Management of Patients with VS 167

In the early postoperative period, the patient rapidly. At discharge the patient had complete
complained of headache and was drowsy due to right-sided hearing loss and HB grade IV facial
a moderate pneumocephalus. Within several palsy. One year later the facial nerve function
days he recovered completely. His gait improved was HB grade II and the gait was normal.

e f

g h

i j
168 5 Vestibular Schwannomas

Opening of the IAC

The important anatomical landmarks are the
tentorium, the superior petrosal sinus, and the
caudal cranial nerves. In smaller tumors the porus
and the seventh-eighth cranial nerves can be
directly seen. During the IAC opening, the struc-
tures that are at highest risk are the common crus,
the vestibular aqueduct, and the posterior semi-
circular canal: if hearing is to be preserved, these
structures should not be destroyed. The bone
structure of this area of the pyramid, the degree
of its pneumatization, and the location of semi-
circular canals are highly variable. Since no ana-
a
tomical landmarks exist to indicate their location
during surgery [86], these variations can be best
appreciated when studying the high-resolution
bone-window thin-slice CT images (Figs. 5.13,
5.14, and 5.15). CT-based neuronavigation guid-
ance has been shown to be useful for the intraop-
erative localization of the pyramid structures. Its
accuracy is highest if bone-implanted reference
markers are used [193]. Although neuronaviga-
tion support has been recommended during the
early phases of the learning curve [171], its prac-
tical value is rather limited.
The dura behind the IAC is incised circumfer-
entially, stripped off the petrous bone, and b
excised (Fig. 5.16a). If the dura is not removed
adequately, drilling may tear it and cause rupture Fig. 5.13 Variable position of the labyrinth in relation to
of the venous sinuses. The incision starts imme- the IAC
diately below the superior petrosal sinus. In
order to avoid injury to the endolymphatic sac however, BAEP monitoring shows that the con-
and duct, the dura should not be incised inferior duction of the cochlear nerve might be disturbed
to the level of the small bone ridge, projecting during bone removal, regardless how careful it
along the lower edge of the IAC (Fig. 5.16a). has been performed. The pathophysiological
High-speed pneumatic drill provides safe and mechanism behind this indirect functional
controlled bone removal. It is advisable to begin disturbance is still unknown, and its evolution
the drilling with a large-sized diamond burr cannot be predicted. In most cases, however, it
(4–5 mm in diameter) and then change to smaller recovers spontaneously. The last bone layer or
burrs. Highly experienced surgeons might prefer lamina may be safely removed with a small
to begin with a cutting burr in order to spare platelet knife.
time. Irrigation is used for cooling and for The extent of lateral opening of the IAC in the
removing the bone dust during drilling. The uti- direction of the fundus is determined by the
lization of a suction–irrigation device has proven extension of the tumor, as estimated at surgery.
to be very helpful throughout the surgery. The canal is opened or “unroofed” over 180° of
Removing the bone layer by layer in 1-mm incre- its circumference in order to obtain sufficient
ments under direct visual control helps to avoid access (Fig. 5.16): an unobstructed view of supe-
injury to underlying structures. Not infrequently, rior and inferior tumor poles should be provided.
Management of Patients with VS 169

a b

c d

e f

Fig. 5.14 Variable structure of the pyramid, posterior to
the IAC (arrows). The bone layer that has to be drilled off
can vary from very thick (a) to moderate one (b, c). Note
the left–right difference of the posterior wall thickness on
image a. It may contain air cells (d–f), which have to be
recognized before beginning the bone removal in order to
avoid inadvertent injury to the IAC components. The air
cells have to be differentiated from the jugular bulb: this is
possibly due to their different CT characteristics (compare
Figs. 5.14e and 5.21)
170
a b
Fig. 5.15 CT (a, b) and MR (c, d) (images of a patient
with a moderately large VS on the left side (a, b).
(a) Preoperative and (b) Postoperative bone-window CT
In case that only a narrow trough is made over
the posterior aspect of the IAC, the identification
and preservation of the cranial nerves are
extremely difficult. The distance to the fundus is
estimated intermittently by palpating with an
angled microhook (Fig. 5.16c). As discussed
earlier in the text, MRI gives only an approxi-
mate estimation of the extent of lateral extension
of the VS and is not a fully reliable guidance. If
hearing-preserving surgery is planned and the
position of the labyrinth precludes further lateral
bone removal, part of the tumor may remain hid-
den (Figs. 5.17 and 5.18). The technique to
remove such tumors completely and to preserve
5 Vestibular Schwannomas
scan, demonstrating the amount of bone removal from the
posterior wall of the IAC. See Fig. 5.16 for intraoperative
details
the cranial nerves function is discussed later in
this chapter.
The amount of bone removal is not equal in
the medial and lateral parts of the canal: the
medial part is opened wider. The superior and
inferior lips of the porus of the IAC should be
drilled off (Figs. 5.15 and 5.16). Ideally, the dura
covering the intracanalicular tumor part should
be preserved during bone removal.
In order to prevent CSF leaks at the end of sur-
gery, the IAC is filled with multiple small fat
pieces, harvested from the subcutaneous fat layer
of the incision, and sealed with fibrin glue. Our
experience has shown that the application of fat
Management of Patients with VS 171

a a

b b

Fig. 5.17 Bone-window CT demonstrating the extent of

lateral opening of the IAC. (a) The position of the semicir-
cular canals allow for safe opening of the IAC up to the
fundus. (b) In this case the most lateral part of the poste-
rior IAC wall was not removed in order to avoid injury to
the labyrinth: a small bone lip hinders the direct visualiza-
tion of the fundus. Its tiny size, however, did not hinder
the safe and complete tumor removal. (c) Wide opening of
the IAC lateral to the fundus: the bony wall of the lateral
semicircular canal wall has been slightly opened (the
patient had no useful hearing prior to surgery)

Fig. 5.16 Opening of the posterior wall of the IAC—

intraoperative images. (a) The dura behind the IAC is
excised widely, taking care not to damage the endolym-
c phatic sac and duct. The arrow points at the inferior extent
of dural excision, which is usually marked by a small bone
ridge. SPS superior petrosal sinus. (b) Bone removal should
be approximately 180° of the circumference of the IAC.
Note the wide removal of the superior and inferior lips of the
porus of the IAC (asterisks). (c) The distance to the fundus
can be determined by palpating with an angled microhook
172 5 Vestibular Schwannomas

in the IAC area should be explicitly stated in the

discharge letters to avoid misinterpretations at
follow-up MR imaging (Fig. 5.19). Not
infrequently, the fat signal is considered as tumor
remnant or recurrence.

High Jugular Bulb

An important consideration during the approach
planning is the jugular bulb position. The wall of
the bulb is thinner and more vulnerable if com-
pared to that of the sigmoid sinus, the latter being
composed by two layers [100]. Inadvertent injury
to the jugular bulb can lead to massive bleeding
and air embolism. Normally, the bulb is situated
c at the inferior surface of the pyramid, inferopos-
terior to the IAC [214]. The position of the bulb
Fig. 5.17 (continued)

a b

c d

Fig. 5.18 In this patient the VS did not extend up to the (b) Bone-window CT before surgery. (c) MR examination
fundus, and it was not necessary to remove the whole pos- following the complete tumor removal (T2-weighted
terior wall of the IAC. Therefore, a targeted bone removal sequence). (d) Postoperative bone-window CT
was performed. (a) Preoperative CISS MRT sequence.
Management of Patients with VS
a b
c d
Fig. 5.19 Postoperative MR images demonstrating the
fat plug in the area of the right IAC. The fat has typical
signal characteristics in various MRI sequences (a) Native
is variable but can be precisely estimated on the
preoperative bone-window CT. In case the jugu-
lar fossa is above the plane of the inferior border
of the IAC, the jugular bulb is considered as
“high.” In a previous study, we found high
jugular bulb in 9 % of all patients [214].
The classification of the high jugular bulbs’
grade that we apply currently is presented below
and is illustrated on Fig. 5.20:
• Grade I: The jugular bulb is less than 2 mm
above the inferior border of IAC (the jugular
bulb and IAC are seen concomitantly at only
one axial CT slice of 2 mm).
• Grade II: The jugular bulb is between 2 and
4.0 mm above the inferior border of the IAC
173
T-1 weighted sequence. (b) T-1 weighted sequence fol-
lowing contrast administration. (c) T-2 weighted sequence.
(d) T2-CISS sequence)
(jugular bulb and IAC are seen concomitantly
in two axial CT slices).
• Grade III: The jugular bulb is more than
4.0 mm above the inferior border of IAC (jug-
ular bulb and IAC are seen in three or more
axial CT slices).
The position of the bulb has never been an
obstacle for sufficient opening of the IAC and—
if the drilling is performed carefully—does not
increase the morbidity rate. In order to avoid
complications, the opening of the IAC should be
planned carefully according to the CT findings
(Fig. 5.21).
In patients with highly located jugular bulb,
corresponding to grade I or II, drilling is
174
IAC IAC
JB
JB
Grade I Grade II
Fig. 5.20 Illustration of the 3 grades of bulb position (Reprinted from Shao et al. [214]. Copyright © 1993; with
permission from Wolters Kluwer Health)
performed superior to the jugular fossa; thus, the
jugular bulb can be avoided (Figs. 5.22, 5.23, and
5.24). In grade III cases, opening of the jugular
fossa is usually necessary in order to open the
IAC sufficiently. The drilling is continued in 0.1-
mm increments until the jugular bulb is identified
under a thin shell of bone. Performing jugular
vein compression at the neck enhances its
identification. The last bone layer is removed
carefully, and the bony wall of the jugular fossa
is opened. The jugular bulb is then mobilized
slightly from all sides and compressed with a
microdissector. Its shrinkage creates more space
to proceed with the drilling around it (Figs. 5.25
and 5.26). In case bleeding from the bulb occurs,
it can be controlled with Tabotamp (or Surgicel);
occasionally, it has to be sealed off with fat pieces
and fibrin glue.
Evaluation of the surgical outcome, in terms
of completeness of tumor removal and func-
tional results, did not show any major differ-
ences between patients with low and high bulb
position [214].
5 Vestibular Schwannomas
IAC
JB
Grade III
Vascular Considerations
The location of the CP angle vessels cannot be
inferred from the diagnostic images with certainty.
Good knowledge of the normal microanatomy
and the possible relations of the main arteries and
veins to the tumor are a prerequisite for successful
VS surgery.
The AICA is the artery most closely related to
VS (see Chap. 2). According to the study of
Sunderland [237], in 64 % of all cases the artery
is intimately related to the seventh-eighth nerve
complex within or at the orifice of the IAC. The
growing VS usually displaces the artery inferi-
orly and posteriorly. In more than 91 % of large
VSs, it is adherent to the tumor capsule [202]. In
some cases a loop of the artery may be covered
by the dura of the pyramid or even protrude deep
into the IAC. If this is not recognized timely and
tumor removal proceeds as usual, the vessel may
be damaged, which has usually catastrophic con-
sequences. The vessel should be initially dis-
sected free from the arachnoidea and from the
dura and only then mobilized. A cuff of dura can
Management of Patients with VS
a
b medullary syndrome.
c tion, performed by the assistant, ensures a clear
Fig. 5.21 Bone-window CT of three patients demon-
strating high position of the jugular bulb (arrow). If the
IAC is opened in the standard technique (dotted line on a),
both the jugular bulb and the integrity of the labyrinth
would be endangered
175
be left around it to avoid any perforator injury.
The dissection of the artery may require occa-
sionally partial tumor debulking and drilling off a
part of the petrous bone (Fig. 5.27).
Infrequently, bleeding can occur from a small
artery under the dura or in the petrous bone—the
subarcuate artery—that enters the subarcuate
fossa on the posterior wall of the IAC. This artery
can be safely coagulated. The internal auditory
artery or the “labyrinth artery” originates from
the anteroinferior cerebellar artery. It runs close
to the seventh-eighth nerve complex, usually
along the inferior surface of the cochlear nerve,
and gives several small feeding vessels to these
nerves and supplies the dura and bony labyrinth.
Injury to the labyrinth artery may cause hearing
loss or rarely facial nerve palsy.
The PICA is located inferior to the tumor and
may also be strongly adherent to its capsule. In large
tumors the PICA or its branches may pass through
the tumor. Recurrent perforating branches—from
AICA or PICA—that supply the brain stem may
run toward the IAC initially and then turn medially;
see Fig. 5.28. It is of utmost importance to identify
and preserve all these vessels in order to prevent
major neurological dysfunction, such as the lateral
Although VSs have variable degree of vascu-
larization, most tend to bleed strongly during
surgery. Hypervascular variants, demonstrated on
MRI as multiple flow voids in and around the
tumor, however, are rarely encountered. Normal
vessels should be differentiated from pathologi-
cal tumor vessels, which may be safely coagu-
lated. End arteries that terminate in the tumor can
also be coagulated but have to be differentiated
form en passage vessels. If the patient is operated
in the semi-sitting position, coagulation during
tumor removal is seldom required: only in case of
bleeding from larger vessel. The frequent irriga-
operative field. Thus, the risk of thermal injury to
the cranial nerves can be avoided. Coagulation is
reserved for final hemostasis.
The bridging petrosal vein/s are easy to pre-
serve in small VS. In large tumors they may be
176 5 Vestibular Schwannomas

a b

c d

e f

Fig. 5.22 High jugular bulb: grade I, preoperative CT (a–c) and MRI (d–f), in a patient with VS grade 3. The
intraoperative findings are presented on Fig. 5.23
Management of Patients with VS 177

a b

c d

Fig. 5.23 High jugular bulb, grade I. Intraoperative
images illustrating the consecutive steps of IAC opening
and tumor removal. (a) Initial view of the CP angle; dot-
ted line is area of the pyramid, posterior to the IAC, that
will be drilled off. (b) The dura overlying this area has
been excised. (c) A few mm from the bone have been
removed, and the dome of the jugular bulb has been
exposed (arrow). (d) Further drilling with a diamond burr,
wider exposure of the bulb (e and f arrows), and careful
mobilization with a dissector (arrowhead). The IAC is
then opened by drilling superior to the jugular bulb (g),
the intracanalicular tumor part is removed, and the CP
angle part is debulked with an ultrasonic aspirator (h).
The tumor capsule is dissected from the cochlear nerve (i;
CN cochlear nerve) and the facial nerves (j; 7 facial nerve).
(k) At the end the most adherent part of the capsule close
to the porus of the IAC is removed. (l) Complete tumor
removal with preservation of the anatomical integrity of
the cranial nerves. (m) Schematic presentation of the tech-
nique of IAC opening in case of grade I high jugular bulb
(IAC internal auditory canal, JB jugular bulb, DD dia-
mond burr) (m: Reprinted from Shao et al. [214].
Copyright © 1993; with permission from Wolters Kluwer
Health)
178 5 Vestibular Schwannomas

e f

g h

i j

Fig. 5.23 (continued)

Management of Patients with VS 179

k l

IAC IAC

JB

JB

Fig. 5.23 (continued)

180 5 Vestibular Schwannomas

a b

c d

Fig. 5.24 High jugular bulb, grade I: postoperative CT (a, b) and MRI (c–e) images of the patient from Fig. 5.23
Management of Patients with VS 181

a b

c d

IAC IAC

JB

JB
DD

e f

Fig. 5.25 High jugular bulb, grade III. (a–c) Bone-
window CT demonstrating the high jugular bulb (grade
III). (d) T1-weigheted contrast-enhanced MRI of the VS.
(e) T2-CISS. (f) Schematic presentation of the technique
of IAC in case of grade III high jugular bulb (IAC internal
auditory canal, JB jugular bulb, DD diamond burr). (g)
Postoperative CT demonstrating the amount of petrous
bone opening, which allowed for safe and complete tumor
removal (h) (f: Reprinted from Shao et al. [214]. Copyright
© 1993; with permission from Wolters Kluwer Health)
182 5 Vestibular Schwannomas

g h

Fig. 5.25 (continued)

a b

Fig. 5.26 CT scans of a patient with a facial nerve The high jugular bulb (grade III) was not an obstacle to
schwannoma, located in the CP angle and protruding deep the complete opening of the canal up to its fundus (b)
into the IAC. (a) Preoperative. (b) Postoperative images.
Management of Patients with VS 183

a b

c d

Fig. 5.27 VS grade 3a on the right side (a–c). A loop of exposing the underlying artery (e). Then, the tumor could
AICA was seen to enter the IAC (d). The posterior wall of be dissected from the cochlear and facial nerves (f) and
the canal was removed and the dura incised carefully, removed completely (g)
184 5 Vestibular Schwannomas

e f

Fig. 5.27 (continued)

Management of Patients with VS 185

a b

c d

Fig. 5.28 VS grade 4b on the left side (a and b:
T1-weighted axial and coronal images following gadolin-
ium administration. c: T2- weighted sequence. d: CISS
sequence). (e–i) Intraoperative images illustrating the dis-
section of the tumor from the vascular structures and their
preservation. (e) Initial view of the tumor. The dura overly-
ing the posterior wall of the IAC has been excised. (f) A
large vessel, which later on proved to be a branch of the
AICA, was strongly displaced posteriorly and adherent to
the tumor capsule (Tu VS, arrows branch of the AICA).
(g) In order to avoid injury to the vascular structures or cra-
nial nerves, “controlled aspiration” with the ultrasonic aspi-
rator (CUSA or Sonoca) is performed: the VS is held with
tumor forceps (left hand), while careful aspiration is per-
formed with the right hand. (h) Gradually the facial nerve
was identified (FN). (i) Complete removal of the tumor with
preservation of the facial nerve (FN), the AICA (arrow), the
petrous vein (asterisk), and the trigeminal nerve (5)
186 5 Vestibular Schwannomas

e f

g h

Fig. 5.28 (continued)

Management of Patients with VS
dislocated or adherent. Still, every effort should
be made to save them because their interruption
might have unpredictable consequences.
Removal of the Intracanalicular Tumor
Portion
The meatal dura is incised parallel to the inferior
lip of the IAC, stripped off the underlying tumor,
and excised. The dural attachments of the VS in
the area of the superior and inferior lips of the
porus should be incised, which allows for its
greater degree of mobilization. The nerves in
their intracanalicular segment are with preserved
anatomical shape almost always: the facial nerve
is displaced typically anterior to the tumor, supe-
rior to the cochlear nerve. An attempt is made to
identify the facial nerve between the superior
tumor pole and the upper wall of the IAC. If this
is not possible without undue traction, the tumor
mass can be removed layer by layer with an
angled hook or microdissector until the nerve is
seen. In case the tumor is of harder consistency, it
may be necessary to debulk it initially with an
ultrasonic aspirator (CUSA or Sonoca). The
cochlear nerve is identified below the facial nerve
at the anteroinferior or inferior part of the IAC. In
larger tumors the vestibular nerves are partially
or completely destroyed by the tumor. In tumors
that do not fill the entire IAC, they can be
identified: either proximal or distal to the tumor.
These nerves are incised several millimeters away
from the tumor attachment, at a point that is com-
pletely free from tumor infiltration. In small
tumors the healthy vestibular nerve can be pre-
served. The tumor is dissected and mobilized
from the facial and cochlear nerves in a lateral-
to-medial direction (Figs. 5.29 and 5.30). In case
the tumor is very adherent to the nerves, this step
is performed later, following the identification of
the nerve in the CP angle. The tumor part that
extends to the fundus is removed with an angled
microhook. Performing gentle palpations with
the hook even tiny tumor parts can be identified.
This lateral part of the IAC can be directly
inspected with a mirror or an angled endoscope.
187
In some VSs this part is not adherent to the nerves
and can be easily mobilized and removed. In case
of intense adhesions, however, the dissection
should be performed with utmost care not to
injure the nerves.
Removal of the Extrameatal Tumor Part
Tumor growth in the CP angle dislocates the
adjacent cranial nerves in different directions. In
a study of 1,006 patients, Sampath et al. [202]
found that the facial nerve is most commonly
located on the anterior middle third and on the
anterior superior third of the tumor capsule,
regardless of tumor size. Much more rarely it is
located on the superior tumor pole, on its anterior
inferior third, or on the inferior pole. The authors
found that in some patients (<3 %) the nerve was
located posterior. The senior author (MS), how-
ever, had never encountered a facial nerve poste-
rior to a VS. Sampath et al. [202] showed that the
facial nerve passed through the tumor in 2.9–
3.4 % of the cases, with similar frequency in
small and in large tumors. We believe this can be
observed in tumors infiltrating the nerve, facial
schwannomas or in multilobulated tumors, asso-
ciated with NF-2. The cochlear nerve has a more
constant location: it is usually splayed along the
anterior inferior third or the inferior surface of
the tumor [41, 197].
The location of the nerves in relation to the
tumor may be directly visualized preoperatively
in patients with small VS with the constructive
interference in steady-state (CISS) MRI study. In
large tumors that fill the CP angle cistern, the
location of the nerves cannot be determined. In
such VSs the facial nerve can run rostrally—even
reach the axilla of the trigeminal nerve—and then
curve down and laterally toward the IAC [43].
DTI-based fiber tracking appears to be a promis-
ing technique for the depiction of the facial nerve
in tumors filling the CP angle cistern [76].
The first step of removal of the extrameatal
tumor, regardless of its size, is tumor debulking
or internal decompression (Fig. 5.32a). Slight
pulling of the remaining tumor part into the
188 5 Vestibular Schwannomas

a b

Fig. 5.29 Bone-window CT (a), T1-weighted MR image following gadolinium administration (b axial, c coronal
slices), demonstrating a small right-sided VS with minimal protruding into the CP angle (see Fig. 5.30)

operative field and dissection of its capsule in the
arachnoid plane follows. In large and giant
tumors, these steps are repeated alternatively in
all directions. Debulking is best performed with
an ultrasonic aspirator (CUSA or Sonoca—
Fig. 5.31) with a middle- or small-sized tip,
selected according to tumor size. The ultrasonic
aspirator allows for gentle and controlled removal
and avoids excessive pressure on the underlying
structures. Alternatively, a platelet-shaped knife
can be used to debulk the tumor. Tumor debulking
should proceed very carefully and systematically
in all directions. Creation of a deep and narrow
hole should be avoided. If the debulking is con-
tinued too far anteriorly, the tumor capsule can be
penetrated and the facial nerve can be damaged.
The main principle is that attempt to mobilize the
remaining tumor and to perform dissection should
be made only after sufficient internal decompres-
sion has been achieved: if the remaining tumor is
not easily mobilized, further debulking should be
performed.
Optimally the facial and cochlear nerves are
identified medial to the tumor close to their entry
Management of Patients with VS
a b
d e
Fig. 5.30 Intraoperative images illustrating the steps of
tumor removal (Patient from previous figure). (a) Initial view
of the CP angle and the seventh-eighth nerve complex. (b)
Dural incision around the posterior wall of the IAC. (c) The
IAC has been opened and its dura has been excised, exposing
the intracanalicular tumor. (d) The tumor is dissected from the
or exit zone on the brain stem surface by slightly
pulling the tumor in a superior direction. In large
VSs this is possible only after considerable reduc-
tion of the tumor volume. The identification of the
nerves medially is guided by their relationship to
the anatomical landmarks (see Chap. 2). The
nerve electrical stimulator is applied frequently:
for mapping of the nerve or for testing its func-
189
c
f
facial and cochlear nerves and removed completely. (e) The
anatomical and functional integrity of the nerves has been
preserved, as proven by the electrical stimulation of the facial
nerve and the preservation of the BAEP wave amplitude and
latency. (f) The dura was used to seal the IAC—a practice that
has been abandoned by the senior author since a long time
tional condition. The integrity of the facial nerve is
monitored throughout the surgery by continuous
facial electromyography and facial nerve motor
evoked potentials recording.
The nerve–tumor interface should be clearly
identified, and the dissection should proceed
always in the arachnoid cleavage plane
(Fig. 5.32b–d). The three-hand technique, which
190
a b
Fig. 5.31 Ultrasonic aspirators that we use for tumor debulking: (a) CUSA (Integra Neurosciences) and (b) Sonoca
(Söring)
is possible if the patient is in the semi-sitting
position, is extremely useful: the surgeon uses
both his hands for tumor preparation and removal,
while the assistant irrigates the operative field.
The operative field is thus cleared and coagula-
tion can be avoided. The dissection is performed
bimanually: the arachnoid layer on the tumor sur-
face is gripped and gently peeled off the tumor
with microforceps. At the same time the second
hand is holding the tumor. In case the dissection
is not in the correct plane, the fragile nerves can
be destroyed or their vascular blood supply, which
is in the subarachnoid space, can be interrupted.
The dissection proceeds in a methodical fash-
ion from the brain stem toward the IAC. It should
be performed in alternative directions, and over-
stretching in one direction should be avoided.
Importantly, the tumor rest should be elevated in
a superior direction, which allows visualizing the
lower margin of the facial nerve. The attempt to
5 Vestibular Schwannomas
dissect the facial nerve from superior (pulling the
tumor inferior) may be very dangerous for the
nerve and should be avoided. Another important
consideration is that the dissection should be
performed from medially to laterally even if both
ends of the facial nerve are well visible. The
attempt to dissect the tumor from the facial nerve
in a lateral-to-medial direction—from the IAC
toward the brain stem—is also related to a high
risk of nerve transection.
In the area near the porus, the nerves are espe-
cially fragile and adherent to the tumor capsule.
This portion is dissected at the end when both the
proximal and distal portions of the facial nerve
have been freed from the tumor. Following tumor
removal the facial nerve is stimulated at its most
proximal region of the brain stem root exit zone
to verify its functional condition.
Tumor variables influencing the surgical strategy:
• Tumor size
Management of Patients with VS 191

• Tumor extension in the IAC • Arachnoid membrane/cleavage plane

• Tumor extension in the CP angle
• Tumor consistency
• Tumor capsule
• Tumor vascularization
• Cystic tumors
• Adherence to neural structures
• Previous surgeries
• Previous radiotherapy/radiosurgery

Fig. 5.32 Schematic

illustration of the steps of VS
removal. (a) Internal
decompression of the
extracanalicular tumor part.
(b, c) Dissection from the
cranial nerves, using the
2-hands technique.
(d) Closure of the IAC with
b
fat pieces or muscle
192 5 Vestibular Schwannomas

Fig. 5.32 (continued)

d
Management of Patients with VS
Intracanalicular (Extension Grade 1) VS
Pure intracanalicular VS or VS grade T1,
according to our tumor extension classification,
comprised less than 10 % of all VSs operated
by the senior author [194, 198]. Some clinical
and epidemiological observations indicate that
these VSs may form a separate subgroup with a
specific natural evolution. The diagnostic delay
in such schwannomas is shorter. In a previous
publication we showed that age distribution
has an inverse linear correlation to tumor exten-
sion and size [145]. Patients with intrameatal
tumors were symptomatic at a more advanced
age than those with larger tumors (48.9 vs. 44.4
years). The duration of subjective deafness and
dizziness were shorter but the duration of tin-
nitus was longer. The incidence of tinnitus was
highest in intracanalicular tumors: 86 % in VS
extension grade T1, 65 in grade 2, 66 % in
grade T3, and 51 % in grade T4. Cranial nerve
symptoms/signs correlated positively with the
degree of tumor extension: vestibular nerve
signs were most pronounced in VSs grade T1.
Expectedly functional hearing rate was highest
in intracanalicular tumors.
Intracanalicular VSs occur in several varieties
according to the extent of IAC involvement:
• VSs limited to the medial IAC near the porus
acusticus
• VSs limited to the lateral part of the IAC near
the fundus
• VSs involving the entire IAC
In regard to the bony changes, grade T1 tumors
cause local, diffuse, or no dilatation of the IAC.
The management options of intracanalicular
VSs are microsurgery, stereotactic radiosurgery,
and follow-up or “wait-and-scan” strategy. The
last two options have been discussed comprehen-
sively elsewhere in the text. In summary, initial
observation may be acceptable option in elderly or
somatic unstable patients with mild nonprogres-
sive symptoms, in patients with complete hearing
193
loss, in patients with VS on the only hearing side,
or those unwilling to undergo surgery. Radiosurgery
could be recommended to patients whose somatic
condition prohibits open surgery or to those insist-
ing on this type of management.
Considering the size of the tumor and its slow
growth tendency, the decision when to perform
surgery should be taken by the patient. If surgery
is not scheduled, a thorough radiological follow-
up to detect early any change in VS’s size is war-
ranted. Surgical removal, however, is indicated in
patients with useful hearing and good BAEP: in
order to have a chance for hearing-preserving
surgery, it should be done prior to its deteriora-
tion. As pointed earlier in the text, the occurrence
of hearing loss is not related to the size of VS and
can be of sudden nature. Rare indications for
early surgery are severe intractable vestibular
problems. Some small VS may cause so severe
vertigo that removal of the tumor with sectioning
of the involved vestibular nerve/s is the only
therapeutic solution (Fig. 5.33).
The principles of intracanalicular VS surgery
were described above in the section on Removal
of Intracanalicular VS. Important consideration
is that they are a heterogeneous group of tumors
and the surgery should always be targeted to the
specific features of each individual case (see Case
Illustrations 5.2 and 5.3).
Tumor extension and size are main predic-
tors of the functional and general outcome of
surgery. Complete tumor removal is possible in
more than 99 % of intracanalicular schwanno-
mas—exceptions are those cases in which the
tumor infiltrates the facial nerve. The facial
nerve anatomical preservation rate in intrac-
analicular VSs in our experience is 100 % and
that of the cochlear nerve, 94 % [179]. Excellent
facial function (House–Brackmann grades I
and II) is achieved in almost all patients. Useful
hearing is preserved in 57 % of those patients
with good preoperative hearing level.
194 5 Vestibular Schwannomas

a b

Fig. 5.33 This 29-year-old patient presented with severe
vertigo and nystagmus to the left side. Hearing on the right
side was only slightly decreased (class II according to the
New Hannover classification) and on the left side was nor-
mal. The MRI demonstrated very small VS in the lateral
part of the IAC on the right side (a–c, arrows). The epi-
sodes of vertigo were so frequent and severe that the
patient was unable to have normal life. Therefore, consid-
ering all possible management options, she decided for
surgery. The tumor was removed via the RS approach;
facial nerve function and serviceable hearing were pre-
served. The vertigo was initially stronger but within 2
weeks disappeared almost completely and in 2 months
resolved
Management of Patients with VS
Case Illustration 5.2
Elongated VS Extension Grade T1, Normal
IAC
This 55-year-old patient presented to our insti-
tute complaining of tinnitus and progressive
hearing loss since 1 year. Her neurological
examination revealed no abnormalities, besides
a complete loss of hearing on the right side.
a b
c d
Preoperative CT and MRI revealing the grade T1 VS on
the right side: the tumor has an elongated shape and did
not cause dilatation of the IAC. (a) Bone-window CT.
(b, c) T1-weighted sequences after contrast administra-
tion. (d) CISS sequence. Intraoperative images. (e)
Dural incision along the transverse and sigmoid sinuses.
A retractor (R) is used to elevate the cerebellar hemi-
sphere, and the lateral cerebellomedullary cistern is
opened, releasing CSF. The lower cranial nerves are vis-
ible. (f) Only when sufficient CSF is drained, a retractor
is placed to hold the cerebellum without any tension. (g)
195
MRI study demonstrated an intracanalicular
tumor in the right IAC, extending lateral up to
the fundus. The tumor had an elongated form,
and the IAC was not dilated. The patient decided
for surgery, and the tumor was removed com-
pletely via the RS approach (a–d). The postop-
erative period was completely uneventful. At
discharge she had normal facial nerve function.
Excision of the dura around the posterior wall of the
IAC. (h, i) Exposure and removal of the intracanalicular
VS. The facial (7) and cochlear nerves (8) are preserved.
(j) The dotted line indicated the amount of bone that had
to be drilled off additionally in order to gain access to
the most lateral part of the tumor. The distance to the
fundus has been determined by palpation with an angled
microhook. (k) The hook points to the transverse crest
of the fundus. (l) Complete tumor removal and preser-
vation of the facial and cochlear nerves (V—vestibular
nerve, from which the tumor originated)
196 5 Vestibular Schwannomas

e f

g h

i j
Management of Patients with VS 197

k l
198
Case Illustration 5.3
VS of the Lateral IAC, Slightly Enlarged
IAC
Sixty-year-old male patients with com-
plaints of strong vertigo and slight hearing
loss on the left side. The audiogram and the
speech discrimination test showed preserved
useful hearing (class H1 according to the
New Hannover hearing classification). The
MRI study of the patient is presented on
figures (a–c): a small intracanalicular VS is
seen, located in the lateral part of the IAC.
a b
Preoperative MRI of the patient demonstrating the VS
located in the lateral part of the IAC: (a, b) T1-weighted
sequences after gadolinium administration. (c) CISS
sequence. Intraoperative images. (d) The posterior wall
of the IAC has been removed and the tumor located in
the lateral part of the IAC is exposed. (e–h) Stepwise
5 Vestibular Schwannomas
The patient was operated via the RS
approach with the goal to remove the tumor
completely and preserve the facial nerve
function, as well as to attempt hearing pres-
ervation. The details of surgery are pre-
sented on figures (d–j) and the postoperative
MRI on figures (k, l).
The postoperative period was completely
uneventful. At discharge—14 days after
surgery—the patient had normal facial nerve
function and useful hearing (class H2 according
to the New Hannover hearing classification).
dissection of the tumor from the cranial nerves and
removal. (i) Complete tumor removal with preservation
of the functions of the facial and cochlear nerves. (j) The
IAC has been sealed off with multiple fat pieces and
fibrin glue. (k, l) Postoperative MRI performed 6 months
after surgery: no recurrences or remnants are detectable
Management of Patients with VS 199

d e

f g

h i
200 5 Vestibular Schwannomas

j k

l
Management of Patients with VS
Giant (Extension Grade T5) VS
Giant VSs or grade 5 VSs are defined as those
that measure >4.0 cm or extend over the midline.
They always cause significant brain stem com-
pression and typically contact and/or compress
both the trigeminal and the lower cranial nerves.
The successful management of such tumors is
especially challenging: the operation is more
complex, the general operative risks are higher,
and the functional facial and cochlear nerves
preservation is extremely demanding. Surgery
with the primary goal of brain stem decompres-
sion and alleviation of the intracranial hyperten-
sion is the only treatment option in giant VSs.
The opinions on the extent of surgery in the lit-
erature vary: from partial or subtotal removal fol-
lowed by observation or radiosurgery to complete
removal in 1, 2 or more stages [1, 5, 21, 98, 186].
We believe that even in giant VSs complete surgi-
cal removal and preservation of facial nerve func-
tion should be the goal. In case useful hearing is
present before surgery, an attempt to preserve it
should always be made [196].
Patients with giant VSs typically have severe
hearing loss at presentation (96–100 % of all
patients in different series). The trigeminal nerve
is affected in 40–78 % of the patients, the lower
cranial nerve in 10–14 %, the facial nerve in
0–14 %, and the abducens nerve in 4–7 %
[4, 104, 107, 125]. Gait instability caused by the
compression of the middle cerebellar peduncle
and cerebellum is in 44–88 % of the patients and
long-tract signs due to brain stem compression
in 14–25 %. The mass effect of the tumor and
the frequent hydrocephalus cause intracranial
pressure elevation: we observed signs of intrac-
ranial hypertension in 26 % of our patients with
giant VSs.
The advantages offered by the RS approach
are crucial for achieving optimal operative
results in giant VSs (Fig. 5.34). In such cases
the CP angle neural and vascular structures are
more vulnerable due to the long-standing com-
pression. The underlying brain parenchyma is
frequently softened and tends to bleed dif-
fusely even after slight manipulations. In giant
201
schwannomas it may be extremely difficult to
identify the correct arachnoid plane, in partic-
ular the one to the brain stem. The pontine sur-
face veins can be used as a guide for finding
the proper dissection layer. With the 2-hands
technique—holding the tumor capsule with
one hand with a forceps and performing
dissection with the other hand—inadvertent
injuries to the neural tissue can be avoided
(see Case Illustrations 5.4 and 5.5)
The risk of hemorrhagic complications due
to venous bleeding after surgical removal of
large or giant VSs is higher [186, 266]. The
draining veins in the CP angle might be engorged
and extremely fragile, with increased bleeding
tendency during and after surgery. In order to
detect bleeding from any open or torn bridging
veins, we apply jugular venous compression
twice: at the end of the procedure, while the
retractor is still in place, and after the removal
of the retractor to detect.
The size of the VS is one major predicting fac-
tor for the chance of facial nerve anatomical and
functional preservation [26, 106, 223]. In a
recently published series, including 50 patients
with giant VSs, we found that complete tumor
removal was possible in all cases [196]. The
anatomical integrity of facial nerve was preserved
in 92 % of the patients. In 4 patients facial recon-
struction with hypoglossal–facial anastomosis
was performed. At 12 months follow-up, the
facial nerve function recovered to HB grade IV in
2 of them and to HB grade III in the other 2.
Overall at last follow-up, 75 % of all patients had
excellent or good facial nerve function (defined
as HB grade I–III). Some surgeons believe that it
is even theoretically impossible to preserve hear-
ing in patients with large VS and therefore favor
the hearing-destructing translabyrinthine
approach [23, 269]. Our experience shows though
that even in patients with giant VSs, there is a
chance of hearing preservation, provided the
patient has useful hearing level and normal or
near-normal BAEP preoperatively. In 11 % of
these patients, useful hearing could be preserved
and 33 % had some hearing.
202 5 Vestibular Schwannomas

a b

c d

Fig. 5.34 Preoperative MR images (a–c) of a patient with giant VS on the right side (T1-weighted scans following
gadolinium administration). (d) Postoperative CT, performed prior to discharge 13 days after surgery
Management of Patients with VS
Case Illustration 5.4
Giant VS 1
This 41-year-old patient presented with com-
plaints of progressive hearing loss since 5
years. During the last year his gait became
unstable; since 5–6 months strong headache
a b
(a–c) MRI of the patient (T1-weighted sequences fol-
lowing gadolinium administration), demonstrating a
giant VS—maximal diameter of 4.6 cm. Intraoperative
images. (d) Initial view of the CP angle. (e) The dura
around the posterior wall of the IAC has been incised
and is about to be stripped off the bone with the platelet
knife. (f) Drilling of the posterior IAC wall. (g) The
jugular bulb (large arrow), which was located high
(grade I), was partially exposed. The IAC was opened
203
and blurred vision developed. Ophthalmoscopy
revealed bilateral papilledema. MR examina-
tion demonstrated a very large VS on the left
side, causing severe compression and disloca-
tion of the brain stem (a–c). No signs of hydro-
cephalus were, however, evident. The patient
superior to the bulb (double small arrows). (h) Exposure
of the intracanalicular tumor part and identification of
the facial nerve (i). (j) Removal of the extracanalicular
tumor part. The facial nerve next to the porus of IAC is
marked with an arrowhead. (k, l) Complete tumor
removal and anatomical preservation of the facial nerve
(arrowheads). The abducens nerve (6) is well visible in
the depth due to the massive dislocation of the brain stem
caused by the tumor. (m, n) CT scan 1 day after surgery
204 5 Vestibular Schwannomas

d e

f g

h i
Management of Patients with VS 205

j k

n
206
had complete hearing loss, as shown by the
audiogram, and partial abducens nerve palsy
on the left side.
He has been operated via the RS approach
(d–l) and the tumor was removed completely.
The anatomical integrity of the facial nerve
5 Vestibular Schwannomas
was preserved. No new neurological deficits
appeared and the postoperative period was
uneventful. He has been transferred to the nor-
mal ward the day after surgery and discharged
14 days later. At discharge he had an HB grade
II facial nerve function.
Management of Patients with VS
Case Illustration 5.5
Giant VS 2
The patient presented to our institute with a
history of headache, gait instability, taste dis-
turbance, and hearing loss on the left side
since several years. Neurological examination
revealed trigeminal nerve hypesthesia (V1–3)
and hypacusis (class H3 according to the New
Hannover classification) on the left side, as
well as severe gait ataxia and a central right-
a b
Preoperative bone-window MRI (a–c) and CT (d) of
the patient: giant VS on the left side with a maximal
diameter >5 cm, extreme compression of the brain
stem, and dilation of the lateral and third ventricles.
(a, b) T1-weighted contrast-enhanced sequences. (c)
T2-weighted sequence. The tumor extended up to the
fundus (c) but did not cause significant dilatation of
the IAC (d). Intraoperative images. (e) Initial view of
the tumor in the CP angle. (f) The IAC is opened with
a diamond drill. (g) The adhesions at the inferior and
superior borders of the porus acusticus internus are
released, which allowed better mobilization of the
tumor. (h) Tumor exenteration with an ultrasonic aspi-
207
sided hemiparesis (grade 4/5). MRI demon-
strated a very large tumor on the left side with
imaging characteristics of VS (a–d). The
tumor was removed completely via the RS
approach (the details are presented on figures
e–o). After surgery the patient had complete
hearing loss and HB grade III facial nerve
palsy. At the first follow-up examination 6
months after the operation, however, the func-
tion had already improved to HB grade II.
rator. (i) Identification of the proximal segment of the
facial nerve (7) at the brain stem. (j) Further tumor
removal: internal decompression and dissection at the
arachnoid plane. Longer segment of the facial nerve
(arrows) is now visible. (k) Identification of the lateral
intracanalicular segment of the facial nerve. (l–n) The
facial nerve was further dissected from the tumor cap-
sule: it was splayed (umbrellalike) on the anteroinfe-
rior tumor surface (BS brain stem). (o) Complete tumor
removal and preservation of the facial nerve. (p, q)
Early postoperative CT demonstrating the wide open-
ing of the IAC. Note the preserved semicircular
canals
208 5 Vestibular Schwannomas

c d

e f

g h
Management of Patients with VS 209

i j

k l
210 5 Vestibular Schwannomas

m n

p
o

q
Management of Patients with VS
Case Illustration 5.6
VS with Large Petrous Bone Involvement
This patient (44-year-old male) presented with
a history of a gradual hearing loss on the left
side. Later on, a feeling of heaviness in the ear
appeared, and neuroimaging was performed,
which demonstrated a large VS on the left side
with massive destruction and involvement of
the petrous bone (a–h). At admission he had
left-sided anacusis and otherwise normal neu-
rological status. He was operated via the ret-
rosigmoid route. Initially, the dura and the thin
layer of bone overlying the petrous tumor
were removed, and this part, which reached
from the petrous apex to the jugular foramen
and compressed the lower cranial nerves and
the jugular bulb, was debulked (i). The facial
a b
Preoperative imaging: (a, b) Bone-window CT demon-
strating the wide destruction of the petrous bone. (c–f)
T1-weighted MR images with contrast. (g, h) T2-CISS
images. (Intraoperative images: (i) Exposure of the
tumor; a small bone septum (small arrow) divides the
petrous from the CP angle tumor parts (P petrous bone,
large arrow petrous tumor part). (j) Identification of
211
nerve was identified laterally in the fundus
area and could be preserved (j, k). Then the
CP angle tumor part was debulked and dis-
sected from the cerebellum (l). The facial
nerve was identified at the brain stem and fol-
lowed laterally (m–o). The tumor was removed
completely, and the facial nerve anatomical
integrity was preserved (p). The whole opened
area of the petrous bone was filled with fat
pieces and sealed with fibrin glue.
The patient recovered quickly after surgery.
His early postoperative CT scan is shown on
figures (q–s). On the eighth postoperative day,
however, a rhinoliquorrhoea appeared and
treated with a lumbar drainage for 1 week. At
discharge he had only HB grade IV facial weak-
ness, which at follow-up improved to grade II.
the facial nerve at the fundus and its gradual dissection.
(k) (FN—facial nerve). (l) Debulking of the CP angle
tumor part. (m) Identification of the facial nerve at the
brain stem and its gradual dissection from the tumor
capsule (n, o). (p) Complete tumor removal. (q–s)
Early postoperative CT control. The opened petrous
bone is filled with fat pieces
212 5 Vestibular Schwannomas

c d

e
f

g
Management of Patients with VS 213

i j

k l

m n
214 5 Vestibular Schwannomas

o p

q r

s
Management of Patients with VS
Tumor Adhesion/Presence of Capsule
The degree of adhesion of the tumor capsule to
the cranial nerves is a major predictor of func-
tional and general outcome after VS surgery. VSs
are traditionally described as being well encapsu-
lated. At surgery the tumor surface tends to be
firmer and more vascular than its central regions.
Histologically, however, only an extremely thin
connective tissue layer (3–5 mm) appears to cover
the tumor [121]. The most probable origin of this
layer appears to be the perineurium of the ves-
tibular nerves [205]. VSs represent a heteroge-
neous group of tumors in terms of presence of
such surgical capsule or of an arachnoid plane
between the tumor and the neural structures. The
treatment strategy in each case should be there-
fore individualized according to these character-
istics. In case a distinct arachnoid cleavage plane
is present, the dissection of the tumor is straight-
forward (see Case Illustration 5.7). In a consider-
able number of cases, however, the tumor is
densely adherent to the surrounding CP angle
structures, in particular to the facial and cochlear
nerves. The extent of adhesion cannot be demon-
a b
Fig. 5.35 Preoperative MR images (a–d) of VS with
pronounced perifocal edema. The fourth ventricle was
obstructed with resulting hydrocephalus. Despite the
radiological evidence of edema, however, the dissection
of the facial nerve did not prove to be more difficult and a
good arachnoid plane was available. The adjacent cerebel-
lar parenchyma, however, was softened and prone to
215
strated even with the most advanced neuroimag-
ing techniques and cannot be predicted before
surgery (see Case Illustration 5.8). In contrast to
meningiomas, the presence of perilesional edema
does not necessarily correlate with a lack of
arachnoid plane (Fig. 5.35).
Keeping to the principles of VS microsurgery
allows achieving optimal results even in very
adherent tumors: dissection plane can almost
always be found and should be meticulously fol-
lowed. The tumor should be dissected from the
nerve, not the nerve from the tumor! The stan-
dard technique of dissection by gripping the
arachnoid sheath with two forceps and carefully
stripping it of the capsule, however, is not appli-
cable if the arachnoid plane does not exist. If no
capsule exists, the “onion-skin technique” of
tumor removal may be applied: the tumor is
removed layer by layer—from the inner to outer
one—until the outermost layer is reached and
removed piecemeal (see Case Illustration 5.9).
This modified technique prolongs the operative
time but allows for cochlear and facial nerve
functional preservation.
diffuse bleeding. On the early postoperative CT (e)—1
day after surgery—only air in the CP angle was visible.
The next CT performed 3 days later to control the hydro-
cephalus (f), however, demonstrated small hematoma in
the tumor bed area. The patient remained asymptomatic
and was managed conservatively
216 5 Vestibular Schwannomas

c d

e f

Fig. 5.35 (continued)

In case the adhesion is so strong that the tumor remnant correlates with the risk of recur-
integrity of the nerve is at risk, the most adherent rence, as shown earlier in the text, every attempt
part of the capsule should not be removed (see should be made to remove as much tumor as
Case Illustration 5.10). Since the size of the safely possible.
Management of Patients with VS 217

Case Illustration 5.7 and MR imaging showed an intracanalicular

Intracanalicular VS with Well-Developed
Capsule and Lack of Adhesions
This 43-year-old female presented with a his-
tory of tinnitus, vertigo, and progressive hear-
ing loss on the right side since 12 months. CT
VS (extension grade T1) on the right side,
spreading to the fundus of the IAC (a, b).
Audiogram and speech discrimination test
showed that she still had good hearing (class
H2 according to the New Hannover

a b

c d

7–8

(a) Preoperative MRI of the patient: intracanalicular
VS on the right side (T1-weighted sequences follow-
ing gadolinium administration). (b) Intraoperative
images. (c) Initial view of the CP angle and vestibulo-
cochlear nerve complex (7–8). The dura around the
posterior IAC wall is incised (arrowheads). (d) The
IAC has been opened, but the dura overlying the tumor
is still intact. (e) Partial removal of the tumor. The
superior vestibular nerve, from which the tumor origi-
nated, is visible (VN). (f) The tumor had a well-devel-
oped capsule and was not adherent. It could be
relatively easily dissected from the facial (FN) and
cochlear nerves (CN) and removed completely (g). (h)
The IAC is sealed with fat pieces
218 5 Vestibular Schwannomas

e f

g h
Management of Patients with VS
Case Illustration 5.8
Intracanalicular VS with Very Strong
Adhesions to the Nerves
This 54-year-old male patient was admitted
with a history of hearing loss, tinnitus, and
vertigo since several months. He had been
diagnosed to have a very small tumor in the
lateral part of the left IAC with imaging char-
acteristics of VS (a, b). The audiogram
showed that he had still useful hearing (class
H2 according to the New Hannover
classification.) The patient was informed in
detail on the alternative management options
a b
Preoperative MRI of the patient: small intracanalicular
VS on the left side, located in the lateral part of the IAC
(a) T1-weighted sequences following gadolinium admin-
istration. (b) CISS sequence. Intraoperative images.
(c) Initial view of the CP angle and the vestibulocochlear
nerve complex. The cerebellomedullary cistern has been
opened, and sufficient amount of CSF has been drained.
(d, e) The IAC has been opened, and the tumor with the
219
and decided for surgery, which was performed
via the RS approach (c–h). The tumor was
found to be very adherent to the facial and
cochlear nerves; its removal required pro-
longed and meticulous dissection. Following
surgery the patient had transient mild facial
nerve palsy (HB grade II) that recovered com-
pletely within 3 months. Hearing was slightly
worse than before surgery—class H3 accord-
ing to the New Hannover classification. The
vertigo resolved completely within several
weeks, but the tinnitus became slightly more
annoying.
superior and inferior vestibular nerves (SVN and IVN,
respectively) is seen. (f) The lateral part of the tumor is
dissected from the facial (FN) and cochlear nerves (CN)
and mobilized medially. (g) Despite its small size, the
tumor was extremely adherent to the nerves, and its
removal required prolonged and meticulous dissection.
Still, it could be removed completely, preserving the
integrity of all neural structures (h)
220 5 Vestibular Schwannomas

c d

e f
Management of Patients with VS 221

g h
222
Case Illustration 5.9
VS Without Obvious Tumor Capsule
Fifty-three-year-old male patient was diag-
nosed to have VS on the left side 6 months
prior to presentation to our institute. He suf-
fered from strong left-sided headache, imbal-
ance, and progressive hearing loss since
several years. At admission he had moderate
hearing on the left side (class H3 according to
a b
c d
(a) Preoperative MRI of a patient with a VS in the
left CP angle—extension grade T3b (T1-weighted
sequences following gadolinium administration). (b)
Intraoperative images illustrating the steps of tumor
removal. (c) The posterior wall of IAC has been
drilled off, and the intracanalicular tumor portion is
exposed. (d) Initial removal of the intracanalicular
tumor and identification of the distal segments of
facial and cochlear nerves. (e) Then, the CP angle
5 Vestibular Schwannomas
the New Hannover classification). The MRI
study is presented on figures (a, b).
The tumor was approached via the RS
route. It had no capsule and was “sticking” to
the neural structures (c–h). Therefore, it had
to be removed with the “onion-skin peeling
technique”—piecemeal removal from inside
to outside. The VS was removed completely,
and the anatomical integrity of the facial and
tumor part was approached. Internal debulking has
been performed and the facial nerve identified
(arrow). The VS had no capsule and appeared
“sticked” to the neural structures. (f) Further tumor
removal—mainly piecemeal—from inside to outside.
(g) Last pieces of the tumor are dissected from the
facial nerve. (h) Complete tumor removal. The elec-
trical stimulation of the facial nerve evoked good
response
Management of Patients with VS 223

cochlear nerves was preserved. Still, the was uneventful. At the first follow-up exami-
patient had an HB grade V facial nerve func- nation, the facial nerve function had improved
tion after surgery, and the hearing was not to HB grade II.
useful. Otherwise, the postoperative period

e f

g h
224
Case Illustration 5.10
Incomplete Tumor Removal Due to Strong
Adhesions
The patient presented with a history of pro-
gressive right-sided hearing loss and intermit-
tent tinnitus since 2 years and unsteadiness
since 3 months. At admission he had service-
able hearing on the right side, gait ataxia, and
horizontal spontaneous nystagmus toward the
side of the tumor. The MR examination dem-
onstrated a large right-sided VS (grade T4a),
causing moderate compression of the brain
stem.
The patient was operated via the RS
approach (figures c–n). During surgery very
a b
(a, b) Large VS (grade T4b) on the right side
(T1-weighted sequences after gadolinium adminis-
tration). Intraoperative images. (c) Overview of the
CP angle and the VS (Tu). TS transverse sinus, SS
sigmoid sinus. (d) The pyramid bone has been widely
drilled off (arrowheads), exposing the IAC (black
dots). (e) The intracanalicular tumor part has been
removed, and the facial nerve (7) has been dissected
free. The cochlear nerve is visible inferior to the
facial nerve. BAEP at the beginning of surgery
showed increased latency and decreased amplitude
of both waves III and V. During the bone removal,
they were suddenly completely lost. The retractor
was removed and a pause was made, but the waves
did not recover. (f) Internal decompression of the
5 Vestibular Schwannomas
intense adhesions between the tumor capsule
and the facial nerve in the area next to the
porus acusticus internus were found. The
attempt to dissect this part seemed to be related
to a very high risk of nerve injury or complete
nerve disruption. Hence, a decision was taken
not to remove this tiny tumor part. The patient
recovered well from surgery and was dis-
charged with an improved gait, anacusis, and
an HB grade III facial nerve function on the
right side. He is being followed up with yearly
MR studies. Within 12 months the facial nerve
function improved to an HB grade II. Up to
now—36 months after surgery—the remnant
remains unchanged.
extracanalicular tumor part with CUSA. (g) Gradually
the brain stem (BS) and the facial nerve (7) in its
proximal segment were exposed. PV—petrous vein.
(h) Dissection of the facial nerve. The tumor rest is
held with slight tension in a superior and lateral
direction. (i, j) Further dissection of the nerve by
pulling the tumor laterally. Note the splayed or
umbrellalike shape of the nerve (arrows). (k, l) The
last part to be dissected is the one close to the porus
acusticus internus. (m) Due to the intense adhesions
between the tumor rest and the facial nerve and the
impossibility to dissect the nerve without endanger-
ing its integrity, this small part of the tumor was not
removed (double arrow). The electrical stimulation
of the facial nerve evoked good response (n)
Management of Patients with VS 225

c d

e f
226 5 Vestibular Schwannomas

g h

i j
Management of Patients with VS 227

k l

m n
228
Medial VSs
Medial VSs are located in the CP angle and do
not extend or extend minimally into the IAC,
while the lateral part of the IAC is free of tumor
[96, 248]. Although they are relatively rare—2 %
of all VSs—they deserve special attention because
the tumor may reach a considerable size before
diagnosis [230, 248]. Nevertheless, as shown by
Strauss et al. [230] and by our own experience, the
facial and cochlear nerves are less adherent to the
tumor than in case of tumors extending into the
IAC. The MRI may be misleading when the lateral
tumor extension is evaluated: not uncommonly it
5 Vestibular Schwannomas
does not show an intracanalicular component,
but at surgery tumor extension up to the fundus
may be found. Therefore, regardless of the pre-
operative imaging, the initial step is always to
open the IAC and to identify all relevant nerves
distal to the tumor (see Case Illustration 5.11).
The extent of lateral tumor extension should be
considered when planning the IAC opening: tai-
lored bone removal affords enough space for
tumor removal and avoids unnecessary risks.
The functional outcome of surgery of medial
VSs has been shown to be better than in usual VS
of comparable size [230].
Management of Patients with VS
Case Illustration 5.11
Medial VS
This 45-year-old male patient presented with
a history of progressive hearing loss on the left
side since 5 years and intermittent tinnitus
since 3 years. His neurological examination
was normal, besides the hypacusis and a slight
gait ataxia. The audiogram showed a hearing
a b
c d
(a) Preoperative MRI demonstrating a medial VS in
the left CP angle. The IAC is free of tumor (c, d). Note
the pronounced caudal tumor extension (b). (a, b)
T1-weighted sequences following gadolinium admin-
istration. (c) T2- weighted sequence. (d) T2-CISS
sequence. Intraoperative images illustrating the steps
of tumor removal. (e) Limited opening of the IAC has
been performed, and the facial nerve (FN) has been
identified, applying electrical stimulation (f). The VS
229
loss of more than 90 dB in the main speech
area. MR images demonstrated relatively large
VS with a maximal diameter of 3.7 cm and a
predominant craniocaudal extension (a–d)
with a minimal growth into the IAC, and
medial VS was suspected. The caudal tumor
extension could explain the early and severe
hearing loss.
did not extend into the canal, as demonstrated on the
images. (g) The lower cranial nerves were than dis-
sected free from the tumor (*, **, ***—ninth, tenth,
and 11th cranial nerves, respectively). Image (h) dem-
onstrates the splayed “umbrellalike” facial nerve after
the complete tumor removal (BS brain stem). Despite
this shape, the nerve reacted normally to electrical
stimulation (i)
230 5 Vestibular Schwannomas

The typical RS approach was performed, case of medial VS (e). The tumor originated
but the IAC was opened only in its medial 1/3: from the lower vestibular nerve. The facial
a limited, 2–3-mm, intracanalicular tumor nerve was seen and verified with electrical
extension was seen, proving that this was a stimulation (f). Next, the extrameatal part was

e f

g h

i
Management of Patients with VS
addressed: the VS was found to extend cau-
dally below the level of the caudal cranial
nerves (g).
Therefore, the caudal tumor part was
approached initially. It was debulked, and the
caudal cranial nerves, which were severely
compressed by the tumor, were dissected free.
Then, the more cranial portion was debulked
and dissected from the cerebellum and brain
stem. The facial nerve could not be seen at its
usual location and was identified somewhat
231
later—it was displaced more cranially. The
nerve was further dissected by slightly luxat-
ing the remaining tumor cranially. The lateral
cisternal segment of the facial nerve was
spread like an umbrella (h). A small tumor
piece was strongly adherent to the nerve and—
following the principle of intermittent dissec-
tion from different sides—the dissection
proceeded from lateral to medial. The tumor
was removed completely, and the preserved
facial nerve responded well to stimulation (i).
232 5 Vestibular Schwannomas

Cystic VS
Cystic VS should be considered as a separate
subgroup. They have different pathological
structure and a tendency for more aggressive or
unpredictable biological behavior. Frequently a
more rapid growth rate is observed. A higher
degree of nuclear atypia and differences in the
gene expression profiles of such tumors have
been found [38, 261].
It is supposed that cyst formation is caused
by central necrosis, coalescence of microcysts,
and/or repeated intratumoral hemorrhage
[164]. Their further enlargement can be
explained by the formation of an osmotic gra- a
dient between the cyst and the solid tumor and
impairment of the blood–tumor barrier. In a
recent study, Moon et al. [153] reported to have
identified high levels of proteolytic enzymes,
such as matrix metalloproteinase, in cyst fluid
and cyst walls. These enzymes are proposed to
play an important role in the pathogenesis of
cyst formation and peritumoral adhesion in
cystic VS.
Cystic VSs comprise between 2 and 13 % of
all VSs [33, 72, 170, 194]. The criteria for cys-
tic VS are radiological and pathological: on
T1-weighted sequences, the cystic tumor parts
are hypointense, while on T2-weighted b
sequences, they are hyperintense [62]
(Fig. 5.36). The cysts do not enhance with gad-
olinium administration, while their wall
enhances—a feature that allows their differen-
tiation from arachnoid and epidermoid cysts.
Cystic tumors should be differentiated from
VS with an arachnoid pseudocyst in the periph-
ery, which is not part of the tumor. The wall of
such pseudocysts neither enhances with gado-
linium administration, nor does it have the
structure of a typical arachnoid cyst (Fig. 5.36).
Occasionally cystic changes in the brain stem
may be observed, which appear to be similar to
those developing in case of intramedullary c
tumors. The mechanism of their development
in VS is unclear. Fig. 5.36 MR images of patients with solid (a) and cys-
tic VSs (b, c). The peripherally located large cysts in cases
a and b is a CSF accumulation (arachnoid pseudocyst),
while the contrast-enhancing peripheral cyst in case c is a
real tumor cyst
Management of Patients with VS
Case Illustration 5.12
Cystic VS 1
Forty-two-year-old male patient presenting
with a history of progressive hearing loss,
unsteadiness, and vertigo. His neurological
examination revealed trigeminal nerve hypes-
thesia (V2–3), absent corneal reflex, and pro-
found hearing loss on the right side. The MRI
demonstrated a large cystic VS on the right
side with maximal diameter of 3.6 cm (a–c).
a b
Cystic VS. MR images of a patient with a large cystic VS
on the left side (a, b) T1-weighted sequences following
contrast administration. (c) CISS sequence. Note the typi-
cal contrast enhancement of the cyst wall, which differen-
tiates it from arachnoid cysts or pseudocysts. Intraoperative
images. (d) The IAC has been opened, and the intrac-
analicular tumor part has been exposed (arrows). (e) The
partially cystic CP angle tumor part is well visible (arrow-
head). (f) Tumor debulking. (g) Dissection of the distal
segment of the facial nerve (white arrow) in the IAC.
(h) The proximal segment of the nerve at the brain stem
233
The patient was operated via the RS approach
(see figures d–n), complete tumor removal was
achieved, and the facial nerve was preserved.
The postoperative period was uneventful, and
at discharge the only new deficit was an HB
grade IV facial nerve palsy. The patient was
examined again 13 months after the surgery. He
had no complaints, and the facial nerve recov-
ered completely (HB grade I). The follow-up
MRI is presented on figures (o–r).
is exposed and consecutively dissected from the tumor
capsule (i–k). (l) The most adherent part of the tumor,
near the porus of the IAC, was removed at the end. (m)
Complete tumor removal with preservation of the facial
nerve. (n) Overview of the CP angle: BS brain stem, 7
facial nerve, 6 abducens nerve. Postoperative images
demonstrating the completeness of tumor removal. (o)
Bone-window CT. Note the wide exposure of the IAC up
to its fundus. (p, q) Axial and coronal MR sequences fol-
lowing contrast administration. The fat plug in the proxi-
mal IAC is visualized. (r) T2-weighted sequence
234 5 Vestibular Schwannomas

d e

f g
Management of Patients with VS 235

h i

j k
236 5 Vestibular Schwannomas

l m

n o

p
Management of Patients with VS 237

q r
238
Many VSs appear internally heterogeneous
containing interspersed regions of soft and firm
consistency. In case of real cystic VS, however, at
surgery fluid is seen within the cysts. Although it
has been proposed to subdivide homogeneous
and heterogeneous VSs, this subclassification has
no practical significance.
Duration of symptoms in patients with cystic
VSs prior to diagnosis is shorter than in those
with solid tumors. The cystic component may
expand rapidly and cause brain stem compres-
sion and hydrocephalus. Cystic VSs tend to
have a more unfavorable surgical outcome in
regard to facial nerve function, hearing, and
morbidity rate [72, 198]. Nevertheless, surgery
is definitely the optimal treatment option of
such tumors (Figs. 5.37 and 5.38). “Wait-and-
scan” policy is not recommended because of the
frequently observed rapid enlargement with
neurological deterioration [38]. Cystic VS may
respond poorly to radiosurgery. Furthermore,
the rate of facial nerve weakness after radiosur-
gery is higher than in solid or NF-2-associated
schwannomas [169]. Close adherence to the
principles of VS microsurgery, meticulous
hemostasis, and extremely careful observation
of the patients after surgery allow achieving
acceptable operative results (Fig. 5.37).
Surgery may be complicated by the tighter
adherence of the cyst wall to neurovascular struc-
tures. The solid portions of cystic VSs are fre-
quently hypervascularized and more difficult to
handle (see Case Illustration 5.12). During sur-
gery the abrupt change in the shape of the lesion,
following the aspiration of the fluid content, can
lead to rupture of the surface vessels and cause
excessive traction to the facial nerve.
The cranial nerves are typically splayed over
the cysts, and an adequate subarachnoid dissec-
tion plane is frequently absent. The facial nerve
can be markedly distorted and may be encoun-
tered unexpectedly. Cystic tumor components
are one of the most reliable predictors for the
5 Vestibular Schwannomas
facial nerve function after surgery [272]. Most
large clinical series confirm this negative corre-
lation [72, 170, 194]. Charabi et al. [35] reported
on the outcome of treatment of 571 VS; among
them 23 were cystic. In the cystic tumor group,
the facial nerve was accidentally transected in
43 %—a much higher rate than the facial nerve
transection rate for noncystic VS of similar size
in their experience. In a previous study of 1,000
patients, we found that the anatomic preserva-
tion rate of the facial nerve in cystic VS was
reduced from 93 to 88 % and of the cochlear
nerve from 68 to 55 % [199].
In case of very severe adhesions, a reasonable
option is to leave behind a small part of the cyst
not to destroy the nerve. In a recently evaluated
series of 200 VSs [194], we performed subtotal
tumor removal in four cases (2 %). Three of
them had cystic VSs: in all the adhesion was so
tight that the attempt to dissect the nerve from
the tumor capsule led to intense electromyo-
graphically demonstrated changes. Moreover,
from the 3 patients (1.5 %), in whom the facial
nerve could not be preserved anatomically, 2
had cystic VS.
Patients with cystic tumors are at higher risk
for postoperative hemorrhagic complications. A
common observation during surgery is that the
cerebellar surface in contact with the cystic
schwannoma is softened and has a propensity for
diffuse bleeding, probably as a consequence of
the proteolytic action of the cyst enzymes. In case
perfect hemostasis has not been achieved, this
can cause delayed bleeding in the CP angle.
Therefore, in addition to the “standard” hemosta-
sis, we coagulate the whole softened cerebellar
surface and cover it abundantly with Tabotamp.
In summary, although cystic VSs pose
specific challenges to the surgeon, strict adher-
ence to the principles of VS removal and use of
meticulous dissection technique allow to
achieve results, comparable to that in common
tumors.
Management of Patients with VS
a
b
c
d
e f
Fig. 5.37 MR images of a patient with a cystic left-sided
VS, presenting with complete hearing loss, vertigo, head-
ache, and trigeminal nerve sensory dysfunction.
Preoperative images: (a) Bone-window CT, (b, c)
T1-weighted images after contrast administration, and (d)
T2-weighted image. The tumor was removed completely
and the facial nerve was preserved. At the follow-up
239
examination 12 months after surgery, the patient had nor-
mal facial nerve function. The postoperative MR study is
presented on figures (e–h). (e, f) Native T1-weighted
sequence. (g) T1-weighted sequence after contrast admin-
istration. Note the fat plug in the area of the drilled IAC,
visible as high intensity signal on images (e and f). (h)
T2-CISS image
240
g h
Fig. 5. 37 (continued)
Surgery of Recurrent VS
Patients with tumor remnants after incomplete
removal are followed with regular yearly MR and
clinical examinations. If the remnant shows a
growth tendency, redo surgery should be per-
formed and the tumor should be removed com-
pletely. The recurrence rate after VS removal
ranges from 0.5 to 18 % in the literature [133,
5 Vestibular Schwannomas
194, 266]. In the senior authors’ series, it is
approximately 0.5 % of patients without NF-2
(0.8 % in the older series). In case a small recur-
rence is detected in asymptomatic patient, initial
follow-up is warranted. If the recurrence shows a
steady growth tendency or causes neurological
symptoms, operative removal should be recom-
mended (Figs. 5.39 and 5.40).
Management of Patients with VS 241

a b

Fig. 5.38 MRI of a patient with a large cystic VS. (a, b) T1-weighted sequences after contrast application. (c, d) CISS
sequences. (e) Intraoperative image: complete tumor removal with preservation of the facial nerve (arrow)
242 5 Vestibular Schwannomas

a b

c d

Fig. 5.39 MRI images of a 45-year-old patient, obtained
24 months after his initial surgery (radiologically verified
complete removal of a right-sided intracanalicular VS in
another department via an RS approach). Tumor recur-
rence with extrameatal extension is obvious. Note the
irregular shape of the tumor, in particular on images (b
and d), which is not typical for primary VSs. (a)
T1-weighted MRI. (b, c) T1-weighted contrast-enhanced
sequences. (d) T2 sequence. (e) CISS sequence
Management of Patients with VS 243

a b

c d

Fig. 5.40 (a–e) Preoperative MR images of a patient pre-
senting with a large recurrent VS 14 years after the first
surgery. Besides the headache that he had since several
months, he had no further complaints. Hearing on the
right side was lost at initial surgery. The tumor was
removed completely and the facial nerve was preserved.
At discharge he had an HB grade IV facial palsy and no
further deficits. The facial nerve function improved to HB
grade II at 12 months follow-up examination. (f–h)
Postoperative MRI (12 months after surgery)
244 5 Vestibular Schwannomas

e f

g h

Fig. 5.40 (continued)

Management of Patients with VS 245

Case Illustration 5.13 ataxia, rendering walking—even with help—

Giant VS Operated Seven Times
Previously
This patient has been operated seven times in
different neurosurgical facilities before pre-
sentation in INI. At admission he had trigemi-
nal hypesthesia, abducens nerve palsy, HB
grade V facial palsy, anacusis, and lower cra-
nial nerve dysfunction on the right side. He
had severe discoordination syndrome and
impossible. A ventriculoperitoneal shunt has
been placed after the third surgery and was
still patent. The patient had furthermore signs
of respiratory insufficiency but was not venti-
lator dependent.
Still, taking into account the size of the
tumor, the compression of the brain stem,
and the lower cranial nerve deficit, a plastic
tracheostomy was done prior to surgery. The

a b

c d

Preoperative MRI images (a–d). Postoperative CT—2 weeks after surgery (e)
246 5 Vestibular Schwannomas

e tumor could be removed completely via the

standard RS approach despite the very severe
adhesions and lack of any arachnoid plane.
The facial nerve, however, could not be
identified—probably it was destroyed
previously.
After surgery the patient received pro-
longed intensive treatment. Gradually, how-
ever, he could be weaned off the ventilator.
His initially worsened swallowing improved,
and the tracheal cannula could be removed.
The balance and body control also improved,
and at discharge he was able to walk with
support.
Management of Patients with VS
Preservation of functions after previous sur-
gery can be very challenging. Typically adhe-
sions and scar tissue obscure the normal
anatomical landmarks. The initial orientation
within the CP angle and the exposure of the
tumor are more demanding (see Case Illustration
5.13). The tumor is frequently very adherent to
the cranial nerves, and the nerves themselves
may be more vulnerable. Hence, tumor removal
should proceed only after definitive identification
of the neural structures. The facial nerve should
be identified initially in its healthy segment; the
scar/adhesion area along its course should be
approached last. The surgical strategy should be
adapted to the findings in each individual case.
Factors to consider are type of previous surgery
247
(approach, extent of tumor removal), size and
location of the remnant/recurrent tumor, neuro-
logical status of the patient, and goal of surgery
(see Case Illustration 5.14). Thus, if the patient
presents with complete facial palsy prior to sur-
gery, an EMG should be performed and opera-
tive reconstruction of the nerve should be
planned. Even in patients with incomplete but
severe facial palsy that did not recover after
previous surgery, the optimal management may
be to remove the tumor, sacrifice the nerve
(which in such circumstances is typically atro-
phic and responds poorly to electrostimulation),
and reconstruct it with an interposition graft or
perform a hypoglossal–facial nerve anastomo-
sis (see Chap. 12 for details).
248
Case Illustration 5.14
Recurrent VS After Middle Fossa Removal
This 42-year-old male patient complained
since 2007 from progressive hearing loss on
the right side. A pure intracanalicular VS was
diagnosed, and he has been operated at another
department in the early 2008: the tumor was
removed partially via the MF approach.
Following surgery the patient had HB grade V
facial nerve palsy that later improved. The
follow-up MRI controls, however, revealed
that the residual tumor had a growth tendency
and expanded into the CP angle cistern (a–d).
The patient presented to our institute with a
a b
MRI examination performed at our institute: the large
tumor remnant in the right IAC with a small extension
in the CP angle is seen. (a–c) T1-weighted sequences
following gadolinium administration. (d) T2-weighted
sequence. Intraoperative images. See the text for
detailed description of the operative steps. (e) Initial
5 Vestibular Schwannomas
grade T3a VS, HB grade III facial nerve palsy,
and hypacusis (PTA of 50–60 dB).
The RS approach was used in the case, and
the IAC was opened in the standard technique
(e–h). In the area of the fundus, however, some
fibrotic changes were seen, which led us to
conclude that the facial nerve might be densely
adherent or even involved by the scar tissue
(during the MF approach, the facial nerve lies
between the tumor and the surgeon, as
described in the Chap. “MF Approach”). The
surgical strategy in case of previous surgery
via the MF approach should be modified,
taken into consideration the expected adhe-
view of the CP angle and the vestibulocochlear nerve
complex, dislocated posteriorly by the tumor. (f) The
IAC has been opened, and the tumor has been partially
removed. (g) The most lateral tumor part is the last to
be removed. (h) Complete tumor removal with preser-
vation of the facial and cochlear nerves
Management of Patients with VS 249

sions of the facial nerve and the tumor
capsule/fibrous tissue in the area of the previ-
ous surgery. Tumor debulking is performed
initially in the CP angle, and the facial and
cochlear nerves are identified medially to the
tumor (f). Even in case of large VSs, the nerves
can be identified at the brain stem.
Then, the tumor dissection and removal
proceeded laterally with constant verification
of the location of the facial nerve with electro-
stimulation. In this particular case, it was
especially difficult to differentiate the tumor
from fibrous tissue in the fundus area (g), and
we had to rely on the electrostimulation to
identify the nerve. The cochlear nerve was
found to be less involved by the scar.
Ultimately, the tumor could be completely
removed, and both nerves were preserved (h).
After the surgery the patient did not have
any new deficit, and the preoperative hearing
level was preserved. He was discharged on the
tenth postoperative day.

e f

g h
250
Facial Nerve Preservation in VS Surgery
The facial nerve function is a major factor deter-
mining the quality of life of patients after CP
angle surgery. Facial nerve palsy besides the cos-
metic disfigurement increases the risk of ocular
complications, such as corneal damage, ulcer-
ation, and even blindness. The increased or
reduced tearing (dry eye), salivation disturbances,
increased nasal secretions, and sensitivity to light
or taste abnormalities may cause significant dis-
comfort. The weakness of orbicularis oris and
buccinators muscles impairs speech and chew-
ing. The negative effects are further exaggerated
by the development of synkinesis and the relative
hyperactivity of the contralateral side.
Patients with facial dysfunction experience
distress that—according to some studies—is not
correlated to the clinical grade of the facial
nerve paralysis. Even an incomplete facial
weakness can have devastating impact on the
social life and psychological well-being of the
patients [176].
Facial Nerve Grading Systems
The generally accepted facial nerve function
grading system is the House–Brackmann grad-
ing scale, proposed in 1985 (Table 5.3) [95]. It
has been widely validated and allows for estima-
tion of facial nerve function with small interob-
server variability. The major weakness of this
Table 5.3 House–Brackmann grading system [95]
Grade Description
1. Normal Normal function in all muscles
2. Mild dysfunction Slight weakness noticeable
only on close inspection
3. Moderate Obvious but not disfiguring
dysfunction weakness, no functional
impairment
4. Moderately severe Obvious weakness, disfiguring
dysfunction asymmetry; possible functional
impairment
5. Severe dysfunction Only barely perceptible motion
6. Total paralysis No motion
5 Vestibular Schwannomas
system, as pointed by Kanzaki et al. [110], is the
definition of grade 3 dysfunction. Patients cate-
gorized in this grade may have dry eye and/or
exposure keratitis that is not accounted for.
Furthermore, the function of the intermediate
nerve is not taken into consideration. At the
Tokyo Consensus Meeting, a modification of the
House–Brackmann grading scale was proposed,
claiming to allow more objective and precise
evaluation [110]. In this facial motion
classification, grade III is subdivided into grade
IIIA and grade IIIB, and criteria for the degree of
synkinesis and hemifacial spasm are added. The
classification allows further a record of nervus
intermedius function (Table 5.4).
Predictive Factors for the Preservation of
the Facial Nerve
Several factors are known to influence the ability
of the surgeon to preserve facial nerve function:
tumor size, tumor type, existence of arachnoid
plane, intraoperative facial nerve monitoring,
previous surgery or radiosurgery, surgical
approach, and experience of the surgical team.
Tumor Characteristics
Tumor size is perhaps the most important predic-
tor of the quality of facial nerve function after
surgery. During tumor growth the facial nerve is
progressively stretched and becomes extremely
Characteristics at rest Characteristics in motion
Normal Normal
Normal symmetry and No synkinesis, contracture, or
tone hemifacial spasm
Normal symmetry and Noticeable, but not severe
tone synkinesis or contracture,
hemifacial spasm, or both
Normal symmetry and Obvious synkinesis or mass
tone action, hemifacial spasm
Asymmetry w/ droop at Synkinesis, contracture, and
corner of mouth and hemifacial spasm usually
decreased nasal labial absent
fold
No tone, asymmetry, no
synkinesis, contracture,
or hemifacial spasm
Management of Patients with VS 251

Table 5.4 Facial motion classification

Grade Tonus at rest Global aspect Forehead Mimic eye Mouth Hemispasm
1. Normal Normal Normal Normal Normal occlusion Normal Absent
2. Light Normal Light Light Eyelid occlusion: Very light Absent or
asymmetry asymmetry complete without asymmetry very light
effort
3. Moderate Normal or very Moderate Moderate Eyelid occlusion: Clear Present,
A (light) light asymmetry asymmetry asymmetry complete with asymmetry moderate
effort comfortable protracted lips
eye occluded
3. Moderate Normal or very Clear Clear Eyelid occlusion: Clear Present,
B (heavy) light asymmetry asymmetry asymmetry complete with asymmetry clear
effort ocular protracted lips
discomfort not occluded
4. Real Real asymmetry Real Real Eyelid occlusion: Real Present,
asymmetry asymmetry incomplete asymmetry greater
5. Heavy Greater Greater Greater Eyelid occlusion: Greater Absent
asymmetry asymmetry asymmetry roughed asymmetry
6. Total Flaccidity Greater Flaccidity No eyelid Flaccidity Absent
asymmetry occlusion
Note Crocodile tears: Taste troubles: Eye: not dry
CO, C+ T0, T+
Kanzaki et al. [110]

vulnerable. It is frequently splayed over the
tumor capsule and consists of multiple small
bundles or has an “umbrellalike” appearance.
Any traction or manipulation during tumor
removal may easily lead to the rupture of these
nerve fibers even if the continuity of the bundles
is not obviously affected.
The correlation between tumor size and facial
outcome has been proven by numerous studies
[125, 194, 198]. The anatomical preservation rate
in our general series was 98.5 %, while in tumor
grades T1, T2, and T3, it was 100 %. Functional
outcome correlated to tumor size and best results
were achieved in VSs grade T1 and in grade T3a
(HB grade I in 90 and 96 %, respectively).
Recently we analyzed the predictive value of
various preoperative existing factors. We evalu-
ated patient-related and tumor-related factors,
such as tumor diameter (anteroposterior or paral-
lel to the petrous ridge, mediolateral or perpen-
dicular to the petrous ridge, and sagittal or vertical
diameters); cranial, caudal, anterior, and poste-
rior tumor extension in relation to the IAC; width
and length of the intracanalicular tumor portion;
tumor–fundus distance; and tumor shape [77].
Our analysis showed that immediate postopera-
tive facial nerve function correlated significantly
to tumor stage, diameter, and tumor volume. The
increase of any of the extrameatal tumor diame-
ters led to worse outcome. However, the anterior
and/or caudal tumor extension had more
significant correlation than the posterior and/or
cranial extensions. VSs with significant anterior
extension could lead to more severe stretching
and thus vulnerability of the facial nerve. This
may be the explanation for the above-mentioned
correlations.
The shape of the tumor also showed significant
correlation to facial nerve outcome: the outcome
is poorer in polycyclic VSs compared to round or
oval ones. The characteristics of the intracanali-
cular tumor portion, including tumor length,
tumor width, and tumor–fundus distance, as well
as the bony changes and characteristics of the
ICA did not have significant relationship to
immediate facial nerve outcome. Other authors
have also reported that the rate of good facial
nerve function does not correlate to the extent of
IAC destruction and/or widening [265].
The extent of adherence of the tumor to the
nerve—or the presence of a good arachnoid dis-
section plane—is of even greater importance.
252
While the chance to preserve the facial nerve in
large VSs is related mainly to the experience of
the surgeon, the presence of dense adherence
may hinder complete tumor removal even in
experienced hands.
Patient Age
Another important parameter is the patients’ age.
If increased age is associated with poor facial
outcomes, as reported by Grey et al. [84], a deci-
sion in favor of radiosurgery for older patients
would be logical. Multiple authors, however,
showed that age is not a negative predictor [41,
116]. Couloigner et al. [48] evaluated patient’s
characteristics and clinical features, such as age,
gender, and symptom duration, and found no cor-
relation to postoperative facial nerve function.
Our analysis showed that age at initial symptom
and surgery did not correlate with immediate
postoperative facial function. Furthermore, symp-
tom duration before surgery and patient’s gender
also did not correlate with outcome. The only
neurological symptoms at presentation that cor-
related significantly with worse immediate facial
nerve function were gait instability and preopera-
tive facial palsy.
NF-2
Patients with NF-2-associated VSs tend to have
poorer facial nerve outcome after surgery [195].
The extent of adhesion of such tumors to the
nerve is more intense, and a clear histologic
cleavage plane may be missing. Furthermore, the
location of the facial nerve is highly variable and
less predictable. It may pass between the separate
tumor lobules, and hence the risk of its inadver-
tent injury is higher.
Cystic Tumors
The dissection and preservation of facial nerve in
cystic VSs are more difficult (see section “Cystic
VSs”), and its preservation rates are respectively
lower. In our experience, the anatomic preserva-
tion rate of the facial nerve was reduced from 93
in solid VSs to 88 % in cystic tumors and of the
cochlear nerve from 68 to 55 %, respectively.
Similar correlation exists in regard to the func-
tion of the nerves.
5 Vestibular Schwannomas
Reoperations
During revision surgery, intense adhesions are
generally encountered between the neurovascular
structures and the tumor remnant. Occasionally it
might be difficult even to identify the facial nerve.
Frequent electrostimulation of the suspicious
areas in such cases is extremely helpful.
Radiosurgery
The clinical experience with VSs treated opera-
tively after failed radiosurgery shows that generally
abundant adhesions are present and the cleavage
plane to the nerve is not so obvious. Some histo-
logical examinations revealed no clear interface
between the vestibular and facial nerves (see sec-
tion “Radiosurgery”). Not infrequently, the patients
have been both operated and irradiated previously,
which further complicates the surgery. Despite the
difficulties encountered in case of previously oper-
ated or irradiated patients, facial nerve preservation
is realistic, provided the general microsurgical
principles of VS removal are followed.
Intraoperative Facial Nerve Monitoring
In a historical study, Harner et al. [91] compared
48 matched cases operated with and without mon-
itoring and demonstrated that this technique sub-
stantially improved the rate of facial nerve
preservation, especially in patients with larger VS.
Nowadays the constant monitoring of the facial
nerve is an absolute prerequisite for the preserva-
tion of its functions. As shown recently, the facial
nerve motor evoked potential monitoring is a valu-
able adjunct to conventional facial nerve monitor-
ing and may predict the functional outcome [2].
Facial nerve stimulation thresholds have been
shown to have prognostic significance to the
degree of postoperative function. According to
the study of Neff et al. [159], stimulus threshold
of 0.05 mA or less and a response amplitude of
240 mV or greater predicted a House–Brackmann
grade 1 or 2 function with 98 % probability. Any
of the parameters alone, however, had a much
lower predictive value.
Preoperative Facial Palsy
Facial palsy is a rare symptom prior to surgery:
its rate was found to be 2.3 % in a series of 1,000
Management of Patients with VS
patients [145]. Patients who presented with facial
nerve palsy have a higher risk of poor postopera-
tive facial nerve functioning. However, preopera-
tive facial nerve dysfunction is more frequent in
large tumors. These two factors may, therefore,
influence independently the functional outcome.
Experience of the Surgical Team
The personal experience of the surgeon is of par-
amount importance in regard to facial nerve pres-
ervation. Many studies have demonstrated that
the outcome is inferior in the initial phases of the
learning curve. Welling et al. [262] demonstrated
a learning curve of 20 patients to be necessary for
attaining acceptable standards in the surgical
removal of VS. Elsmore and Mendoza [60] found
that the improvements occurred mainly between
the 40th and 100th cases. Buchmann et al. [29]
presented similar numbers: 60 cases are neces-
sary before the new team achieved results similar
to those of highly experience surgeons. Moffat
et al. [151] in a review of 300 patients demon-
strated obvious improvement in facial nerve pres-
ervation from the first to the sixth group of 50
patients: from 88 to 98 %. Interestingly, the
authors noted that a significant improvement
occurred after the first 50 patients and then again
after patient 200. In our recent analysis of a series
of 200 patients, we demonstrated that the facial
nerve outcome could be improved even after an
experience of more than 3,000 cases [194]. The
improved outcome allowed us to conclude that an
ultimate plateau of learning is never reached.
For the achievement of optimal results, the
experience of the whole team involved in the
patients’ management is critical. In a profound
meta-analysis, Hastan et al. [93] showed that a
linear relation between hospital volume and pres-
ervation ratio of facial function exists. The
authors concluded that concentration of surgical
treatment might be a consideration to optimize
clinical outcome.
Operative Approach
During the past three decades, a major topic in
the literature has been the relation of the opera-
tive approach to the outcome. Numerous authors
have claimed that their operative technique is
253
superior. We consider this controversy as a part
of VS history. It is obvious that optimal results
are obtained if the surgeon applies the technique
that he best masters.
Causes of Facial Nerve Injury
(Modified from Sampath et al. [200])
• Direct trauma, stretch, or torsion on the nerve.
This mechanism is probably the most frequent
cause of facial nerve dysfunction and occurs
during its dissection from the tumor capsule.
Less severe stretching causes neuropraxia and
axonotmesis, which are potentially reversible.
• Mechanical disruption or nerve transection.
Risk factors are less operative experience,
cystic VSs, as well as the attempt to dissect
the nerve from the tumor from above.
• Devascularization of nerve segments. Most of
the microvascular blood supply to the facial
nerve is in the subarachnoid space and may be
damaged if the correct dissection plane is not
followed.
• Compromise of facial nerve vascular supply.
The facial nerve vascular supply is variable
and is derived from three separate systems: the
labyrinthine artery, the greater superficial pet-
rosal branch of the middle meningeal artery,
and the stylomastoid artery off the external
carotid system. Therefore, interruption of a
feeding artery or prolonged vasospasm can
cause varying degree of facial nerve palsy.
Topical application of nimodipine or papaver-
ine has been recommended, but their efficacy
is not definitely proven.
• Thermal injury due to bipolar coagulation
close to the nerve or due to the heat generated
while drilling.
Prevention of Facial Nerve Palsy
Prevention of facial nerve injury is based on the
knowledge of its location, on its early identification
according to constant anatomical landmarks, and
on the refinement of the microsurgical technique
of dissection and tumor manipulation. Ideally,
neuroimaging should allow to define the position
of the facial nerve and to plan the surgery accord-
ingly. The anatomical imaging, however, does
not provide reliable information on the facial
254
nerve course. DTI and fiber tracking is a rapidly
evolving field that allows for visualization of the
nerve. Its reliability has been proven, but the clin-
ical value of this information remains to be estab-
lished [39, 76, 240]. Postoperative use of steroid
medications (dexamethasone) is recommendable
to minimize both cerebellar and perineurial swell-
ing. Electrolyte homeostasis and appropriate
intravenous hydration should be achieved.
Outcome
Anatomical preservation of the facial nerve is
achieved in more than 90 % of the cases in expe-
rienced hands. The functional outcome correlates
closely with tumor size, but in small-to-medium
large tumors, excellent or good functional result
is achieved in more than 80 % [56, 124, 235].
In a series of 1,000 VSs operated by the senior
author in the period 1978 to 1993, the facial nerve
anatomical preservation rate was 93 % [199]. The
early facial nerve function was normal in 51 % of
them. In 45 % the function recovered within 1–12
months to HB grade II in 13 %, HB grade III in
15 %, HB grade IV in 6 %, HB grade V in 11 %,
and HB grade VI (no recovery) in 4 %, despite
nerve continuity. Of these last patients, 1.7 %
showed no recovery. In 11 of them, hypoglossal–
facial nerve anastomosis was done and good
facial reanimation achieved.
The anatomical continuity of the nerve was
lost in 60 cases: in 23 of them the facial nerve
was reconstructed in the CP angle, in 16 cases
intracranial–intratemporal reconstruction, and in
3 cases intracranial–extracranial transplantation
was performed. The facial nerve was reanimated
by a donor nerve in 18 cases (the contralateral
facial nerve or the hypoglossal nerve).
In a later series (2000–2003) of 200 patients,
the rate of excellent facial function 2 weeks post-
operatively increased to 62 % (vs. 51 % in the
above-mentioned series) [194]. Good function
(HB grade III) had 14 % of the patients, fair func-
tion (HB grade IV) 16 %, poor function (HB
grade V) 5 %, and no function (HB grade VI)
3 %. At the last follow-up examination, 81 % of
patients had excellent or good facial nerve func-
tion (vs. 79 % in the previous series). All patients
with complete palsy immediately after surgery
showed improvement after at least 1 year after
5 Vestibular Schwannomas
surgery, and at least 1 year after surgery, there
were no patients with complete facial palsy.
Delayed Facial Nerve Palsy
Delayed facial nerve palsy is a term used to
describe those cases in which spontaneous wors-
ening of facial nerve function is seen in patients
with normal or near-normal function early after
surgery. The reported incidence is highly vari-
able—between 2.3 and 29 %—due to the lack of
consensus on the precise inclusion criteria, in par-
ticular the definition of the time range [140, 201].
The pathogenetic mechanisms leading to delayed
facial nerve palsy are poorly understood, and fac-
tors such as perineural edema, vasospasm, delayed
immune response, viral reactivation, and nerve
entrapment within its bone canal have been
implied. Respectively, various management
options have been proposed: treatment with nimo-
dipine, acyclovir, methylprednisolone, or dexam-
ethasone, as well as operative decompression of
the nerve in its labyrinthine segment. In the series
of 200 patients, we observed facial palsy develop-
ing 4 days to 2 weeks postoperatively in seven
patients (3.5 %). A course of dexamethasone
treatment was performed in all patients, and the
outcome at 1 year was favorable. The good prog-
nosis in case of delayed facial palsy is confirmed
by the experience of other authors who point that
recovery of facial nerve function to its original
postoperative status nearly always occurs.
Intermediate Nerve Dysfunction
Although this nerve is rarely evaluated after sur-
gery and its disorders are classified as “minor,”
its deficit can cause significant discomfort to the
patient, in particular increased or reduced tearing
(dry eye), salivation disturbances, increased nasal
secretions, and taste abnormalities. The reduced
frequency of blinking in addition to the decreased
tearing contributes to the severity of the dry eye
syndrome.
Some studies showed that nervus intermedius
abnormalities following VS surgery are more com-
mon than generally anticipated. Stripf et al. [231]
reported that 45 % of their patients experienced
crocodile tears, 40 % had dry eyes, whereas 44 %
complained of increased nasal secretion. In the
series of Magliulo et al. [139], 42.3 % of the cases
Management of Patients with VS 255

Table 5.5 Gardner–Robertson Grade PTA or SRT SDS (%)

scale [73]
1 0–30 And 70–100
2 31–50 And 50–69
3 51–90 And 5–49
4 91 (ML) And 1–4
5 NR And NR
PTA pure-tone average, SRT speech reception threshold, SDS speech discrimina-
tion score, NR no response, ML maximum loss

Table 5.6 AAO-HNS Class Pure-tone threshold Speech discrimination (%)

hearing classification A £30 dB ³70
B ³30 dB, £50 dB ³50
C >50 dB ³50
D Any level <50
Note: classes A, B, and C require both the pure-tone average and speech discrimi-
nation criteria to be met

had crocodile tears and 59.6 % had altered tearing.
Irving et al. [97] evaluated a series of 224 patients:
44 % had postoperative crocodile tears, absence or
significant reduction in the production of tears was
noted in 72 %, and a taste abnormality in 48 % of
the patients. The overall rate of recovery of normal
tearing within 7 months was 27 %. In patients with
better facial nerve motor function (HB grades
I–III), the tearing recovered in almost 50 %.
Taste Disturbance
The incidence of taste disturbance after surgery is
surprisingly high: between 29 and 38 % of the
patients with normal preoperative function had
such deficit postoperatively [40, 215]. Although
Watanabe et al. [259] reported that the function
recovered completely in 65 %, the extent of
recovery is variable and unpredictable. The risk
for taste disturbance is higher with the MF
approach than with the RS approach. The study
of Stripf et al. [231] also revealed strong tendency
to a higher rate of intermediate nerve-related
symptoms following the MF approach.
The time course and degree of facial nerve
recovery depend upon the extent of its injury. In
less severe injuries, the function recovers gradually
within several weeks to months after surgery. In
case of more severe injuries, it may take more than
12 months to recover. Preoperative counseling on
this particular aspect in all patients undergoing sur-
gery for VS is essential to adequately motivate them
and to reduce their psychological discomfort.
Vestibular Schwannomas and Hearing
In 1954 Elliot and McKissock first reported suc-
cessful hearing preservation in 2 of 3 operated
patients with VSs [59]. Hearing is an important
determinant of quality of life, and its preservation
should be hence attempted in every patient who
has functional/useful hearing before surgery,
regardless of tumor size.
Hearing Classification
The first hearing classification was proposed by
Silverstein et al. in 1985 [218]. In 1988, Gardner
and Robertson [73] presented a classification
similar to that of Silverstein et al. It was later
named the “Gardner and Robertson classification”
(Table 5.5). This system gained popularity,
although it has some weaknesses: 30 dB—and
not the physiological 25 dB—is accepted as
upper limit of useful hearing, and the last studied
frequency is 3,000 Hz.
The AAO-HNS classification is also com-
monly applied (Table 5.6). Its drawbacks are that
it separates hearing into only four categories and
is not sufficiently refined [110]. Normal and sub-
normal hearing are not separated [203].
Other popular classifications are those of
Gardner, Shelton, and House and of the Mayo
Clinic. Sanna et al. [203] classified hearing in steps
of 10 % for SDS and by steps of 20 dB for PTA. At
the Tokyo Consensus Meeting, an agreement was
met that the PTA evaluation should be made at
500, 1,000, 2,000, and 4,000 Hz [110]. Six hearing
256
Table 5.7 New Hannover
hearing classification [197] Class Hearing
H1 Normal hearing
H2 Useful hearing
H3 Moderate hearing
H4 Poor hearing
H5 No functional hearing
PTA pure-tone average: the average of air conduction data at 1,000, 1,500, 2,000,
and 3,000 kHz
classes were defined using a diagram on which the
hearing result is located on the cross point of SDS
(speech discrimination score) and PTA.
Word recognition is a more sensitive parame-
ter of communication ability than pure-tone
thresholds. Patients with good word recognition
should benefit significantly from amplification
than those with little word recognition and good
pure-tone average [79].
Very important issue in regard to hearing
preservation is the definition of serviceable (or
functional or useful) hearing. This issue has
been variably presented in the literature, pro-
hibiting any meaningful comparison between
different series [70, 86, 110, 182, 201]. The
“more relaxed” criterion defined useful hearing
as PTA £ 50 dB and SDS ³ 50 % [203].
According to the “strict criteria,” useful hearing
corresponds to PTA better than 30 dB and SDS
better than 70 % in the presence of a normal
contralateral ear [81].
We favor the New Hannover classification
[197], presented at Table 5.7. If the PTA is 40 dB
or less and an SDS is 70 % or greater, hearing is
defined as useful, functional, or serviceable. If
hearing level is worse, balance cannot be achieved
between the good ear and the impaired ear, direc-
tional hearing is impossible, and hearing in noisy
environments becomes very bothersome. Hearing
level worse than 90 dB is defined as “functional
deafness.”
Factors Related to the Preoperative Hearing
Level and Predictors of Hearing Preservation
In a recently published study [75], we evaluated
the factors that correlate with preoperative hear-
ing level. Patients’ sex and age at onset of clinical
symptoms and at surgery were found to be impor-
tant predictive parameters: younger and female
5 Vestibular Schwannomas
Audiometry Speech discrimination score
(PTA) (SDS) (%)
0–20 dB 95–100 %
21–40 dB 70–95 % or better
41–60 dB 40–65 % or better
61–80 dB 10–35 % or better
>80 dB 0–5 % or better
patients had worse preoperative hearing. The
studies of Lapsiwala et al. [127] and Badie et al.
[9] showed that a positive correlation exists
between hearing loss and the increased intrac-
analicular pressure due to tumor expansion.
Respectively, only the size of the intrameatal
tumor component and not the total tumor size
were found to correlate to the hearing level.
Interestingly, a correlation was found between
the pressure elevation and BAEP features, such
as I to wave V intervals and wave V latency. The
authors speculated that an ischemic injury to the
auditory apparatus or pressure to the intrameatal
part of the cochlear nerve is the cause of hearing
dysfunction. Our data, however, showed that both
intrameatal and extrameatal tumor extension
influenced the hearing level, suggesting that some
additional mechanism is involved [75]. Tumor
growth perpendicular to the pyramid toward the
brain stem correlates significantly with hearing
loss. A possible explanation for these findings
might be that the underlying mechanisms are
stretching, rather than compressing, of the
cochlear nerve fibers. Caudal tumor growth has
similar effect on the cochlear nerve [147, 154].
The relation of tumor size and hearing level has
been controversially discussed in the literature.
Although some authors could not find significant
correlation, in our material both the size and vol-
ume of the VS correlated positively with the
hearing level [75].
The most reliable predictive factors for hear-
ing preservation after surgical removal of VSs are
tumor size, preoperative hearing levels, and qual-
ity of the BAEP. Other features that might be rel-
evant for hearing preservation are tumor location,
tumor consistency, presence of transient evoked
otoacoustic emissions and a short duration of
hearing loss [149, 152, 174, 206].
Management of Patients with VS
The extent of lateral extension of the VS has
been reported to be an independent prognostic
factor [152]. Our analysis proved that the degree
of intrameatal tumor growth and the lateral VSs
extension toward the fundus correlated with pre-
operative hearing loss. The extensive osseous
erosion or dilatation of the IAC is another uncer-
tain predictor of hearing preservation.
The degree of tumor adhesion and surgical
expertise are intuitively known as major predic-
tive factors for hearing preservation but are
difficult to quantify [29, 154, 194]. With the cur-
rently available neuroimaging techniques, it is still
impossible to determine the degree of adhesion
between tumor capsule and cranial nerves, hence
the difficulty of tumor dissection. The experience
of the surgeon is perhaps the single most impor-
tant factor determining the outcome of surgery.
Younger patients, as well as those with tinni-
tus as initial symptom or at surgery, have better
postoperative hearing. Obviously the impact of
VS on the cochlear nerve might be via two differ-
ent pathophysiological mechanisms causing
either nerve inhibition (hearing loss) or irritation
(tinnitus).
VS Removal and Hearing Preservation
Some authors suggest that hearing preservation
surgeries should be undertaken only in carefully
selected cases in regard to tumor size and preop-
erative hearing level: attempt at hearing preser-
vation is worthwhile only when hearing is
excellent or when the tumor extends less than
20–25 mm outside the IAC. They state that in
larger tumors the chances of hearing preserva-
tion are negligible and this consideration should
not influence the approach selection. Further
arguments are that the attempt to preserve hear-
ing prolongs operative time and may increase
the risk of injury to the facial nerve and the risk
of recurrences. The concept that in VS ³ 2–3 cm
the hearing-destructive translabyrinthine
approach should be utilized has therefore sup-
porters [21, 26, 83, 269].
We, as well as an ever-increasing number of
surgeons worldwide, believe that cochlear nerve
preservation should be attempted in all VS sur-
geries and hearing preservation should be the
goal in all patients who have good or useful hear-
257
ing, whatever the tumor size. Adhering to this
philosophy, our rate of anatomically preserving
the cochlear nerve increased from 68 % in our
first 1,000 cases to 75.8 % in a recent series (79
and 84 % in patients with preoperative hearing)
[194]. Preservation of hearing was achieved in
46 % of patients in our previous study and
improved to 51 % in the later series. Furthermore,
if at least some hearing is available before sur-
gery, we always attempt to preserve the cochlear
nerve, whatever the size of the tumor. According
to some studies [68], microscopic involvement of
the cochlear nerve is frequent even when the sur-
geon sees no gross involvement. The extreme low
rate of tumor recurrences in our series even in
case of cochlear nerve preservation is an obvious
argument against such possibility.
Hearing might be lost at any stage of VS
removal. Most frequent causes are destruction of
the inner ear structures during IAC opening,
vibration-induced injury to inner ear, traction
injury to the myelin sheath, thermal injury or
direct trauma to the cochlear nerve, and ischemia
due to vessel occlusion or vasospasm [45].
Detailed knowledge of the normal and patho-
logical microanatomy of the cochlear nerve and
inner ear structures is essential. Similar to the
principle of facial nerve preservation, early
identification of the cochlear nerve is important.
Its constant relations to anatomical landmarks in
the IAC and medially, at the brain stem, are uti-
lized: in the fundus of the IAC, it occupies the
anteroinferior quadrant below the transverse plate
and anterior to the vertical crest. In the CP angle
the cochlear nerve is generally located on the
anteroinferior tumor surface. More rarely it is
found on the anterior middle tumor portion and at
the inferior tumor pole [202]. It joins the brain
stem in the lateral part of the pontomedullary sul-
cus, approximately 1–2 mm posterior to the exit
site of the facial nerve [182].
The cochlear nerve is extremely sensitive to
traction, which may destroy the nerve fibers at
their weakest point at the Obersteiner–Redlich
zone. Therefore, excessive retraction of the cere-
bellum with the self-retaining retractor should be
avoided. Monitoring of the BAEP allows avoid-
ing this complication: whenever the extent of
traction exceeds a certain physiological value,
258
the wave V latency increases or its amplitude
decreases. Actually, the retractor should be used
only to support and protect the cerebellar
hemisphere.
The integrity of the labyrinth and vestibular
aqueduct should be preserved, as described ear-
lier (see Chap. 4). During drilling of the IAC,
the local temperature increases significantly.
Continuous irrigation is required to avoid ther-
mal injury to the underlying neural structures.
Mohr et al. [152] showed that the presence of
CSF space between the tumor and the fundus
may enhance the dissection of the tumor and,
consequently, may improve the outcome.
Hearing preservation in VSs reaching the fun-
dus is more difficult because the attempt to
expose the entire IAC can lead to violation of
the labyrinth [86, 242]. Extensive drilling of the
posterior wall of the IAC, which is needed to
expose the entire tumor, may increase the risk
of opening the posterior semicircular canal
[147]. As shown by Tatagiba et al. [242], the
extent of labyrinthine opening correlates with
postoperative hearing preservation. The amount
of bone removal should be therefore carefully
planned.
The dissection of the cochlear nerve from the
tumor capsule is of key importance. It should
proceed from known to unknown structures and
should be performed always in the arachnoid
plane (see Chap.“RS Approach” in VSs). The
dissection should be alternated from different
directions, and prolonged stretching of the tumor–
nerve complex in one direction has to be
avoided.
The BAEP waves amplitude or latency reflect
the functional integrity of the auditory pathway
and are highly sensitive to any manipulation of or
even near to the auditory structures. This tech-
nique provides almost real-time feedback infor-
mation every 30–90 s. In a study published in
1997, Matthies and Samii [146] investigated the
significance of the presence or absence of ABR
waves I, III, and V as functional representatives
of the cochlea, the nucleus cochlearis, and the
colliculus inferior, respectively. They could show
that certain microsurgical maneuvers were likely
5 Vestibular Schwannomas
Fig. 5.41 MRI of a patient with an intracanalicular VS
on the right side and normal preoperative hearing. See
Fig. 5.42 for details
to cause isolated or combined component losses
and subsequent hearing losses:
• Wave I: 86 % risk of deafness existed if it was
lost permanently, and an 11 % risk existed if
the loss was temporary. Highest risk for its
loss was associated with pulling the tumor–
nerve complex down or medially away from
the cochlea.
• Wave III: 82 % risk of deafness existed if it
was lost permanently, and a 12 % risk existed
if it was temporary. The wave is the most sen-
sitive warning sign.
• Wave V: 90 % risk of deafness existed if the
loss was permanent, and a 14 % risk existed if
it was temporary. Loss of wave V is the sign
most closely associated with deafness.
Importantly, earlier warning signs—disap-
pearance of wave I or III—precede wave V
loss. The most frequent actions, during which
wave V disappeared, were pulling the tumor–
nerve complex back or down, nerve dissec-
tion, hemostasis, and plugging of the IAC.
As soon as deterioration of BAEP trace
appears—usually increase of latency or decrease
of amplitude of waves III or V—the ongoing sur-
gical action is stopped. The cause of BAEP trace
change or component loss is not always obvious,
and its dynamics cannot be predicted: in some
cases it recovers completely, while in others the
waves disappear completely (Figs. 5.41, 5.42,
Management of Patients with VS 259

a b

c d

Fig. 5.42 Intraoperative images of the patient from
Fig. 5.41. After the tumor has been removed, an unex-
plained change in the BAEP trace occurred: decreased
amplitude and increased latency of wave V. At the end of
surgery, the BAEP trace recovered completely. The steps
of tumor removal and the corresponding BAEP wave
characteristics are demonstrated. (a, b) Dura opening
(baseline wave amplitude and latency) (11:07–11:11). (c,
d) Drilling of the IAC (slight decrease of the amplitude)
(11:12–11:17). (e, f) Initial tumor removal (further
decrease of the amplitude and increase of the latency)
(11:18–11:21). (g, h) The tumor has been completely
removed, and the facial and cochlear nerves are preserved
(sudden worsening of the BAEP, in particular of the wave
V amplitude and latency) (11:22–11:28). (i, j) Plugging of
the IAV with fat pieces and fibrin glue (slight improve-
ment BAEP) (11:28–11:32). (k, l) Dura closure (recovery
of the BAEP trace) (11:34–11:47)
260 5 Vestibular Schwannomas

e f

g h

Fig. 5.42 (continued)

Management of Patients with VS 261

i j

k l

Fig. 5.42 (continued)

262
Fig. 5.43 Preoperative MRI: intracanalicular VS that
extends up to the fundus area of the IAC. See Fig. 5.44 for
details
5.43, 5.44, 5.45, 5.46, and 5.47). In case of quick
recovery, the surgery is continued. If the change
persists, a short break is made and the retractor is
loosened or removed completely. The site and
type of surgical dissection or manipulation after-
Fig. 5.44 Loss of the BAEP response, occurring without
any obvious cause after the tumor removal. The intraop-
erative images and the corresponding BAEP wave charac-
teristics are presented. (a, b) The dura has been incised,
CSF has been evacuated, and a retractor has been posi-
tioned—baseline BAEP trace (11:24–11:30). (c, d) Drill-
ing of the posterior wall of the IAC—sudden worsening of
the BAEP trace (11:30–11:37). (e, f) Complete tumor
removal—improvement of the amplitude of wave V and
of the latency (11:44–11:49). (g, h) During the plugging
of the IAC with fat pieces, the BAEP trace worsened—the
5 Vestibular Schwannomas
wards are modified. Calcium channel blockers
(nimodipine) can be applied locally if BAEP
deterioration persists.
One highly sensitive technique is the use of
direct brain stem recording of the auditory evoked
potentials. The potentials are recorded by placing
a retractor with electrodes attached to its tip at the
cerebellomedullary junction [144]. Any move-
ment of the retractor, however, causes artifacts,
and monitoring throughout the procedure may
prove difficult.
Outcome
Hearing preservation rates reported in the liter-
ature vary from 17 to 71 % [79, 119, 189, 232,
245, 264]. This wide range of variation reflects
the use of different criteria for patient selection
and outcome evaluation, as well as the individ-
ual/institutional surgical expertise. The out-
come correlates with tumor size: hearing is
most likely to be preserved in VSs < 15 mm [79,
152]. The rate of hearing preservation in the
series of Yates [269] decreased from 72 % in
intracanalicular tumors to 34 % in tumors with
extension above 1 cm in the CP angle. Wiet
et al. [264] described a tendency toward wors-
ening hearing outcome for VSs beyond 1.1 cm:
useful hearing rate dropped from 57 % for intra-
canalicular tumors to 48 % for all tumors with
<1.0 cm extension into the CP angle and to only
17 % in case of tumors with <1.6 cm
extension.
The two hearing-preserving operative
approaches are the MF and the RS. If one
amplitude decreased severely and the latency increased
(11:55–11:59). Therefore, assuming that a compression-
induced cochlear nerve dysfunction occurred, the fat was
removed (one such piece is still visible). (i, j) Further
deterioration of the BAEP trace. A Gelfoam soaked with
nimodipine was placed over the cochlear nerve (assumed
vasospasm) (11:59–12:04). (k, l) Despite the removal of
the fat pieces and application of a nimodipine, no recovery
was seen. The IAC was plugged again—almost complete
loss of all BAEP components (12:04–12:09). (m, n) Dural
closure—complete loss of the BAEP (12:08–12:20)
Management of Patients with VS 263

a b

c d
264 5 Vestibular Schwannomas

e
f

g h

Fig. 5.44 (continued)

Management of Patients with VS 265

i j

k l

Fig. 5.44 (continued)

266
m n
Fig. 5.44 (continued)
compares only tumors of equal size, the hearing
preservation rates in experienced hands are simi-
lar, whatever the approach. The experience of the
surgeon rather than the advantages of a particular
technique determines the outcome. In large
tumors, however, the RS approach is the only
hearing-preserving option.
The hearing preservation rate in our general
series is above 51 %. The best overall preserva-
tion rates were found in VSs with slight exten-
sion in the CP angle (grade T2), 56 %, followed
by pure intracanalicular VSs (grade T1), 51 %,
and VSs filling the CP cistern (grade T3), 44 %.
These rates increased in the recent 200 cases to
72, 60, and 56 % in VSs grade 2, grade 1, and
grade 3a, respectively. Normal preoperative hear-
ing provided the highest preservation scores
(£88 %), whereas good or moderate hearing pro-
vided a chance of preservation of one-third to
more than two-thirds.
5 Vestibular Schwannomas
Long-Term Outcome
Durability of preserved postoperative hearing is
an important issue, more often discussed in the
neurootological literature. The exact incidence
and pathophysiological mechanisms of late
hearing loss have to be yet determined. Fibrosis
and scarring in the IAC, which gradually involve
the cochlear nerve, has been suggested as
provoking factor [252]. Histopathologic studies
of the temporal bone have demonstrated new
bone formation within the cochlea. Progressive
vascular changes have also been implicated
[149]. The important conclusion from these
studies is that hearing loss late after surgical
removal of VSs does not necessarily mean
tumor recurrence.
Hearing Preservation in Large VSs
Hearing preservation in patients with large VS
is very difficult to achieve but is possible, pro-
Management of Patients with VS 267

a b

Fig. 5.45 T2 vestibular schwannoma on the right side. (a, b) T1-weighted sequences after contrast administration. (c)
T2-weighted sequence. See Fig. 5.46 for details

vided the patient has good preoperative hearing
level and well-preserved BAEP. Increasing
number of surgeons report on successful hear-
ing preservation in such patients [52, 61, 174,
178, 258]. Wanibuchi et al. [258] reported
recently hearing preservation rate of 54 % of the
patients who had tumors larger than 20 mm. Di
Maio et al. [52] achieved hearing preservation in
21.4 % of the patients with VSs 3 cm or larger:
in those with normal preoperative hearing
(Gardner–Robertson class I hearing preopera-
tively), the rate was 30.8 %. In our experience,
in tumors extending to and compressing the
brain stem (grade T4a), the rate of hearing pres-
ervation (hearing classes I–III) after surgery was
high as 29 %, while in VSs grade T4b, the rate
268
a b
Fig. 5.46 Intraoperative images of the steps of tumor
removal and the corresponding changes of the BAEP
trace. (a, b) Exposure of the CP angle (Tu tumor, 7 facial
nerve, 8 vestibular nerve, P posterior pyramid) and inci-
sion of the dura around the posterior lip of the IAC (arrow-
heads). (c) Baseline BAEP trace. (d) Drilling of the
posterior wall of the IAC. (e) Worsening of the BAEP.
5 Vestibular Schwannomas
(f) The IAC is opened up to the fundus, as proven by the
palpation with the angled microhook. (g) Further worsen-
ing of the BAEP. (h) The dura of the IAC has been excised.
(i) Tumor removal. (j) Removal of the last tumor part in
the fundus. (k) Complete tumor removal. (l) The BAEP is
discernible but worse than initially
Management of Patients with VS 269

h g

Fig. 5.46 (continued)

270 5 Vestibular Schwannomas

i j

Fig. 5.46 (continued)

Management of Patients with VS 271

a b

Fig. 5.47 Postoperative images of the patient from the previous figure

was 27 % [194] (Fig. 5.48). Hearing preserva-
tion is possible even in patients with giant VSs.
In a recent study [196], we found that if the
patient has useful hearing before surgery, the
rate of hearing preservation (hearing classes
I–III) was 33 %; useful hearing had 11 % of the
patients in the series.
Hydrocephalus Associated with VSs
Patients with VSs are prone to develop hydro-
cephalus, either as a consequence of tumor-
induced obstruction at the level of fourth
ventricle and its outlets or due to malabsorption
of CSF. The incidence of hydrocephalus varies
strongly in published series—from 4 to
272 5 Vestibular Schwannomas

a b

d
c

Fig. 5.48 Large VS, operated by the senior author in the early 1990s. (a) Preoperative MR image. (b, c) Tumor expo-
sure and complete removal. (d) Normal postoperative facial nerve function. (e) Preserved functional hearing
Management of Patients with VS
42 %—reflecting the variable tumor size and cri-
teria for hydrocephalus [78, 239, 255]. The
radiological criteria for hydrocephalus that we
apply are those described by Pirouzmand et al.
[172]: the ratio of the maximal width of the fron-
tal horns to the maximal diameter of the inner
table of the cranium at the same level. If the ratio
is 30–39 %, the hydrocephalus is assessed as
mild, if the ratio is 40–45 % the hydrocephalus is
moderate, and if it is >46 % it is severe. The dif-
ferentiation of VS-associated hydrocephalus
from ventricular enlargement due to other causes
should be based on additional criteria: progres-
sion in the clinical symptoms that are not related
to the tumor or progressive ventricular enlarge-
ment [172].
Communicating or malabsorptive hydroceph-
alus is the more frequent type (61–87.2 % of all
patients with hydrocephalus) (Figs. 5.49 and
5.50). Its precise pathogenesis is still unclear, and
factors such as protein sloughing from the tumor
secondary to increased vascular endothelial per-
meability, development of meningeal adhesions,
arachnoiditis, or seeding of tumor cells are
believed to play an important role [145].
Obstructive hydrocephalus is caused by obstruc-
tion of the CSF flow at the level of the fourth ven-
tricle, its outlets, or basal cisterns (Fig. 5.51).
Characteristic radiological findings are the dis-
273
proportionally small fourth ventricle in relation
to the lateral and third ventricles in patients with
large VSs [185]. Complete VS removal does not
always lead to resolution of the hydrocephalus,
indicating that at least in some of these patients, a
malabsorptive component coexists. Hence, the
actual incidence of such “mixed hydrocephalus”
is still not known.
The clinical manifestation of hydrocephalus is
related to its severity and rate of development:
either NPH-like symptoms or symptoms/signs of
intracranial hypertension.
Management of Patients with Hydrocephalus
The VS-associated hydrocephalus is potentially
reversible condition, and complete tumor
removal frequently leads to resolution of the
symptoms. This strategy allows avoiding an
additional invasive procedure, such as EVD or
VP shunt placement, and shunt dependence in
most patients. In patients presenting with severe
hydrocephalus-related symptoms, however, the
hydrocephalus should be treated before tumor
surgery by placing an external ventricular
drainage (EVD). When the intracranial pres-
sure is controlled, the VS can be safely removed.
In case the tumor surgery has to be postponed
for some reason, a VP shunt has to be inserted
(see Case Illustration 5.15).
274 5 Vestibular Schwannomas

a b

c d

Fig. 5.49 MR study of a patient with severe VS-associated communicating hydrocephalus. Note the unobstructed
fourth ventricle, indicating that the cause of hydrocephalus is CSF malabsorption
Management of Patients with VS 275

Fig. 5.49 (continued) b

Fig. 5.50 Communicating hydrocephalus. (a) Patient

with VS grade T2. (b) Patient with VS grade T4a (b)
276 5 Vestibular Schwannomas

a b

Fig. 5.51 Obstructive hydrocephalus due to occlusion of the fourth ventricle (arrow)
Management of Patients with VS
Case Illustration 5.15
Large VS with Associated Hydrocephalus
This 69-year-old patient presented with a his-
tory of progressive hearing loss and gait insta-
bility for many years. During the last few
weeks, the gait worsened very rapidly and
strong headache with nausea appeared.
Neurological examination revealed profound
left-sided hearing loss, decreased corneal
reflex on the left side, and severe gait ataxia.
The MRI demonstrated large VS on the left
side (grade T4b) and communicating hydro-
cephalus (a–f). As an incidental finding,
inflammatory changes involving the mastoid
process and middle ear on the side of the
tumor were found. Considering the potential
risk related to the retrosigmoid craniotomy in
such circumstances, a decision was taken to
perform the tumor removal only after healing
the mastoid infection. An EVD was inserted
to control the intracranial hypertension, and
the mastoiditis was treated surgically (g, h).
The recovery period was protracted and
277
required 2 weeks of intensive therapy. Once
the acute infection was healed and the infec-
tious parameters were normalized, a VP shunt
was inserted and the patient was discharged.
The patient was admitted again in 3 months,
and the VS was removed completely via the
retrosigmoid approach (i–m). Although the
facial nerve was preserved anatomically, early
after surgery the patient had severe facial palsy
(HB grade V). She had furthermore swallow-
ing difficulty, which was interpreted as a
sequence of the manipulation of the lower cra-
nial nerves during tumor removal (note the
extent of caudal tumor extension on figure f).
During her hospital stay, however, peroral feed-
ing could be successfully initiated. The facial
nerve function improved rapidly, and at dis-
charge she was able to close the eye completely.
At follow-up 12 months after the surgery, she
had normal gait and balance (she could ski
again), very good facial nerve function (HB
grade II), and normal swallowing. The MR
study at follow-up is presented on figures n–q.
278 5 Vestibular Schwannomas

a b

c d

Preoperative CT (a, b) and MRI (c–f) of the patient
demonstrating the VS and the hydrocephalus. The
inflammatory changes of the mastoid can be well rec-
ognized on both CT and MRI sequences. (a, b) Bone-
window CT. (c) T1-weighted MR sequence after
gadolinium administration. (d) CISS sequence. (e)
T2-weighted sequence. (f) Coronal slice demonstrat-
ing the tumor and the dilatation of the ventricular sys-
tem. (g, h) CT scans after the operative treatment of
the suppurative mastoiditis. Intraoperative images
illustrating the steps of tumor removal. (i) Initial over-
view of the CP angle and of the VS. (j) The IAC had
been opened, and the facial nerve is identified. (k, l)
Dissection of the extracanalicular part of the facial
nerve by slightly pulling the tumor in lateral (k) or
superior direction (l). (m) Complete tumor removal
and preservation of the integrity of the facial nerve (Tu
tumor, 5 trigeminal nerve, 7 facial nerve, BS brain
stem). Postoperative images of the patient demonstrat-
ing the completeness of tumor removal. (n, o)
T1-weighted sequence after gadolinium administra-
tion. Note the artifact caused by the valve of the VP
shunt. (p) CISS MR sequence. (q) CT scan showing
the normal size of the ventricular system
Management of Patients with VS 279

e f

g h
h
280 5 Vestibular Schwannomas

i j

k l

m
Management of Patients with VS 281

n o

p q
282
Following tumor removal the patients should
be observed closely, both radiologically and clini-
cally. If clinical deterioration occurs and the CT
examination shows a persisting or increasing
widening of the ventricles, an EVD is inserted.
Initially the EVD, regardless if it is placed before
or after surgery, is positioned 10–12 cm H2O
above the level of the external auditory canal;
once the patients’ condition is stabilized, a pro-
cess of gradual “weaning from the EVD” is initi-
ated. If no symptoms/signs of intracranial
hypertension appear, the level of the EVD is ele-
vated progressively. In case the patient becomes
drowsy or other symptoms/signs occur, the level
is decreased and more CSF is allowed to drain for
several days. The whole period of weaning may
last up to 3 weeks. The drain is ultimately closed
for 24 h, and if the patient tolerates it well and the
intracranial pressure does not exceed 25 cm H2O
for more than 5 min, a CT is performed and the
EVD is removed. A VP shunt is inserted only in
case the “weaning” from the EVD fails. In case
hydrocephalus develops in a patient after surgery,
it was managed similarly with a temporary EVD.
Recently we evaluated the management of 53
patients with VS-associated hydrocephalus. In
48/53 patients, the tumor was removed initially,
and 42 of them (87.5 %) did not need additional
hydrocephalus treatment. Only 3/53 patients
required ultimately a VP shunt. Contrary to the
opinion of many authors [172, 239], we found
that the general and functional outcome in
patients with primary VS removal did not corre-
late to the existence of hydrocephalus. Moreover,
the surgery was not more complex or risky.
Important prerequisites for safe surgery are the
sufficient and adequate anesthesia and relaxation
of the patient, operating the patient in the semi-
sitting position, and drainage of sufficient CSF
from the lateral cerebellomedullary cistern.
Extremely important is the prevention of brain
edema during surgery, which is frequently caused
by an excessive cerebellar retraction or interrup-
tion of the venous drainage. We avoid any unnec-
essary opening of the basal cisterns in order to
prevent the wide distribution of blood, bone dust,
and tumor cells, thereby reducing the risk of
obstruction of CSF flow or its resorption.
5 Vestibular Schwannomas
The postoperative follow-up of the patients
should be mainly clinical because the ventriculo-
megaly may persist for months. As noted by
Pyrasmound et al. [172], the slow resolution of
the ventriculomegaly could be related to a mild
derangement in the CSF circulation or absorp-
tion or to a change in the elastance of the brain
tissue.
Radiosurgical Management of VS
In 1969 Lars Leksell was the first to treat a patient
with VS with gamma knife radiosurgery [129,
175]. During the following decades, the radiation
techniques and protocols continued to evolve,
and the procedure was established as one of the
main VS treatment modalities. Increasing num-
ber of patients are currently being treated with
radiation therapy—most frequently with radio-
surgery (gamma knife or cyber knife) or with
fractionated radiotherapy [7, 175]. The reported
tumor growth control rate in recent series is up to
91–98 % [16, 92, 117, 118, 132, 181, 192, 267].
Shrinkage of the tumor is observed in 21–75 % of
the cases, and tumor growth control (or absence
of progressive growth) is achieved in 18–59 %.
The facial nerve is preserved in more than 80 %
of the patients, and the probability of preserving
useful hearing ranges from 33 to 74 %.
These reported good results; the relatively
noninvasiveness and the cost-effectiveness of the
procedure promoted its wide spreading. According
to a recent survey by the Acoustic Neuroma
Association (2007–2008), 20 % of the patients
reported treatment by radiation compared with
5 % of patients in the 1998 survey [15].
On the other hand, radiation therapy is not
without hazards, in particular of cranial neuropa-
thy, balance disturbances, headache, and hydro-
cephalus. The reported rate of permanent facial
neuropathy ranges from 4 to 19 %, and the
trigeminal neuropathy rate is from 11 to 16 %.
Notably, radiation therapy is not curative: its goal
is to achieve tumor growth control. Treatment
failure or secondary regrowth after radiosurgery
that necessitates additional treatment is observed
in 2–9 % of the patients [7, 16, 42, 156].
Management of Patients with VS
We believe that complete tumor removal is the
mainstay of VS management: it cures the patient
and in experienced hands is related to very low
risk of morbidity. Radiosurgery is an option in
those patients that cannot be operated in general
anesthesia for some somatic problem and those
that are unwilling to have surgery. Chronological
age per se is not a contraindication for surgery.
Surgery of VSs, however, is very demanding and
follows a steep learning curve. An experience of
at least 200–300 cases is required to achieve an
acceptable proficiency level. Furthermore, opti-
mal results are achieved in centers of excellence,
in which at least 50–100 patients with VS are
operated annually. Contrary to that in case of
radiosurgery, high standards may be achieved
even with less experience, provided that the rec-
ommended protocols are followed. Therefore, we
believe that in neurosurgical settings with lower
case volume, radiosurgery should be the first line
of VS management.
Radiation therapy can be safely applied only
in small-to-medium-sized VSs. Recent popular
trend in the management of large VSs is to per-
form initially tumor debulking and later on radio-
surgery of the tumor remnant [71, 98]. The
cumulative effect of these two different types of
tissue injury, however, has still not been ade-
quately studied. The ultimate outcome has not
been systematically evaluated, and data from
larger series with sufficient follow-up period have
not yet been published. In our experience surger-
ies in patients that have undergone radiosurgery
after prior incomplete operative tumor removal
are most challenging and have worse outcome.
The detailed presentation and evaluation of
this management option is beyond the scope of
this book. We would refer to the multiple recent
publications on the topic.
Surgical Removal of VS After Failed
Radiosurgery
The focus of this chapter will be the surgical
management of VSs after failed previous radia-
tion therapy. Considering the rising number of
patients that undergo radiosurgery/radiotherapy,
it is obvious that this issue is becoming especially
important.
283
The probable explanation for the unrespon-
siveness of some VS to radiotherapy may be
related to their inherently low proliferation index
and the radioresistance of the tumor bulk [131,
270]. Treatment failure after radiosurgery/radio-
therapy is usually defined as progressive tumor
enlargement demonstrated on at least 2 imaging
studies [173]. Patients with failed radiosurgery
are managed either with repeated radiosurgery or
surgically [69, 74, 173]. Surgery is the only rea-
sonable option in case of large VSs that compress
the brain stem and in patients with progressive
neurological deterioration or major neurological
symptoms. Tumor enlargement or development
of new neurological symptoms per se, however,
is not an indication for immediate surgery. During
the first year after radiosurgery, VS frequently
increases in tumor volume and then shrinks spon-
taneously [175]. Such tumor enlargement may
cause transient neurological worsening and
should be differentiated from the real treatment
failure. New neurological deficits do not neces-
sarily indicate tumor activity: they may be a
delayed sequence of radiation, and a trial with
steroids should be undertaken. Therefore, surgery
in the early phase after radiosurgery is indicated
very rarely.
The goal of surgery should be total tumor
removal, as it is the only option that cures the
patient [74]. Some authors advocate tumor deb-
ulking or internal decompression as the preferred
management strategy for patients after radiosur-
gical failure in order to decrease the complication
and morbidity rates and to improve the chances
of preservation of cranial nerves function [128,
173, 216]. Subtotal removal may indeed lead to
better functional outcome in some cases, but it is
a short-term solution. It is difficult to assume that
a remnant of VS that expanded despite the previ-
ous therapy will change its biological behavior
and stop growing. Furthermore, even subtotal
tumor removal does not guarantee high rates of
functional preservation if performed from less
experienced surgeons.
Surgery after previous radiosurgery is indi-
cated in patients with large tumors compressing
the brain stem, in case of sustained tumor growth,
which is documented on serial MR examina-
284
tions, or in case of progression of symptoms,
such as significant cerebellar ataxia, pyramidal
tract signs, or intracranial hypertension, that
could be definitely related to tumor enlargement
and do not respond to steroid therapy. In a
recently evaluated series of 28 patients with VS
operated after failed previous radiosurgery by
the senior author, tumor enlargement was docu-
mented on serial MR examinations in 27 patients
[74]. Thirteen patients presented with progres-
sive neurological symptoms: intracranial hyper-
tension in 5 patients, gait imbalance in 7 patients,
and contralateral hemiparesis in 1 patient. The
indications for surgery were sustained tumor
enlargement and progression of neurological
symptoms in 12 patients and sustained tumor
enlargement in 15 patients. One patient was
operated despite the absence of progressive
tumor growth. He had a large VS (maximal
extrameatal diameter of 4.2 cm) and signs of
intracranial hypertension with papilledema.
Surgery in the early stage after radiosurgery was
performed only in one patient. He presented 6
months following radiosurgery with rapid neuro-
logical deterioration and a 4.7-cm-large VS that
severely compressed the brain stem.
Operative Technique
Microsurgery following previously radiosurgery
is complicated by the development of extensive
5 Vestibular Schwannomas
postradiational changes, causing tight adherence
of the tumor capsule or lack of clear arachnoid
plane, increased vulnerability of the facial nerve,
and radiation-induced vasculopathy with the
related tendency for delayed rupture and bleed-
ing [209, 220]. Our surgical concept and opera-
tive technique in such cases do not differ from
those in non-treated VSs of the same size (see
Case Illustrations 5.16 and 5.17). Important is the
sufficient initial CSF drainage to prevent addi-
tional retraction injury to the cerebellum. During
surgery especially important is the delicate dis-
section in the arachnoid plane. Although it might
be difficult to identify it, such a plane usually
exists. The 2-hand technique for tumor dissection
is especially helpful in this regard. Every effort
should be made to preserve even the small drain-
ing veins. The blood vessels are often more frag-
ile, and thorough hemostasis at the end of surgery
is mandatory.
The analysis of our results showed that total
removal of VSs after failed previous radiosur-
gery/radiotherapy can be achieved without mor-
tality and major morbidity [74]. Total tumor
removal was achieved in all patients. The post-
operative facial nerve function is indeed worse
than in untreated VSs of similar size, and the
risk of new neurological deficits or CSF leak
tended to be higher, but the differences were not
significant.
Management of Patients with VS
Case Illustration 5.16
Surgery Following Previous Radiosurgery
This patient (46-year-old female) presented 4
years after cyber knife treatment of her VS
(a–d). The follow-up MRI studies showed a
sustained tumor growth tendency, as well as
progressive dilatation of the ventricular system
(e–l). She complained of gradually worsening
gait and headache. At presentation the patient
had diminished corneal reflex, complete hear-
285
ing loss, tinnitus, and slight facial nerve palsy
(HB grade II) on the left side, as well as pro-
nounced gait ataxia. She was operated via the
retrosigmoid approach, and the tumor was
removed completely (see figures m–x). The
postoperative period was unremarkable, and the
patient was discharged 2 weeks after surgery.
Her facial nerve function early after surgery
was HB grade IV but improved to grade III at
discharge (the follow-up is just 3 months).
286 5 Vestibular Schwannomas

a b

c d

MR examination performed 1 year after radiosurgery:
(a, b) T1-weighted sequence after gadolinium admin-
istration. (c) CISS sequence. (d) T2-weighted
sequence. MRI performed 36 months after radiosur-
gery: the growth tendency of the tumor is obvious on
all slices: (e, f) T1-weighted sequence after gadolin-
ium administration. (g) CISS sequence. (h)
T2-weighted sequence. The MRI study 46 months
after radiosurgery demonstrated further tumor enlarge-
ment, as well as dilatation of the lateral and third ven-
tricles: (i, j) T1-weighted sequence after gadolinium
administration. (k) CISS sequence. (l) T2-weighted
sequence. A decision for surgery was taken at this
point. The patient was operated via the retrosigmoid
approach. (m) Initial view of the CP angle and the VS
(TS transverse sinus, SS sigmoid sinus, P posterior
pyramid, Tu tumor). (n) The tumor was covered with
an unusually firm capsule, which was as thick as the
dura overlying the pyramid (arrows) and had to be
excised with microscissors. (o) The IAC had been
widely opened (arrowheads—superior and inferior
walls of the IAC). (p) Partial removal of the intrac-
analicular tumor part and identification of the facial
nerve (FN). (q) Verification of the facial nerve with
electrostimulation (small arrows—petrous vein). (r)
Further removal of the intracanalicular tumor allowed
to expose the cochlear nerve (CN). (s) The arachnoidea
surrounding the extracanalicular tumor part (asterisks)
was very tightly attached to neighboring structures and
required meticulous dissection (small arrows—petrous
Management of Patients with VS 287

e f

g h

vein). (t, u) Once the tumor was debulked sufficiently,
its capsule could be dissected, and the facial nerve
close to the brain stem was exposed. The nerve was
dissected in the arachnoid plane further laterally; note
the slight lateral (t) and superior (u) traction of the
tumor remnant, which allows to find and to follow the
correct plane (the tumor is hold with a tumor forceps).
(v, w) The tumor part immediately next to the porus of
the IAC was most adherent and was dissected at the
end. Both the cisternal and the intracanalicular seg-
ments of the facial nerve (FN) are well visible on the
images. (x) Complete tumor removal; the facial nerve
was preserved anatomically and showed a good
response to electrical stimulation. Through hemostasis
and sealing of the IAC with fat pieces followed
288 5 Vestibular Schwannomas

i j

k l
Management of Patients with VS 289

m n

o p
290 5 Vestibular Schwannomas

q r

s t
Management of Patients with VS 291

u v

w x
292
Case Illustration 5.17
VS Removal Following Previous Surgery
and Radiosurgery
Twenty-year-old female patient presenting
with headache, nausea, severe gait ataxia, as
well as trigeminal hypesthesia, abducens
nerve palsy, complete facial nerve palsy, and
anacusis on the right side. She had been diag-
nosed approximately 2 years earlier of having
large VS on the right side. An attempt was
made to remove the VS surgically twice in her
city of origin; however, both surgeries had to
be interrupted after prolonged (>14 h) tumor
removal. The VS could be removed only par-
tially. Hearing and facial nerve function were
completely lost. Gamma knife treatment was
performed 5 months later, followed by implan-
tation of a VP shunt for hydrocephalus. Several
months after the gamma knife treatment, hyp-
esthesia in all three branches of the trigeminal
nerve appeared and could not be influenced by
the dexamethasone therapy. The MRI follow-
up examinations showed that the tumor con-
tinued to grow steadily (a–e). Her neurological
condition also deteriorated, and she developed
iatrogenic Cushing’s syndrome.
5 Vestibular Schwannomas
The patient was therefore reoperated in our
institute via the RS approach. The surgery
was complicated by the extensive thick scar
tissue surrounding the tumor. Interestingly,
the tumor core was stone hard, which was a
surprise, considering the MRI findings (typi-
cal “postradiational” hypointensity on
T1-weighted images). The tumor extended
into the dilated Meckel’s cave—in order to
remove this part, the petrous apex was drilled
off (suprameatal extension of the retrosigmoid
approach). Thus, complete tumor removal
was achieved, and the ventrally dislocated
abducens nerve was decompressed. Expectedly
the facial nerve was not identified. The early
postoperative CT control is shown on figure f.
The patient had an uneventful recovery, and 2
weeks later an end-to-side facial-to-hypoglos-
sal nerve anastomosis was performed (h–j).
One year later she could close the eyes com-
pletely with minimal effort. There was almost
complete symmetry at rest with slight asym-
metry of the mouth. Her Cushing’s syndrome
recovered completely (k–n). The MRI is
shown on figure g.
Management of Patients with VS 293

a b

c d

Preoperative MRI (a–e) and postoperative CT images
(e) of the patient. Note the hypointense on T1
sequences tumor core (a–c) and the edema of cerebel-
lum and ipsilateral temporal lobe (d, e). The CT was
made the day after surgery and shows moderate pneu-
mocephalus and hyperdense areas surrounding the
resection cavity (probable blood accumulation). The
occipital bone had not been reconstructed after the ini-
tial surgeries. Despite the watertight dural closure, we
did not make methyl methacrylate (Palacos) cranio-
plasty at this stage in order to prevent CSF leak.
(g) Postoperative MRI study. End-to-side facial-to-
hypoglossal nerve anastomosis was performed 2
weeks after tumor removal. (h) Exposure of the facial
(7) and hypoglossal nerves (12). (i) Exposure of the
proximal segment of the facial nerve in the mastoid
(7). (j) Fixation of the facial nerve stump on the par-
tially incised hypoglossal nerve (12) with 2 sutures.
Photos of the patient before (k, l) and 12 months after
reconstruction of the facial nerve (m, n). Note the ini-
tial cushingoid appearance. The patient could close the
eyes completely with minimal effort; there was almost
complete symmetry at rest with slight asymmetry of
the mouth
294 5 Vestibular Schwannomas

g h
Management of Patients with VS 295

i j

k l
296 5 Vestibular Schwannomas

m n
Management of Patients with VS
Operative Complications: Avoidance
and Management
The risk for surgery-related complications corre-
lates with certain patient-, tumor- and surgeon-
related factors. Although the morbidity is higher
in the elderly, as shown by some studies [161],
we believe that patient’s general physical condi-
tion—but not his age—is a more important con-
sideration. Large tumors and NF-2-associated
tumors are generally related to higher morbidity
rate. The experience of the surgeon and of the
whole team involved in the management of the
patient, along with tumor size, is the key factor
determining the outcome. It has been repeatedly
shown those both functional and general out-
comes reflect the level of the learning curve of
the operator [29, 194, 262].
When evaluating the morbidity rates reported
in the literature, an issue of utmost importance is
whether the outcome is correlated with tumor
size and with the completeness of tumor removal.
The comparisons of different surgical approaches
or modes of VS management are of little value if
these issues, as well as the surgeons and institu-
tions experience with a given approach, are not
taken into account.
Cranial Nerves
The main principle for avoiding cranial nerve-
related complications is their early identification
and avoiding their injury during surgical manipu-
lations (stay in the arachnoid plane!). Importantly,
patients at risk for developing such complications
should be actively managed in the postoperative
period to avoid potential problems.
Large tumors may cause compression and/or
dislocation of the trigeminal, trochlear, abducens,
and lower cranial nerves, which renders them
more susceptible to injury during surgical manip-
ulations. The trigeminal nerve is more resistant to
compression and manipulations than the facial or
cochlear nerves. Patients with VS present very
rarely with trigeminal neuralgia. Removal of the
tumor usually leads to improvement or resolution
of the pain. Nevertheless, its dissection from the
tumor may lead to numbness in some of its
branches. Trigeminal neuralgia after surgical
removal of VS has been also reported but is an
297
exception (0.3 % in the series of Sanna et al.
[204]). Extreme care is required in case of com-
bined lesions to both the trigeminal and facial
nerves because this may cause major eye trophic
problems and corneal ulcerations.
Large tumors with major inferior extension
may cause lower cranial nerve deficits, which
usually become even more severe after surgery. If
preoperative deficit related to these nerves is
absent, however, the risk of new deficits after the
surgery is lower. The lower cranial nerves are
usually protected by an arachnoid layer and can
be safely dissected. New lower nerve deficit has
been reported to occur in 0.14–4.2 % of the
patients [198, 204, 234]. In the recent series of
the senior author, three patients (1.5 %) had lower
cranial nerve disturbances in the early postopera-
tive period. Two of them had caudal nerve signs
preoperatively [194]. Lower cranial nerve dys-
function can cause diminished gag reflex, dys-
phagia, hoarseness, and paralysis of vocal cord,
with a threat of aspiration. Even in the presence
of gag reflex at examination, however, the coordi-
nation of the swallowing mechanism might be
dysregulated (see Chap. 10 for detailed
discussion).
The ocular motor nerves are at risk in case of
large tumors with medial extension in the petro-
clival area. As pointed earlier, the RS approach
allows for early identification of the abducens
nerve, either in the area of Dorello’s canal or at
its brain stem exit site. The trochlear nerve can be
identified along the tentorial incisura. The
reported rates of such deficits range between 1.7
and 7 % [109, 198, 204]. Frequently the patients
complain of difficulty in focusing the eyes or
diplopia but have no obvious strabismus: such
complaints usually resolve in few weeks.
Cerebellar Complications
Cerebellar symptoms usually improve after sur-
gery. Most patients complain of temporary rela-
tive mild unsteadiness that clears over several
days to several weeks. In case of severe walking
difficulty, discoordination, and dysarthria the
recovery period is, of course, longer [198].
Exacerbation of the gait instability or cerebellar
dysfunction occurs rarely after VS removal. In
298
the previously evaluated series of 1,000 patients
with VS operated by the senior author, the cere-
bellum was hemorrhagic or ischemic in seven
patients. Severe permanent ataxia persisted in
two of them, while the ability to walk freely
recovered in the rest.
Vestibular Symptoms
Removal of a VS usually destroys completely the
vestibular function on the side of the lesion.
Exceptions are patients with small tumors when
one of the vestibular nerves is preserved.
Vestibular symptoms, such as disequilibrium,
dizziness, vertigo, and imbalance, are therefore
frequent complaints after VS resection [138,
228]. The decompensated state after surgery,
characterized by strong vertigo, nystagmus, nau-
sea, and vomiting, lasts usually for only up to 3
days. Vestibular dysfunction after surgery tends
to be transient, and the vast majority of the
patients recover due to the phenomenon of ves-
tibular compensation. Some patients, however,
need much longer to recover—up to several
weeks. The complaints are more severe after
removal of small VS when normal or near-normal
vestibular function was present preoperatively
[228]. The preservation of a given vestibular
nerve neither prevents vestibular disturbances
nor does it promote functional recovery. Of para-
mount importance for the recovery are the physi-
cal activity and the systematic vestibular
training.
Tinnitus
Tinnitus is the second most frequent symptom in
patients with VSs and may severely affect the
quality of life [10, 108]. Removal of the tumor
does not, however, influence its occurrence or
characteristics. The evolution of tinnitus follow-
ing surgery cannot be predicted. Furthermore,
our experience shows that the cochlear nerve sec-
tioning does not have an effect on the tinnitus
either.
Vascular Complications
The vascular complications are perhaps the most
serious morbidity that can occur after surgery.
They may have serious and lasting consequences
5 Vestibular Schwannomas
and even cause the death of the patient. The inci-
dence of vascular complications in large series
ranges from 1 to 7 % [25, 83, 198, 204, 234].
Hemorrhagic complications are more frequent
than ischemic. In a recently published study, Sade
et al. evaluated a series of patients with VS that
underwent 413 procedures. Vascular complica-
tions were detected in 11 patients (2.7 %): hem-
orrhage in 8 patients and ischemia in 3 patients.
The comparison of the incidence of these compli-
cations after the RS approach and the translaby-
rinthine approach showed that it was similar.
Bleeding in the CP angle is the most common
hemorrhagic complication, followed by cerebel-
lar bleeding. Occasionally epidural or subdural
hematomas occur. The hemorrhage is usually
diagnosed early after the patients’ extubation: in
33–40 % they are detected within the first 24 h
[56, 198, 204]. Early recognition and active man-
agement of such complications are prerequisite
for avoiding worse outcome. Patients with a pro-
longed awakening phase, those who remain
drowsy or show progressive deterioration in their
level of consciousness or worsening neurological
deficit, should undergo immediate CT scanning.
Delayed diagnosis, for example, when the patient
is already comatose, can explain the high mor-
bidity rates after CP angle bleeding. In our expe-
rience cystic and/or uncapsulated tumors have a
higher risk of postoperative hematoma. Patients
with such tumors, as well as those with large VS
or in case of difficult hemostasis, should be fol-
lowed especially carefully. The blood pressure
should be permanently monitored—initially with
an intra-arterial sensor—and arterial hyperten-
sion should be treated aggressively (the hypoten-
sion may have also a deleterious effect!).
Patients with a postoperative epidural hema-
toma have a relatively better outcome (Fig. 5.52).
They are most frequently caused by a bleeding
artery in the suboccipital area: the blood collec-
tion is in the soft tissue and secondarily extends
into the epidural space. We have never had hem-
orrhagic complications, such as subdural or
intracerebral hemorrhages, related to the semi-
sitting position.
In some cases, small blood collections in the
tumor cavity or CP angle are detected on routine
Management of Patients with VS
a Electrophysiological monitoring is advisable,
b cause of ischemic complications. Vasospasm is
c
Fig. 5.52 Patient with an acute postoperative epidural
hematoma in the craniotomy area. (a) Preoperative MRI
study showing the VS. (b, c) Postoperative CT, performed
because of the prolonged awaking phase. The early detec-
tion of the hematoma allowed rapid treatment and avoid-
ance of any related complications
299
postoperative CT scans. If they are not causing
mass effect or clinical symptoms, they are man-
aged conservatively and the blood is absorbed
within several days. Still, the possibility of grad-
ual increase of the blood volume should be con-
sidered and regular imaging follow-up should be
performed. Immediate evacuation is indicated if
the hemorrhage has a mass effect and/or the con-
dition of the patient deteriorates. The blood cloths
should be removed and a meticulous hemostasis
should be made. The cranial nerves are at higher
risks in case of large hemorrhages with absent
normal anatomical landmarks.
although not always available in case of emer-
gency. Frequently, the source of bleeding cannot
be identified, and a venous origin may be sus-
pected. The exposed brain surfaces are covered
with a layer of Tabotamp.
Brain stem infarcts occur usually during the
dissection of adherent tumor parts from the brain
stem when perforating vessels are interrupted
(Darrouzet). Sade et al. refer to the description of
Rhoton that recurrent perforating arteries can ini-
tially course toward the IAC but subsequently
turn medially and supply the brain stem.
Occlusion of a large artery is currently a rare
another proposed but not proven mechanism
leading to infarction.
Although some surgeons state that the petrosal
veins can be sacrificed with relative safety, we
believe that the venous anatomy of the CP angle
is so variable that the consequences of such inter-
ruption cannot be predicted.
Sinus Thrombosis
This is a relatively underestimated complication,
probably due to its more subtle and late manifes-
tation. According to one study, its rate after VS
surgery may reach 4.7 % [112]. The thrombosis
may occur after injury or laceration to the sinus,
drying and shrinkage of the sinus if it is overex-
posed and the protective layer of bone is missing,
or after venous stasis due to its prolonged retrac-
tion (required in the translabyrinthine approach).
The symptoms and speed of clinical deterioration
depend on the anatomical structure of a given
300
patient: dominance and size of the sinus, pres-
ence of collaterals and of a communicating torcu-
lar herophili [191].
Sinus occlusion may have a milder evolution
with headache and visual deterioration beginning
days after the surgery. Alternatively, it may cause
a dramatic clinical picture, especially if concomi-
tant hemorrhages appear.
Pneumocephalus
Pneumocephalus occurs relatively frequently in
patients operated in the semi-sitting position.
Symptomatic pneumocephalus is, however, rarely
seen. The volume of trapped air tends to correlate
with the duration of surgery, the amount of evac-
uated CSF, and the degree of brain atrophy of the
patient. If pneumocephalus is suspected, a CT
should be made to assess its space occupying
effect. Patients with symptomatic tension pneu-
mocephalus and radiological evidence of more
severe compression of the ventricles may require
burr hole trepanation and evacuation of the air
collection (a detailed discussion on the topic is
provided in the Chap. “RS Approach”).
Postoperative Suboccipital Headache
The issue of postoperative suboccipital headache
related to the RS approach and its prevention is
discussed earlier (section “Retrosigmoid
Approach”). In summary, the technique of skull
base reconstruction with methyl methacrylate
reduces significantly the risk of such headache,
most probably by preventing scar tissue forma-
tion between the dura and neck muscles: less than
1 % of the patients in our experience have persis-
tent headache. Very strong occipital pain with
possible cranial irradiation, however, may be
caused by the formation of posttraumatic scar
neuroma or scar tissue around the occipital
nerves. In such cases, a trigger point can always
be found near the incision line. A reliable test
with potential therapeutic effect is the infiltration
of the area around the trigger point with local
anesthetics: it usually ameliorates the symptoms.
In case the pain subsides but later on recurs, the
scar should be widely excised. A more limited
excision of the scar or of the neuroma may lead to
a recurrence.
5 Vestibular Schwannomas
CSF Leak
CSF leak is one of the relatively common compli-
cations after CP angle surgery, whatever approach
is utilized. The reported incidence in the litera-
ture varies widely—from 2 to 30 % [24, 64, 134,
150, 184, 198]. Higher rates have been reported
in the older series. In the last decade, however,
the improved techniques of dura and wound clo-
sure lead to significant reduction of the CSF leak
rate: in large current series, the rate is below
5 %.
CSF leaks appear typically during the first 2
weeks after surgery. Delayed leak is rare but pos-
sible, and the patients should be instructed
accordingly. Although some authors reported that
the most frequent site of CSF leakage is the skin
[24], in our experience this is exceptional. Key
factors are the good operative technique, meticu-
lous attention to dura and wound closure—allow-
ing quick wound healing—and avoidance of
infection. Rhinorrhea is the most frequent type of
CSF leak in our experience. The communication
between the cisternal space and the external
structures is usually air cells surrounding the
IAC. The extent of pneumatization of the bone
correlates with the risk of CSF leak. These air
cells are frequently opened during the meatal
drilling and should be thoroughly occluded at the
end of surgery. As shown by Hoffman, the leak
rate is 16 % after drilling the IAC and 0 % if the
IAC is not drilled. Of course, this is a historical
observation, and the need to open the IAC is a
basic principle of VS surgery.
In case of paradoxical rhinorrhea, the CSF
leak pathway is through a dural defect to the epi-
dural space and then to the mastoid cells. If these
cells are not sealed adequately, the CSF may
enter the middle ear and the Eustachian tube.
Otorrhea with the RS approach is an exceptional
finding reported in the literature when the tym-
panic membrane has been perforated while posi-
tioning the cochlear electrodes [24].
Factors associated with the risk for CSF leak
are related to the surgical technique, the clinical
management, the patient’s anatomy, and the
tumor features [134, 227].
Surgical Technique: The factors discussed in
the literature are suboptimal dural and/or wound
Management of Patients with VS
closure, wound infection, inadequate wound
healing, increased intracranial pressure, or
opened and not occluded air cells in the petrous
bone or mastoid. Inadequate dura closure may
cause CSF leak through the wound or fluid accu-
mulation under the skin (pseudomeningocele).
The closure of air cells opened during the
craniotomy or IAC drilling is a crucial issue.
Various materials have been applied in the attempt
to achieve a leakproof occlusion. Bone wax has
been used widely in the past. The experience has
shown, however, that this material cannot provide
reliable sealing. Furthermore, if a large amount
of wax is used, it may cause foreign body reac-
tions and/or infectious complications.
Hydroxyapatite bone cement for the reconstruc-
tion of the petrous bone or of the drilled IAC [11]
is also not recommendable. In a recent study,
Kerr et al. [114] showed that in 7.3 % of the
patients, a delayed extrusion of the hydroxyapa-
tite cement used for petrous reconstruction
occurred. Autologous materials are utilized most
frequently: muscle, fat, bone dust, fascia, and
even soft tissue flaps. These materials are plugged
into the defect and fixed with fibrin glue. We con-
sider the use of fat instead of muscle pieces sealed
with fibrin glue as a major breakthrough in the
prevention of CSF leaks. Muscle tends to shrink
over the time and is reorganized to a scar. The
CSF leak rate in our older series, published in
1996, was 9.2 %. In a later patient series, oper-
ated between 2001 and 2004, the rate was reduced
to 5.4 %. Since we started to apply fat in the
described above technique in 2004, the rate
dropped to 2.2 % [134]. Furthermore, almost all
patients with CSF leak are managed
conservatively.
It has been frequently discussed which
approach is related to higher risks for CSF leak.
Many authors found no significant correlation
between the approach and the leak incidence. We
believe that whatever approach is used, if the dura
and wound are closed meticulously, the risk of
this complication can be reduced significantly.
Patient Factors: The extent of petrous bone
pneumatization correlates with the risk of CSF
leak. The CSF leak rate is somewhat lower in
female compared to male patients (3.4 % vs.
301
5.6 %) [227], which may reflect the more pro-
nounced pneumatization of the mastoid bone in
males. A predisposing factor for CSF leak is the
increased intracranial pressure. In patients with
hydrocephalus, the leak rates are significantly
higher [239]. If the CSF leak persists despite
sufficient drainage via the lumbar drainage, CT
of the head has to be performed to exclude
hydrocephalus.
Tumor Factors: The relation between tumor
size and CSF leak rate is controversially dis-
cussed in the literature. Large tumor size is con-
sidered by some authors as a factor increasing the
leak rate [25, 28], while others did not find any
significant correlation [63, 125]. The evaluation
of our series showed that highest CSF leak rate
had patients with small intrameatal schwanno-
mas (class T1), which was 9.7 %, while in those
with large VS (grade T4a), the rate was 2.7 %.
Similar data have been presented by Sanna et al.
[204]: the CSF leak rate was higher in patients
with small tumors. Previous radiotherapy is con-
sidered as a factor that significantly increases the
risk of CSF leak [55]. In our series we could not
proof that such correlation exists. Previous sur-
gery, however, is a negative prognostic factor
[55]. We also found that the CSF leak rate in such
patients is 11.1 % compared to a 4.4 % rate in the
control group of patients.
Prevention and Management: The operative
technique is the key to CSF leak prevention (dis-
cussed elsewhere in the text). The patients should
be closely monitored in the postoperative period.
They have to be instructed to avoid activities
leading to increased intracranial pressure, such as
blowing the nose or pressing against the closed
glottis, during the early postoperative period.
CSF leaks should be detected early and managed
adequately in order to prevent potential compli-
cations, such as meningitis caused by ascending
bacteria or tension pneumocephalus.
As pointed by Luedemann et al. [134], the
leak of fluid out of the nose after surgery does not
necessarily mean that it is CSF. The source can
be the fluid collected and retained in the mastoid
air cells during surgery or nasal secretion. Nasal
decongestants can be given to exclude a local
nasal cause. The patient’s sensation may give a
302
clue: CSF has a metallic, salty, or sweet taste. In
case a leak is suspected, we perform a provoca-
tion test: the patient is asked to lower his head for
at least 5 min and is observed. If some fluid is
collected, it is tested for glucose or for beta-trace
protein to assess if it is CSF. After albumin, the
beta-trace protein is the protein with highest con-
centration in the CSF. Bachmann-Harildstad [8]
showed recently that both beta2-transferrin and
beta-trace protein are reliable immunological
markers for the detection of CSF traces.
We do not favor an initial period of conserva-
tive measures, consisting only of bed rest, head
elevation, pressure dressings, or frequent lumbar
punctures. If the presence of CSF leak is proven,
a lumbar drain is placed for at least 7 days.
Prophylactic broad-spectrum antibiotic is also
given, and the CSF infectious parameters are
regularly tested. The level of the drainage is gen-
erally adjusted to produce between 150 and
250 ml in 24 h. If the CSF leak stops, the drain-
age system is closed at the seventh day for 24 h
and then the provocation test is repeated.
In our recently analyzed series of 420 patients
operated during the period 2001–2006, CSF leak
had 19 patients (4.5 %). The conservative man-
agement, described above, was successful in all
but 1 of the patients. There were no cases of
meningitis.
Indications for surgical revision are the cases
when the leak continues despite the drainage or
reappears after its closure. The patient is put
supine on the operative table with rotated head.
The area around the IAC is examined for opened
and non-occluded air cells. Frequently, the fat
pieces that have been inserted at the initial sur-
gery are found to be dislocated. The reason for
that can be strong CSF pulsations or inadequate
distribution of the fibrin glue. The fat pieces are
removed; the area is thoroughly inspected and
sealed again.
Meningitis
The reported rates of meningitis after VS surgery
vary from 0 to >10 % [25, 125, 142, 143, 198]. In
the recent series, it is less than 2 % with a ten-
dency for further decline. The optimized preven-
tion of CSF leaks is one of the possible
5 Vestibular Schwannomas
explanations: besides to the general predisposing
factors, meningitis is found to correlate to the
presence of a CSF leak. Aseptic meningitis usu-
ally becomes apparent 2–21 days after surgery
and is treated with tapering dose of steroids.
Postoperative Hydrocephalus
Symptomatic dilatation of the ventricular system
following surgery is a well-known phenomenon
that can cause acute deterioration of the patient.
It has to be detected and treated early enough to
prevent irreversible brain damage. Surgery in
itself may hinder normal CSF dynamics and
thereby potentiate an existing hydrocephalus or
cause the development of new hydrocephalus.
Obstruction at the level of fourth ventricle and its
outlets is provoked most frequently by cerebellar
edema or postoperative hemorrhage. Blood prod-
ucts in the CSF can affect its absorption and
cause chronic hydrocephalus and NPH-like
symptoms.
Careful operative technique and meticulous
hemostasis are critical for the prevention of this
complication. Cerebellar edema can be caused by
excessive cerebellar retraction or interruption of
venous drainage. In large tumors, causing long-
standing neural compression, even the slightest
manipulations can provoke cerebellar edema.
The basal cisterns should be opened only as much
as necessary for the tumor exposure. Redundant
opening of the cisterns is to be avoided in order to
prevent wide spread of blood, bone dust, and
tumor cells or particles within the CSF.
Active management is necessary in case of a
progressive dilatation of the ventricular system
that correlates with a prolonged awakening phase,
incomplete awakening, or clinical deterioration
of the patient (Fig. 5.53). One should not rely
simply on the absolute measurements of the ven-
tricular size. It is important always to compare
the ventricles with the previous images of the
same patient and determine if a tendency for their
enlargement exists. Patients with less severe
hydrocephalus can be initially followed. General
measures, such as head elevation and dexametha-
sone, are applied, and the neurological and clini-
cal status (blood pressure, heart rate!) are
monitored closely. Radiological control with CT
Management of Patients with VS 303

a b

Fig. 5.53 Fifty-five-year-old female patient with a VS on
the left side. The MRI study demonstrated a cystic VS with
highly irregular surface and moderate compression of the
brain stem (a–c). The surgery was straightforward: the
tumor was removed completely, and all neural and vascular
structures were preserved. Several hours after extubation,
however, the patient became somnolent. CT examination
was performed and showed extensive cerebellar edema
causing obstruction of the fourth ventricle and correspond-
ing hydrocephalus (d). An EVD was inserted immediately.
Within 10 days the swelling subsided (e), and the patient
could be successfully weaned from the EVD. At discharge
did not have any new neurological deficits besides an HB
grade II facial nerve function
304
is performed frequently, guided by the patient’s
clinical condition.
In patients with pronounced symptoms, an
EVD is inserted. It is kept as long as necessary to
allow regression of the cerebellar edema and
recovery of the normal CSF circulation.
Afterwards, an attempt for weaning from the
EVD is done, as described in the section on
hydrocephalus. A VP shunt is required if the
weaning fails. In a recently evaluated group of
400 patients, we found five, in whom symptom-
atic hydrocephalus after the VS surgery devel-
oped. In all cases an EVD was inserted. Weaning
from the EVD was successful in 3 cases; the
remaining 2 required a VP shunt.
Mortality
During the last decades, the mortality rate after
VS surgery has been dramatically reduced and
nowadays should be an exception. In the large
series it is between 1 and 2 %. In our earlier expe-
rience [198] the mortality rate was 1.1 %. The
cause of this outcome was myocardial infarction;
acute pulmonary embolism; sinus thrombosis and
contralateral media infarction with a large edema,
secondary to revision surgery for a postoperative
hemorrhage; or aspiration pneumonia due to
severe caudal cranial nerve palsies. One patient
was reoperated for a postoperative CP angle hem-
orrhage but did not recover. The patients at risk
are premorbid patients with previous surgery or
with severe brain stem compression, patients with
very large VSs and complete caudal nerve palsy
after surgery, and patients prone to have postop-
erative hemorrhage (cystic or nonencapsulated
tumors) [198]. In premorbid patients with very
large tumors causing significant brain stem com-
pression, the option of performing only internal
decompression should be considered. In the last
10 years, the mortality rate was 0 %.
References
1. Abe T, Izumiyama H, Imaizumi Y, Kobayashi S,
Shimazu M, Sasaki K, Matsumoto K, Kushima M
(2001) Staged resection of large hypervascular ves-
tibular schwannomas in young adults. Skull Base
11(3):199–206
5 Vestibular Schwannomas
2. Acioly MA, Gharabaghi A, Liebsch M, Carvalho CH,
Aguiar PH, Tatagiba M (2011) Quantitative parame-
ters of facial motor evoked potential during vestibular
schwannoma surgery predict postoperative facial
nerve function. Acta Neurochir (Wien). doi:10.1007/
s00701-011-0995-4
3. Allcutt DA, Hoffman HJ, Isla A, Becker LE,
Humphreys RP (1991) Acoustic schwannomas in
children. Neurosurgery 29(1):14–18
4. Anderson DE, Leonetti J, Wind JJ, Cribari D, Fahey K
(2005) Resection of large vestibular schwannomas:
facial nerve preservation in the context of surgical
approach and patient-assessed outcome. J Neurosurg
102(4):643–649. doi:10.3171/jns.2005.102.4.0643
5. Angeli RD, Piccirillo E, Di Trapani G, Sequino G,
Taibah A, Sanna M (2011) Enlarged translabyrinthine
approach with transapical extension in the manage-
ment of giant vestibular schwannomas: personal expe-
rience and review of literature. Otol Neurotol
32(1):125–131. doi:10.1097/MAO.0b013e3181ff7562
6. Arriaga MA, Carrier D, Houston GD (1995) False-
positive magnetic resonance imaging of small internal
auditory canal tumors: a clinical, radiologic, and
pathologic correlation study. Otolaryngol Head Neck
Surg 113(1):61–70, S0194599895001720 [pii]
7. Arthurs BJ, Fairbanks RK, Demakas JJ,
Lamoreaux WT, Giddings NA, Mackay AR, Cooke
BS, Elaimy AL, Lee CM (2011) A review of treat-
ment modalities for vestibular schwannoma.
Neurosurg Rev 34:265–277. doi:10.1007/s10143-
011-0307-8
8. Bachmann-Harildstad G (2008) Diagnostic values of
beta-2 transferrin and beta-trace protein as markers
for cerebrospinal fluid fistula. Rhinology 46(2):
82–85
9. Badie B, Pyle GM, Nguyen PH, Hadar EJ (2001)
Elevation of internal auditory canal pressure by ves-
tibular schwannomas. Otol Neurotol 22(5):696–700
10. Baguley DM, Humphriss RL, Axon PR, Moffat DA
(2006) The clinical characteristics of tinnitus in
patients with vestibular schwannoma. Skull Base
16(2):49–58. doi:10.1055/s-2005-926216
11. Baird CJ, Hdeib A, Suk I, Francis HW, Holliday MJ,
Tamargo RJ, Brem H, Long DM (2007) Reduction
of cerebrospinal fluid rhinorrhea after vestibular
schwannoma surgery by reconstruction of the drilled
porus acusticus with hydroxyapatite bone cement.
J Neurosurg 107(2):347–351. doi:10.3171/JNS-
07/08/0347
12. Bakkouri WE, Kania RE, Guichard JP, Lot G, Herman
P, Huy PT (2009) Conservative management of 386
cases of unilateral vestibular schwannoma: tumor
growth and consequences for treatment. J Neurosurg
110(4):662–669. doi:10.3171/2007.5.16836
13. Barker GF, Ojemann RG (1993) Surgical approaches
to tumors of the posterior fossa cranial nerves (exclud-
ing acoustic neuromas). In: Barrow DL (ed) Surgery
of the cranial nerves of the posterior fossa. American
Association of Neurological Surgeons, Park Ridge, pp
289–306
References
14. Baser ME, Makariou EV, Parry DM (2002) Predictors
of vestibular schwannoma growth in patients with
neurofibromatosis Type 2. J Neurosurg 96(2):217–
222. doi:10.3171/jns.2002.96.2.0217
15. Bassim MK, Berliner KI, Fisher LM, Brackmann DE,
Friedman RA (2010) Radiation therapy for the treat-
ment of vestibular schwannoma: a critical evaluation
of the state of the literature. Otol Neurotol 31(4):567–
573. doi:10.1097/MAO.0b013e3181d8d3ad
16. Battista RA (2009) Gamma knife radiosurgery
for vestibular schwannoma. Otolaryngol Clin North
Am 42(4):635–654. doi:10.1016/j.otc.2009.04.009,
S0030-6665(09)00064-4 [pii]
17. Bederson JB, von Ammon K, Wichmann WW,
Yasargil MG (1991) Conservative treatment of
patients with acoustic tumors. Neurosurgery 28(5):
646–650; discussion 650–651
18. Bloch DC, Oghalai JS, Jackler RK, Osofsky M,
Pitts LH (2004) The fate of the tumor remnant after
less-than-complete acoustic neuroma resection.
Otolaryngol Head Neck Surg 130(1):104–112,
S0194599803015985 [pii]
19. Bonneville F, Savatovsky J, Chiras J (2007) Imaging
of cerebellopontine angle lesions: an update. Part 2:
intra-axial lesions, skull base lesions that may invade
the CPA region, and non-enhancing extra-axial
lesions. Eur Radiol 17(11):2908–2920. doi:10.1007/
s00330-007-0680-4
20. Brackmann DE, Bartels LJ (1980) Rare tumors of the
cerebellopontine angle. Otolaryngol Head Neck Surg
88(5):555–559
21. Brackmann DE, Green JD (1992) Translabyrinthine
approach for acoustic tumor removal. Otolaryngol
Clin North Am 25(2):311–329
22. Brackmann DE, Green JD Jr (2008) Translabyrinthine
approach for acoustic tumor removal 1992. Neurosurg
Clin N Am 19(2):251–264. doi:10.1016/j.nec.2008.
02.007, S1042-3680(08)00008-9 [pii], vi
23. Brackmann DE, Owens RM, Friedman RA,
Hitselberger WE, De la Cruz A, House JW, Nelson
RA, Luxford WM, Slattery WH 3rd, Fayad JN (2000)
Prognostic factors for hearing preservation in vestibu-
lar schwannoma surgery. Am J Otol 21(3):417–424
24. Brennan JW, Rowed DW, Nedzelski JM, Chen JM
(2001) Cerebrospinal fluid leak after acoustic neu-
roma surgery: influence of tumor size and surgical
approach on incidence and response to treatment. J
Neurosurg 94(2):217–223. doi:10.3171/jns.2001.94.
2.0217
25. Briggs RJ, Fabinyi G, Kaye AH (2000) Current man-
agement of acoustic neuromas: review of surgical
approaches and outcomes. J Clin Neurosci 7(6):521–
526. doi:10.1054/jocn.2000.0728, S0967-5868(00)
90728-8 [pii]
26. Briggs RJ, Luxford WM, Atkins JS Jr, Hitselberger
WE (1994) Translabyrinthine removal of large acous-
tic neuromas. Neurosurgery 34(5):785–790; discus-
sion 790–791
27. Bruder CE, Ichimura K, Tingby O, Hirakawa K,
Komatsuzaki A, Tamura A, Yuasa Y, Collins VP,
305
Dumanski JP (1999) A group of schwannomas with
interstitial deletions on 22q located outside the NF2
locus shows no detectable mutations in the NF2 gene.
Hum Genet 104(5):418–424
28. Bryce GE, Nedzelski JM, Rowed DW, Rappaport JM
(1991) Cerebrospinal fluid leaks and meningitis in
acoustic neuroma surgery. Otolaryngol Head Neck
Surg 104(1):81–87
29. Buchman CA, Chen DA, Flannagan P, Wilberger JE,
Maroon JC (1996) The learning curve for acoustic
tumor surgery. Laryngoscope 106(11):1406–1411
30. Cafer S, Bayramoglu I, Uzum N, Yilmaz M, Memis
L, Uygur K (2008) Expression and clinical significance
of Ki-67, oestrogen and progesterone receptors in
acoustic neuroma. J Laryngol Otol 122(2):125–127.
doi:10.1017/S0022215107000229,
S0022215107000229 [pii]
31. Caye-Thomasen P, Borup R, Stangerup SE, Thomsen
J, Nielsen FC (2009) Deregulated genes in sporadic
vestibular schwannomas. Otol Neurotol 31(2):256–
266. doi:10.1097/MAO.0b013e3181be6478
32. Chandler CL, Ramsden RT (1993) Acoustic schwan-
noma. Br J Hosp Med 49(5):335–343
33. Charabi S, Mantoni M, Tos M, Thomsen J (1994)
Cystic vestibular schwannomas: neuroimaging and
growth rate. J Laryngol Otol 108(5):375–379
34. Charabi S, Thomsen J, Mantoni M, Charabi B,
Jorgensen B, Borgesen SE, Gyldensted C, Tos M
(1995) Acoustic neuroma (vestibular schwannoma):
growth and surgical and nonsurgical consequences of
the wait-and-see policy. Otolaryngol Head Neck Surg
113(1):5–14, S0194-5998(95)70138-9 [pii]
35. Charabi S, Tos M, Borgesen SE, Thomsen J (1994)
Cystic acoustic neuromas. Results of translabyrin-
thine surgery. Arch Otolaryngol Head Neck Surg
120(12):1333–1338
36. Charabi S, Tos M, Thomsen J, Charabi B, Mantoni M
(2000) Vestibular schwannoma growth – long-term
results. Acta Otolaryngol Suppl 543:7–10
37. Charabi S, Tos M, Thomsen J, Charabi B, Mantoni M
(2000) Vestibular schwannoma growth: the continu-
ing controversy. Laryngoscope 110(10 Pt 1):
1720–1725. doi:10.1097/00005537-200010000-
00030
38. Charabi S, Tos M, Thomsen J, Rygaard J, Fundova P,
Charabi B (2000) Cystic vestibular schwannoma –
clinical and experimental studies. Acta Otolaryngol
Suppl 543:11–13
39. Chen DQ, Quan J, Guha A, Tymianski M, Mikulis D,
Hodaie M (2011) Three-dimensional in vivo model-
ing of vestibular schwannomas and surrounding cra-
nial nerves with diffusion imaging tractography.
Neurosurgery 68(4):1077–1083. doi:10.1227/
NEU.0b013e31820c6cbe
40. Chishti AH, Kim AC, Marfatia SM, Lutchman M,
Hanspal M, Jindal H, Liu SC, Low PS, Rouleau GA,
Mohandas N, Chasis JA, Conboy JG, Gascard P,
Takakuwa Y, Huang SC, Benz EJ Jr, Bretscher A,
Fehon RG, Gusella JF, Ramesh V, Solomon F,
Marchesi VT, Tsukita S, Hoover KB et al (1998) The
306
FERM domain: a unique module involved in the link-
age of cytoplasmic proteins to the membrane. Trends
Biochem Sci 23(8):281–282, S0968000498012377
[pii]
41. Cho YD, Choi GH, Lee SP, Kim JK (2003) (1)H-MRS
metabolic patterns for distinguishing between menin-
giomas and other brain tumors. Magn Reson Imaging
21(6):663–672, S0730725X03000973 [pii]
42. Chung WY, Pan DH, Lee CC, Wu HM, Liu KD, Yen
YS, Guo WY, Shiau CY, Shih YH (2010) Large
vestibular schwannomas treated by Gamma Knife
surgery: long-term outcomes. J Neurosurg 113(Suppl):
112–121. doi:10.3171/2010.8.GKS10954
43. Ciric I, Zhao J, Rosenblatt S, Wiet R, O’Shaughnessy
B (2005) Suboccipital retrosigmoid approach for
removal of vestibular schwannomas: facial nerve
function and hearing preservation. Neurosurgery 56:
560–570
44. Clemis JD, Ballad WJ, Baggot PJ, Lyon ST
(1986) Relative frequency of inferior vestibular
schwannoma. Arch Otolaryngol Head Neck Surg
112(2):190–194
45. Colletti V, Fiorino F (2003) Middle fossa versus ret-
rosigmoid-transmeatal approach in vestibular schwan-
noma surgery: a prospective study. Otol Neurotol
24(6):927–934
46. Comey CH, Jannetta PJ, Sheptak PE, Joh HD,
Burkhart LE (1995) Staged removal of acoustic
tumors: techniques and lessons learned from a series
of 83 patients. Neurosurgery 37(5):915–920; discus-
sion 920–921
47. (2001) Consensus meeting on systems for reporting
results in acoustic neuroma. November 7–9, 2001.
Tokyo, Japan. Abstracts (2001). Keio J Med 50(Suppl
4):13–77
48. Couloigner V, Gervaz E, Kalamarides M, Ferrary E,
Rey A, Sterkers O, Henin D (2003) Clinical and histo-
logic parameters correlated with facial nerve function
after vestibular schwannoma surgery. Skull Base
13(1):13–19. doi:10.1055/s-2003-820553
49. Dandy W (1925) An operation for the total removal of
cerebellopontine (acoustic) tumors. Surg Gynecol
Obstet 41:129–148
50. Deguine O, Maillard A, Bonafe A, el Adouli H H,
Tremoulet M, Fraysse B (1998) Pre-operative and
per-operative factors conditioning long-term facial
nerve function in vestibular schwannoma surgery
through translabyrinthine approach. J Laryngol Otol
112(5):441–445
51. Delsanti C, Regis J (2004) Cystic vestibular schwan-
nomas. Neurochirurgie 50(2–3 Pt 2):401–406, MDOI-
NCHIR-06-2004-50-2-3-C2-0028-3770-101019-
ART26 [pii]
52. Di Maio S, Malebranche AD, Westerberg B,
Akagami R (2011) Hearing preservation after micro-
surgical resection of large vestibular schwannomas.
Neurosurgery 68(3):632–640. doi:10.1227/NEU.
0b013e31820777b1; discussion 640
53. Dispenza F, De Stefano A (2009) Imaging of vestibular
schwannoma with prevalent cystic component: cystic
5 Vestibular Schwannomas
vestibular schwannoma. Otol Neurotol 30(5):681–682.
doi:10.1097/MAO.0b013e3181859a3e
54. Doherty JK, Ongkeko W, Crawley B, Andalibi A,
Ryan AF (2008) ErbB and Nrg: potential molecular
targets for vestibular schwannoma pharmacotherapy.
Otol Neurotol 29(1):50–57. doi:10.1097/mao.0b013e
31815d4429, 00129492-200801000-00012 [pii]
55. Duong DH, O’Malley S, Sekhar LN, Wright DG
(2000) Postoperative hydrocephalus in cranial base
surgery. Skull Base Surg 10(4):197–200
56. Ebersold MJ, Harner SG, Beatty CW, Harper CM Jr,
Quast LM (1992) Current results of the retrosigmoid
approach to acoustic neurinoma. J Neurosurg
76(6):901–909. doi:10.3171/jns.1992.76.6.0901
57. El-Kashlan HK, Zeitoun H, Arts HA, Hoff JT,
Telian SA (2000) Recurrence of acoustic neuroma
after incomplete resection. Am J Otol 21(3):389–392
58. Eldridge R, Parry D (1992) Vestibular schwannoma
(acoustic neuroma). Consensus development confer-
ence. Neurosurgery 30(6):962–964
59. Elliott FA, McKissock KW (1954) Acoustic neuroma;
early diagnosis. Lancet 267(6850):1189–1191
60. Elsmore AJ, Mendoza ND (2002) The operative learn-
ing curve for vestibular schwannoma excision via the
retrosigmoid approach. Br J Neurosurg 16(5):448–455
61. Fahlbusch R, Neu M, Strauss C (1998) Preservation
of hearing in large acoustic neurinomas following
removal via suboccipito-lateral approach. Acta
Neurochir (Wien) 140(8):771–777; discussion 778
62. Falcioni A, Piccirillo E, Mancini F (2000) Cystic ves-
tibular schwannoma. Am J Otol 21(4):595–596
63. Falcioni M, Mulder JJ, Taibah A, De Donato G, Sanna
M (1999) No cerebrospinal fluid leaks in translabyrin-
thine vestibular schwannoma removal: reappraisal of
200 consecutive patients. Am J Otol 20(5):660–666
64. Fayad JN, Schwartz MS, Slattery WH, Brackmann DE
(2007) Prevention and treatment of cerebrospinal fluid
leak after translabyrinthine acoustic tumor removal.
Otol Neurotol 28(3):387–390. doi:10.1097/01.
mao.0000265188.22345.d4, 00129492-200704000-
00019 [pii]
65. Fernandez-Valle C, Tang Y, Ricard J, Rodenas-
Ruano A, Taylor A, Hackler E, Biggerstaff J, Iacovelli
J (2002) Paxillin binds schwannomin and regulates its
density-dependent localization and effect on cell mor-
phology. Nat Genet 31(4):354–362. doi:10.1038/
ng930, ng930 [pii]
66. Fitzgerald DC, Grundfast KM, Hecht DA, Mark AS
(1999) Intralabyrinthine schwannomas. Am J Otol
20(3):381–385
67. Flint D, Fagan P, Panarese A (2005) Conservative
management of sporadic unilateral acoustic neuro-
mas. J Laryngol Otol 119(6):424–428. doi:10.1258/
0022215054273089
68. Forton GE, Cremers CW, Offeciers EE (2004)
Acoustic neuroma ingrowth in the cochlear nerve:
does it influence the clinical presentation? Ann Otol
Rhinol Laryngol 113(7):582–586
69. Friedman RA, Brackmann DE, Hitselberger WE,
Schwartz MS, Iqbal Z, Berliner KI (2005) Surgical
References
salvage after failed irradiation for vestibular
schwannoma. Laryngoscope 115(10):1827–1832.
doi:10.1097/01.mlg.0000175063.76945.75,
00005537-200510000-00022 [pii]
70. Frohlich AM, Sutherland GR (1993) Epidemiology
and clinical features of vestibular schwannoma in
Manitoba, Canada. Can J Neurol Sci 20(2):126–130
71. Fuentes S, Arkha Y, Pech-Gourg G, Grisoli F, Dufour
H, Regis J (2008) Management of large vestibular
schwannomas by combined surgical resection and
gamma knife radiosurgery. Prog Neurol Surg 21:
79–82. doi:10.1159/000156709, 156709 [pii]
72. Fundova P, Charabi S, Tos M, Thomsen J (2000)
Cystic vestibular schwannoma: surgical outcome. J
Laryngol Otol 114(12):935–939
73. Gardner G, Robertson JH (1988) Hearing preserva-
tion in unilateral acoustic neuroma surgery. Ann Otol
Rhinol Laryngol 97(1):55–66
74. Gerganov V, Giordano M, Samiii A, Samii M (2012)
Surgical treatment of patients with vestibular schwan-
nomas after failed previous radiosurgery. J Neurosurg
116(4):713–720
75. Gerganov V, Nouri M, Stieglitz L, Giordano M,
Samii M, Samii A (2009) Radiological factors related
to pre-operative hearing levels in patients with ves-
tibular schwannomas. J Clin Neurosci 16(8):1009–
1012. doi:10.1016/j.jocn.2008.08.029, S0967-5868
(08)00486-4 [pii]
76. Gerganov VM, Giordano M, Samii M, Samii A (2011)
Diffusion tensor imaging-based fiber tracking for
prediction of the position of the facial nerve in rela-
tion to large vestibular schwannomas. J Neurosurg.
doi:10.3171/2011.7.JNS11495
77. Gerganov VM, Klinge PM, Nouri M, Stieglitz L,
Samii M, Samii A (2009) Prognostic clinical and
radiological parameters for immediate facial nerve
function following vestibular schwannoma surgery.
Acta Neurochir (Wien) 151(6):581–587. doi:10.1007/
s00701-009-0288-3; discussion 587
78. Gerganov VM, Pirayesh A, Nouri M, Hore N,
Luedemann WO, Oi S, Samii A, Samii M (2011)
Hydrocephalus associated with vestibular schwanno-
mas: management options and factors predicting the
outcome. J Neurosurg 114(5):1209–1215.
doi:10.3171/2010.10.JNS1029
79. Gjuric M, Rudic M (2008) What is the best tumor size
to achieve optimal functional results in vestibular
schwannoma surgery? Skull Base 18(5):317–325.
doi:10.1055/s-0028-1086056
80. Gjuric M, Wigand ME, Wolf SR (2001) Enlarged
middle fossa vestibular schwannoma surgery: experi-
ence with 735 cases. Otol Neurotol 22(2):223–230;
discussion 230–231
81. Glasscock ME 3rd, Hays JW, Minor LB, Haynes DS,
Carrasco VN (1993) Preservation of hearing in sur-
gery for acoustic neuromas. J Neurosurg 78(6):
864–870. doi:10.3171/jns.1993.78.6.0864
82. Godefroy WP, Kaptein AA, Vogel JJ, van der Mey AG
(2009) Conservative treatment of vestibular schwan-
noma: a follow-up study on clinical and quality-
307
of-life outcome. Otol Neurotol. doi:10.1097/
MAO.0b013e3181b4e3c9
83. Gormley WB, Sekhar LN, Wright DC, Kamerer D,
Schessel D (1997) Acoustic neuromas: results of cur-
rent surgical management. Neurosurgery 41(1):50–
58; discussion 58–60
84. Grey PL, Moffat DA, Palmer CR, Hardy DG, Baguley
DM (1996) Factors which influence the facial nerve
outcome in vestibular schwannoma surgery. Clin
Otolaryngol Allied Sci 21(5):409–413
85. Guermazi A, Lafitte F, Miaux Y, Adem C, Bonneville
JF, Chiras J (2005) The dural tail sign – beyond men-
ingioma. Clin Radiol 60(2):171–188. doi:10.1016/j.
crad.2004.01.019, S0009-9260(04)00074-1 [pii]
86. Haberkamp TJ, Meyer GA, Fox M (1998) Surgical
exposure of the fundus of the internal auditory canal:
anatomic limits of the middle fossa versus the ret-
rosigmoid transcanal approach. Laryngoscope 108(8
Pt 1):1190–1194
87. Hajioff D, Raut VV, Walsh RM, Bath AP, Bance ML,
Guha A, Tator CH, Rutka JA (2008) Conservative
management of vestibular schwannomas: third review
of a 10-year prospective study. Clin Otolaryngol
33(3):255–259. doi:10.1111/j.1749-4486.2008.
01705.x, COA1705 [pii]
88. Hakyemez B, Erdogan C, Bolca N, Yildirim N,
Gokalp G, Parlak M (2006) Evaluation of different
cerebral mass lesions by perfusion-weighted MR
imaging. J Magn Reson Imaging 24(4):817–824.
doi:10.1002/jmri.20707
89. Hanemann CO, Evans DG (2006) News on the genet-
ics, epidemiology, medical care and translational
research of Schwannomas. J Neurol 253(12):
1533–1541. doi:10.1007/s00415-006-0347-0
90. Hardy M, Crowe S (1936) Early asymptomatic acous-
tic tumor. Arch Surg 32:292–301
91. Harner SG, Daube JR, Ebersold MJ, Beatty CW
(1987) Improved preservation of facial nerve func-
tion with use of electrical monitoring during removal
of acoustic neuromas. Mayo Clin Proc 62(2):
92–102
92. Hasegawa T, Fujitani S, Katsumata S, Kida Y,
Yoshimoto M, Koike J (2005) Stereotactic radiosur-
gery for vestibular schwannomas: analysis of 317
patients followed more than 5 years. Neurosurgery
57(2):257–265, 00006123-200508000-00009 [pii];
discussion 257–265
93. Hastan D, Vandenbroucke JP, van der Mey AG (2009)
A meta-analysis of surgical treatment for vestibular
schwannoma: is hospital volume related to preserva-
tion of facial function? Otol Neurotol. doi:10.1097/
MAO.0b013e3181b0d04a
94. House JW, Bassim MK, Schwartz M (2008)
False-positive magnetic resonance imaging in
the diagnosis of vestibular schwannoma. Otol
Neurotol 29(8):1176–1178. doi:10.1097/MAO.
0b013e318187e199
95. House JW, Brackmann DE (1985) Facial nerve grad-
ing system. Otolaryngol Head Neck Surg 93(2):
146–147
308
96. Inamasu J, Shiobara R, Kagami H, Sato S, Kawase
T, Kanzaki J (2000) Medial (intra-cisternal) acoustic
neuromas. Acta Otolaryngol 120(5):623–626
97. Irving RM, Viani L, Hardy DG, Baguley DM,
Moffat DA (1995) Nervus intermedius function after
vestibular schwannoma removal: clinical features
and pathophysiological mechanisms. Laryngoscope
105(8 Pt 1):809–813. doi:10.1288/00005537-
199508000-00007
98. Iwai Y, Yamanaka K, Ishiguro T (2003) Surgery
combined with radiosurgery of large acoustic neuro-
mas. Surg Neurol 59(4):283–289,
S0090301903000259 [pii]; discussion 289–291
99. Jackler R (1994) Acoustic neuroma (vestibular
schwannoma). In: Jackler RK, Brackmann DE (eds)
Neurotology. Mosby-Year Book, St. Louis, pp
729–785
100. Jackson CG (1993) Basic surgical principles of
neurotologic skull base surgery. Laryngoscope
103(11 Pt 2 Suppl 60):29–44
101. Jacoby LB, Jones D, Davis K, Kronn D, Short MP,
Gusella J, MacCollin M (1997) Molecular analysis
of the NF2 tumor-suppressor gene in schwannoma-
tosis. Am J Hum Genet 61(6):1293–1302.
doi:10.1086/301633, S0002-9297(07)60230-4 [pii]
102. Jacoby LB, MacCollin M, Barone R, Ramesh V,
Gusella JF (1996) Frequency and distribution of
NF2 mutations in schwannomas. Genes Chromo-
somes Cancer 17(1):45–55, 10.1002/(SICI)1098-
2264(199609)17:1<45::AID-GCC7>3.0.CO;2-2
[pii]. 10.1002/(SICI)1098-2264(199609)17:1<45::
AID-GCC7>3.0.CO;2-2
103. Jacoby LB, MacCollin M, Louis DN, Mohney T,
Rubio MP, Pulaski K, Trofatter JA, Kley N, Seizinger
B, Ramesh V et al (1994) Exon scanning for muta-
tion of the NF2 gene in schwannomas. Hum Mol
Genet 3(3):413–419
104. Jain VK, Mehrotra N, Sahu RN, Behari S, Banerji D,
Chhabra DK (2005) Surgery of vestibular schwan-
nomas: an institutional experience. Neurol India
53(1):41–45; discussion 45
105. James MF, Lelke JM, Maccollin M, Plotkin SR,
Stemmer-Rachamimov AO, Ramesh V, Gusella JF
(2008) Modeling NF2 with human arachnoidal
and meningioma cell culture systems: NF2 silencing
reflects the benign character of tumor growth.
Neurobiol Dis 29(2):278–292. doi:10.1016/j.
nbd.2007.09.002, S0969-9961(07)00211-2 [pii]
106. Jung S, Kang SS, Kim TS, Kim HJ, Jeong SK, Kim SC,
Lee JK, Kim JH, Kim SH, Lee JH (2000) Current sur-
gical results of retrosigmoid approach in extralarge
vestibular schwannomas. Surg Neurol 53(4):370–377,
S0090301900001968 [pii]; discussion 377–378
107. Jung S, Kim SH, Kim HW, Kim TS, Kim JH,
Kang SS, Lee JH (1998) Prediction of facial nerve
displacement in extra large vestibular schwannoma.
Acta Neurochir (Wien) 140(11):1143–1145
108. Kameda K, Shono T, Hashiguchi K, Yoshida F,
Sasaki T (2010) Effect of tumor removal on tinni-
tus in patients with vestibular schwannoma. J
5 Vestibular Schwannomas
Neurosurg 112(1):152–157. doi:10.3171/2009.3.
JNS081053
109. Kanzaki J, Ogawa K, Tsuchihashi N, Inoue Y,
Yamamoto M, Ikeda S (1991) Postoperative compli-
cations in acoustic neuroma surgery by the extended
middle cranial fossa approach. Acta Otolaryngol
Suppl 487:75–79
110. Kanzaki J, Tos M, Sanna M, Moffat DA, Monsell EM,
Berliner KI (2003) New and modified reporting sys-
tems from the consensus meeting on systems for
reporting results in vestibular schwannoma. Otol
Neurotol 24(4):642–648; discussion 648–649
111. Kartush JM, Lundy LB (1992) Facial nerve outcome
in acoustic neuroma surgery. Otolaryngol Clin North
Am 25(3):623–647
112. Keiper GL Jr, Sherman JD, Tomsick TA, Tew JM Jr
(1999) Dural sinus thrombosis and pseudotumor
cerebri: unexpected complications of suboccipital
craniotomy and translabyrinthine craniectomy. J
Neurosurg 91(2):192–197. doi:10.3171/jns.1999.91.
2.0192
113. Kemink JL, Langman AW, Niparko JK, Graham MD
(1991) Operative management of acoustic neuro-
mas: the priority of neurologic function over com-
plete resection. Otolaryngol Head Neck Surg
104(1):96–99
114. Kerr RG, Hearst MJ, Samy RN, van Loveren HR,
Tew JM Jr, Pensak ML, Theodosopoulos PV (2009)
Delayed extrusion of hydroxyapatite cement after
transpetrosal reconstruction. Neurosurgery
64(3):527–531. doi:10.1227/01.NEU.0000338070.
85190.3B, 00006123-200903000-00015 [pii]; dis-
cussion 531–532
115. Khrais T, Romano G, Sanna M (2008) Nerve origin
of vestibular schwannoma: a prospective study. J
Laryngol Otol 122(2):128–131. doi:10.1017/
S0022215107001028, S0022215107001028 [pii]
116. Kino T, Takeshima H, Nakao M, Nishi T,
Yamamoto K, Kimura T, Saito Y, Kochi M, Kuratsu
J, Saya H, Ushio Y (2001) Identification of the cis-
acting region in the NF2 gene promoter as a potential
target for mutation and methylation-dependent
silencing in schwannoma. Genes Cells 6(5):
441–454, gtc432 [pii]
117. Kondziolka D, Lunsford LD, Flickinger JC (2000)
Gamma knife radiosurgery for vestibular schwanno-
mas. Neurosurg Clin N Am 11(4):651–658
118. Kondziolka D, Lunsford LD, McLaughlin MR,
Flickinger JC (1998) Long-term outcomes after
radiosurgery for acoustic neuromas. N Engl J Med
339(20):1426–1433. doi:10.1056/NEJM199811123
392003
119. Koos WT, Day JD, Matula C, Levy DI (1998)
Neurotopographic considerations in the microsurgi-
cal treatment of small acoustic neurinomas. J
Neurosurg 88(3):506–512. doi:10.3171/jns.1998.
88.3.0506
120. Koos WT, Lang J (1993) Tumors of the cerebello-
pontine angle. In: Color atlas of microneurosurgery.
Thieme Medical Publishers, New York, pp 510–608
References
121. Kuo TC, Jackler RK, Wong K, Blevins NH, Pitts LH
(1997) Are acoustic neuromas encapsulated tumors?
Otolaryngol Head Neck Surg 117(6):606–609,
S0194-5998(97)70040-8 [pii]
122. Kveton JF, Tarlov EC, Drumheller G, Katcher P,
Abbott C (1989) Cochlear nerve conduction block:
an explanation for spontaneous hearing return after
acoustic tumor surgery. Otolaryngol Head Neck
Surg 100(6):594–601
123. Lallemand D, Manent J, Couvelard A, Watilliaux A,
Siena M, Chareyre F, Lampin A, Niwa-Kawakita M,
Kalamarides M, Giovannini M (2009) Merlin
regulates transmembrane receptor accumulation
and signaling at the plasma membrane in primary
mouse Schwann cells and in human schwannomas.
Oncogene 28(6):854–865. doi:10.1038/onc.2008.
427, onc2008427 [pii]
124. Lalwani AK, Butt FY, Jackler RK, Pitts LH,
Yingling CD (1994) Facial nerve outcome after
acoustic neuroma surgery: a study from the era of
cranial nerve monitoring. Otolaryngol Head Neck
Surg 111(5):561–570, S0194599894000938 [pii]
125. Lanman TH, Brackmann DE, Hitselberger WE,
Subin B (1999) Report of 190 consecutive cases of
large acoustic tumors (vestibular schwannoma)
removed via the translabyrinthine approach. J
Neurosurg 90(4):617–623
126. Lanser MJ, Sussman SA, Frazer K (1992) Epide-
miology, pathogenesis, and genetics of acoustic
tumors. Otolaryngol Clin North Am 25(3):499–520
127. Lapsiwala SB, Pyle GM, Kaemmerle AW, Sasse FJ,
Badie B (2002) Correlation between auditory func-
tion and internal auditory canal pressure in patients
with vestibular schwannomas. J Neurosurg
96(5):872–876. doi:10.3171/jns.2002.96.5.0872
128. Lee CC, Yen YS, Pan DH, Chung WY, Wu HM, Guo
WY, Chen MT, Liu KD, Shih YH (2010) Delayed
microsurgery for vestibular schwannoma after
gamma knife radiosurgery. J Neurooncol 98(2):203–
212. doi:10.1007/s11060-010-0178-9
129. Leksell L (1971) A note on the treatment of acoustic
tumours. Acta Chir Scand 137(8):763–765
130. Lescanne E, Francois P, Bakhos D, Velut S, Robier
A, Pollak A (2008) Vestibular schwannoma: dissec-
tion of the tumor and arachnoidal duplication. Otol
Neurotol 29(7):989–994. doi:10.1097/MAO.
0b013e3181845812
131. Linskey ME (2000) Stereotactic radiosurgery versus
stereotactic radiotherapy for patients with vestibular
schwannoma: a Leksell Gamma Knife Society 2000
debate. J Neurosurg 93(Suppl 3):90–95. doi:10.3171/
jns.2000.93.supplement3.0090
132. Lobato-Polo J, Kondziolka D, Zorro O, Kano H,
Flickinger JC, Lunsford LD (2009) Gamma knife
radiosurgery in younger patients with vestibular
schwannomas. Neurosurgery 65(2):294–300.
doi: 10.1227/01.NEU.0000345944.14065.35 ,
0006123-200908000-00010 [pii]; discussion 294–300
133. Lownie SP, Drake CG (1991) Radical intracapsular
removal of acoustic neurinomas. Long-term follow-
309
up review of 11 patients. J Neurosurg 74(3):422–425.
doi:10.3171/jns.1991.74.3.0422
134. Ludemann WO, Stieglitz LH, Gerganov V, Samii A,
Samii M (2008) Fat implant is superior to muscle
implant in vestibular schwannoma surgery for
the prevention of cerebrospinal fluid fistulae.
Neurosurgery 63(1 Suppl 1):ONS38–ONS42.
doi: 10.1227/01.neu.0000335009.53122.a3 ,
00006123-200807001-00006 [pii]; discussion 42–43
135. Luetje C (2000) Spontaneous involution of acoustic
tumors. Am J Otol 21:393–398
136. Luetje CM, Whittaker CK, Calloway LA, Veraga G
(1983) Histological acoustic tumor involvement of
the VIIth nerve and multicentric involvement of the
VIIIth. Laryngoscope 93:1133–1139
137. Lutchman M, Rouleau GA (1995) The
neurofibromatosis type 2 gene product, schwan-
nomin, suppresses growth of NIH 3T3 cells. Cancer
Res 55(11):2270–2274
138. Lynn SG, Driscoll CL, Harner SG, Beatty CW,
Atkinson EJ (1999) Assessment of dysequilibrium after
acoustic neuroma removal. Am J Otol 20(4):484–494
139. Magliulo G, Cordeschi S, Sepe C, de Vincentiis M
(1998) Taste and lacrimation after acoustic neuroma
surgery. Rev Laryngol Otol Rhinol (Bord) 119(3):
167–170
140. Magliulo G, D’Amico R, Di Cello P (2003) Delayed
facial palsy after vestibular schwannoma resection:
clinical data and prognosis. J Otolaryngol 32(6):
400–404
141. Malis LI (2001) Nuances in acoustic neuroma sur-
gery. Neurosurgery 49(2):337–341
142. Mamikoglu B, Wiet RJ, Esquivel CR (2002)
Translabyrinthine approach for the management of
large and giant vestibular schwannomas. Otol
Neurotol 23(2):224–227
143. Mass SC, Wiet RJ, Dinces E (1999) Complications
of the translabyrinthine approach for the removal of
acoustic neuromas. Arch Otolaryngol Head Neck
Surg 125(7):801–804
144. Matthies C, Samii M (1997) Direct brainstem
recording of auditory evoked potentials during ves-
tibular schwannoma resection: nuclear BAEP
recording. Technical note and preliminary results. J
Neurosurg 86(6):1057–1062. doi:10.3171/jns.
1997.86.6.1057
145. Matthies C, Samii M (1997) Management of 1000
vestibular schwannomas (acoustic neuromas): clini-
cal presentation. Neurosurgery 40(1):1–9; discus-
sion 9–10
146. Matthies C, Samii M (1997) Management of vestib-
ular schwannomas (acoustic neuromas): the value of
neurophysiology for evaluation and prediction of
auditory function in 420 cases. Neurosurgery
40(5):919–929; discussion 929–930
147. Matthies C, Samii M, Krebs S (1997) Management
of vestibular schwannomas (acoustic neuromas):
radiological features in 202 cases–their value for
diagnosis and their predictive importance. Neuro-
surgery 40(3):469–481; discussion 481–482
310
148. McFarland WH, Linthicum FH, Waldorf RA (1989)
Auditory and vestibular tests (in acoustic neuroma).
Semin Hear 10:313–326
149. McKenna MJ, Halpin C, Ojemann RG, Nadol JB Jr,
Montgomery WW, Levine RA, Carlisle E, Martuza
R (1992) Long-term hearing results in patients after
surgical removal of acoustic tumors with hearing
preservation. Am J Otol 13(2):134–136
150. Millen SJ, Meyer G (1993) Surgical management of
CSF otorhinorrhea following retrosigmoid removal
of cerebellopontine angle tumors. Am J Otol 14(6):
585–589
151. Moffat DA, Hardy DG, Grey PL, Baguley DM
(1996) The operative learning curve and its effect on
facial nerve outcome in vestibular schwannoma
surgery. Am J Otol 17(4):643–647
152. Mohr G, Sade B, Dufour JJ, Rappaport JM (2005)
Preservation of hearing in patients undergoing
microsurgery for vestibular schwannoma: degree of
meatal filling. J Neurosurg 102(1):1–5. doi:10.3171.
jns.2005.102.1.0001
153. Moon KS, Jung S, Seo SK, Jung TY, Kim IY,
Ryu HH, Jin YH, Jin SG, Jeong YI, Kim KK, Kang
SS (2007) Cystic vestibular schwannomas: a possi-
ble role of matrix metalloproteinase-2 in cyst devel-
opment and unfavorable surgical outcome. J
Neurosurg 106(5):866–871. doi:10.3171/jns.2007.
106.5.866
154. Moriyama T, Fukushima T, Asaoka K, Roche PH,
Barrs DM, McElveen JT Jr (2002) Hearing preserva-
tion in acoustic neuroma surgery: importance of
adhesion between the cochlear nerve and the tumor.
J Neurosurg 97(2):337–340. doi:10.3171/
jns.2002.97.2.0337
155. Muranen T, Gronholm M, Renkema GH, Carpen O
(2005) Cell cycle-dependent nucleocytoplasmic
shuttling of the neurofibromatosis 2 tumour suppres-
sor merlin. Oncogene 24(7):1150–1158. doi:10.1038/
sj.onc.1208283, 1208283 [pii]
156. Murphy ES, Suh JH (2011) Radiotherapy for
vestibular schwannomas: a critical review.
Int J Radiat Oncol Biol Phys 79(4):985–997.
doi:10.1016/j.ijrobp.2010.10.010, S0360-
3016(10)03428-0 [pii]
157. Nedzelski JM, Schessel DA, Pfleiderer A, Kassel
EE, Rowed DW (2008) Conservative management
of acoustic neuromas 1992. Neurosurg Clin N Am
19(2):207–216. doi:10.1016/j.nec.2008.02.013, v.
S1042-3680(08)00014-4 [pii]
158. Neff BA, Oberstien E, Lorenz M, Chaudhury AR,
Welling DB, Chang LS (2006) Cyclin D(1) and D(3)
expression in vestibular schwannomas. Laryngo-
scope 116(3):423–426. doi:10.1097/01.mlg.
0000195076.05466.6c, 00005537-200603000-
00017 [pii]
159. Neff BA, Ting J, Dickinson SL, Welling DB (2005)
Facial nerve monitoring parameters as a predictor of
postoperative facial nerve outcomes after vestibular
schwannoma resection. Otol Neurotol 26(4):
728–732, 00129492-200507000-00031 [pii]
5 Vestibular Schwannomas
160. Niemczyk K, Vaneecloo FM, Lecomte MH,
Lejeune JP, Lemaitre L, Skarzynski H, Vincent C,
Dubrulle F (2000) Correlation between Ki-67 index
and some clinical aspects of acoustic neuromas (ves-
tibular schwannomas). Otolaryngol Head Neck Surg
123(6):779–783. doi:10.1067/mhn.2000.111356,
S0194-5998(00)41879-6 [pii]
161. Oghalai JS, Buxbaum JL, Pitts LH, Jackler RK
(2003) The effect of age on acoustic neuroma sur-
gery outcomes. Otol Neurotol 24(3):473–477
162. Ohata K, Tsuyuguchi N, Morino M, Takami T,
Goto T, Hakuba A, Hara M (2002) A hypothesis of
epiarachnoidal growth of vestibular schwannoma at
the cerebello-pontine angle: surgical importance. J
Postgrad Med 48(4):253–258; discussion 258–259
163. Okamoto K, Furusawa T, Ishikawa K, Sasai K,
Tokiguchi S (2006) Focal T2 hyperintensity in the
dorsal brain stem in patients with vestibular schwan-
noma. AJNR Am J Neuroradiol 27(6):1307–1311,
27/6/1307 [pii]
164. Park CK, Kim DC, Park SH, Kim JE, Paek SH,
Kim DG, Jung HW (2006) Microhemorrhage, a pos-
sible mechanism for cyst formation in vestibular
schwannomas. J Neurosurg 105(4):576–580.
doi:10.3171/jns.2006.105.4.576
165. Parry DM, Eldridge R, Kaiser-Kupfer MI,
Bouzas EA, Pikus A, Patronas N (1994) Neuro-
fibromatosis 2 (NF2): clinical characteristics of 63
affected individuals and clinical evidence for
heterogeneity. Am J Med Genet 52(4):450–461.
doi:10.1002/ajmg.1320520411
166. Patel AK, Alexander TH, Andalibi A, Ryan AF,
Doherty JK (2008) Vestibular schwannoma
quantitative polymerase chain reaction expression
of estrogen and progesterone receptors. Laryngo
scope 118(8):1458–1463. doi:10.1097/MLG.
0b013e318177e20b, 00005537-200808000-00024
[pii]
167. Patni AH, Kartush JM (2005) Staged resection of
large acoustic neuromas. Otolaryngol Head Neck
Surg 132(1):11–19. doi:10.1016/j.otohns.2004.09.
094, S0194599804018777 [pii]
168. Pelton PD, Sherman LS, Rizvi TA, Marchionni MA,
Wood P, Friedman RA, Ratner N (1998) Ruffling
membrane, stress fiber, cell spreading and prolifera-
tion abnormalities in human Schwannoma cells.
Oncogene 17(17):2195–2209. doi:10.1038/sj.onc.
1202141
169. Pendl G, Ganz JC, Kitz K, Eustacchio S (1996)
Acoustic neurinomas with macrocysts treated with
Gamma Knife radiosurgery. Stereotact Funct
Neurosurg 66(Suppl 1):103–111
170. Piccirillo E, Wiet MR, Flanagan S, Dispenza F,
Giannuzzi A, Mancini F, Sanna M (2009) Cystic
vestibular schwannoma: classification, management,
and facial nerve outcomes. Otol Neurotol 30(6):826–
834. doi:10.1097/MAO.0b013e3181b04e18,
00129492-200909000-00024 [pii]
171. Pillai P, Sammet S, Ammirati M (2009) Image-
guided, endoscopic-assisted drilling and exposure of
References
the whole length of the internal auditory canal and
its fundus with preservation of the integrity of the
labyrinth using a retrosigmoid approach: a labora-
tory investigation. Neurosurgery 65(6 Suppl):53–59.
doi: 10.1227/01.NEU.0000343521.88537.16 ,
00006123-200912001-00004 [pii]; discussion 59
172. Pirouzmand F, Tator CH, Rutka J (2001) Management
of hydrocephalus associated with vestibular
schwannoma and other cerebellopontine angle
tumors. Neurosurgery 48(6):1246–1253; discussion
1253–1254
173. Pollock BE (2006) Management of vestibular
schwannomas that enlarge after stereotactic radio-
surgery: treatment recommendations based on a 15
year experience. Neurosurgery 58(2):241–248.
doi: 10.1227/01.NEU.0000194833.66593.8B ,
00006123-200602000-00005 [pii]; discussion
241–248
174. Post KD, Eisenberg MB, Catalano PJ (1995) Hearing
preservation in vestibular schwannoma surgery:
what factors influence outcome? J Neurosurg
83(2):191–196. doi:10.3171/jns.1995.83.2.0191
175. Prasad D, Steiner M, Steiner L (2000) Gamma sur-
gery for vestibular schwannoma. J Neurosurg
92(5):745–759. doi:10.3171/jns.2000.92.5.0745
176. Prasad S, Hirsch BE, Kamerer DB, Durrant J, Sekhar
LN (1993) Facial nerve function following cerebel-
lopontine angle surgery: prognostic value of intraop-
erative thresholds. Am J Otol 14(4):330–333
177. Preston-Martin S, Thomas DC, Wright WE,
Henderson BE (1989) Noise trauma in the aetiology
of acoustic neuromas in men in Los Angeles County,
1978–1985. Br J Cancer 59(5):783–786
178. Raftopoulos C, Abu Serieh B, Duprez T, Docquier
MA, Guerit JM (2005) Microsurgical results with
large vestibular schwannomas with preservation of
facial and cochlear nerve function as the primary
aim. Acta Neurochir (Wien) 147(7):697–706.
doi:10.1007/s00701-005-0544-0; discussion 706
179. Rangwala R, Banine F, Borg JP, Sherman LS (2005)
Erbin regulates mitogen-activated protein (MAP)
kinase activation and MAP kinase-dependent inter-
actions between Merlin and adherens junction pro-
tein complexes in Schwann cells. J Biol Chem
280(12):11790–11797. doi:10.1074/jbc.
M414154200, M414154200 [pii]
180. Raut VV, Walsh RM, Bath AP, Bance ML, Guha A,
Tator CH, Rutka JA (2004) Conservative manage-
ment of vestibular schwannomas – second review of
a prospective longitudinal study. Clin Otolaryngol
Allied Sci 29(5):505–514. doi:10.1111/j.
1365-2273.2004.00852.x, COA852 [pii]
181. Regis J, Roche PH, Delsanti C, Thomassin JM,
Ouaknine M, Gabert K, Pellet W (2007) Modern
management of vestibular schwannomas. Prog
Neurol Surg 20:129–141. doi:10.1159/0000100100,
100100 [pii]
182. Rhoton AL Jr (2000) The cerebellopontine angle and
posterior fossa cranial nerves by the retrosigmoid
approach. Neurosurgery 47(3 Suppl):S93–S129
311
183. Roche PH, Bouvier C, Chinot O, Figarella-Branger
D (2008) Genesis and biology of vestibular schwan-
nomas. Prog Neurol Surg 21:24–31. doi:10.1159/
000156556, 156556 [pii]
184. Rodgers GK, Luxford WM (1993) Factors affecting
the development of cerebrospinal fluid leak and
meningitis after translabyrinthine acoustic tumor
surgery. Laryngoscope 103(9):959–962. doi:10.1288/
00005537-199309000-00003
185. Rogg JM, Ahn SH, Tung GA, Reinert SE, Noren G
(2005) Prevalence of hydrocephalus in 157 patients
with vestibular schwannoma. Neuroradiology
47(5):344–351. doi:10.1007/s00234-005-1363-y
186. Roland JT Jr, Fishman AJ, Golfinos JG, Cohen N,
Alexiades G, Jackman AH (2004) Cranial nerve
preservation in surgery for large acoustic neuromas.
Skull Base 14(2):85–90. doi:10.1055/s-2004-828699;
discussion 90–91
187. Rosenberg S (2000) Natural history of vestibular
schwannomas. Laryngoscope 110:497–508
188. Rouleau GA, Merel P, Lutchman M, Sanson M,
Zucman J, Marineau C, Hoang-Xuan K, Demczuk S,
Desmaze C, Plougastel B et al (1993) Alteration in a
new gene encoding a putative membrane-organizing
protein causes neuro-fibromatosis type 2. Nature
363(6429):515–521. doi:10.1038/363515a0
189. Rowed DW (1999) Hearing preservation in vestibu-
lar schwannoma surgery. J Neurosurg 90(2):
374–376
190. Ruttledge MH, Rouleau GA (2005) Role of the
neurofibromatosis type 2 gene in the development of
tumors of the nervous system. Neurosurg Focus
19(5):E6, 190506 [pii]
191. Sade B, Mohr G, Dufour JJ (2006) Vascular
complications of vestibular schwannoma surgery: a
comparison of the suboccipital retrosigmoid and
translabyrinthine approaches. J Neurosurg 105(2):
200–204. doi:10.3171/jns.2006.105.2.200
192. Sakamoto GT, Borchers DJ 3rd, Xiao F, Yang HJ,
Chang SD, Adler JR Jr (2009) Cyberknife radiosur-
gery for trigeminal schwannomas. Neurosurgery
64(2 Suppl):A14–18. doi:10.1227/01.NEU.
0000341629.57676.DB, 00006123-200902001-
00004 [pii]
193. Samii A, Brinker T, Kaminsky J, Lanksch WR,
Samii M (2000) Navigation-guided opening of the
internal auditory canal via the retrosigmoid route for
acoustic neuroma surgery: cadaveric, radiological,
and preliminary clinical study. Neurosurgery
47(2):382–387; discussion 388
194. Samii M, Gerganov V, Samii A (2006) Improved
preservation of hearing and facial nerve function in
vestibular schwannoma surgery via the retrosigmoid
approach in a series of 200 patients. J Neurosurg
105(4):527–535. doi:10.3171/jns.2006.105.4.527
195. Samii M, Gerganov V, Samii A (2008) Microsurgery
management of vestibular schwannomas in
neurofibromatosis type 2: indications and results.
Prog Neurol Surg 21:169–175.
doi:10.1159/000156905, 156905 [pii]
312
196. Samii M, Gerganov VM, Samii A (2009) Functional
outcome after complete surgical removal of giant
vestibular schwannomas. J Neurosurg 112(4):
860–867. doi:10.3171/2009.7.JNS0989
197. Samii M, Matthies C (1997) Management of 1000
vestibular schwannomas (acoustic neuromas): hear-
ing function in 1000 tumor resections. Neurosurgery
40(2):248–260; discussion 260–262
198. Samii M, Matthies C (1997) Management of 1000
vestibular schwannomas (acoustic neuromas): surgi-
cal management and results with an emphasis on
complications and how to avoid them. Neurosurgery
40(1):11–21; discussion 21–13
199. Samii M, Matthies C (1997) Management of 1000
vestibular schwannomas (acoustic neuromas): the
facial nerve – preservation and restitution of func-
tion. Neurosurgery 40(4):684–694; discussion
694–685
200. Sampath P, Holliday MJ, Brem H, Niparko JK,
Long DM (1997) Facial nerve injury in acoustic neu-
roma (vestibular schwannoma) surgery: etiology and
prevention. J Neurosurg 87(1):60–66. doi:10.3171/
jns.1997.87.1.0060
201. Sampath P, Rhines LD, Holliday MJ, Brem H,
Long DM (1998) Late-onset facial nerve degenera-
tion after vestibular schwannoma surgery: incidence,
putative mechanisms, and prevention. Neurosurg
Focus 5(3):e6, 050306 [pii]
202. Sampath P, Rini D, Long DM (2000) Microanatomical
variations in the cerebellopontine angle associated
with vestibular schwannomas (acoustic neuromas): a
retrospective study of 1006 consecutive cases. J
Neurosurg 92(1):70–78. doi:10.3171/jns.2000.92.
1.0070
203. Sanna M, Khrais T, Russo A, Piccirillo E, Augurio A
(2004) Hearing preservation surgery in vestibular
schwannoma: the hidden truth. Ann Otol Rhinol
Laryngol 113(2):156–163
204. Sanna M, Taibah A, Russo A, Falcioni M, Agarwal M
(2004) Perioperative complications in acoustic
neuroma (vestibular schwannoma) surgery. Otol
Neurotol 25(3):379–386, 00129492-200405000-
00029 [pii]
205. Sasaki T, Shono T, Hashiguchi K, Yoshida F,
Suzuki SO (2009) Histological considerations of
the cleavage plane for preservation of facial
and cochlear nerve functions in vestibular schwan-
noma surgery. J Neurosurg 110(4):648–655.
doi:10.3171/2008.4.17514
206. Satar B, Yetiser S, Ozkaptan Y (2003) Impact of
tumor size on hearing outcome and facial function
with the middle fossa approach for acoustic neu-
roma: a meta-analytic study. Acta Otolaryngol
123(4):499–505
207. Saunders JE, Luxford WM, Devgan KK,
Fetterman BL (1995) Sudden hearing loss in acous-
tic neuroma patients. Otolaryngol Head Neck Surg
113(1):23–31, S0194-5998(95)70140-0 [pii]
208. Schuknecht H (1977) Pathology of vestibular
schwannoma (acoustic neuroma). In: Silverstein H,
5 Vestibular Schwannomas
Norrell H (eds) Neurological surgery of the ear.
Aesculapius, Birmingham, pp 193–197
209. Schulder M, Sreepada GS, Kwartler JA, Cho ES
(1999) Microsurgical removal of a vestibular
schwannoma after stereotactic radiosurgery: surgical
and pathologic findings. Am J Otol 20(3):364–367;
discussion 368
210. Seedat RY, Claassen AJ, Mol DA (2002) Incidence
and management of acoustic neuromas in South
Africa. Otol Neurotol 23(6):996–998
211. Sekhar LN, Gormley WB, Wright DC (1996) The
best treatment for vestibular schwannoma (acoustic
neuroma): microsurgery or radiosurgery? Am J Otol
17(4):676–682; discussion 683–689
212. Sekhar LN, Tzortzdis F (1999) Retrosigmoid
approach to the cerebellopontine angle. In: Sekhar
LN, de Oliveira E (eds) Cranial microsurgery.
Approaches and techniques. Thieme, New York, pp
352–377
213. Seol HJ, Kim CH, Park CK, Kim DG, Chung YS,
Jung HW (2006) Optimal extent of resection in ves-
tibular schwannoma surgery: relationship to recur-
rence and facial nerve preservation. Neurol Med
Chir (Tokyo) 46(4):176–180; discussion 180–181.
JST.JSTAGE/nmc/46.176 [pii]
214. Shao KN, Tatagiba M, Samii M (1993) Surgical
management of high jugular bulb in acoustic
neurinoma via retrosigmoid approach. Neurosurgery
32(1):32–36; discussion 36–37
215. Sheptak PE, Jannetta PJ (1979) The two-stage
excision of huge acoustic neurinomas. J Neurosurg
51(1):37–41. doi:10.3171/jns.1979.51.1.0037
216. Shuto T, Inomori S, Matsunaga S, Fujino H (2008)
Microsurgery for vestibular schwannoma after
gamma knife radiosurgery. Acta Neurochir (Wien)
150(3):229–234. doi:10.1007/s00701-007-1486-5;
discussion 234
217. Silverstein H, McDaniel A, Norrell H, Wazen J (1985)
Conservative management of acoustic neuroma in the
elderly patient. Laryngoscope 95(7 Pt 1):766–770
218. Silverstein H, McDaniel AB, Norrell H (1985)
Hearing preservation after acoustic neuroma surgery
using intraoperative direct eighth cranial nerve mon-
itoring. Am J Otol (Suppl):99–106
219. Skinner A (1923) The origin of acoustic nerve
tumors. Br J Surg 16:440–463
220. Slattery WH 3rd (2009) Microsurgery after radiosur-
gery or radiotherapy for vestibular schwannomas.
Otolaryngol Clin North Am 42(4):707–715.
doi:10.1016/j.otc.2009.04.007, S0030-6665(09)
00062-0 [pii]
221. Smouha EE, Yoo M, Mohr K, Davis RP (2005)
Conservative management of acoustic neuroma: a
meta-analysis and proposed treatment algorithm.
Laryngoscope 115(3):450–454. doi:10.1097/01.
mlg.0000175681.52517.cf, 00005537-200503000-
00011 [pii]
222. Somers T, Casselman J, de Ceulaer G, Govaerts P,
Offeciers E (2001) Prognostic value of magnetic
resonance imaging findings in hearing preservation
References
surgery for vestibular schwannoma. Otol Neurotol
22(1):87–94
223. Staecker H, Nadol JB Jr, Ojeman R, Ronner S,
McKenna MJ (2000) Hearing preservation in
acoustic neuroma surgery: middle fossa versus
retrosigmoid approach. Am J Otol 21(3):399–404
224. Stangerup SE, Caye-Thomasen P, Tos M, Thomsen J
(2006) The natural history of vestibular schwan-
noma. Otol Neurotol 27(4):547–552. doi:10.1097/01.
mao.0000217356.73463.e7, 00129492-200606000-
00018 [pii]
225. Stangerup SE, Tos M, Thomsen J, Caye-Thomasen
P (2010) True incidence of vestibular schwannoma?
Neurosurgery 67(5):1335–1340. doi:10.1227/
NEU.0b013e3181f22660; discussion 1340
226. Sterkers JM, Perre J, Viala P, Foncin JF (1987) The
origin of acoustic neuromas. Acta Otolaryngol
103(5–6):427–431
227. Stieglitz LH, Giordano M, Gerganov V, Raabe A,
Samii A, Samii M, Ludemann WO (2010) Petrous
bone pneumatization is a risk factor for cerebrospi-
nal fluid fistula following vestibular schwannoma
surgery. Neurosurgery 67(2 Suppl Operative):
509–515. doi:10.1227/NEU.0b013e3181f88884,
00006123-201012002-00027 [pii]
228. Stieglitz LH, Samii A, Kaminsky J, Gharabaghi A,
Samii M, Ludemann WO (2005) Nausea and
dizziness after vestibular schwannoma surgery: a
multivariate analysis of preoperative symptoms.
Neurosurgery 57(5):887–890; discussion 887–890.
00006123-200511000-00009 [pii]
229. Stokowski RP, Cox DR (2000) Functional analysis
of the neurofibromatosis type 2 protein by means of
disease-causing point mutations. Am J Hum Genet
66(3):873–891. doi:10.1086/302812, S0002-9297
(07)64015-4 [pii]
230. Strauss C, Bischoff B, Romstock J, Rachinger J,
Rampp S, Prell J (2008) Hearing preservation in
medial vestibular schwannomas. J Neurosurg
109(1):70–76. doi:10.3171/JNS/2008/109/7/0070
231. Stripf T, Braun K, Gouveris H, Stripf EA, Mann WJ,
Amedee RG (2007) Influence of different approaches
to the cerebellopontine angle on the function of the
intermediate nerve. J Neurosurg 107(5):927–931.
doi:10.3171/JNS-07/11/0927
232. Sughrue ME, Yang I, Aranda D, Kane AJ, Parsa AT
(2010) Hearing preservation rates after microsurgi-
cal resection of vestibular schwannoma. J Clin
Neurosci 17(9):1126–1129. doi:10.1016/j.jocn.2010.
01.018, S0967-5868(10)00157-8 [pii]
233. Sughrue ME, Yang I, Aranda D, Lobo K, Pitts LH,
Cheung SW, Parsa AT (2010) The natural history of
untreated sporadic vestibular schwannomas: a com-
prehensive review of hearing outcomes. J Neurosurg
112(1):163–167. doi:10.3171/2009.4.JNS08895
234. Sughrue ME, Yang I, Aranda D, Rutkowski MJ,
Fang S, Cheung SW, Parsa AT (2011) Beyond audio-
facial morbidity after vestibular schwannoma sur-
gery. J Neurosurg 114(2):367–374. doi:10.3171/
2009.10.JNS091203
313
235. Sughrue ME, Yang I, Rutkowski MJ, Aranda D,
Parsa AT (2010) Preservation of facial nerve func-
tion after resection of vestibular schwannoma. Br J
Neurosurg 24(6):666–671. doi:10.3109/02688697.2
010.520761
236. Sughrue ME, Yeung AH, Rutkowski MJ, Cheung SW,
Parsa AT (2011) Molecular biology of familial and
sporadic vestibular schwannomas: implications for
novel therapeutics. J Neurosurg 114(2):359–366.
doi:10.3171/2009.10.JNS091135
237. Sunderland S (1945) The arterial relations of the
internal auditory meatus. Brain 68:23
238. Tallan EM, Harner SG, Beatty CW, Scheithauer BW,
Parisi JE (1993) Does the distribution of Schwann
cells correlate with the observed occurrence of
acoustic neuromas? Am J Otol 14(2):131–134
239. Tanaka Y, Kobayashi S, Hongo K, Tada T, Sato A,
Takasuna H (2003) Clinical and neuroimaging char-
acteristics of hydrocephalus associated with vestibu-
lar schwannoma. J Neurosurg 98(6):1188–1193.
doi:10.3171/jns.2003.98.6.1188
240. Taoka T, Hirabayashi H, Nakagawa H, Sakamoto M,
Myochin K, Hirohashi S, Iwasaki S, Sakaki T,
Kichikawa K (2006) Displacement of the facial
nerve course by vestibular schwannoma: preopera-
tive visualization using diffusion tensor tractogra-
phy. J Magn Reson Imaging 24(5):1005–1010.
doi:10.1002/jmri.20725
241. Tarlov E (1980) Total one-stage suboccipital micro-
surgical removal of acoustic neuromas of all sizes:
with emphasis on arachnoid planes and on saving the
facial nerve. Surg Clin North Am 60(3):565–591
242. Tatagiba M, Samii M, Matthies C, el Azm M M,
Schonmayr R (1992) The significance for postopera-
tive hearing of preserving the labyrinth in acoustic
neurinoma surgery. J Neurosurg 77(5):677–684.
doi:10.3171/jns.1992.77.5.0677
243. Thamburaj K, Radhakrishnan VV, Thomas B, Nair S,
Menon G (2008) Intratumoral microhemorrhages on
T2*-weighted gradient-echo imaging helps differen-
tiate vestibular schwannoma from meningioma.
AJNR Am J Neuroradiol 29(3):552–557.
doi:10.3174/ajnr.A0887, ajnr.A0887 [pii]
244. Theodosopoulos PV, Pensak ML (2012) Contem-
porary management of acoustic neuromas. Laryngo-
scope 121(6):1133–1137. doi:10.1002/lary.21799
245. Tonn JC, Schlake HP, Goldbrunner R, Milewski C,
Helms J, Roosen K (2000) Acoustic neuroma sur-
gery as an interdisciplinary approach: a neurosurgi-
cal series of 508 patients. J Neurol Neurosurg
Psychiatry 69(2):161–166
246. Tos M (1998) Increasing incidence of vestibular
schwannoma: is it real and realistic? Ugeskr Laeger
160(45):6538
247. Tos M, Charabi S, Thomsen J (1999) Incidence of
vestibular schwannomas. Laryngoscope 109(5):
736–740
248. Tos M, Drozdziewicz D, Thomsen J (1992) Medial
acoustic neuromas. A new clinical entity. Arch
Otolaryngol Head Neck Surg 118(2):127–133
314
249. Tos M, Thomsen J (1991) Management of acoustic neu-
romas. A review. Acta Otolaryngol 111(4):616–632
250. Tran Ba Huy P, Kania R, Legac MS (2008) Vestibular
schwannoma (acoustic neurinoma). Natural history
and quality of life. Bull Acad Natl Med 192(9):1725–
1737; discussion 1738–1740
251. Trofatter JA, MacCollin MM, Rutter JL, Murrell JR,
Duyao MP, Parry DM, Eldridge R, Kley N, Menon
AG, Pulaski K et al (1993) A novel moesin-, ezrin-,
radixin-like gene is a candidate for the neurofi-
bromatosis 2 tumor suppressor. Cell 72(5):791–800,
0092-8674(93)90406-G [pii]
252. Umezu H, Aiba T, Tsuchida S, Seki Y (1996) Early
and late postoperative hearing preservation in
patients with acoustic neuromas. Neurosurgery
39(2):267–271; discussion 271–272
253. Utermark T, Alekov A, Lerche H, Abramowski V,
Giovannini M, Hanemann CO (2003) Quinidine
impairs proliferation of neurofibromatosis type
2-deficient human malignant mesothelioma cells.
Cancer 97(8):1955–1962. doi:10.1002/cncr.11275
254. Valvassori GE, Shannon M (1991) Natural history of
acoustic neuromas. Skull Base Surg 1(3):165–167
255. Wada K, Nawashiro H, Shimizu A, Shima K (2003)
MRI analysis of hydrocephalus associated with acous-
tic neurinoma. Acta Neurochir Suppl 86:549–551
256. Walsh RM, Bath AP, Bance ML, Keller A, Rutka JA
(2000) Consequences to hearing during the conser-
vative management of vestibular schwannomas.
Laryngoscope 110(2 Pt 1):250–255. doi:10.1097/
00005537-200002010-00012
257. Walsh RM, Bath AP, Bance ML, Keller A, Tator CH,
Rutka JA (2000) The natural history of untreated
vestibular schwannomas. Is there a role for conser-
vative management? Rev Laryngol Otol Rhinol
(Bord) 121(1):21–26
258. Wanibuchi M, Fukushima T, McElveen JT Jr,
Friedman AH (2009) Hearing preservation in sur-
gery for large vestibular schwannomas. J Neurosurg
111(4):845–854. doi:10.3171/2008.12.JNS08620
259. Watanabe K, Saito N, Taniguchi M, Kirino T,
Sasaki T (2003) Analysis of taste disturbance before
and after surgery in patients with vestibular schwan-
noma. J Neurosurg 99(6):999–1003. doi:10.3171/
jns.2003.99.6.0999
260. Welling DB, Guida M, Goll F, Pearl DK,
Glasscock ME, Pappas DG, Linthicum FH,
Rogers D, Prior TW (1996) Mutational spectrum in
the neurofibromatosis type 2 gene in sporadic and
familial schwannomas. Hum Genet 98(2):189–193
5 Vestibular Schwannomas
261. Welling DB, Lasak JM, Akhmametyeva E,
Ghaheri B, Chang LS (2002) cDNA microarray
analysis of vestibular schwannomas. Otol Neurotol
23(5):736–748
262. Welling DB, Slater PW, Thomas RD, McGregor JM,
Goodman JE (1999) The learning curve in vestibular
schwannoma surgery. Am J Otol 20(5):644–648
263. Whitehouse K, Foroughi M, Shone G, Hatfield R (2009)
Vestibular schwannomas – when should conservative
management be reconsidered? Br J Neurosurg
24(2):185–190. doi:10.3109/02688690903272634
264. Wiet RJ, Mamikoglu B, Odom L, Hoistad DL (2001)
Long-term results of the first 500 cases of acoustic
neuroma surgery. Otolaryngol Head Neck Surg
124(6):645–651, S0194599801514382 [pii]
265. Yamakami I, Uchino Y, Kobayashi E, Saeki N,
Yamaura A (2002) Prognostic significance of
changes in the internal acoustic meatus caused by
vestibular schwannoma. Neurol Med Chir (Tokyo)
42(11):465–470; discussion 470–471
266. Yamakami I, Uchino Y, Kobayashi E, Yamaura A,
Oka N (2004) Removal of large acoustic neurinomas
(vestibular schwannomas) by the retrosigmoid
approach with no mortality and minimal morbidity. J
Neurol Neurosurg Psychiatry 75(3):453–458
267. Yang I, Sughrue ME, Han SJ, Aranda D, Pitts LH,
Cheung SW, Parsa AT (2009) A comprehensive anal-
ysis of hearing preservation after radiosurgery for
vestibular schwannoma. J Neurosurg. doi:10.3171/
2009.8.JNS0985
268. Yasargil M (1996) Acoustic neuromas, vol IVb,
Microneurosurgery. Thieme, New York
269. Yates PD, Jackler RK, Satar B, Pitts LH, Oghalai JS
(2003) Is it worthwhile to attempt hearing preserva-
tion in larger acoustic neuromas? Otol Neurotol
24:460–464
270. Yeung AH, Sughrue ME, Kane AJ, Tihan T, Cheung
SW, Parsa AT (2009) Radiobiology of vestibular
schwannomas: mechanisms of radioresistance and
potential targets for therapeutic sensitization.
Neurosurg Focus 27(6):E2. doi:10.3171/2009.9.
FOCUS09185
271. Yoshimoto Y (2005) Systematic review of the natu-
ral history of vestibular schwannoma. J Neurosurg
103(1):59–63. doi:10.3171/jns.2005.103.1.0059
272. Zaouche S, Ionescu E, Dubreuil C, Ferber-Viart C
(2005) Pre- and intraoperative predictive factors
of facial palsy in vestibular schwannoma surgery.
Acta Otolaryngol 125(4):363–369. doi:10.1080/
00016480410025216, K682N0KGGLRKMN0D [pii]
 Neurofibromatosis Type 2
and Other Bilateral
Cerebellopontine Angle Tumors
Neurofibromatosis Type 2
NF-2 is a dominantly inherited tumor predispo-
sition syndrome characterized by a tendency for
developing bilateral VSs and other CNS tumors
(meningiomas, ependymomas, astrocytomas, or
other schwannomas). Typically the syndrome
manifests itself during young adulthood—in the
second or third decade of life. The severity of the
disease ranges from mild to aggressive in regard to
age of onset, tumor types, and biological aggres-
siveness [50]. Traditionally NF-2 is subdivided
into two groups: the Wishart and the Gardner type
[13]. The Wishart type is characterized by more
severe clinical presentation, earlier onset (in the
late teens or early 20s), and occurrence of multiple
tumors. In addition to bilateral VSs, the patients
frequently have multiple associated brain/spinal
tumors. Patients with Gardner-type NF-2 have later
onset, slower development, and less severe pre-
sentation. The incidence of associated intracranial
tumors is lower. The birth incidence of NF-2 was
estimated to vary between 1/25,000 and 1/87,410,
while the population-based disease prevalence is
1/210,000 [13, 14]. Disease prevalence has now
risen to around 1/60,000 due to earlier diagnosis
and longer survival of the patients [12].
Recently a third subgroup—the mosaic
NF-2—was outlined, which is caused by a
somatic mutation occurring during embryogen-
esis [16]. Only a portion of the patients’ cells
carry the mutation hence, and the germline DNA
is not affected [30, 61]. Approximately 25 % of
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_6, © Springer-Verlag Berlin Heidelberg 2013
6
NF-2 cases in patients, whose parents did not display
the disease, are mosaic [30]. If the mutation
occurs early in embryogenesis, bilateral VSs may
develop rendering the disease clinically indistin-
guishable from nonmosaic forms. Mutation at
a later stage of embryogenesis causes segmen-
tal NF-2, characterized by unilateral VS and an
additional ipsilateral intracranial tumor, usually
meningioma. This NF-2 subtype has understandably
a very low inheritance risk [47].
Criteria for Diagnosis
Several different sets of diagnostic criteria for NF-2
are used currently [3, 13, 24, 49], but the modified
NIH criteria and the Revised Clinical criteria are
regarded as most sensitive (Tables 6.1 and 6.2).
Genetics
NF-2, as well as sporadic VSs, is caused by
inactivation of the NF2 tumor suppressor gene
on chromosome 22q12 (see Chap. 5). NF-2 is
caused by a single germline mutation of the
chromosome band 22q12. The normal allele is
lost due to a somatic mutation in the cells giv-
ing rise to the tumor. It has been estimated that
NF-2 has the highest spontaneous mutation rate
of any human genetic disorder. If a person
inherits the abnormal gene, there is a 95 %
chance that he will develop bilateral VSs.
315
316 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Table 6.1 Modified NIH Main criteria Bilateral VS or

criteria for the diagnosis of Family history of NF-2 plus
NF2 [13]
1. Unilateral VS or
2. Any two of: meningioma, glioma, posterior subcapsular
lenticular opacities
Additional criteria Unilateral VS plus any two of meningioma, glioma,
neurofibroma, schwannoma, and posterior subscapular opacities
Or
Multiple meningioma (2 or more) plus
Unilateral VS or any two of glioma, neurofibroma, schwannoma,
and cataract

Table 6.2 NNFF criteria or revised clinical criteria for NF-2

A. Confirmed or definite
NF-2
B. Presumptive or probable
NF-2
From Gutmann et al. [24]
Characteristics
1. Bilateral vestibular schwannomas
2. First-degree family relative with NF2 and unilateral VS at less than 30 years of
age or any two of meningioma, schwannoma, glioma, juvenile posterior
subcapsular lenticular opacities
1. Unilateral vestibular schwannoma at less than 30 years of age and at least one of
meningioma, schwannoma, glioma, juvenile lens opacity (posterior subcapsular
cataract or cortical cataract)
2. Multiple meningiomas (two or more) and unilateral vestibular schwannoma at
less than 30 years of age or at least one of schwannoma, glioma, juvenile lens
opacity (posterior subcapsular cataract or cortical cataract)

Approximately 50 % of the patients represent Table 6.3 Clinical manifestation in NF-2 patients in the
new mutations that were not inherited. It is sup- largest published series
posed that the functional inactivation of the Clinical finding Rate (%)
NF2 gene is caused by two germline mutation Bilateral VS 71–95
(“double-hit” hypothesis). The penetrance of Spinal tumor 26–90
NF-2 is 100 %, but it has wide phenotypic vari- Intracranial meningioma 18–58
ability [16]. Practically all individuals who Peripheral nerve tumor 34
have pathogenic mutation develop the disease Skin tumors 32–68
in an average lifetime [13]. Unilateral VS 26
Café-au-lait macules 42–47
Intracranial astrocytoma 1.6–4.1
Ependymoma 2.5–6
Clinical Presentation
Nonvestibular cranial nerve 23
schwannoma
The mean age at onset of the disease is Presenile cataract 14–81
17–22 years [15, 44, 46, 54]. Clinical presenta- Cranial calcification 6
tion is highly variable and is related to tumor References: [15, 28, 44, 54]
types, size and extension, and more rarely to
the cutaneous or non-nervous-system-related Bilateral VSs are the hallmark of the disease
abnormalities. The clinical manifestation in and occur in about 95 % of adult patients with NF-2
NF-2 patients in the largest published series is (Fig. 6.1). Children with NF-2, however, often
presented on Table 6.3. present initially with nonvestibular tumors [45].
Neurofibromatosis Type 2
Fig. 6.1 MRI studies of patient with NF-2 and bilateral VSs. Note the characteristic multilobular structure of the VS
on the left side
The VS may reach large size before the onset of
audiological or vestibular symptoms. Compared
to sporadic VSs, they tend to be more invasive,
have a higher degree of dividing cells, and are
frequently multifocal. A polycyclic tumor shape
is more often observed. The growth rate of VSs
is highly variable and remains unpredictable. It is
generally higher in younger patients, but great
variations exist, both between different patients
and in the same patient over time [4, 43].
Patients may present with cranial meningioma/s
or with spinal tumor/s prior to the appearance
317
of a VS [13]. The likelihood of developing
NF-2 in young patients (<18 years) present-
ing with isolated meningioma or VS is 20 and
10 %, respectively. This rate drops dramati-
cally afterward and is very unlikely in patients
>30 years [17].
Approximately half of all NF-2 patients develop
meningiomas, and the lifetime risk is estimated to
be as high as 75 % [13]—see Case Illustration 6.1.
These meningiomas have a higher degree of divid-
ing cells than sporadic meningiomas and are usu-
ally fibroblastic [13].
318 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Case Illustration 6.1 recovery. Since several months, however, her

Multiple NF-2-Associated Meningiomas
and Schwannoma
This 21-year-old female patient presented after
multiple cranial and spinal surgeries at another
institution. She had been diagnosed of having
NF-2 seven years ago and has been operated
for a parasagittal meningioma, craniocervi-
cal meningioma, and schwannoma of the C5
nerve root on the right side. One year ago the
VS on the right side was partially removed.
After the surgery she had transient swallowing
difficulty and facial palsy with almost complete
gait ataxia became more severe. The MRI
showed significant enlargement of the right
CP angle tumor.
At presentation the patient had right-sided
slight facial weakness (HB grade II), bilat-
eral hypacusis, more pronounced on the
right side (PTA of 70 dB vs. PTA of 15 dB
on the left side), slight weakness in both
arms (grade 4/5), and severe gait ataxia with
positive Romberg test. The MRI examina-
tion (a–f) showed a large tumor in the right
CP angle with characteristics compatible with

a b

c d

(a–f) MRI study of the patient with bilateral VSs and multiple supra- and infratentorial meningiomas (arrows).
(g–i) Follow-up MRI performed 12 months after the surgery
Neurofibromatosis Type 2 319

e f

g h

i
320 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
VS, extension grade T4b. The tumor com-
pressed severely and dislocated the brain stem.
A smaller tumor was seen in the left CP angle.
Several other contrast-enhancing lesions were
seen in the right CP angle, posterior cranial
fossa, and supratentorially with imaging char-
acteristics of meningiomas. Multiple spinal
extramedullary tumors (probably schwanno-
mas) were also visualized.
Considering the fact that the large right-
sided VS caused the current neurological
deterioration, as well as the size of this tumor
and the absence of useful hearing on this side,
a decision was taken to remove it surgically.
A standard RS approach (re-craniectomy)
was performed, and the VS, as well as the
accessible meningiomas, were removed com-
pletely. Although the facial nerve was pre-
served anatomically, it was very atrophic and
reacted weakly when stimulated. After sur-
gery the patient had complete facial palsy
(HB grade 6/6), anacusis on the right side,
and dysphagia. The swallowing difficulty
proved to be transient and at discharge recov-
ered almost completely. A tendency for
improvement of the facial palsy was also
noted. Two weeks later it was assessed as HB
grade V. At 12 months follow-up, she could
close the eye and her face was symmetrical at
rest (HB grade III). The other tumors remained
asymptomatic and the MRI showed no growth
tendency (g–i).
Neurofibromatosis Type 2
Spinal schwannomas arising from the dorsal
spinal roots are very common findings—Fig. 6.2
[42]. Most of them are small and asymptomatic. A
dumbbell appearance is frequently seen (Fig. 6.2c).
Radiologically these schwannomas cannot be dif-
ferentiated from neurofibromas arising from spi-
nal roots in NF-1 patients [73]. Close follow-up is
required because some schwannomas may enlarge
and cause compression of the spinal cord or may
even compress adjacent organs (Fig. 6.2e).
Peripheral schwannomas can arise from any
nerve; sensory nerves are, however, more fre-
quently affected than motor nerves [13, 73]. The
clinical manifestation is caused by impaired
motor or sensory functions or by nerve irritation,
causing pain or paresthesias. Superficial periph-
eral schwannomas present either as subcutaneous
nodules or as slightly raised well-circumscribed
cutaneous lesions [15, 73]. Neurofibromas pre-
senting as skin tumors are very rare.
The ependymomas and astrocytomas in NF-2
are most commonly intramedullary spinal or
cauda equina tumors but may be also intracranial.
Patients with NF-2, in contrast to those with
NF-1, rarely develop non-tumor-related neuro-
logic manifestations, such as peripheral neuropa-
thy and hyporeflexia [22]. Seizures, epilepsy, and
cognitive dysfunction are not typical. Visual
impairment is common in the NF-2 population—
up to 75 %—and is due to posterior subcapsular
lenticular opacities, retinal abnormalities, partic-
ularly hamartomas, or epiretinal membrane [57].
Between 14 and 81 % of patients have presenile
cataracts, which rarely require active treatment.
The average actuarial survival from the time of
diagnosis was estimated to be 15 years in the
1970s–1980s but is constantly improving with earlier
diagnosis and better treatment [2, 13, 15]. According
to the study of Baser et al. [4], published in 2002, age
at diagnosis, intracranial meningiomas, and type of
constitutional NF2 mutation are predictors of the
relative risk of mortality. Another interesting finding
was that patients who are treated in specialty centers
had a significantly lower risk of mortality than those
who were treated in nonspecialty centers.
321
General Management
The treatment of patients with bilateral VSs is
different from that of patients with sporadic
unilateral VSs. The lifelong tendency to forma-
tion of new CNS tumors predetermines the
impossibility of definitive cure of NF-2 patients.
Therefore, management is focused on life pro-
longation, preservation or rehabilitation of cra-
nial nerve function, and maintenance of quality
of life [6, 63]. The treatment should be individ-
ualized and performed in specialized treatment
centers in close collaboration with the patient
and his family. Patient’s clinical presentation,
attitude, and expectations, as well as the tumor
characteristics, should guide the decision-making
process. The tumor size and the function of the
cochlear nerves on both sides are issues of par-
amount importance.
Management guidelines of NF-2-associated
VSs are controversial in regard to timing and
type of treatment. The options are observation,
radiosurgery, or surgery [50]. Several surgi-
cal strategies have been put forward: partial or
total tumor removal aiming hearing preserva-
tion (via the retrosigmoid or the middle fossa
approaches), total removal of the tumor with
placement of an auditory brain stem implant
(via the retrosigmoid or the translabyrinthine
approaches), or decompression of the cochlear
nerve in the IAC. Some surgeons advocate sub-
total tumor removal in all cases. Regrowth of
the residual tumor, however, is highly probable
because of the typical young patients’ age and
the fact that VSs are typically rapidly growing in
NF-2. Surgeries in case of recurrences are more
difficult and more dangerous; clear arachnoidal
dissection plane to facial nerve is often absent.
Our treatment goal has always been total tumor
removal. The only exceptions have been made in
order to preserve hearing function or facial nerve
integrity. Deliberate subtotal resections can be
performed for brain stem decompression and for
hearing preservation in the last hearing ear (see
Case Illustration 6.2).
322 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

a b

c d

Fig. 6.2 Multiple spinal schwannomas (a, b) in a patient bilateral extraspinal component (c–e), extending in the
with NF-2 with bilateral VSs (c, arrowheads). The tumors upper thorax (e)
at the cervicothoracal junction level (arrows) have a major
Neurofibromatosis Type 2 323

Fig. 6.2 (continued)

324 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
Case Illustration 6.2
Subtotal Tumor Removal for Brain Stem
Decompression and Hearing Preservation
in the Last Hearing Ear
This 19-year-old patient presented with NF-2
and multiple intracranial and spinal tumors.
He has been operated three times previously:
a large VS on the right side and a cervical
schwannoma have been removed and a VP
shunt has been inserted. During the last
2 years, he was followed with regular MRI
examinations (a–d). At presentation he was
in poor general condition and was unable to
walk independently. He had oculomotor dis-
turbances, bilateral trigeminal hypoesthesia,
facial weakness (HB grade III) on the right
side, complete bilateral hearing loss, lower
cranial nerve dysfunction, and quadriparetic
syndrome. The MRI examination demon-
strated significant enlargement of the left-
sided VS when compared to the previous
images (e–i). Multiple cranial meningiomas
and schwannomas with extracranial exten-
sion, as well as multiple spinal tumors, were
seen as well. Obviously, the clinical deterio-
ration correlated mostly to the growth of the
left-sided VS. Therefore, a decision was
taken to remove this tumor in an attempt to
prolong the life expectancy of the patient,
according to his wish and the wish of his
family. A tracheostomy was performed, and
then the tumor was removed completely via
the RS approach. The facial nerve was com-
pletely destroyed by the tumor, even in its
petrous segment. All other structures could
be preserved. The postoperative CT scans (j)
showed small blood collections in the tumor
bed area, which were clinically irrelevant and
gradually resorbed spontaneously (k). The
patient had no new neurological deficits
besides the facial nerve palsy and was trans-
ferred to a rehabilitation center in a stable
condition with improvement of gait and
quadriparesis.
Neurofibromatosis Type 2 325

a b

c d

(a–d) MRI of the head and spine, performed 10 months
prior to admission. (e–i) MRI examination of the patient,
performed at our institute. Almost all tumors showed a
growth tendency. Note the significant enlargement of
the left-sided VS when compared to the initial images
(j, k) Postoperative CT scans (day 1 after surgery):
small blood collection in the tumor bed area is visible,
which had no clinical significance and was managed
conservatively
326 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

e f

g h
Neurofibromatosis Type 2 327

j k
328 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Fig. 6.3 Very large bilateral VSs, extreme compression of the brain stem: the only management option is surgical
removal of both tumors

Large VSs Smaller VS, Useful Hearing

Symptomatic large tumors have to be removed
surgically in order to decompress the brain stem
(Figs. 6.3, 6.4, and 6.5). An attempt to pre-
serve available hearing should always be made
(Fig. 6.4). Alternatively, the tumor may be
removed partially, and the remaining part may be
subjected to radiosurgery. Drawback of this lat-
ter management concept, however, is the added
morbidity risk of the two procedures. Therefore,
we do not recommend it.
Some surgeons advocate a more conservative
approach in such cases in order to preserve hear-
ing. Surgery is postponed as long as possible: the
patients are followed with serial MRIs, and sub-
total intracapsular resection is done if the VS
enlarges [49]. Surgery on the better hearing ear is
avoided, regardless of tumor size. With this man-
agement strategy, surgery is performed only
when the tumors have reached a considerable
size, which significantly worsens the outcome.
Neurofibromatosis Type 2 329

a b

Preoperative audiogram

c R L
Frequency in kHz
01.5 0.25 0.5 1 1.5 2 3 4 5 8 10 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10

–10 –10
0 0
10 10
20 20
Hearing loss in dB HL

30 30
40 40
50 50
60 60
70 70
80 80
90 90
100 100
110 110

Speech discrimination in %
0 20 40 60 80 100 0 20 40 60 80 100
0 0

10 10

20 20
Sound pressure level in dB

30 30

40 40

50 50

60 60

70 70

Fig. 6.4 Patient with NF-2 and very large bilateral VSs. He follow-up examination was HB grade II on both sides. (a, b)
was operated in 1982: both tumors were removed and the Preoperative CT scans. (c) Preoperative audiogram. (d)
hearing was preserved. The facial nerve function at 12 months’ Postoperative CT scan. (e) Postoperative audiogram
330 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Postoperative audiogram

18 months after bilateral removal

e R Frequency in kHz
L

01.5 0.25 0.5 1 1.5 2 3 4 5 8 10 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10

–10 –10
0 0
10 10
Hearing loss in dB HL

20 20
30 30
40 40
50 50
60 60
70 70
80 80
90 90
100 100
110 110

Speech discrimination in %
0 20 40 60 80 100 0 20 40 60 80 100
0 0

10 10

20 20
Sound pressure level in dB

30 30

40 40

50 50

60 60

70 70

Fig. 6.4 (continued)

Neurofibromatosis Type 2 331

a b

c d

Fig. 6.5 Removal of a giant VS on the nonhearing side.
Preoperative CT (a) and MRI (b–e) of a patient with NF-2.
A giant VS on the left side and a smaller one on the right
side are visible. At presentation hearing was completely
lost on the left and was still functional on the right side.
The management decision was to remove the large tumor
operatively and to follow the smaller one. The tumor
could be removed completely via the RS approach, and
the facial nerve could be preserved. (f) Postoperative CT
332 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
e f
Fig. 6.5 (continued)
We accept an initial observational period in
selected cases: in older medically unstable
patients, in those with poor surgical risks, or in
those who refuse surgery. We, as well as other
experienced surgeons, favor early proactive
treatment of NF-2 patients, based on the obser-
vation that VSs can be removed and hearing
preserved [6, 63, 66, 67]. Our goal has always
been to preserve useful hearing for as long as
possible. If the chances for useful hearing pres-
ervation are realistic, our recommendation is
for early surgery. Tumor extension, hearing
level, and BAEP features, which are the main
predictors of successful hearing preservation,
determine which side should be operated ini-
tially. The side with best chances for hearing
preservation—smaller tumor and better hear-
ing level—is treated first. In case the VSs are
of similar size, the side with better hearing
level is operated initially (Fig. 6.6). If the hear-
ing and the tumor size are similar on both sides,
our decision is based on the preoperative BAEP.
The side with better BAEP quality is operated
first because the chances of successful hearing
preservation are higher (Fig. 6.7).
Surgery on the Only Hearing Side
Management of VSs on the only hearing side (or
“in the single ear”) is especially challenging. In
case of large tumors with compressive effect, sur-
gery is without alternatives. In smaller tumors,
however, the consideration is whether to treat
them actively early, accepting the risk of complete
hearing loss, or to observe the patient until hear-
ing deteriorates. With the later strategy, the patient
may enjoy hearing for a certain time. The draw-
back, however, is that sooner or later the hearing
will be completely lost. Ultimately, the patient
should take the decision on the type of treatment.
We recommend early surgical treatment in
those patients that have realistic chances for
hearing preservation: useful hearing and good
quality of the preoperative BAEP. The goal of
surgery is to decompress the IAC and to remove
the tumor. The extent of tumor removal, however,
is determined intraoperatively. As shown ear-
lier, BAEP monitoring is a highly sensitive test
for the function of auditory pathways. Surgery
proceeds as far as the BAEP remains stable. In
case that even slight microsurgical actions are
Neurofibromatosis Type 2
Fig. 6.6 Patient with relatively small VSs and good bilateral hearing. The tumor on the left side (better hearing level)
was removed initially
followed by severe deterioration of the auditory
evoked responses, partial resection is performed.
Applying this strategy, we could preserve hearing
in 15 patients. Follow-up examinations indicated
that preserved hearing remained functional for
periods of up to 15 years. If the BAEP is of low
quality before surgery, with loss of waves or con-
siderable interval prolongation, this feedback is
not available. In such case, we would not recom-
mend early surgery.
If the patient decides against surgery, the
tumor should be followed and removed when
hearing is lost or the tumor progression leads
to serious neurological symptoms. The res-
toration of hearing with an auditory implant
should be planned and performed at the same
surgery or in several weeks. Bilateral deafness
of NF-2 patients is often inevitable, and an
ABI implantation is the only option to provide
some hearing sensation.
Approximately 40 % of NF-2-associated VSs
are multilobular, and the nerves and vessels may
pass between the tumor lobules. The tumor can
infiltrate the fibers of individual nerves, which is
seldom the case in unilateral tumors. The man-
agement of non-CP angle tumors associated with
NF-2 follows the general neurosurgical principles.
333
These tumors are generally very slow growing.
Only symptomatic tumors or those showing a
steady growth tendency should be treated (see
Case Illustration 6.2).
Radiosurgery is another treatment option.
Some studies, however, showed that the outcomes
are not as good as in individuals with sporadic
unilateral VS (see Case Illustration 6.3). The
long-term tumor control rate ranges between 60
and 81 % [41, 55, 60]. In a study of Rowe et al.
[60], 20 % of all patients required further treat-
ment 8 years after radiosurgery, and in 30 % there
was some concern about tumor control. Hearing
preservation in the published series is approxi-
mately 33–43 %, although late deterioration is
observed in up to 40 % of the patients [41, 55,
60]. As pointed by Brackmann et al. [6], hearing
preservation rate and facial nerve function after
radiosurgery are not superior to those follow-
ing microsurgery by experienced surgeons. The
trigeminal nerve dysfunction rate is, however,
higher. Radiosurgery is therefore not the optimal
primary therapy in NF-2. It is best reserved for
those patients who have particularly aggressive
tumors, those with medical contraindications for
microsurgery, patients who refuse surgery, or for
the elderly [2].
334 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

01.5 0.25 0.5 1 1.5 2 3 4 5 8 10

a b–10
0
10
20
30
40
50
60
70
80
90
100
110

c 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10 d

–10
0
10
20
30
40
50
60
70
80
90
100
110

Fig. 6.7 This is the first patient with successfully
removed by the senior author bilateral VSs and normal
postoperative facial nerve function and preserved useful
hearing after surgery, done. She has been operated in
1981. Initially the larger tumor on the side with worse
hearing—the left side—was removed. In 3 months the
second tumor was also removed, and the hearing and
facial nerve function were preserved. (a) Preoperative CT.
(b, c) Preoperative audiogram (b, right side; c, left side).
(d) Postoperative photo of the patient, demonstrating the
normal facial nerve function on both sides. (e, f) Post-
operative audiogram
Neurofibromatosis Type 2 335

e 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10 f 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10

–10 –10
0 0
10 10
20 20
30 30
40 40
50 50
60 60
70 70
80 80
90 90
100 100
110 110

Fig. 6.7 (continued)

336 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Case Illustration 6.3
Initial Follow-Up, Radiosurgery, and
Microsurgery
This 18-year-old male patient was diagnosed
of having NF-2 with bilateral VSs in 1996 (a).
Initially he was followed radiologically; the MRI
examination in 1 year showed clear enlargement
of both tumors (b). He had profound hearing loss
on the left side and still useful hearing on the right
side. Gamma knife treatment was performed in
1997. One year later, when he presented for the
first time to the senior author, he had no hear-
ing on both sides, bilateral trigeminal hypesthe-
sia, severe gait ataxia, and weakness of the right
extremities, as well as intracranial hypertension.
The MRI study demonstrated that both VSs
have enlarged further (c, d). The brain stem was
severely compressed, and the ventricles were
dilated. A new intra-axial tumor was also visible
in the low medulla oblongata. The left-sided VS
was removed initially; in 3 months the second
VS was also removed successfully.

a b

c d

(a–d) Bilateral VSs that have been initially observed, then treated with radiosurgery, and then removed operatively.
See the text for details
Neurofibromatosis Type 2
Personal Series
Over a period of approximately 40 years, the
senior author has treated surgically 175 patients
with NF-2. The total number of VS surgeries in
these patients was 228. Complete tumor removal
rate was 85 %, while in 15 % deliberately subto-
tal removal was performed for brain stem decom-
pression and hearing preservation in the only
hearing ear. Facial nerve preservation was usu-
ally possible unless the schwannoma (or multiple
337
schwannomas) originated from the facial nerve
or no cleavage plane between the cranial nerves
and the tumor could be found (see Case Illustration
6.4). In 205 cases (90 %) the facial nerve could
be separated from the VS and its continuity pre-
served. The overall rate of hearing preservation
in the series was 35 %. If only patients with pre-
served functional preoperative hearing are
included, however, the rate was 65 %. Bilateral
hearing after surgery had 23 % of the patients.
See Case Illustrations 6.5 and 6.6.
338 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Case Illustration 6.4 a

Siblings with Bilateral VSs
These two siblings with NF-2 presented to
the senior author in the early 1990s. Their
father had been operated for bilateral VSs
5 years earlier. The boy was 17 years old,
and although his VSs were of similar size,
hearing level was better on the left side
(a–c). The girl was 13 years old at presen-
tation. Her hearing was better on the side
of the larger VS (d–f). Both siblings were
admitted together and operated the same
day (g, h). See the text to the figures for
details.

Left side
May 1992 June 1992 June 1993
Preoperative Postoperative Postoperative
Frequency in kHz
b 01.5 0.25 0.5 11.5 2 3 4 5 8 10 01.5 0.25 0.5 11.5 2 3 4 5 8 10 01.5 0.25 0.5 11.5 2 3 4 5 810
–10 –10 –10
Hearing loss in dB HL

0 0 0
10 10 10
20 20 20
30 30 30
40 40 40
50 50 50
60 60 60
70 70 70
80 80 80
90 90 90
100 100 100
110 110 110
Speech discrimination in %
0 20 40 60 80 100 0 20 40 60 80 100 0 20 40 60 80 100
0 0 0
Sound pressure level in dB

10 10 10
20 20 20
30 30 30
40 40 40
50 50 50
60 60 60
70 70 70

Preoperative MRI demonstrating bilateral VSs in a
17-year-old boy (a). The tumor on the left side was
removed initially, and hearing could be preserved (b).
One year later the right-sided tumor was removed, the
facial nerve was preserved, but hearing was lost (c). The
13-year-old sister was operated initially on the left side,
and later on the right-sided VS was removed (d). Hearing
could be preserved on both sides (e, f). (g, h) Postoperative
photos of the siblings, approximately 3 months follow-
ing last surgery: normal bilateral facial nerve function
Neurofibromatosis Type 2 339

Right side
May 1993 May 1993
Preoperative Postoperative
Frequency in kHz
c 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10

–10 –10
0 0
10 10
20 20
Hearing loss in dB HL

30 30
40 40
50 50
60 60
70 70
80 80
90 90
100 100
110 110

d
340 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Left side
May 1992 June 1992 June 1993
Preoperative Postoperative Postoperative
Frequency in kHz
e 01.5 0.25 0.5 11.5 2 3 4 5 8 10 01.5 0.25 0.5 11.5 2 3 4 5 810 01.5 0.25 0.5 11.5 2 3 4 5 8 10
Hearing loss in dB HL

–10 –10 –10

0 0 0
10 10 10
20 20 20
30 30 30
40 40 40
50 50 50
60 60 60
70 70 70
80 80 80
90 90 90
100 100 100
110 110 110

Speech discrimination in %
0 20 40 60 80 100 0 20 40 60 80 100 0 20 40 60 80 100
0 0 0
Sound pressure level in dB

10 10 10
20 20 20
30 30 30
40 40 40
50 50 50
60 60 60
70 70 70
Neurofibromatosis Type 2 341

Right side
May 1993 May 1993
Preoperative Postoperative
Frequency in kHz
f 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10 01.5 0.25 0.5 1 1.5 2 3 4 5 8 10

–10 –10
0 0
10 10
Hearing loss in dB HL

20 20
30 30
40 40
50 50
60 60
70 70
80 80
90 90
100 100
110 110

Speech discrimination in %
0 20 40 60 80 100 0 20 40 60 80 100
0 0

10 10
Sound pressure level in dB

20 20

30 30

40 40

50 50

60 60

70 70
342 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

h
Neurofibromatosis Type 2 343

Table 6.4 Modified from Yohay [73]

Features NF-1 NF-2
Inheritance Autosomal dominant Autosomal dominant
Incidence 1:3,500–4,000 1:25,000–40,000
Chromosome abnormality 17q11 22q12
Gene product Neurofibromin Merlin
Typical clinical presentation/age Six or more café-au-lait macules/infancy Hearing loss or vestibular dysfunction/
or early childhood young adults
Nerve sheath tumors Neurofibroma, plexiform neurofibroma Schwannoma
Intracranial tumors Gliomas (optic gliomas, other low-grade Bilateral vestibular schwannomas,
astrocytomas/gliomas), ependymomas, meningiomas
meningiomas, and/or PNETs
Spinal tumors Nodular plexiform neurofibromas Schwannomas, ependymomas,
astrocytoma
Cognitive Learning disability, AHDH Normal
Other tumors Chronic myeloid leukemia, None
pheochromocytoma
Ophthalmologic findings Lisch nodules, congenital glaucoma Posterior subcapsular lenticular
opacities, presenile cataracts, retinal
hamartomas, or epiretinal membrane

Differential Diagnosis The neurological symptoms in patients with

Neurofibromatosis Type 1
NF-2 is distinctly different disease from neuro-
fibromatosis type 1 (NF-1). NF-1 or the “von
Recklinghausen disease” is an autosomal-domi-
nant disorder and results from mutation of a tumor
suppressor gene mapped to chromosome 17: the
NF1 gene [32]. It occurs with an incidence of 1
per 3,500–4,000 live births. The diagnostic cri-
teria for NF1, formulated by the NIH Consensus
Development Conference on NF, include six
or more café-au-lait macules, two or more
neurofibromas of any type or 1 or more plexiform
neurofibroma, two or more Lisch nodules, distinct
osseous lesions, or first degree relative with NF-1.
When two or more of these signs are present in
one patient, the diagnosis NF-1 is accepted.
Patients with NF-1 have specific clinical pre-
sentation, which is completely different from that
seen in case of NF-2 (see Table 6.4). Cutaneous,
ophthalmological, musculoskeletal, and/or neuro-
logical symptoms occur in varying combinations.
The cutaneous and ocular manifestations—
typically café-au-lait macules, axillary freckling,
and Lisch nodules—are the most common [72].
Ninety-nine percent of the patients have café-au-lait
macules.
NF-1 are caused by brain, spine, or peripheral
nerves tumors; epilepsy; macrocephaly; hydro-
cephalus; meningoceles; and/or peripheral neu-
ropathy [72]. Typical finding is multiple peripheral
neurofibromas: either cutaneous neurofibromas,
subcutaneous neurofibromas, nodular plexiform
neurofibromas, or diffuse plexiform neurofibromas.
Plexiform neurofibromas may undergo malig-
nant transformation. A higher risk for developing
intracranial tumors is observed in NF-1 patients,
compared to the general population. The tumor
types, associated with NF-1 and NF-2, however,
are different. NF-1 patients tend to have most fre-
quently gliomas, ependymomas, meningiomas,
and/or PNETs. Gliomas are typically low grade and
involve the optic pathways, hypothalamus, cerebel-
lum, brain stem, and spinal cord. Optic pathway
gliomas generally have a more indolent natural
history and are less aggressive than optic gliomas
in patients without NF-1 [68]. They involve fre-
quently the anterior visual pathways and occur in
11–19 % of NF-1 patients. Vascular complications,
such as carotid or middle cerebral artery occlusion,
can occur [32]. NF-1 has a significant effect on
learning and cognition and causes frequently psy-
chiatric disorders.
344 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
Schwannomatosis
Schwannomatosis is a recently defined entity,
characterized by multiple spinal, peripheral nerve
or subcutaneous schwannomas in the absence of
VSs [39, 64]. Schwannomatosis is segmental or
localized to a certain body part, such as a single
limb, several contiguous segments of the spine,
or one side of the body, in approximately 30 % of
patients. Most schwannomas are caused by
somatic NF2 gene inactivation; less frequently
mutations in the INI1/SMARCB1 gene are
responsible [25]. The disease is rarely familial.
Patients with schwannomatosis frequently pres-
ent with intractable pain rather than cranial nerve
deficits. Other intracranial tumors are not typical
for this condition.
Multiple Meningiomas (See Chap. 7)
Follow-Up and Screening of NF-2 Patients
Patients with known NF-2 should be followed
closely. In case no new symptoms/signs appear,
annual audiological and ophthalmological exam-
inations, as well as MRI of the brain, are manda-
tory. Once the diagnosis of NF-2 is made, relatives
who are at risk should be screened for the dis-
ease: a thorough dermatological, ophthalmologi-
cal, and neurological examinations, as well as
MRI of the whole neuraxis, should be performed
(NIH Consensus conference). All patients with
NF-2 and their families (relatives at risk) should
have access to genetic testing because early, at
best presymptomatic, diagnosis improves clinical
outcome.
Presently, prenatal diagnosis of pregnancies is
possible: the DNA of fetal cells obtained by
amniocentesis (15–18 weeks of gestation) or
chorionic villous sampling (10–12 weeks of ges-
tation) can be examined [13]. Such test is recom-
mended for those at 50 % risk for developing
NF-2. A prerequisite for the test is the identification
of the disease-causing allele of an affected family
member or the establishing of a linkage in the
family.
The offspring of patients with NF-2 should be
followed and examined at regular intervals for
earliest possible detection of NF-2. Annual oph-
thalmological examinations should start soon
after birth, while the annual neurological exami-
nations should start at 7 or 10 years of age
[18, 51]. Audiogram should be performed twice
per year. MRI studies of the brain are recom-
mended annually or at 2 years interval starting at
10–12 years of age. Spine MRI has to be per-
formed in case signs or symptoms suggestive of
myelopathy or radiculopathy appear.
Neurofibromatosis Type 2
Case Illustration 6.5
Bilateral NF-2-Associated VSs
The patient presented with a history of hearing
loss on the left side and dizziness since more
than 2 years. Gradually his gait became unsta-
ble and headache appeared. At admission he
had anacusis on the left side and slightly
reduced but useful hearing on the right side,
trigeminal hypesthesia on the left side (V1–3),
decreased gag reflex, and gait ataxia. MRI
revealed giant VS on the left side, with a maxi-
mal diameter of approximately 5 cm, causing
severe compression of the brain stem (a–c). No
signs of hydrocephalus were, however, visual-
ized. The tumor on the right side was consider-
ably smaller—extension grade 3a.
The left-sided VS had to be removed at the
first step for obvious reasons: giant size, severe
compressive effect, no hearing on this side.
The surgery was extremely difficult: all neigh-
boring neural structures were very vulnerable
due to the long-standing severe compression.
The facial nerve was atrophic and splayed in
an umbrella-like fashion over the anterior
tumor surface. Still, its anatomical integrity
could be preserved and complete tumor
removal achieved. The patient recovered
345
quickly and had only a slight facial weakness
(HB grade II)—figures d, e. He decided for
initial follow-up of the right-sided VS. Two
years later hearing remained stable and the
tumor showed no major growth tendency.
Afterward, however, the right-sided VS began
to grow quickly, the patients’ condition dete-
riorated again, and hearing worsened.
Furthermore, he complained of worsened gait
and very annoying tinnitus. At presentation he
had bilateral anacusis and gait ataxia, prohib-
iting independent walking. The MRI study,
shown on figures f–i, showed that the tumor
on the right side had reached a large size and
caused compression of the brain stem. The
patient was therefore operated again: com-
plete removal of the tumor was achieved, and
the facial nerve was preserved. Figures j, k
show the CT 1 day after surgery with moder-
ate pneumocephalus. The whole postoperative
period was smooth, and the patient was dis-
charged with a slight (HB grade II facial weak-
ness on the right side) and improved balance.
Implantation of an ABI was discussed with
the family but was not plausible because the
patient did not fulfill the criteria outlined pre-
viously in text.
346 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

a b

T1-weighted contrast-enhanced (a, b) and CISS (c) MRI
images of the patient, demonstrating a small VS on the
right side and a giant one on the left side. The degree of
brain stem compression and deformation of the fourth
ventricle, as well as the large intracanalicular tumor part,
are obvious. (d) MRI, performed before discharge,
confirming the complete removal of the left VS. The
right-sided VS is visualized, as well as the fat in the left
IAC. Fat has characteristic signal on T1 and T2 and
should not be interpreted as tumor residual. (e) Photo of
the patient prior to discharge. Slight weakness of the
inferior facial branch on the left side is noted (HB grade
II). MRI images before (f–i) the second surgery: the
enlargement of the right-sided tumor is obvious. Note
the fat plug in the left IAC and a small contrast-enhanc-
ing structure lateral to it, which is suspicious for local
recurrence. (f) T1-weighted native MRI. (g, h)
T1-weighted MRI after contrast administration. (i) CISS
sequence. (j, k) CT 1 day after surgery with moderate
pneumocephalus
Neurofibromatosis Type 2 347

d e

f g

h i
348 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

j k
Neurofibromatosis Type 2
Case Illustration 6.6
Removal of Both VSs During the Same
Hospital Stay and Hearing Preservation
This patient suffered from a brain stem stroke
at the age of 15 years. He recovered partially
and still had some phonation difficulty, right-
sided hearing loss, and hemiparesis. At the
age of 24 years, he started complaining of
vertigo and tinnitus on the left side. MRI was
performed that showed bilateral CP angle
tumors. At presentation he was 25 years old
and complained of hearing loss on the right
side, swallowing difficulty, and vertigo. The
audiogram revealed non-useful hearing on
the right side (hearing loss of more than
80 dB in the main speech area) and normal
hearing on the left side (hearing loss of
15 dB). MRI showed bilateral VSs (a–d): on
the right side the tumor was larger (grade IV
B, approximately 3.5 cm in its maximal
diameter) and polycyclic; the left tumor was
grade III B (reaching but not compressing the
brain stem).
A decision was taken to remove initially the
larger tumor on the side with non-useful
hearing—the right-sided VS. The tumor was
349
removed completely, and both the facial and ves-
tibular nerves were preserved anatomically (e).
The patient recovered quickly and had only a
slight facial weakness (HB grade II). The man-
agement options of the tumor on the only hear-
ing side were discussed with the patient and
his family. Our recommendation was to per-
form the surgery in 3–6 months—after the
recovery from the initial surgery—because of
the good chances of hearing preservation (good
hearing level, good BAEP). The patient and his
family, however, insisted on having early sur-
gery during the same hospital stay. Taking into
account the excellent postoperative condition
of the patient and the fact that he came from a
very distant country to our department, we
decided to perform the second surgery 2 weeks
after the first one. Notably, the BAEP were
stable throughout the surgery, which allowed
us to proceed with tumor removal. The VS was
removed completely and the cranial nerves
were preserved (e). Postoperative audiogram
demonstrated preservation of the preoperative
hearing level on the left side (PTA of 15 dB)
(h). Until discharge the patient’s facial weak-
ness improved to HB grade II.
350 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

a b

c d

e f

Preoperative MRI demonstrating the bilateral VSs. (a–c) following the second surgery. Complete tumor removal
T1-weighted MRI with contrast enhancement (axial on both sides. The fat used to plug the IAC is well seen.
and sagittal). (c) CISS sequence. (e) CT of the head 1 Preoperative (g) and postoperative (h) audiograms—
day after the initial surgery. (f) MRI performed 3 days left ear
Neurofibromatosis Type 2 351

h
352 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Other Bilateral CP Angle Tumors
Bilateral CP angle tumors are usually seen in
patients with NF-2 and are typically vestibu-
lar or facial nerves schwannomas or—infre-
quently—meningiomas [1]. Other bilateral CP
angle lesions have been rarely reported: bilateral
choroid plexus papillomas [8], bilateral endo-
lymphatic sac tumors [29], bilateral primary
central nervous system lymphoma [27], bilat-
eral arachnoid cysts [70], or bilateral metasta-
ses (Fig. 6.8) [5, 10, 33, 56]. Infrequently fourth
ventricle tumors may extend into both CP angles
via the foramina of Luschka (Fig. 6.9). Some
extensive skull base or intra-axial tumors may
extend into both CP angles but per definition
should not be regarded as “bilateral CP angle
tumors” (Fig. 6.10).
The diagnosis may be straightforward in the
presence of NF-2 disease or if the typical MR
characteristics of schwannomas are seen. The
presence of multiple intracranial or extracranial
tumors or of a primary focus, as well as rapid
clinical deterioration, should arise the suspicion
of metastatic disease. In some cases, however,
clinical examination and neuroradiological
images do not allow to ascertain preoperatively
the tumor type.

Fig. 6.8 Metastatic lesions in both cerebellar hemispheres with extension in the CP angles in a patient with a primary
pulmonary adenocarcinoma
Other Bilateral CP Angle Tumors 353

Fig. 6.9 MR images of a 62-year-old male patient with a The tumor was removed completely via a median suboc-
subependymoma of the fourth ventricle, which extends cipital craniotomy
into both CP angles (arrows) via the foramina of Luschka.
354 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

Fig. 6.10 Extensive skull base meningioma with involvement of both CP angles
Other Bilateral CP Angle Tumors
Case Illustration 6.7
Bilateral Osteomas
A 30-year-old female patient presented to our
department with a history of increasing ver-
tigo and tinnitus on the left side. Her neuro-
logical examination revealed no deficits. The
audiogram showed hearing loss of 25 dB in
the main speech area on the left side and nor-
mal hearing on the right side. HRCT with
bone window showed hyperdense lesions with
bone density and irregular margins close to
both IAC (a, b). MRI showed a lesion in the
area of the porus of the IAC, but the image
characteristics were unspecific (c, d).
Considering the size of the lesion and the pro-
gressive nature of the symptoms, a decision
was made to remove the left-sided tumor, to
verify it pathologically, and to decompress the
vestibulocochlear nerve.
355
The standard RS approach was used and the
tumor, which had the features of an osteoma,
was seen attached to the anterosuperior lip of
the IAC (e, f). It was anterior and superior to
the seventh-eighth nerve bundle, compressing
and dislocating them posteriorly, although not
adherent to the lesion. The osteoma was
removed completely with a diamant micro-
drill, and the nerves were decompressed.
Histopathological examination proved the
diagnosis of an osteoma.
The patient recovered rapidly from surgery,
and at discharge the vertigo and tinnitus were
completely resolved (g). Hearing even
improved with a mean of 20 dB in the main
speech area (h, i). Considering the small size
of the contralateral lesion and the lack of any
related symptoms, we recommended regular
CT scan follow-up.
356 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

a c

b
d

(a, b) Preoperative bone window CT showing the
lesions attached to both IAC (arrowheads). The left
tumor has a maximal diameter of 1.2 cm and the right
one, 0.3 cm. The diagnosis of osteomas relies entirely
on CT scanning, which demonstrates well-demarcated,
dense bone outgrowth. (c, d) MR imaging: the lesion
in the area of the porus has unspecific characteristics.
(e, f) Intraoperative images. (e) Osteoma attached to
the left porus acusticus internus. (f) The osteoma has
been completely removed. The seventh-eighth nerves
have been decompressed. Postoperative bone window
CT. (g) Preoperative and (h) postoperative audio-
grams—improvement of hearing following the
cochlear nerve decompression (Reprinted from
Gerganov et al. [21]. Copyright © 2012, with permis-
sion from Wolters Kluwer Health)
Other Bilateral CP Angle Tumors 357

e f

g
358 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

h
Other Bilateral CP Angle Tumors
Case Illustration 6.8
Melanoma Metastases
This 46-year-old woman presented with a his-
tory of a Clark Level IV cutaneous malignant
melanoma of the right calf and involvement of
the inguinal lymphatic nodes. Her primary
tumor and the metastasis have been removed.
Subsequently alpha-interferon therapy was
instituted. One year later she began to com-
plain of dizziness and vomiting and later on of
acute hearing loss and tinnitus in the left ear.
CT and MR examinations were performed,
showing bilateral enhancing intra- and extra-
canalicular lesions (a–e).
Neurological examination revealed hearing
loss on the left side (PTA of 50 dB). No other
somatic metastases were detected. MR study
of the spine, however, showed further tumors:
at the level of T-12 and L-3 spinal nerve roots.
The differential diagnosis was therefore NF-2
and melanoma metastases.
A decision for surgery was taken with the
goal of obtaining histopathological diagnosis
and removing the tumor. The left-sided lesion
was operated initially due to the hearing loss
on this side. At surgery a black-colored tumor,
slightly protruding from the IAC, was seen.
The IAC was widely opened thus exposing the
whole tumor, which infiltrated the facial and
cochlear nerves. Frozen section showed that
the lesion was a malignant melanoma. Taking
into account this diagnosis and the extensive
infiltration of the facial nerve, partial tumor
resection was performed (e, f).
The postoperative period was uneventful.
Besides the complete loss of hearing on the
359
left side, no further neurological deficits devel-
oped. The final histopathological examination
confirmed the lesion to be a lightly pigmented
epithelioid malignant melanoma.
Surgery on the right side was postponed in
favor of palliative radiotherapy of the entire
neural axis. The patient decided, however, to
continue with the interferon therapy and to
undergo adjunctive chemotherapy instead of
radiation treatment. Follow-up MR imaging
showed that the intracranial and spinal lesions
remained constant in size. Twenty-four months
later the patient presented again with progres-
sive hearing loss on the right side. MRI demon-
strated slight enlargement of both intracanalicular
tumors. The right tumor was approached via the
retrosigmoid route, and a similar tumor in the
right IAC was detected. It could be removed
completely because a good dissection plane to
the cranial nerves was found (g–m). The post-
operative MR images are presented at figures n,
o. After surgery the patient had normal facial
nerve function and preserved useful hearing.
The histopathological diagnosis was lightly pig-
mented epithelioid malignant melanoma.
Summary: The presence of a primary malig-
nant melanoma with regional metastases, the
patient’s relatively advanced age for an initial
diagnosis of NF-2, and the absence of family
history of NF-2 suggested that the intracranial
lesions could be metastases. The clinical
course, however, did not give us clues to the
diagnosis. The finding of bilateral contrast-
enhancing intracanalicular lesions, along with
two spinal nerve root lesions, argued for the
diagnosis of NF-2.
360 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

a b

c d

Bilateral contrast-enhancing lesion in the IAC; the left
one has a maximal diameter of 1.2 cm and the right
one, 1.3 cm. (a, b) T1-weighted sequence after gado-
linium administration. (c, d) CISS sequence. (e, f)
MRI after the partial resection of the left-sided tumor
(T1-weighted sequence after gadolinium administration).
(g–m) Intraoperative images. (g) Initial view of the CP
angle and of the tumor (tu). (h–j) Exposure of the
intracanalicular tumor part. (k, l) Dissection from the
facial and vestibulocochlear nerves. (m) Complete
tumor removal. (n, o) MRI after the complete removal
of the right-sided tumor
Other Bilateral CP Angle Tumors 361

e f

g h
362 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

i j

k l
Other Bilateral CP Angle Tumors 363

n o
364 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
Hearing Rehabilitation
A central auditory prosthesis that bypasses the
cochlea and the eighth nerve is the only option
for restoration of some hearing sensations in
patients with bilateral loss of integrity and con-
ductivity of the cochlear nerve. Auditory brain
stem implants (ABI) were designed to stimulate
directly the second-order neurons and bypass
both the cochlea and cochlear nerve. The cochlear
nucleus complex is stimulated directly by placing
the ABI electrode into the lateral recess of the
fourth ventricle. ABI was applied for the first
time in 1979 by House and Hitselberger in the
House Ear Clinic in a patient with NF-2 after the
removal of her second VS [7, 52]. It has been
estimated that up to year 2008, more than 600
patients worldwide have received the ABI, most
of whom with NF-2 [9].
Indications
Implantation of the ABI is only the first step of a
complex auditory rehabilitation program. The
ultimate success is determined to a certain extent
by the capability and motivation of the patient to
complete this rehabilitation. Therefore, strict cri-
teria for patient selection have been introduced in
regard to age, diagnosis, cognitive level, and gen-
eral condition.
The criteria accepted in Hannover [36] are:
• Diagnosis of NF-2 with bilateral retrocochlear
deafness caused by tumor growth or surgical
tumor removal
• Absence of actual signs of serious disease
progression
• Age at least 18 years
• Stable psychosocial condition
• Realistic preoperative expectations
• Acceptable lip-reading ability
• Motivation to take part in the postimplantation
rehabilitation program
The indications for ABI implantation have
been expanded to include non-NF-2 patients with
bilateral profound hearing loss and impossibility
of cochlear implantation, such as ossification or
major cochlear malformations, aplasia, or avul-
sion of the cochlear nerves [9, 23]. A recently
published report describes the application of ABI
in patients who develop VS in the only hearing
ear, the other having been deaf from birth [58].
Prosthesis
The device used initially by House and
Hitselberger was a ball electrode ABI [52].
Multichannel devices replaced this early proto-
type single-channel device in 1992. Currently
several multichannel devices, sharing similar
basic constructional principles, are available [9,
26, 71]. The device consists of an implanted com-
ponent and an external component. The implanted
receiver–stimulator is attached to an electrode
array; the external component consists of a micro-
phone, speech processor, and a transcutaneous
transmitter coil (Figs. 6.11 and 6.12).
Sound is transformed to an analog electrical
signal by a microphone. The electrical signal is
modified by a speech processor afterward and
delivered to the set of electrodes (electrode array
composed of 21 platinum disks) mounted on a
silicone and Dacron mesh carrier. These elec-
trodes stimulate the cochlear nucleus and evoke
responses that can be interpreted by the brain as
sound [31].
Technique
Accurate placement of the prosthesis is essential to
achieve good functional outcome and avoid com-
plications. The target for placement of the ABI
electrode array is the cochlear nucleus complex,
composed of the dorsal and ventral cochlear nuclei.
These nuclei cannot be directly visualized on the
brain stem surface and must be located using sev-
eral anatomical landmarks. In case of large VSs
causing significant compression of the brain stem,
their identification may be challenging. The ABI
is implanted either via the RS approach or via the
translabyrinthine route [9, 40]. The RS approach
has the advantage of providing wide exposure of
the CP angle and allowing for removal of any
additional existing tumors, as frequently observed
in NF-2 (Figs. 6.13 and 6.14). From a retrosig-
moid perspective, the entry of the cochlear nerve
into the brain stem is obscured by the cerebellar
flocculus and covered by the choroid plexus [9].
The plexus protrudes from the foramen of
Luschka, which is found between the roots of the
Other Bilateral CP Angle Tumors 365

a c

b d

Fig. 6.11 One of the first ABIs implanted by the senior tumor. (b) Skin incision. (c) Initial view of the tumor. (d)
author. (a) Initial CT demonstrating both VSs. The tumor The schwannomas have been removed completely. (e, f)
on the left side was removed initially. The implantation of Implantation of the external device. (g) Postoperative CT,
the ABI was planned after the removal of the right-sided which shows the correct position of the electrode
366 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

e f

Fig. 6.11 (continued)

Other Bilateral CP Angle Tumors 367

a b

Fig. 6.12 Implantation of a magnetless ABI. (a) Planning (c) View after the reconstruction of the bone with methyl
of the skin incision and site of device implantation. methacrylate and fixation of the device
(b) Insertion of the electrode after the tumor removal.
368 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
Fig. 6.13 MRI of an NF-2 patient with a right-sided VS.
The larger left-sided VS has been removed earlier (note
the fat plug in the right IAC area). Implantation of an ABI
was planned after the removal of the right-sided tumor
facial and glossopharyngeal nerves. With a slight
caudal-to-cranial retraction of the cerebellum, these
structures can be seen. In case a stump of the
cochlear nerve remains after VS removal, it may be
a useful landmark for the lateral recess of the fourth
ventricle. The lateral recess can be palpated and
opened with a microhook. If adhesions are found,
they should be dissected. The observation of CSF
egress through the foramen of Luschka during a
Valsalva maneuver is another helpful clue. The use
of an angled endoscope may be helpful to control
the placement of the ABI electrode [20].
The placement of the electrode completely
within the lateral recess provides optimal contact
to the nuclei. Furthermore, this aids in the stabili-
zation of the electrode [19, 65]. The array is thus
Fig. 6.14 Removal of a right-sided VS and implantation
of an ABI—intraoperative images. (a) Initial view of the
tumor from an RS perspective. (b) The IAC has been
opened, and the intracanalicular part of the tumor has been
removed, exposing the facial nerve. (c) Tumor debulking
with the ultrasonic aspirator. (d) Dissection and removal of
located immediately adjacent to the dorsal cochlear
nucleus and the posterior tip of the ventral cochlear
nucleus [36]. Telemetry and electrical BAEP are
used to optimize the electrode position [9]. The
receiver/stimulator is placed in a bony well, drilled
in the temporoparietal area—superior and anterior
to the craniotomy—and fixed.
Initially, the ABI was placed in patients with
bilateral VSs at the second VS surgery. Some
authors advocate device placement during sur-
gery for the first tumor, even though the patient
has useful hearing in the opposite ear. The theo-
retical advantage of early implantation is that it
allows the patient to learn to use the device before
complete loss of hearing occurs in both ears.
Furthermore, if the initial device fails to provide
useful auditory sensation, it can be implanted on
the second side after tumor removal. The benefit
of this approach, however, remains unproven.
Complications
The specific complications, related to ABI place-
ment, include electrode migration, non-auditory
side effects, and CSF leaks. Placement of the
electrode array deep into the lateral recess can
prevent its migration, which usually occurs as a
result of unstable positioning or changes of the
brain stem after VS removal.
If the electrode is positioned too low, the
glossopharyngeal nerve can be stimulated, result-
ing in a sense of tingling or constriction in the
throat. If it is placed too high, the facial nerve
could be stimulated and cause facial twitching.
These non-auditory side effects can be avoided or
at least reduced by switching reference elec-
trodes, increasing the duration of the stimulus
pulse or turning off the more medial or lateral
electrodes. Previously turned off electrodes can
the last part of the tumor from the facial nerve. (e) The posi-
tion of the retractor is changed, and the cerebellum is
retracted slightly in a caudal-to-cranial direction, thus
exposing the flocculus (double arrows) and the choroid
plexus (single arrow). (f) A microhook is used to open the
lateral recess. (g) The electrode of the ABI has been placed
Other Bilateral CP Angle Tumors 369

a b

c d

e f
370 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
g the impact of brain stem compression or distor-
Fig. 6.14 (continued)
be reactivated later because with time some adap-
tation occurs and the severity of the non-auditory
sensations often decreases [36].
CSF leak along the electrode lead is avoided
by accurately sealing the dura opening with fat
pieces and fibrin glue.
Rehabilitation
The device is activated usually 4–8 weeks after
implantation. Rehabilitation program starts after
the first fitting with a basic training period of about
2 weeks consisting of intensive speech and hearing
therapy program every day [35, 36]. Thereafter,
refitting and reevaluation of the auditory perfor-
mance is performed initially at 3-month intervals
and then at 6-month intervals for 3 years, provided
the patient’s hearing condition remains stable.
Results
Patients should be informed and comprehend that
the ABI does not provide a normal quality of
sound. The level of performance and the quality
of sound from the ABI are variable and do not
reach the level obtained by the latest multichannel
cochlear implants. The reasons for these are not
completely elucidated yet. Major contributing
factors are the unfavorable tonotopic organization
of the cochlear nuclei, the distance from the sur-
face of the fourth ventricle to the cochlear nuclei,
tion caused by large VSs, and the presence of
other tumors, common in NF-2 patients. Another
unsolved problem is related to the more complex
processing of afferent signals at the level of
second-order auditory pathway neurons [35].
The performance of patients with ABIs is
comparable to that of patients with earlier single-
channel cochlear implants. Most patients acquire
environmental-sound awareness and understand-
ing of closed-set words, consonants, and vowels.
Ebinger et al. [11] report that 85 % of patients
undergoing implantation receive auditory sensa-
tions. Combined with lip-reading cues, 93 % of
patients demonstrate improved sentence under-
standing. Open-set speech recognition, however,
remains an exception.
In our series (Samii and T. Lenarz), more than
50 % of the patients developed lip-reading
enhancement in the first 3 months after device
activation [36]. Optimal lip-reading enhancement
was mostly observed 6 months after device acti-
vation. Regarding the speech tracking and
Freiburger numbers test, the plateau effect was
less prominent. Sound recognition and speech
perception generally continue to improve for up
to 8 years following implantation. oto
Lenarz et al. [35] reported on the patients’
assessment of their hearing and communication
abilities with the help of ABI. All patients profited
from the ABI: due to the increased environmental
awareness, certain dangers could be avoided and
the patients’ self-confidence increased. Most of
them distinguished not merely speech from envi-
ronmental sounds but could perceive difference
between various voice qualities and differentiate
environmental sounds.
New Developments
Penetrating Electrode ABI
The cochlear nucleus has a highly complex struc-
ture and contains several distinct tonotopic repre-
sentations composed of functionally different cell
types. They are organized perpendicular to the
surface [48]. Hence, the two-dimensional elec-
trode array, which is designed for surface stimu-
lation, makes poor connection with all of these
structural layers [31, 62].
References
A new generation of ABI with penetrating
microelectrodes—the penetrating electrode ABI
(or PABI)—was developed to achieve a better
alignment along the tonotopic gradient of the
cochlear nucleus [19, 53]. The PABI uses 8 or 10
penetrating microelectrodes in conjunction with
a separate array of 10 or 12 surface electrodes.
A recent review of the initial experience with ten
patients showed, however, that a major difficulty
is the reliable placement of the penetrating elec-
trodes into cochlear nucleus. Due to the individ-
ual anatomic variability and the tumor-induced
anatomical distortions, only 22 of 90 penetrating
electrodes produced auditory sensations.
The speech perception level of the patients could
not be improved [53]. Still, the study demonstrated
that the procedure is safe and that with the penetrat-
ing microelectrodes auditory sensations are achieved
at lower levels of electrical stimulation than with
surface electrodes; the pitch range was increased.
Auditory Midbrain Implant
The limited success of the ABI in NF-2 patients
may be associated with tumor-induced alterations
at the level of the cochlear nucleus. Stimulation at a
more proximal to the damage level may be a rea-
sonable alternative [34]. The inferior colliculus, in
particular the inferior colliculus central nucleus, is
a promising site for a central auditory prosthesis in
NF-2 patients because it provides access to neural
projections necessary for speech perception [34, 38].
The auditory midbrain implant (AMI) is a new
hearing prosthesis designed for stimulation of
this nucleus. An initial clinical trial included five
patients, in which a single shank AMI array (20
electrodes) was implanted. A modified lateral
suboccipital approach was developed to ensure
safe removal of large VS and to provide adequate
exposure of the inferior colliculus for ideal AMI
implantation in one stage [62].
The preliminary results have been presented
by Lim et al. [37]. They were interpreted as
encouraging in terms of the safety and function-
ality of the implant. Similar to the PABI, a major
problem remains the optimal position of the elec-
trodes. Although all patients obtained improve-
ments in hearing capabilities, the performance
varied dramatically across patients depending on
371
the implant location. Open-set speech perception
without lip-reading cues could not be achieved.
Cochlear Implants
Cochlear implantation may be an alternative in
case the cochlear nerve is preserved anatomically
and has maintained a positive electric stimulation
response. As pointed by Roehm et al. [59], they
bypass the external auditory canal, ossicles, and
inner-ear hair cells and stimulate directly the
cochlear nerve within the cochlea. Some literature
data indicate that such cochlear implant patients
performed better than auditory brain stem implant
patients and might even achieve open-set speech
communication [69]. In the recently published
study [59], the authors found that cochlear implants
offer superior auditory outcomes in patients with
retained cochlear nerves and positive results on
promontory stimulation testing. The experience is
though still very limited, and any conclusions
should be evaluated critically.
References
1. Baldwin D, King TT, Chevretton E, Morrison AW
(1991) Bilateral cerebellopontine angle tumors in
neurofibromatosis type 2. J Neurosurg 74(6):910–915.
doi:10.3171/jns.1991.74.6.0910
2. Baser ME, Evans DGR, Gutmann DH (2003) Neuro-
fibromatosis 2. Curr Opin Neurol 16(1):27–33.
doi:10.1097/01.wco.0000053583.70044.ab
3. Baser ME, Friedman JM, Joe H, Shenton A, Wallace
AJ, Ramsden RT, Evans DG (2011) Empirical develop-
ment of improved diagnostic criteria for
neurofibromatosis 2. Genet Med 13(6):576–581.
doi:10.1097/GIM.0b013e318211faa9
4. Baser ME, Makariou EV, Parry DM (2002) Predictors
of vestibular schwannoma growth in patients with
neurofibromatosis type 2. J Neurosurg 96(2):217–222.
doi:10.3171/jns.2002.96.2.0217
5. Bono P, Bartoloni Saint Omer F (1994) A rare case of
bilateral metastasis in the cerebellopontine angle.
Pathologica 86(3):324–328
6. Brackmann DE, Fayad JN, Slattery WH 3rd,
Friedman RA, Day JD, Hitselberger WE, Owens RM
(2001) Early proactive management of vestibular
schwannomas in neurofibromatosis type 2. Neuro-
surgery 49(2):274–280; discussion 280–283
7. Brackmann DE, Hitselberger WE, Nelson RA, Moore J,
Waring MD, Portillo F, Shannon RV, Telischi FF (1993)
Auditory brainstem implant: I. Issues in surgical implan-
tation. Otolaryngol Head Neck Surg 108(6):624–633
372 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
8. Casar Borota O, Jacobsen EA, Scheie D (2006)
Bilateral atypical choroid plexus papillomas in cere-
bellopontine angles mimicking neurofibromatosis 2.
Acta Neuropathol 111(5):500–502. doi:10.1007/
s00401-006-0067-7
9. Colletti V, Shannon RV, Carner M, Veronese S,
Colletti L (2009) Progress in restoration of hearing
with the auditory brainstem implant. Prog Brain Res
175:333–345. doi:10.1016/S0079-6123(09)17523-4,
S0079-6123(09)17523-4 [pii]
10. Delerue O, Destee A, Devos P (1991) Bilateral
metastases in the cerebellopontine angle. J Neurol
Neurosurg Psychiatry 54(6):562–563
11. Ebinger K, Otto S, Arcaroli J, Staller S, Arndt P
(2000) Multichannel auditory brainstem implant: US
clinical trial results. J Laryngol Otol Suppl 27:50–53
12. Evans DG (2009) Neurofibromatosis 2 [Bilateral
acoustic neurofibromatosis, central neurofibromatosis,
NF2, neurofibromatosis type II]. Genet Med
11(9):599–610. doi:10.1097/GIM.0b013e3181ac9a27
13. Evans DG (2009) Neurofibromatosis type 2 (NF2): a
clinical and molecular review. Orphanet J Rare Dis 4:16.
doi:10.1186/1750-1172-4-16, 1750-1172-4-16 [pii]
14. Evans DG, Baser ME, O’Reilly B, Rowe J, Gleeson M,
Saeed S, King A, Huson SM, Kerr R, Thomas N,
Irving R, MacFarlane R, Ferner R, McLeod R, Moffat
D, Ramsden R (2005) Management of the patient
and family with neurofibromatosis 2: a consensus
conference statement. Br J Neurosurg 19(1):5–12.
doi:10.1080/02688690500081206,
M877P25502X236M6 [pii]
15. Evans DG, Huson SM, Donnai D, Neary W, Blair V,
Newton V, Harris R (1992) A clinical study of type 2
neurofibromatosis. Q J Med 84(304):603–618
16. Evans DG, Huson SM, Donnai D, Neary W, Blair V,
Teare D, Newton V, Strachan T, Ramsden R, Harris R
(1992) A genetic study of type 2 neurofibromatosis in
the United Kingdom. I. Prevalence, mutation rate,
fitness, and confirmation of maternal transmission
effect on severity. J Med Genet 29(12):841–846
17. Evans DG, Ramsden RT, Gokhale C, Bowers N,
Huson SM, Wallace A (2007) Should NF2 mutation
screening be undertaken in patients with an appar-
ently isolated vestibular schwannoma? Clin Genet
71(4):354–358. doi:10.1111/j.1399-0004.2007.
00778.x, CGE778 [pii]
18. Evans DG, Sainio M, Baser ME (2000) Neuro-
fibromatosis type 2. J Med Genet 37(12):897–904
19. Fayad JN, Otto SR, Brackmann DE (2006) Auditory
brainstem implants: surgical aspects. Adv Otorhino-
laryngol 64:144–153. doi:10.1159/000094649, 94649
[pii]
20. Friedland DR, Wackym PA (1999) Evaluation of sur-
gical approaches to endoscopic auditory brainstem
implantation. Laryngoscope 109(2 Pt 1):175–180
21. Gerganov VM, Samii A, Paterno V, Stan AC, Samii M
(2008) Bilateral osteomas arising from the internal
auditory canal: case report. Neurosurgery 62(2):
E528–E529
22. Gijtenbeek JM, Gabreels-Festen AA, Lammens M,
Zwarts MJ, van Engelen BG (2001) Mononeuropathy
multiplex as the initial manifestation of
neurofibromatosis type 2. Neurology 56(12):
1766–1768
23. Grayeli AB, Bouccara D, Kalamarides M, Ambert-
Dahan E, Coudert C, Cyna-Gorse F, Sollmann WP,
Rey A, Sterkers O (2003) Auditory brainstem implant
in bilateral and completely ossified cochleae. Otol
Neurotol 24(1):79–82
24. Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks
J, Pyeritz RE, Rubenstein A, Viskochil D (1997) The
diagnostic evaluation and multidisciplinary manage-
ment of neurofibromatosis 1 and neurofibromatosis 2.
JAMA 278(1):51–57
25. Hanemann CO, Evans DG (2006) News on the genet-
ics, epidemiology, medical care and translational
research of Schwannomas. J Neurol 253(12):1533–
1541. doi:10.1007/s00415-006-0347-0
26. Jackson KB, Mark G, Helms J, Mueller J, Behr R
(2002) An auditory brainstem implant system. Am J
Audiol 11(2):128–133
27. Jaiswal AK, Chandra PS (2001) Cerebellopontine
angle meningioma with acquired chiari and syringo-
myelia: neuroimage. Neurol India 49(3):323
28. Kanter WR, Eldridge R, Fabricant R, Allen JC,
Koerber T (1980) Central neurofibromatosis with
bilateral acoustic neuroma: genetic, clinical and bio-
chemical distinctions from peripheral
neurofibromatosis. Neurology 30(8):851–859
29. Kempermann G, Neumann HP, Scheremet R, Volk B,
Mann W, Gilsbach J, Laszig R (1996) Deafness due to
bilateral endolymphatic sac tumours in a case of von
Hippel-Lindau syndrome. J Neurol Neurosurg
Psychiatry 61(3):318–320
30. Kluwe L, Mautner V, Heinrich B, Dezube R,
Jacoby LB, Friedrich RE, MacCollin M (2003)
Molecular study of frequency of mosaicism in
neurofibromatosis 2 patients with bilateral vestibular
schwannomas. J Med Genet 40(2):109–114
31. Kuchta J, Otto SR, Shannon RV, Hitselberger WE,
Brackmann DE (2004) The multichannel auditory
brainstem implant: how many electrodes make
sense? J Neurosurg 100(1):16–23. doi:10.3171/jns.
2004.100.1.0016
32. Lee MJ, Stephenson DA (2007) Recent developments
in neurofibromatosis type 1. Curr Opin Neurol
20(2):135–141.doi:10.1097/WCO.0b013e3280895da8,
00019052-200704000-00004 [pii]
33. Lee WT, Weber PC (2005) Melanoma metastasis mas-
querading as bilateral acoustic neuromas. Otolaryngol
Head Neck Surg 132(3):505–506. doi:10.1016/j.
otohns.2004.05.004, S0194599804005546 [pii]
34. Lenarz M, Lim HH, Lenarz T, Reich U, Marquardt N,
Klingberg MN, Paasche G, Reuter G, Stan AC (2007)
Auditory midbrain implant: histomorphologic effects
of long-term implantation and electric stimulation
of a new deep brain stimulation array. Otol
Neurotol 28(8):1045–1052. doi:10.1097/MAO.
References
0b013e318159e74f, 00129492-200712000-00013
[pii]
35. Lenarz M, Matthies C, Lesinski-Schiedat A, Frohne C,
Rost U, Illg A, Battmer RD, Samii M, Lenarz T (2002)
Auditory brainstem implant part II: subjective assess-
ment of functional outcome. Otol Neurotol 23(5):
694–697
36. Lenarz T, Moshrefi M, Matthies C, Frohne C,
Lesinski-Schiedat A, Illg A, Rost U, Battmer RD,
Samii M (2001) Auditory brainstem implant: part I.
Auditory performance and its evolution over time.
Otol Neurotol 22(6):823–833
37. Lim HH, Lenarz M, Lenarz T (2009) Auditory
midbrain implant: a review. Trends Amplif 13(3):
149–180. doi:10.1177/1084713809348372, 13/3/149
[pii]
38. Lim HH, Lenarz T, Joseph G, Battmer RD, Samii A,
Samii M, Patrick JF, Lenarz M (2007) Electrical stim-
ulation of the midbrain for hearing restoration: insight
into the functional organization of the human central
auditory system. J Neurosci 27(49):13541–13551.
d o i : 1 0 . 1 5 2 3 / J N E U RO S C I . 3 1 2 3 - 0 7 . 2 0 0 7 ,
27/49/13541 [pii]
39. MacCollin M, Woodfin W, Kronn D, Short MP (1996)
Schwannomatosis: a clinical and pathologic study.
Neurology 46(4):1072–1079
40. Maini S, Cohen MA, Hollow R, Briggs R (2009)
Update on long-term results with auditory brainstem
implants in NF2 patients. Cochlear Implants Int
10(Suppl 1):33–37. doi:10.1002/cii.383
41. Mathieu D, Kondziolka D, Flickinger JC, Niranjan A,
Williamson R, Martin JJ, Lunsford LD (2007)
Stereotactic radiosurgery for vestibular schwannomas
in patients with neurofibromatosis type 2: an analysis
of tumor control, complications, and hearing preser-
vation rates. Neurosurgery 60(3):460–468.
doi:10.1227/01.NEU.0000255340.26027.53; discus-
sion 468–470. 00006123-200703000-00005 [pii]
42. Mautner VF (1996) Spinal tumors in patients with
neurofibromatosis type 2: MR imaging study of fre-
quency, multiplicity and variety. AJR Am J Roentgenol
166(5):1231
43. Mautner VF, Baser ME, Thakkar SD, Feigen UM,
Friedman JM, Kluwe L (2002) Vestibular schwan-
noma growth in patients with neurofibromatosis type
2: a longitudinal study. J Neurosurg 96(2):223–228.
doi:10.3171/jns.2002.96.2.0223
44. Mautner VF, Lindenau M, Baser ME, Hazim W,
Tatagiba M, Haase W, Samii M, Wais R, Pulst SM
(1996) The neuroimaging and clinical spectrum of
neurofibromatosis 2. Neurosurgery 38(5):880–885;
discussion 885–886
45. Mautner VF, Tatagiba M, Guthoff R, Samii M,
Pulst SM (1993) Neurofibromatosis 2 in the pediatric
age group. Neurosurgery 33(1):92–96
46. Moffat DA, Quaranta N, Baguley DM, Hardy DG,
Chang P (2003) Management strategies in
neurofibromatosis type 2. Eur Arch Otorhinolaryngol
260(1):12–18. doi:10.1007/s00405-002-0503-9
373
47. Mohyuddin A, Neary WJ, Wallace A, Wu CL,
Purcell S, Reid H, Ramsden RT, Read A, Black G,
Evans DG (2002) Molecular genetic analysis of the
NF2 gene in young patients with unilateral vestibular
schwannomas. J Med Genet 39(5):315–322
48. Moore JK, Osen KK (1979) The cochlear nuclei in
man. Am J Anat 154(3):393–418. doi:10.1002/
aja.1001540306
49. (1994) National Institutes of Health Consensus
Development Conference Statement on Acoustic
Neuroma, December 11–13, 1991. The Consensus
Development Panel. Arch Neurol 51(2):201–207
50. Neff BA, Welling DB (2005) Current concepts in
the evaluation and treatment of neurofibromatosis
type II. Otolaryngol Clin North Am 38(4):671–684.
doi:10.1016/j.otc.2005.01.002, ix. S0030-6665(05)
00003-4 [pii]
51. Neff BA, Welling DB, Akhmametyeva E, Chang LS
(2006) The molecular biology of vestibular schwan-
nomas: dissecting the pathogenic process at the
molecular level. Otol Neurotol 27(2):197–208.
doi:10.1097/01.mao.0000180484.24242.54,
00129492-200602000-00011 [pii]
52. Otto SR, House WF, Brackmann DE, Hitselberger WE,
Nelson RA (1990) Auditory brain stem implant: effect
of tumor size and preoperative hearing level on func-
tion. Ann Otol Rhinol Laryngol 99(10 Pt 1):789–790
53. Otto SR, Shannon RV, Wilkinson EP, Hitselberger WE,
McCreery DB, Moore JK, Brackmann DE (2008)
Audiologic outcomes with the penetrating electrode
auditory brainstem implant. Otol Neurotol 29(8):1147–
1154. doi:10.1097/MAO.0b013e31818becb4
54. Parry DM, Eldridge R, Kaiser-Kupfer MI, Bouzas EA,
Pikus A, Patronas N (1994) Neurofibromatosis 2
(NF2): clinical characteristics of 63 affected individu-
als and clinical evidence for heterogeneity. Am J Med
Genet 52(4):450–461. doi:10.1002/ajmg.1320520411
55. Phi JH, Kim DG, Chung HT, Lee J, Paek SH, Jung HW
(2009) Radiosurgical treatment of vestibular schwan-
nomas in patients with neurofibromatosis type 2:
tumor control and hearing preservation. Cancer
115(2):390–398. doi:10.1002/cncr.24036
56. Preuss M, Stein M, Huegens-Penzel M,
Kuchelmeister K, Nestler U (2008) Metastatic
tumours mimicking vestibular schwannoma. Acta
Neurochir (Wien) 150(9):915–919. doi:10.1007/
s00701-008-0007-5
57. Ragge NK (1993) Clinical and genetic patterns of
neurofibromatosis 1 and 2. Br J Ophthalmol 77(10):
662–672
58. Ramsden R, Khwaja S, Green K, O’Driscoll M,
Mawman D (2005) Vestibular schwannoma in the
only hearing ear: cochlear implant or auditory brain-
stem implant? Otol Neurotol 26(2):261–264,
00129492-200503000-00023 [pii]
59. Roehm PC, Mallen-St Clair J, Jethanamest D,
Golfinos JG, Shapiro W, Waltzman S, Roland JT
(2011) Auditory rehabilitation of patients with
neurofibromatosis type 2 by using cochlear implants.
374 6 Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors
J Neurosurg 115(4):827–834. doi:10.3171/2011.5.
JNS101929
60. Rowe JG, Radatz M, Walton L, Kemeny AA (2002)
Stereotactic radiosurgery for type 2 neurofibromatosis
acoustic neuromas: patient selection and tumour size.
Stereotact Funct Neurosurg 79(2):107–116.
doi:10.1159/000070106, 70106 [pii]
61. Ruggieri M (2001) Mosaic (segmental) neurofibro-
matosis type 1 (NF1) and type 2 (NF2): no longer
neurofibromatosis type 5 (NF5). Am J Med Genet
101(2):178–180. doi:10.1002/ajmg.1332
62. Samii A, Lenarz M, Majdani O, Lim HH, Samii M,
Lenarz T (2007) Auditory midbrain implant: a com-
bined approach for vestibular schwannoma surgery
and device implantation. Otol Neurotol 28(1):31–38.
doi:10.1097/01.mao.0000247819.16325.7d,
00129492-200701000-00006 [pii]
63. Samii M, Gerganov V, Samii A (2008) Microsurgery
management of vestibular schwannomas in neuro-
fibromatosis type 2: indications and results. Prog Neurol
Surg 21:169–175. doi:10.1159/000156905, 156905 [pii]
64. Seppala MT, Sainio MA, Haltia MJ, Kinnunen JJ, Setala
KH, Jaaskelainen JE (1998) Multiple schwannomas:
schwannomatosis or neurofibromatosis type 2? J
Neurosurg 89(1):36–41. doi:10.3171/jns.1998.89.1.0036
65. Shannon RV, Fayad J, Moore J, Lo WW, Otto S,
Nelson RA, O’Leary M (1993) Auditory brainstem
implant: II. Postsurgical issues and performance.
Otolaryngol Head Neck Surg 108(6):634–642
66. Slattery WH 3rd, Brackmann DE, Hitselberger W
(1998) Hearing preservation in neurofibromatosis
type 2. Am J Otol 19(5):638–643
67. Slattery WH 3rd, Fisher LM, Hitselberger W,
Friedman RA, Brackmann DE (2007) Hearing preser-
vation surgery for neurofibromatosis type 2-related
vestibular schwannoma in pediatric patients. J
Neurosurg 106(4 Suppl):255–260. doi:10.3171/
ped.2007.106.4.255
68. Thiagalingam S, Flaherty M, Billson F, North K
(2004) Neurofibromatosis type 1 and optic pathway
gliomas: follow-up of 54 patients. Ophthalmology
111(3):568–577. doi:10.1016/j.ophtha.2003.06.008,
S0161-6420(03)01420-9 [pii]
69. Tran Ba Huy P, Kania R, Frachet B, Poncet C,
Legac MS (2009) Auditory rehabilitation with
cochlear implantation in patients with neurofibro-
matosis type 2. Acta Otolaryngol 129(9):971–975.
doi:10.1080/00016480802510202, 905661602 [pii]
70. Ucar T, Akyuz M, Kazan S, Tuncer R (2000) Bilateral
cerebellopontine angle arachnoid cysts: case report.
Neurosurgery 47(4):966–968
71. Vincent C, Zini C, Gandolfi A, Triglia JM, Pellet W,
Truy E, Fischer G, Maurizi M, Meglio M, Lejeune JP,
Vaneecloo FM (2002) Results of the MXM Digisonic
auditory brainstem implant clinical trials in Europe.
Otol Neurotol 23(1):56–60
72. Ward BA, Gutmann DH (2005) Neurofibromatosis 1:
from lab bench to clinic. Pediatr Neurol 32(4):221–
228. doi:10.1016/j.pediatrneurol.2004.11.002, S0887-
8994(04)00555-7 [pii]
73. Yohay K (2006) Neurofibromatosis types 1 and
2. Neurologist 12(2):86–93. doi:10.1097/01.nrl.
0000195830.22432.a5, 00127893-200603000-00003
[pii]
 Meningiomas
Cerebellopontine Angle Meningiomas
C. Rokitansky was the first to describe a petrous
ridge meningioma in 1855 at autopsy [81]. Later
on, Virchow described a tumor originating from
the posterior lip of the meatus acusticus, which
he called “psammoma” [100]. Sir Charles Balance
in London performed the first successful com-
plete removal of a CP angle tumor on November
19, 1894. According to the original description, it
was most probably a meningioma: firm, well-
encapsulated tumor with wide attachment to the
dura of the petrous bone. The term “meningioma”
was coined in 1922 by Harvey Cushing to
describe a benign neoplasm of the brain menin-
ges [19]. In 1938, Cushing and Eisenhardt
described their experience with the surgical man-
agement of seven patients with meningiomas
“simulating acoustic neuromas” [20].
Epidemiology
Meningiomas are the second most frequent tumor
of the CP angle after VSs. They comprise 6–15 %
of all CP angle tumors and 40–42 % of all poste-
rior cranial fossa meningiomas [10, 16, 74, 90].
Their epidemiological and biological features are
similar to those of meningiomas with other loca-
tions: they are generally slow-growing benign
lesions that occur more frequently in women in
the fifth to sixth decade of their life.
The senior author’s experience comprises more
than 1,000 posterior fossa meningiomas: 59 %
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_7, © Springer-Verlag Berlin Heidelberg 2013
7
(CP angle), 21 % (petroclival), 11 % (foramen
magnum), and 10 % (tentorial meningiomas).
Pathogenesis and Pathology
CP meningiomas are believed to arise from arach-
noid cap cell clusters located around the IAC,
lower border of superior petrosal sinus, lateral
border of the inferior petrosal sinus, around the
cranial nerve foramina, and in the region of the
geniculate ganglion [65]. The variable attach-
ment sites and direction of growth determine the
high heterogeneity of CP angle meningiomas in
terms of clinical presentation and operative
challenge.
Inactivation of both NF2 gene alleles is
regarded essential for meningioma formation in
both sporadic and in NF-2-associated menin-
giomas. The inactivation is caused most fre-
quently by biallelic losses of markers from the
chromosomal region (22q12.2) encompassing
the NF2 gene (40–70 % of meningiomas), muta-
tions in the NF2 gene (30–60 % of sporadic men-
ingiomas), calpain-mediated proteolysis, or
aberrant methylation in the 5¢ region of the NF2
gene [22, 24, 52]. As described in the chapter on
NF-2, the NF2 gene product functions as a reces-
sive tumor suppressor. This protein is believed to
be crucial in regulating leptomeningeal prolifera-
tion. Although inactivation of both copies of the
NF2 gene is necessary for initiation of menin-
gioma formation, its role in malignant menin-
gioma progression is debated [94]. Some studies
375
376
have shown that NF-2 mutations occur with simi-
lar frequencies in WHO grade I, WHO grade II,
and WHO grade III meningiomas [103]. What
alternative mechanism is responsible for the ori-
gin of meningiomas that do not carry NF-2 muta-
tions and for atypical and anaplastic meningiomas
remains unclear. Some genetic aberrations, such
as deletion of the INK4a-ARF locus or altera-
tions of the protein 4.1B family, which belongs to
the same protein superfamily as merlin, might
play a role [94].
CP angle meningiomas are typically globular
dura-based tumors; very rarely the carpet-like or
“en plaque” form is encountered. With progres-
sive growth, the overlying meninges are pushed
by the tumor, thus creating an arachnoidal mar-
gin, which serves as a natural cleavage plane dur-
ing surgery.
The current World Health Organization (WHO)
2000 classification distinguishes between WHO
grade I, atypical WHO grade II, and anaplastic
(malignant) WHO grade III meningioma [53].
Within each grade, however, a wide variability of
tumor biological behavior exists, in terms of
growth rate, adhesion, or infiltration of adjacent
neurovascular structures; brain invasion; and risk
of recurrence (Fig. 7.1). Generally, meningiomas
are two types: encapsulated and infiltrating.
Encapsulated meningiomas may be removed
without or with minimal morbidity. Some menin-
giomas, however, tend to infiltrate the pial cover-
ing and even neural tissue. Microcystic, atypical,
and, especially, anaplastic meningiomas tend to
have more extensive and irregular tumor–brain
interface [55]. Pial infiltration is not restricted to
the aforementioned tumor categories: it may occur
even in WHO grade I meningiomas. Brain inva-
sion does not necessarily indicate malignant
tumor, in particular in the absence of frank histo-
logical anaplasia [52, 78]. Grading of menin-
giomas is based on histopathological criteria,
which do not account for these individual differ-
ences [43]. Criteria for atypical (WHO grade II)
meningiomas are increased mitotic activity or
three or more of the following features: hypercel-
lularity, small cell transformation, macronucleoli,
uninterrupted patternless growth, and foci of
spontaneous or geographic necrosis [79]. Malignant
7 Meningiomas
progression of meningiomas, similar to that of
gliomas, is regarded as a continuum of increasing
atypia and mitotic activity. Anaplastic (WHO
grade III) meningiomas exhibit considerably more
malignant histologic features, compared to atypi-
cal ones [78].
In the future, meningioma grading will be cer-
tainly refined by inclusion of molecular and
genetic criteria, such as presence of genetic alter-
ations on chromosomes 1p, 10q, 14q, and 9p, and
telomerase activation [94].
Peritumoral edema, which is encountered in a
considerable number of patients, is not a feature
pertinent only to atypical or anaplastic menin-
giomas and occurs also in benign WHO grade I
tumors. The extent of edema, however, tends to
correlate with histological tumor grade. More
severe edema is seen in case of tumor transgres-
sion of the arachnoid plane or brain invasion.
Tumor size has also been linked to the develop-
ment of edema, but our experience does not
confirm that completely. Although the precise
pathogenesis of edema is not known, important
role seems to play the extravasation of edemo-
genic cytokines, particularly of VEGF (vascular
endothelial growth factor), which causes vasogenic
edema and neoangiogenesis into brain paren-
chyma [64].
Classification
Meningiomas, arising from the dura of the poste-
rior surface of the pyramid lateral to the trigemi-
nal nerve, are defined as “CP angle meningiomas”
and those arising medial to the trigeminal nerve
as “petroclival meningiomas” [86]. We consider
those meningiomas that arise from adjacent areas
but have their main bulk in the CP angle also as
CP angle meningiomas. These tumors are further
divided into five subgroups (Figs. 7.2, 7.3, 7.4,
7.5, 7.6, and 7.7): premeatal, centered at the IAC,
suprameatal, inframeatal, and retromeatal
[66, 67]. Although the main tumor occupies the
CP angle cistern, any of these meningiomas can
grow secondarily into the IAC. Meningiomas,
arising within the IAC, though very rare, comprise
a separate subgroup. Accurate determination of
Cerebellopontine Angle Meningiomas
a b
Fig. 7.1 Large sphenopetroclival meningioma in a
38-year-old patient. Considering the extension of the
tumor (a), the degree of brain stem compression, and the
neurological status (absence of visual symptoms), a deci-
sion was taken for tumor removal in two steps. Initially,
the infratentorial tumor part was removed via the retrosig-
moid suprameatal approach. The pathological examina-
tion revealed that it is a transitional meningioma (WHO
grade 1); Ki 67 was 2 %. The early postoperative MRI (b)
377
demonstrated the extent of tumor removal and brain stem
decompression. Second surgery—removal of the extra-
cavernous tumor part via a frontotemporal craniotomy—
was scheduled 6 months later. In 5 months, new MRI was
performed: unexpectedly, substantial growth of the
infratentorial part of the tumor was visualized (c). The
patient is to be operated within 1 week and is planned for
postoperative radiotherapy
378 7 Meningiomas

a b

Fig. 7.2 Premeatal meningiomas. (a) T1-weighted MR sequence after contrast administration. (b) CISS sequence

a b

Fig. 7.3 Retromeatal meningiomas. (a) T1-weighted MR sequence after contrast administration. (b) CISS sequence

a b

Fig. 7.4 Suprameatal meningiomas. (a) T1-weighted MR sequence after contrast administration. (b) CISS sequence
Cerebellopontine Angle Meningiomas 379

a b

Fig. 7.5 Inframeatal meningiomas. (a) T1-weighted MR sequence after contrast administration. (b) CISS sequence

a b

Fig. 7.6 Meningiomas centered at the IAC. (a) T1-weighted MR sequence after contrast administration. (b) CISS
sequence

a b

Fig. 7.7 Petroclival meningiomas. (a, b) T1-weighted MR sequences after contrast administration
380
the site of origin, however, is possible only in
small tumors. Large tumors may spread more
widely and occupy several adjacent areas. The
site of origin in such cases might be inferred from
the location of the so-called tumor matrix or dural
attachment. Occasionally it is marked by a small
hyperostosis. In most patients, the blood supply
to the tumor comes primarily through this attach-
ment site.
Pure clival meningiomas are rare; they origi-
nate from the lower, middle, or upper clivus and
are regarded as a separate subtype of the petro-
clival group (Fig. 7.8).
This classification has surgical and prognostic
significance: the more medially located tumors
are related to greater surgical complexity and
poorer outcome. As presented in a previously
published evaluation of the senior author’s expe-
rience with CP angle meningiomas [66], a
specific pattern of dislocation of the seventh-
eighth cranial nerves is observed in each of the
subgroups:
Premeatal meningiomas (originating between
the IAC and the trigeminal nerve):
• seventh and eighth nerves are displaced most
frequently posteriorly (45 %) and inferiorly
(43 %); rarely—superiorly (10 %).
• fifth nerve is displaced primarily superiorly
(59 %), anteriorly (26 %), or inferiorly
(14 %).
Meningiomas centered at the IAC:
• seventh and eighth nerves are surrounded
by the tumor in 29 %; displaced superiorly
or inferiorly (25 % each). Rarely they are
displaced posteriorly (12 %) or anteriorly
(8 %).
Suprameatal meningiomas:
• seventh and eighth nerves are displaced inferi-
orly in 78 % and anteriorly in 17 %.
Inframeatal meningiomas:
• seventh and eighth nerves are displaced supe-
riorly in 69 % and anterior in 19 %.
Retromeatal meningiomas (originate between
the IAC and sigmoid sinus):
• seventh and eighth nerves are displaced ante-
riorly in 62 % and inferiorly in 25 %.
7 Meningiomas
The extension patterns of larger CP angle
meningiomas can be generally classified as:
• Retromeatal meningiomas:
– Extension into the IAC
– No extension into the IAC
• Premeatal meningiomas
– Medial extension to petrous apex
– Medial and superior extension
• Extension into the Meckel’s cave
• No extension into the Meckel’s cave
– Medial and inferior extension
• Extension into the jugular foramen
• No extension into the jugular foramen
• Extension to the foramen magnum
• No extension to the foramen magnum
– Extension into the IAC
– No extension into the IAC
• Large meningiomas located pre- and ret-
romeatal with all extension patterns described
above
Neuroimaging
CP meningiomas display more variable features
than VSs in regard to tumor type and grade, ori-
gin, location, and extension pattern. Preoperative
imaging, besides the information on tumor type
and extension, should provide information on its
relations to the important surrounding neurovas-
cular and bone structures, which is essential for
the surgical planning. The neuroradiological
examination should include at least thin-slice
bone-window CT, native MRI, and MRI follow-
ing contrast administration. In case the surgery is
supposed to be performed in the semi-sitting
position, dynamic X-ray of the cervical spine is
also mandatory.
On CT, meningiomas appear generally as
well-circumscribed, encapsulated tumors. Most
demonstrate some degree of hyperdensity with
respect to surrounding gray matter. Meningiomas
are strongly and uniformly enhancing tumors.
Up to 20–25 % may show evidence of intratu-
moral calcification, occurring in a punctate, rim-
like, or nodular pattern or even as dense
Cerebellopontine Angle Meningiomas 381

a b

Fig. 7.8 Clival meningioma, arising from the middle third facial, and lower cranial nerves are obvious. (d) Complete
of clivus. (a, b) Preoperative MR images. (c) Initial view tumor removal with coagulation of its attachment at the
of the tumor, via a retrosigmoid perspective. The compres- clivus. (e) Postoperative CT scan (Reprinted from:
sion and dislocation of the trigeminal, vestibulocochlear/ Nakamura and Samii [70])
382
e calcification. Calcifications appear hypointense
Fig 7.8 (continued)
calcification of the entire tumor (Fig. 7.9). Bone
alterations, best visible on high-resolution bone-
window CT series, may be caused by actual
tumor infiltration or erosion from direct pres-
sure or are reactive bony changes. These bone
changes contribute to the discrimination of men-
ingiomas from other tumors (Fig. 7.10). VSs
may cause dilatation of the IAC and adjacent
bone erosion but do not produce hyperostosis
and almost never calcify. Chordomas and chon-
drosarcomas display different patterns of bone
destruction.
MRI is currently regarded as the most sensi-
tive modality for detection and characterization
of meningiomas. The ability to obtain images and
visualize the tumor and its surroundings in mul-
tiple planes is important for operative planning.
On T1-weighted MRI studies, meningiomas are
typically isointense to slightly hypointense with
respect to brain [109]. On T2-weighted MRI
studies, they demonstrate greater signal variabil-
ity: more than half are isointense, 35–40 % are
hyperintense, and less than 10 % are hypointense.
Signal heterogeneity on T2-weighted sequences,
which is not uncommon, is due to cystic tumor
parts, necrosis, previous hemorrhages, or areas of
7 Meningiomas
on all sequences. Contrast enhancement is usu-
ally intense and homogeneous. Areas of central
necrosis, which are caused by rapid tumor growth,
are hypointense on T1 and hyperintense on T2
sequences. The extent of meningioma growth
into IAC is best evaluated on axial and/or coronal
T2-weighted and CISS sequences: the presence/
absence of high signal, corresponding to CSF,
lateral to the tumor indicates its lateral extension.
Notably, radiological information may be mis-
leading, and the actual relations may become
obvious only at surgery.
CP meningiomas can be usually reliably dif-
ferentiated from VSs on preoperative images
[13, 105]. Meningiomas have broad contact to
the petrous bone or tentorium; most are centered
away from the IAC; the angle between the tumor
and the pyramid is obtuse; the IAC is not wid-
ened (Fig. 7.11). Although secondary invasion
of the IAC might be observed in 10–22 %, pri-
marily meningiomas of the IAC are very rare
[83, 101]. In contrast, VSs are centered at the
widened IAC, extend almost always into the
IAC, form an acute angle with the posterior sur-
face of the pyramid, and cause different patterns
of bone changes. On T2-weighted sequence,
meningiomas have higher intensity than VSs.
The dural “tail” sign, although not pathogno-
monic, is strongly suggestive of meningioma
(Fig. 7.12). This dural reaction is due to actual
tumor extension (wedge-shaped tumor “tongue”)
but can also be caused by nonneoplastic reac-
tive change, tissue proliferation, or hypervascu-
larity. Other tumors that may have a dural “tail”
are dura-based metastases, lymphomas, sarcoi-
dosis, solitary fibrous tumors, and hemangio-
pericytomas [102].
The possibility to predict accurately tumor type
and grade, tumor consistency (firm or soft menin-
giomas), tumor vascularization, and tumor relation
to the arachnoid (clear arachnoid plane or invasion)
would greatly benefit the decision-making process.
It would allow for more precise preoperative plan-
ning and patient counseling. Although some radio-
logical factors have been shown to have correlation
to the meningiomas’ biological behavior, current
Cerebellopontine Angle Meningiomas 383

a b

c d

Fig. 7.9 Petroclival meningioma, causing reactive bony hyperostosis in the area anterior to the IAC (arrow). (a) Bone-
window CT scan. (b, c) T1-weighted MR images after contrast administration. (d) FLAIR MR image

neuroimaging modalities provide inconclusive
data. Malignant meningiomas tend to have more
frequently increased signal intensity on both T1-
and T2-weighted MR scans than do benign ones.
The presence of irregular tumor projections into
the brain surface, the so-called mushrooming, and
the presence of indistinct margins are further
factors that might rise the suspicion of malignancy
[2] (Fig. 7.13). Peritumoral edema tends to corre-
late well but not always to the loss of a patent
arachnoid plane (See Case Illustration 7.1) [15, 32,
92, 96]. Atypical and anaplastic meningiomas that
invade the pia often provoke considerable cerebral
edema [54, 96]. Some studies showed that edema
is more common in association with meningiomas
having increased MIB-1 labeling indices [40].
Edema appears hypointense on T1-weighted
images and hyperintense on T2-weighted sequences
and is associated with peripheral (or rim) enhance-
ment on fast FLAIR sequences [73].
384 7 Meningiomas

a b

e
f

Fig. 7.10 CT (a–c) and MR images (d–h) of an unusual nal, and sagittal bone-window CT. (d) T1-weighted native
petroclival meningioma with involvement of the cavern- and (e, f) contrast-enhanced MR sequences. (g, h)
ous sinus and retrosellar growth. The dense calcification T2-weighted sequences
of the tumor is the clue to its diagnosis. (a–c) Axial, coro-
Cerebellopontine Angle Meningiomas 385

g h

Fig 7.10 (continued)

a b

Fig. 7.11 Meningioma with

secondary intracanalicular extension.
(a, b) bone-window CT, demonstrat-
ing the IAC on the left and right
sides. Both sides should be compared
always to exclude—as in this
case—an anatomically wider medial
portion of the canal. (c, e) MRI
examination: T1-weighted native
(c), T1-weighted with contrast
administration (d), and T2-CISS
(e) MR sequences. The extent of
intracanalicular tumor growth is best
appreciated on the CISS sequence,
showing both the tumor in the
medial part of the canal and the
CSF lateral to it
386 7 Meningiomas

c d

Fig 7.11 (continued)

Fig. 7.12 The dura “tail” sign (arrows), although not

pathognomonic, is strongly suggestive of meningioma
Cerebellopontine Angle Meningiomas 387

a b

c d

Fig. 7.13 MRI study of a 22-year-old patient with a giant
CP angle meningioma (a–c) T1-weighted sequence after
contrast administration. (d) T2-weighted sequence. Note
the mushrooming effect: irregular tumor projections into
the brain surface and the intense peritumoral edema. The
patient was operated at another facility, but only partial
removal was feasible due to its high vascularization and
absence of arachnoid dissection plane. The patient is
scheduled for surgical treatment via the RS approach at
the INI
388 7 Meningiomas

Case Illustration 7.1
Petroclival Meningioma with Perifocal
Edema
Sixty-five-year-old female patient with recur-
rent headache, progressive gait instability,
hemifacial hypesthesia, and decreased hearing
on the left side. The MRI showed a large sphe-
nopetroclival meningioma on the left side with
supratentorial extension and involvement of
posterior cavernous sinus (a–f). Two fea-
tures—the unclear border to surrounding
structures and the perifocal edema—indicated
a possible infiltration of the brain stem. This
possibility was discussed with the patient, and
a decision was taken that if the tumor would
turn out to be infiltrative, only a subtotal resec-
tion would be performed. The tumor was
exposed via the RS approach and partially
resected. It was indeed infiltrative in regard to
cranial nerves and brain stem. Multiple ves-
sels were identified passing through the men-
ingioma and supplying ultimately the brain
stem. An attempt to proceed with tumor dis-
section would inevitably cause severe iatro-
genic damage. Hence, once decompression of
the brain stem was achieved, no attempt was

a b

c d

Preoperative MRI of an infiltrative sphenopetroclival sequence. Note the brain stem edema, which is best
meningioma. (a–d) T1-weighted sequence after con- visualized on the T2-weighted and FLAIR sequences,
trast administration. (e) T-2 sequence. (f) FLAIR and the unclear border to surrounding structures
Cerebellopontine Angle Meningiomas 389

e f

made to dissect the tumor capsule. MRI was recommended in 6 months: in case
Histopathological diagnosis was meningothe- the remnant would have growth tendency,
lial meningioma (WHO grade I). Follow-up fractionated radiotherapy would be necessary.
390
Generally, T1-weighted signal characteris-
tics do not correlate with either histopathologi-
cal findings or tumor consistency [28]. Higher
signal on T2-weighted images may indicate a
softer tumor consistency. Lower signal suggests
harder tumor consistency; it is associated with
calcifications and fibrous tissue [28, 107]. The
very important from surgical standpoint cleav-
age plane or distinct interface between tumor
and adjacent brain may be visualized as a CSF
“cleft” around the tumor periphery. It has typi-
cal CSF signal characteristics: hypointensity
on T1-weighted images and hyperintensity on
T2-weighted images.
DWI might prove helpful in the differentiation
of the pathological grading of meningiomas:
some data indicate that atypical or malignant
meningiomas tend to have lower ADCs (apparent
diffusion coefficient) than the benign ones [30].
MR spectroscopy finding of a combined elevation
of glutamate/glutamine and presence of alanine at
1.5 ppm are specific for meningiomas [18].
MR angiography and MR venography demon-
strate the degree of tumor vascularity and allow
assessing the patency and dominance of major
venous sinuses. In our experience, digital subtrac-
tion angiography (DSA) as a diagnostic and/or
planning tool is required in exceptional cases, such
as giant tumors with wide extension and involve-
ment of major vessels. Vascular supply to CP angle
meningiomas is from branches of the ascending
pharyngeal artery, occipital artery, vertebral artery,
AICA (subarcuate artery), and middle meningeal
artery. Large tumors may be supplied additionally
by direct branches of the AICA and the superior
cerebellar artery. Preoperative embolization of
tumor-feeding arteries may be helpful in highly
vascular meningiomas. Ideally, it renders the men-
ingioma smaller, softer, and less bloody, which,
ultimately, allows for safer removal. Most menin-
giomas, however, do not need to be embolized
because adequate blood control can be obtained
intraoperatively.
7 Meningiomas
Clinical Presentation
The clinical presentation of patients with CP
angle meningiomas depends mainly on the size,
site of origin, and extension of the tumor. They
cannot be distinguished from other CP angle
tumors based on their clinical presentation
alone. Some clinical clues, however, should
alert the clinician that the lesion may be other
than VS [56].
Neurological symptomatology is due to:
• Involvement of the cranial nerves and dura
• Mass effect on the cerebellum
• Brain stem compression
• Intracranial hypertension, caused by the tumor
mass or due to obstructive hydrocephalus
The onset of clinical symptoms is usually
insidious, and the mean duration of symptoms
before hospital admission is 12–31 months
[10, 56]. At the time of diagnosis, the most fre-
quent symptoms, observed in 50–78 % of the
cases, are otologic: impaired hearing (41–68 %),
dizziness (20–31 %), and tinnitus (12–22 %)
[10, 21, 58, 91, 98, 101]. Hearing loss is of sud-
den onset in 9–14 %. Unspecific headache as ini-
tial complaint is estimated to appear in 15–24 %.
At presentation, trigeminal nerve sensory
deficit (facial numbness or a decreased corneal
reflex) is seen in 13–49 % of the patients. Up to
14 % may present with trigeminal neuralgia.
Two percent of all cases of trigeminal neuralgia
are due to tumor compression at the root entry
zone of the trigeminal nerve, and the tumors
causing such compression most frequently are
meningiomas (see Case Illustration 7.2). Hear-
ing loss has 63–83 % of all patients [9, 14, 101];
this rate is lower than in patients with VSs,
where it is found in the majority of the cases.
Preoperative facial nerve paresis is a rare sign,
observed in 6–8 % of the patients [66, 101].
Lower cranial nerve deficits can occur in case of
caudal tumor extension and are seen in 6–21 %
of all patients [10].
Cerebellopontine Angle Meningiomas
Case Illustration 7.2
Small Premeatal Meningioma Causing
Trigeminal Neuralgia
The patient suffered from symptomatic trigem-
inal neuralgia for approximately 18 months.
Neurological examination revealed hypesthesia
in all branches of the fifth nerve and decreased
corneal reflex on the left side. She was operated
via the standard RS approach. In this case, the
petrous vein had to be coagulated in order to
expose the tumor sufficiently. The meningioma
a b
(a–c) Small meningioma arising from the posterior
petrous dura, immediately lateral to the trigeminal
nerve. The compressive effect of the tumor is well vis-
ible, especially on the CISS sequence (c). Note the
391
could be successfully removed without endan-
gering the surrounding cranial nerves. In order
to decrease the risk of recurrences, the hyper-
ostotic bone and the suprameatal tubercle were
drilled off. Fat pieces were used to prevent
occurrence of CSF leak. Histopathological
finding was a psammomatous meningioma
WHO grade I. After the surgery, the patient
did not have more neuralgic attacks; however,
the hypesthesia persisted longer and started to
resolve only after 6–7 months.
hyperostosis in the area of tumor attachment. (a) Bone-
window CT scan. (b) T1-weighted MR sequence.
(c) CISS sequence
392
Cerebellar dysfunction, mainly gait ataxia, is
found in 25–52 % of all patients with CP angle
meningiomas. Symptoms and signs secondary to
brain stem compression with obstructive hydro-
cephalus occur in late stages of the disease. Signs
of increased intracranial pressure have 16–29 %,
and hydrocephalus have 20–31 % of the patients
in different series [51, 56]. In case of very large
or giant meningiomas, these symptoms are invari-
ably seen.
The origin and location of meningiomas
determine their clinical presentation. Premeatal
and retromeatal meningiomas have distinctive
clinical development and symptomatology
[66, 86, 89, 90]. Premeatal tumors are diag-
nosed earlier and consequently have smaller
size. Their clinical presentation is related to
cranial nerve dysfunction (such as hearing loss,
trigeminal neuralgia, or hypesthesia) and facial
and/or abducens nerve palsy. Retromeatal men-
ingiomas are generally larger; the predominant
symptoms are due to cerebellar compression.
Meningiomas, originating above the IAC, most
often present with facial pain or other sensory
symptoms before developing eighth cranial
nerve symptoms. The clinical presentation of
meningiomas, originating at or near to the IAC,
is similar to that of patients with VSs: hearing
loss, tinnitus, and disequilibrium. Patients with
large meningiomas that occupy the whole CP
angle present with a combination of these
symptoms.
Clinical Examinations
All patients should undergo hearing examination,
including audiogram and evaluation of speech
discrimination. Neuro-ophthalmologic examina-
tion is performed in patients with oculomotor
nerve deficits. In case of more caudal tumor
growth and preoperative swallowing or speech
deficits, an ENT examination with direct laryn-
goscopy allows estimating the function of the
lower cranial nerves. Tumors extending supraten-
torially might compress the pituitary stalk or
extend into the sella. In such cases, pituitary axis
evaluation and—if needed—endocrinologic
treatment preoperatively are indicated.
7 Meningiomas
Management
Perioperative Management
Patients with extensive perifocal edema receive
dexamethasone for 3 days prior to surgery (12–
24 mg/day). Beneficial effect—decrease in capil-
lary permeability—is manifested within the first
hours after its administration [93]. Dexamethasone
after the surgery is given to all patients in similar
dosage—12–24 mg/day—for at least 3 days.
Prophylaxis with broad-spectrum antibiotic (usu-
ally cefazolin) is administered as a single preop-
erative dose and readministered every 6 h during
the procedure. Prophylactic antibiotics are dis-
continued within 24 h. If the risk of infection is
higher, for example, due to local or systemic rea-
sons, prolonged application or more potent anti-
biotics may be given.
The risk of venous thromboembolism in patients
operated for intracranial meningiomas is elevated
due to the existing neurological deficits, the related
immobility of patients and/or the prolonged immo-
bilization during or after surgery. Furthermore,
release of procoagulants and fibrinolysis-inhibiting
factors by the tumor may play a role in the devel-
opment of this procoagulant state [88]. All patients
wear antithrombotic (compression) stockings dur-
ing the postoperative hospital stay. Intermittent
compression devices are utilized in high-risk
patients or in those with contraindications for hep-
arin prophylaxis. All patients receive subcutane-
ous low molecular weight heparin prophylaxis
since the day after surgery, provided the postopera-
tive CT shows no signs of intracranial hemorrhage.
Following surgery, patients are mobilized early;
passive range-of-motion exercises are initiated
early in those with neurological deficits.
Management
The treatment options of CP angle meningiomas
are observation, surgery, radiotherapy/radiosur-
gery, or combination thereof. The decision in
each case should be taken individually, consid-
ering patient’s biological age, expectations, and
activities; his general and neurological status; as
well as tumor size and extension. Meningiomas
are increasingly detected early, during their
Cerebellopontine Angle Meningiomas
“preclinical” stage due to the wide availability
of MRI facilities. Furthermore, MRI is a reliable
method to monitor tumor growth. Initial follow-
up and surgery only in case the meningioma
shows growth potential or new symptoms appear
are frequently recommended. On the other hand,
earlier active management is completely justified
because the outcome of surgery is related to
tumor size and is optimal in neurologically intact
patients. We recommend an initial period of clin-
ical and radiological observation to determine
whether the symptoms are progressive and/or the
tumor is enlarging in elderly or somatic unstable
patients with small tumors and mild stable symp-
toms or in those unwilling to undergo surgery.
Repeat scans should be performed in 6-month
intervals or—if new symptoms appear—earlier.
The optimal treatment of CP meningiomas,
which potentially leads to cure of the patient, is
their complete operative tumor removal. This
goal, however, should not be strived at the expense
of new neurological dysfunction and/or deterio-
ration of the patient’s quality of life.
Different surgical routes are used to approach
CP angle meningiomas: retrosigmoid, extended
retrosigmoid, presigmoid, translabyrinthine,
transcochlear, transpetrosal, far lateral, modified
far lateral, as well as routes through the middle
cranial fossa [6, 11, 21, 65, 73, 91, 98]. The gen-
eral concepts that are important for the successful
removal of most meningiomas, however, are simi-
lar: adequate exposure, interruption of the blood
supply along the dural attachment, internal decom-
pression, and cautious dissection of the tumor cap-
sule from brain stem and cranial nerves in the
arachnoid plane. Due to the progress in neuroanes-
thesia, critical care, and microsurgery, as well as
the introduction of modern skull-base approaches,
the morbidity and mortality related to the surgical
treatment of meningiomas decreased considerably
over the last decades.
In the early ages of the skull base surgery, an
extensive bony exposure allowing complete visual-
ization of the meningioma and all adjacent neural
and vascular structures was considered as a major
prerequisite for complete and safe tumor removal.
The approaches, providing such exposure, however,
393
are related to higher risks of neurological morbid-
ity or operative complications, such as facial nerve
palsy, hearing loss, and CSF leak. Moreover, with
these techniques, a higher risk of venous-related
complications, such as interruption or thrombosis
of major draining veins or sinuses, exists. The
transverse and sigmoid sinuses may be injured dur-
ing their exposure or may desiccate if skeletonized
completely, which increases the risk of thrombosis
with consequent parenchymal swelling or hemor-
rhage. Even their prolonged retraction with the
dura can cause partial or complete interruption of
venous flow, leading to venous congestion or
thrombosis.
Another important issue is the possibility to
preserve hearing. Some authors suggest that hear-
ing preservation should be attempted only in ret-
romeatal meningiomas [89]. Hearing destructing
approaches through the pyramid have been pro-
posed for patients with severe hearing deficit or
premeatal meningiomas. Our experience has
shown, however, that in CP angle meningiomas,
contrary to VSs, hearing loss is usually due to
compression of the cochlear nerve. Following
tumor removal, hearing may improve even in large
tumors. Such improvement has been observed and
reported also by other authors [59, 66, 99].
Having extensive experience with all skull-
base techniques, the senior author’s current con-
cept is that simpler and safer approaches are the
prerequisite for improving outcome and reducing
morbidity. The retrosigmoid suboccipital approach
(RS approach) offers an excellent panoramic visu-
alization of the whole CP angle, wide exposure of
the tumor, whatever its size, and increased safety
when working in the vicinity of the brain stem. It
is related to a very low procedure-related morbid-
ity rate and allows hearing preservation. In case of
pure CP meningiomas, the RS approach is our
favorite technique. In CP meningiomas with
medial extension toward the petroclival area and/
or the Meckel’s cave, we utilize the retrosigmoid
suprameatal approach (Samii’s technique), intro-
duced by the senior author in 1982.
All CP angle meningiomas, regardless of their
location, can display infiltrative or adhesive char-
acteristics. The lack of arachnoid plane, however,
394
a b
Fig. 7.14 MR images of two patients with large CP angle
meningiomas, causing hydrocephalus. The hydrocepha-
lus-related signs/symptoms resolved completely follow-
becomes critical in case of large premeatal or pet-
roclival meningiomas. The attempt to dissect
their capsule may damage the pial vascular sup-
ply or cause direct damage to the brain stem. In
such cases, subtotal tumor removal is justified in
order to avoid major neurological morbidity.
Management of Hydrocephalus
in Patients with CP Angle Meningiomas
Large meningiomas of the CP angle may occlude
the outlets of the fourth ventricle and cause
obstructive hydrocephalus (Fig. 7.14). In contrast
to VSs, meningiomas typically do not cause
impairment of the CSF absorption. Once the
obstruction is removed, the normal flow of the
CSF is reestablished and the hydrocephalus is
resolved. In our practice, VP shunting is very
rarely needed after radical tumor removal.
In patients presenting with severe hydrocepha-
lus-related symptoms, an external ventricular drain-
age (EVD) is inserted. Following the tumor surgery,
stepwise weaning from the EVD is performed, as
described in the section on VS-related hydrocepha-
lus. A VP shunt is inserted only in case the weaning
is unsuccessful. In patients with less severe preop-
erative hydrocephalus symptoms, the tumor is
removed primarily, and the patient is monitored
closely. In case neurological deterioration appears,
7 Meningiomas
ing tumor removal in both cases. The dilatation of the
ventricular system, however, persisted for more than 6
months
which can be attributed to further dilatation of the
ventricles, an EVD is placed. Once the patients’
condition stabilizes, the weaning program is initi-
ated, and a decision on the necessity of a VP shunt
is taken according to its success.
General Operative Technique
Similar to the technique used in case of VSs, the
meningioma is removed by alternating internal
debulking and dissecting the capsule from sur-
rounding structures in the arachnoid plane. Initial
internal decompression or debulking of the tumor
is performed with an ultrasonic surgical aspirator,
suction, or platelet knife. Thereafter, the tumor
capsule is dissected in the arachnoid plane from
the neural and vascular structures.
Retrosigmoid Approach
The main steps of the RS approach in case of CP
angle meningiomas are similar to those in case of
VSs and are described in detail in the chapter on
RS approach. They will be therefore only sys-
temized here:
• The patient is in semi-sitting position.
• Continuous neurophysiological monitoring is
performed throughout the surgery: from the
patient positioning to the skin closure. SSEP,
facial nerve EMG, BAEP, and recently facial
Cerebellopontine Angle Meningiomas
nerve motor-evoked potential are monitored
routinely, while monitoring of the oculomotor
and of the lower cranial nerves is performed if
needed—according to the particular tumor
extension and clinical presentation. During
tumor removal, nerve stimulation is used to
identify the facial nerve.
• The head is flexed and rotated approximately
30° to the involved side. Importantly, occlu-
sion of venous jugular outflow or hyperflexion
of the cervical spine should be avoided. During
patient positioning, SSEP are continuously
monitored and provide feedback information
on the spinal cord function.
• The approach should be planned and per-
formed so that damage to venous sinuses is
avoided. Venous bleeding should be immedi-
ately controlled by bipolar coagulation or with
hemostatic sponges in order to avoid air
entrapment.
• Avoidance of clinically significant air embo-
lism relies on continuous anesthesiological
monitoring (end-tidal carbon dioxide and pre-
cordial Doppler echocardiography) and care-
ful operative technique (preventing of
embolism and immediate control if it occurs).
Good coordination between the anesthesiolo-
gist and the surgeon is essential for avoiding
air embolism-related morbidity. The surgeon
should act systematically to detect and obliter-
ate the source of air entry at the first sign of
venous air embolism.
• A slightly curved skin incision 2.5–3.5 cm
medial to the mastoid process is made.
• The craniectomy should expose just the edges
of the sigmoid and transverse sinuses.
Caudally, the floor of the posterior fossa
should be reached to allow unrestricted access
to the lateral cerebellomedullary cistern. The
mediolateral diameter of the opening is
3.5–4.5 cm.
• Excessive traction to the mastoid emissary
vein/veins may cause sinus laceration. The
vein should be skeletonized with a diamond
drill until it is free of any bony encasement
and can be safely coagulated.
• The dura is incised in a curvilinear manner
1.5–2 mm medial to the sigmoid and inferior
to the transverse sinus.
395
• The lateral cerebellomedullary cistern is then
opened, and sufficient CSF is allowed to
egress.
• Every attempt should be made to preserve the
integrity of petrous or other main draining veins.
The wide variability of the venous system of the
CP angle does not allow predicting the potential
consequences of their interruption [44].
Individual tumor characteristics, such as size
and extension, determine the subsequent steps:
• Generally, the meningioma is removed by
alternating internal debulking and dissecting
the capsule from the surrounding structures.
• The cranial nerves are identified near their
entrance or exit in bone or dura and at the
brain stem. Their dissection is performed by
carefully stripping off the arachnoid from the
tumor with one hand. Simultaneously, a slight
traction is done with the second hand.
• Dissection is started only after sufficient inter-
nal decompression has been achieved. It is
performed always in the arachnoid plane.
Once sufficient internal decompression has
been achieved at one of the CP angle levels,
the surgeon moves to the next one.
• Only the feeding arteries or major bleeding
vessels are coagulated during tumor removal.
The continuous irrigation of the operative
field performed by the assistant obviates the
need for more frequent coagulation. Coagu-
lation is avoided in the vicinity of the cranial
nerves.
• Targeted opening of the IAC, depending on
the degree of intracanalicular tumor extension
and surgical goal, is performed. Violation to
the inner ear structures should be avoided.
• The dural origin of the tumor should be excised
or—if excision is not possible—coagulated
meticulously. High-speed drill is used to
remove any bone hyperostosis, which may
contain viable tumor cells.
• Since the dural tail probably indicates tumor
extension (at least in about 2/3 of the cases), it
should be resected, if possible, to reduce the
risk of tumor recurrence.
• The drilled area of the pyramid as well as any
opened mastoid air cells is sealed with multi-
ple fat pieces and fibrin glue.
• Watertight dura closure.
396
a b
c
Fig. 7.15 Lateral retromeatal meningioma. Note the
broad base to the posterior petrous bone and to the tento-
rium. The tumor is adjacent to the dominant sigmoid
sinus, which has to be considered during surgery. (a–c)
Operative Technique
in Different CP Angle Meningiomas
Retromeatal meningiomas arise from the poste-
rior petrous bone dura between the IAC and the
sigmoid sinus. According to the senior authors’
experience, most of them originate posterior
and inferior to the IAC dorsal to the jugular
foramen. The size and exact site of origin of the
tumor determine their relation to the seventh-
eighth nerve complex and the operative com-
plexity. Small more lateral meningiomas are
located away from the nerves, and their removal
7 Meningiomas
d
Axial, coronal, and sagittal T1-weighted MR images after
contrast application. (d) CISS sequence—the facial and
vestibulocochlear nerves are well visible both in their cis-
ternal and intracanalicular parts
is straightforward (Fig. 7.15). Important issue
to consider is the possibility of sigmoid sinus
involvement, especially on the side of the domi-
nant sinus. In case the tumor invades the wall of
a dominant sinus, complete removal should be
attempted only if its safe reconstruction is pos-
sible. If a large part of the sinus is involved,
small layer of the tumor may be left over the
sinus.
MRI, especially CISS and T2-weighted
sequences, may provide information on the
relation of these nerves to the tumor. Often, a
Cerebellopontine Angle Meningiomas
a b
Fig. 7.16 Retromeatal meningioma. (a, b) T1-weighted
MR image after gadolinium application of a meningioma,
arising posterior to the IAC. The broad base to the dura,
the small dural tail sign (medial), and the homogeneous
well-defined arachnoid layer separates them
(Fig. 7.16).
Large or more medially located meningiomas
have more intimate relationship to the seventh
and eighth cranial nerves and usually displace
them anteriorly (63 %) or inferiorly (25 %). The
nerves are encountered in later stages of tumor
removal, which has to be kept in mind during
surgery. Penetration of the anterior capsule
397
strong contrast enhancement are obvious. (c) On
T2-weighted sequences, the arachnoid layer, separating
the tumor from the seven to eighth nerve complex, can be
appreciated
during tumor debulking should be avoided not to
injure the cranial nerves. Notably, patients with
large or giant meningiomas, which caused pro-
nounced cerebellar compression over long
period, should be very carefully monitored after
surgery: hematomas tend to accumulate in tumor
bed or in cerebellar parenchyma, despite how
thorough the hemostasis had been (see Case
Illustration 7.3).
398
Case Illustration 7.3
Large Retromeatal Meningioma
Seventy-year-old male patient presented with
a history of increasing gait instability and
decreased hearing on the right side since more
than a year. Later on, constant nausea and
frequent vomiting appeared. He was treated
for Ménière’s disease for several months;
only when the therapy turned out to be com-
pletely futile, MRI examination was per-
formed. At presentation, he had very severe
gait and truncal ataxia, so that he had to use
wheelchair. His cranial nerve function was
normal besides the profound right-sided hear-
ing loss. MRI showed a very large retromeatal
meningioma with broad attachment to the
posterior petrosal surface and tentorium (a,
b). The tumor caused pronounced compres-
sion of the cerebellum; however, no signs of
edema were seen.
The patient was operated via the RS
approach (c–f), and complete removal of the
tumor, which was highly vascularized, could
a b
(a, b) Large retromeatal meningioma with a broad base
to the posterior pyramid surface and the tentorium. The
cerebellum is compressed, but the fourth ventricle is not
occluded. (c, d) The images present the steps of tumor
removal. (c) Initial exposure in the CP angle. (d) Internal
debulking with CUSA and suction. (e) Dissection from
the adjacent structures. (f) Electrical stimulation of the
facial nerve to verify its function after complete tumor
removal (the arrow points at the electrode probe). All
7 Meningiomas
be achieved. The facial and cochlear nerves
were dislocated anteriorly. An arachnoid layer
separated them from the tumor capsule, which
allowed for their safe dissection and structural
preservation. The tumor originated from the
dura between the IAC and the jugular fora-
men. In order to prevent recurrences, the dura
in the area was excised and the underlying
bone was drilled off with the diamond tip.
The patient did not have new neurological
deficits after surgery; the facial nerve function
was completely normal. Routine follow-up CT
performed the next day revealed an intracerebel-
lar hematoma on the right side (g). Considering
its relatively small size, a decision was taken for
conservative treatment. Follow-up CT scans
demonstrated the gradual resorption of the blood
(h, i). The histopathological diagnosis was
WHO grade I fibrous meningioma. At dis-
charge, the gait was significantly improved. At 2
years follow-up, the patient recovered com-
pletely. The late follow-up MRI examination
showed no signs of tumor recurrence (j–l).
neurovascular structures in the CP angle have been pre-
served. * fifth cranial nerve, ** seventh-eighth nerves,
*** lower cranial nerves. (g–h) Routine follow-up CT
revealed an intracerebellar hematoma on the right side.
Considering its relatively small size, a decision was
taken for conservative treatment. Follow-up CT scans
demonstrated the gradual resorption of the blood (i). (j,
k, l) T1-weighted MR imaging, performed 24 months
later: no signs of residual tumor or recurrence
Cerebellopontine Angle Meningiomas 399

c d

e f

g h
400 7 Meningiomas

i j

k l
Cerebellopontine Angle Meningiomas
Premeatal and Inframeatal Meningiomas
Premeatal meningiomas are located medial to the
IAC and most frequently displace the facial and
cochlear nerves posteriorly and inferiorly (in 45
and 43 %, respectively) [66]. Some premeatal
meningiomas expand more medially toward the
petroclival junction and may extend into the
Meckel’s cave or even supratentorially. Other
tumors extend caudally toward the jugular fora-
men and foramen magnum or laterally along the
posterior petrous surface. These meningiomas
may invade the IAC (Fig. 7.17) or remain entirely
in the CP angle cistern.
Similar to the technique used in petroclival
meningiomas, the surgeon has to approach the
tumor between the cranial nerves via one of the
CP angle levels, as viewed from a retrosigmoid
perspective: the upper level between tentorium
401
and trigeminal nerve, the second level between
trigeminal and seventh-eighth nerves, the third
level between seventh-eighth nerves and lower
cranial nerves, and the lowest level, defined by the
lower cranial nerves and foramen magnum. The
tumor is approached initially via the most
expanded level, for example, the level with the
widest distance between the respective structures
(see Case Illustration 7.4). Resection of the petrous
apex (the suprameatal extension of the retrosig-
moid approach—see the section “Petroclival
Meningiomas”) may be necessary to achieve
sufficient exposure of the tumor attachment
(Fig. 7.18). Inframeatal meningiomas extending
to the foramen jugulare or foramen magnum level
can be removed via a similar retrosigmiod craniec-
tomy with/without additional C1 hemilaminec-
tomy/laminectomy [85]; see Case Illustration 7.5.
402 7 Meningiomas

a b

c d

f
e

Fig. 7.17 Premeatal meningioma with limited growth enhanced (b, c). T2-weighted (d). T2 CISS (e). (f) MR
into the IAC: only the medial part of the canal is involved. cisternography. The seven to eighth nerve complex is dis-
T1-weighted native MRI (a). T1-weighted contrast- located laterally
Cerebellopontine Angle Meningiomas 403

a b

c d

Fig. 7.18 The complete removal of this premeatal men- (c)—from the brain stem to the Meckel’s cave. (d) Early
ingioma (a, b) required resection of the suprameatal postoperative CT control. (e) The patient at 6-month fol-
tubercle. Note the wide exposure of the trigeminal nerve low-up examination
404
Case Illustration 7.4
Premeatal Meningioma with Perifocal
Edema
This 55-year-old male patient was operated 5
years earlier in another neurosurgical facility
for a CP angle meningioma. The tumor was
removed partially, and the rest was followed
with regular MRI examinations. A growth
tendency was demonstrated, and 3 years later,
the tumor was treated with gamma knife. Over
the following 2 years, further tumor growth
was evident. At presentation to our depart-
ment, he had a hypesthesia on the right side of
the face (V1–V3), right-sided anacusis, and
severe gait difficulty. MRI demonstrated a
relatively large tumor with largest diameter of
3.5 cm with imaging characteristics, compati-
ble with those of a meningioma. The tumor
was located premeatally, had a small exten-
sion into the IAC, and caused significant com-
pression of the brain stem, as well as distortion
of the fourth ventricle (a, b). T2 sequences
demonstrated an intense peritumoral edema,
involving both the brain stem and cerebellum.
Although the ventricles were slightly dilated,
the patient had no symptoms/signs of intracra-
nial hypertension.
The meningioma was very vascularized
and had its attachment lateral and inferior to
the Meckel’s cave. Medially it extended up to
the oculomotor nerve. In order to remove the
part in the Meckel’s cave, the petrous apex
(the suprameatal tubercle) was drilled off. The
tumor capsule was found to be strongly adher-
ent to the trigeminal nerve fascicles. Still, it
could be dissected, and the integrity of the
7 Meningiomas
nerve could be preserved. Targeted opening of
the IAC was performed to remove the intrac-
analicular tumor part, while preserving the
facial and cochlear nerves. During the whole
surgery, the BAEP remained stable. The men-
ingioma was very adherent to the brain stem
but—despite the radiologically visualized
extensive peritumoral edema—did not invade
the pia. Thus, complete removal of the tumor
with preservation of all neurovascular struc-
tures was achieved. Histopathologically the
tumor was WHO grade I meningothelioma-
tous meningioma.
The CT examination performed several
hours after surgery because the patient was
drowsy showed blood collection in the tumor
bed area and severe compression of the
fourth ventricle (c). The dilation of the lat-
eral and third ventricles was more pro-
nounced when compared to the preoperative
CT scans, and a decision was taken in favor
of placing an EVD (d, e). Immediately there-
after, the patient regained his consciousness
completely. Over the following days, he
made rapid recovery and was successfully
weaned from the EVD, which was removed
on the eighth postoperative day. At discharge,
his gait was much more stable, but he had an
HB grade IV facial palsy. The patient was
seen 6 months later: his gait had recovered
almost completely, and the facial nerve func-
tion was HB grade III. MRI at 6 months
demonstrated no pathological finding (f).
Twelve months after the operation, he had
normal gait and very good facial nerve
function (HB grade II).
Cerebellopontine Angle Meningiomas 405

a b

c d

T1-weighted contrast-enhanced (a) and T2- weighted tumor bed area and almost complete occlusion of the
MR images of the tumor (b). Note the extensive peri- fourth ventricle. (d) Dilatation of the lateral ventricles;
focal edema on the T2 sequence. (c) CT performed (e) CT after the insertion of the EVD. (f) MRI per-
after the surgery, demonstrating blood collection in the formed 6 months later: no pathological findings
406 7 Meningiomas

e f
Cerebellopontine Angle Meningiomas 407

Case Illustration 7.5 he had severe gait ataxia, urine incontinence,

Inframeatal Meningioma
This 70-year-old male patient has been diag-
nosed to have a CP angle tumor 3 years prior
to presentation to our institute. He decided
against “early” surgery in favor of a wait-
and-see strategy. During the last several
months, however, he felt progressive gait
instability and headache. MR examination
demonstrated significant tumor growth with
obstructive hydrocephalus (a–f). At admission,
and complained of strong headache.
Audiometry revealed nonfunctional hearing
on the side of the tumor. Considering the
size of the lesion and the progressive wors-
ening of his condition, a decision for opera-
tive treatment was taken. Due to the presence
of hydrocephalus and the clinical signs of
intracranial hypertension, external ventricu-
lar drainage was inserted prior to tumor sur-
gery. The tumor was approached via the

a b

e f

Preoperative MRI, demonstrating a large premeatal meningioma, causing obstructive hydrocephalus. (a–c)
T1-weighted contrast-enhanced images. (d–f) CISS sequences
408
standard RS craniotomy, decompressed
internally, and dissected from the surround-
ing structure. It encased the vertebral artery
and dislocated the lower cranial nerves cau-
dally. Small tumor portions entered the jugu-
lar foramen and the IAC. Following removal
of the CP angle tumor part, the IAC was
opened, and the intracanalicular extension
was also resected. Then, the part in the jugu-
lar canal was approached, and complete
removal with preservation of all neurovascu-
lar structures was achieved. The tumor
matrix was inferior and medial to the IAC;
7 Meningiomas
hence, the dura in the area was intensely
coagulated.
After surgery, the patient had swallowing
difficulty and developed aspiration pneumonia,
which had to be treated with antibiotics over a
period of 2 weeks. Gradually his condition sta-
bilized, and a tendency toward improvement of
the lower nerve function was noted. The EVD
was removed on the 12th postoperative day.
Later on, the nasogastric tube was removed, and
peroral feeding was started. At discharge, he
was fully mobilized: the gait was considerably
better when compared to the preoperative level.
Cerebellopontine Angle Meningiomas
Meningiomas Involving the IAC
Meningiomas may arise primary or extend sec-
ondary into the IAC [65, 83, 101]. It has been
estimated that 14–20 % of all CP angle menin-
giomas have an intracanalicular part [10, 83, 101].
The extracanalicular tumor part in the senior
authors’ series was found to be located anterior to
the IAC (premeatal) in 38 %, superior to the
IAC (suprameatal) in 22 %, inferior to the
IAC (inframeatal) in 29 %, and posterior to the
a b
c
Fig. 7.19 The variable extension patterns of CP angle
meningiomas in regard to the IAC are illustrated on these
images. (a, b) Meningioma centered at the IAC without
any intracanalicular growth. (c, d) Meningioma with
409
IAC (retromeatal) in 11 % [66]. It is still unclear
why some tumors tend to extend into the canal,
while others remain purely cisternal (Fig. 7.19).
Approximately 10 % of all CP angle menin-
giomas arise from the dura of the IAC and gradu-
ally extend toward the CP angle [10, 90]. They
are believed to originate from intrameatal arach-
noid cell clusters [65]. In a widely cited study,
Nager and Masica [65] described such arachnoid
clusters not only along the dural sinuses and in
d
intracanalicular extension and dilatation of the IAC. (e, f)
The intracanalicular part of this tumor extends laterally up
to the fundus of the IAC. (g, h) Petroclival meningioma
with a limited intracanalicular extension
410 7 Meningiomas

e f

g h

Fig 7.19 (continued )

the area of Meckel’s cave but also in the IAC, in
the jugular foramen, along the greater petrosal
nerve, and in the vicinity of the geniculate
ganglion.
In the seniors’ author series, the mean diame-
ter of meningiomas extending into the IAC was
28 and 34 mm in male and female patients,
respectively [83]. The average symptom duration
prior to diagnosis was 48 months. At presenta-
tion, the most frequent neurological finding was
related to cochlear nerve dysfunction: some
degree of hearing loss had 72 %. Functional
hearing (Hannover classes H1–H2), however, had
54 % of all patients. Tinnitus had 42 %, vertigo
had 35 %, ataxia had 22 %, trigeminal neuralgia
had 17 %, headache had 17 %, and facial nerve
palsy had 8 %. Grey et al. reported that 45 % of
their patients with gross intrameatal tumor exten-
sions were deaf preoperatively, compared with
5 % of patients in whom the IAM was not
involved.
Neuroimaging reveals the typical characteris-
tics of CP angle meningiomas and shows the
extent of IAC involvement and the changes of
Cerebellopontine Angle Meningiomas
surrounding bone structures. Such meningiomas
are difficult to differentiate from VSs due to
their similar MR and CT signal intensities and
degree of contrast enhancement. Important
clues, if available, are the dural tail sign, the en
plaque growth, and the bone hyperostosis, typi-
cal for meningiomas. Meningiomas, further-
more, rarely cause dilatation of the IAC. In the
seniors’ author series, dural tail was evident in
21 %, petrous bone hyperostosis in 13 %, and
bone erosion in 20 % of all meningiomas with
IAC extension. En plaque appearance had 6 % of
the tumors [83].
The necessity and the extent of IAC opening,
as in case of VS, are determined by the extension
of the tumor in the canal, its adherence to neural
structures, and the preoperative cranial nerve
function. In patients with normal preoperative
cranial nerve function, every attempt should be
made to avoid new deficits. The attempt to dis-
sect very adherent tumors from the nerves could
cause neurological morbidity. Following this
concept, in 2/20 patients with meningiomas orig-
inating from the IAC, the canal was left intact,
while in the remaining 18/20 patients, it was
opened to achieve more complete resection or to
decompress the neurovascular bundle [83]. The
latter option is used to gain functional time in
case the cranial nerves are infiltrated.
Targeted opening of the posterior wall of the
IAC is performed with the high-speed drill
(Fig. 7.20). Meningiomas with limited intrac-
analicular extension can be safely removed by
drilling off just few millimeters of the posterior
lip of the IAC. Wide opening of the IAC is
required to remove meningiomas extending more
laterally. Thin-slice bone-window CT provides
the information necessary for the precise plan-
ning of this step. Important issues to consider are
the degree of bone pneumatization and the loca-
tion of the jugular bulb and of the inner ear struc-
tures. The dura behind the IAC is incised
circumferentially, stripped off, and excised.
Large-sized diamond burr (4–5 mm in diameter)
is used initially and changed to smaller in the
depth. Bone is drilled off layer by layer in 1-mm
increments, and the last bone layer or lamina is
removed with a small platelet knife. The posterior
411
wall of the canal should be opened sufficiently—
generally approximately 180° of its circumfer-
ence—in order to provide an unobstructed view
of the tumor. In case of meningioma arising from
the dura of the IAC, complete excision of the
dura in that area should be attempted. See Case
Illustration 7.6.
In the series, the IAC was opened to achieve
tumor removal in 16 patients, while in 19
patients, the intracanalicular tumor portion could
be removed completely without opening of the
canal. Radical removal of all affected bone adja-
cent to the tumor site of origin is recommended
to prevent recurrences in cases of severe preop-
erative facial and cochlear nerve dysfunction. In
such patients, surgical radicality may necessitate
even complete resection of the infiltrated nerve
and its primary reconstruction in the CP angle
(see section “Facial Nerve Reconstruction”).
The decision to sacrifice a normally functioning
nerve should be taken on an individual basis.
Factors to consider are the patients wish, the
possibility to achieve radical surgery, and the
tumor grade.
Generally, meningiomas arising from the dura
of the IAC are infiltrative or, at least, are very
adhesive, whereas the degree of secondary intra-
canalicular tumor extension does not affect clini-
cal outcome so strong. In our series, 83 % of the
patients with HB grade I or II preoperative facial
function had similar function immediately after
surgery. Twenty-five percent of the patients
maintained normal hearing after surgery, two
patients improved from preoperative hearing
class H2 to normal hearing, and 32 % of those
with preoperative class H2 retained hearing at
the same level. Interestingly, hearing recovered
in 1 of the 11 preoperatively deaf patients.
Complete tumor resection (defined as Simpson
grades I and II) could be achieved in 86 % of the
cases. Facial nerve function was significantly
better in case the meningioma involved the IAC
secondarily. The extent of resection and hearing
outcome, however, did not correlate significantly
to tumor origin [83]. At long-term follow-up,
four patients had recurrences (mean follow-
up—60 months). Two of them had atypical
meningiomas.
412 7 Meningiomas

a b

c
d

Fig. 7.20 Small premeatal meningioma with intracanali- (c) The IAc has been opened, and the tumor is mobilized.
cular extension. (a) MR image. (b) Initial view of the CP (d) Complete tumor removal. All essential structures have
angle tumor part ventral to the seventh-eighth nerves. been preserved
Cerebellopontine Angle Meningiomas
Case Illustration 7.6
Premeatal Meningioma with Intracanali-
cular Extension
The patient (32-year-old female) presented
with a history of decreased hearing and ver-
tigo since approximately 6 months. Her neu-
a b
(a–c) Preoperative MRI of the patient: (a, b)
T1-weighted sequences after contrast administration.
(c) T2-weighted sequence. The tumor extends laterally
up to the fundus area, as demonstrated on the T2
sequence: no CSF is seen lateral to the tumor. (d) Initial
view of the CP angle, demonstrating the tumor and its
relations (Tu tumor, P posterior pyramid, R retractor, C
cerebellar hemisphere). (e) Removal of the retro- and
inframeatal tumor parts (arrow—lower cranial nerves).
413
rological examination was normal besides the
hypacusis (PTA of 50 dB). MRI revealed a CP
angle meningioma with lateral extension up to
the fundus of IAC. The patient was operated
via the RS approach. Once CSF was drained
from the lateral cerebellomedullary cistern
(f) The dura around the posterior lip of the IAC had
been coagulated. The dotted line depicts the area of the
petrous bone to be drilled off (arrowhead seventh-
eighth nerve complex). (g) The posterior wall of the
IAC has been removed widely, the dura was excised,
and the intracanalicular meningioma part was exposed
and gradually removed (h). (i) The tumor has been
removed completely, and the neural and vascular struc-
tures were preserved (arrow—lower cranial nerves)
414 7 Meningiomas

d e

f g

h i
Cerebellopontine Angle Meningiomas
and the retractor was placed, the tumor, which
covered the seventh-eighth nerve complex
and—partially—the lower cranial nerves, was
seen. The meningioma originated from the
dura anterior to the porus and extended like a
carpet premeatally, inframeatally down to the
jugular foramen, suprameatally, and slightly
retromeatally. Its retro- and inframeatal parts
were dissected from the lower cranial nerves
and removed. A large tumor part was seen to
extend along the seventh-eighth nerves into
the IAC—its dissection, however, was post-
poned until the canal was widely opened. The
dura behind the IAC was coagulated, incised
circumferentially around the porus of the
canal, and excised. Then, the posterior wall
415
was widely removed with the high-speed drill
with diamond burrs. The intracanalicular
tumor part, which was located posterior and
inferior to the nerve complex, was then
removed completely. The integrity of the
nerves, as well as their vasculature, was pre-
served. After thorough hemostasis, the IAC
was sealed off with fat pieces and fibrin glue.
Following surgery, the patient did not have
new neurological deficits and recovered
quickly. The audiogram showed an improve-
ment of the hearing (PTA of 40 dB), which
was stable and confirmed at follow-up exami-
nations. Histopathological examination
showed that this was a case of lymphoplasma-
cyte-rich WHO grade I meningioma.
416
Intracanalicular Meningiomas
Meningiomas confined to the IAC or “intrac-
analicular meningiomas” are extremely rare
[8, 49, 69]. Their clinical and neuroradiological
features are very similar to those of VSs, and in
most cases, the correct diagnosis is made only
at surgery. The initial tumor manifestation is
usually decreased hearing. At presentation, the
majority of patients have vestibulocochlear
nerve disturbances. In the series of the senior
author, including eight patients with intrac-
analicular meningiomas, tinnitus had 88 %,
hearing loss of variable degree had 75 %, and
dizziness and vertigo had 63 % [69]. In contrast
to VSs, meningiomas can cause facial nerve
symptoms, including facial paresis or hemifa-
cial spasm [7, 69].
Preoperative differentiation between these
meningiomas and VSs is difficult because they
have similar neuroradiological characteristics.
Still, some findings could help their differentia-
tion. Meningiomas and VSs have similar signal
intensities; on T2-weighted images, however,
VSs tend to show higher signal intensity than
meningiomas. The visualization of intratumoral
calcification, the type of bone changes, the gen-
eral shape of the tumor, and/or the presence of
“dural tail” may be further helpful clues,
although these findings are nonspecific [7, 46].
7 Meningiomas
Meningiomas rarely cause widening of the IAC
but induce more frequently petrous bone or IAC
hyperostosis.
The relationship of the tumor to the seventh
and eighth cranial nerves is less reliably predict-
able in meningiomas than in VSs. The direction
of nerve displacement is related to the site of
tumor origin. Another fact to consider is that
meningiomas tend more frequently to be severe
adherent or to infiltrate these nerves [7, 69].
Asaoka et al. [7] pointed that the adhesions
between the meningioma and facial nerve are
much more difficult to separate, than in VS, even
in case of smaller tumors.
The operative strategy and technique used in
surgeries of intracanalicular meningiomas do not
differ from those in case of meningiomas involv-
ing secondarily the IAC (see Case Illustration
7.7). The goal is complete tumor removal and
functional preservation of all cranial nerves. In
our series, complete tumor removal was achieved
in all patients without major morbidity. Facial
and cochlear nerves preserved anatomically in all
cases; one patient had postoperative facial weak-
ness that resolved completely. In 71 % of the
patients, hearing was preserved. Interestingly, in
three of the seven patients with tinnitus at presen-
tation, the symptom resolved, in three patients it
improved, and only in one it persisted.
Cerebellopontine Angle Meningiomas
Case Illustration 7.7
Intracanalicular Meningioma
This 47-year-old patient presented with a his-
tory of right-sided hearing loss since more
than 10 years. In the last months, he suffered
from annoying tinnitus and headache on the
right side. His neurological examination was
normal besides the right-sided hypacusis. CT
and MRI demonstrated a pure intracanalicular
tumorous lesion with imaging characteristics,
compatible with those of VS (a–f). The IAC,
a b
(a, b) Bone-window CT of the patient: normal IAC on
both sides. (c–f) MRI clearly demonstrated the pure
intracanalicular tumor. (c, d) T1-weighted sequences
417
however, was not dilated. All management
options were discussed with the patient, but he
decided for surgery.
The CP angle was exposed via an RS route. In
the area of the porus of the IAC, a small protrud-
ing tumor part was seen, which appeared like a
meningioma. Frozen section confirmed this sus-
picion. The strategy of its removal was similar to
the one applied in case of intracanalicular VSs:
wide opening of the IAC up to its fundus, preser-
vation of the inner ear structures, excision of the
c
after contrast application. (e, f) T2-weighted sequences.
(g, h) MRI preformed 12 months after surgery: no
remnant or recurrent tumor is detectable
418
g h
dura of IAC, identification of the cranial nerves,
and stepwise dissection of the meningioma from
surrounding structures. The meningioma, includ-
ing the dura from which it originated, was
removed completely, and the anatomical integ-
rity of the facial and cochlear nerves was pre-
served. The severely impaired BAEP showed
slight improvement after tumor removal: the
7 Meningiomas
waves III and V could be recognized but had still
long latency and low amplitude.
The patient had no new neurological deficits
after surgery, and the hearing level remained at
the same nonfunctional level. Histopathological
diagnosis was psammomatous meningioma
WHO grade I. Follow-up MRI (g, h) confirmed
the completeness of tumor removal.
Cerebellopontine Angle Meningiomas
Intraosseous Meningiomas
Extradural meningiomas comprise 1–2 % of all
meningiomas [25]. Primary intraosseous are
those extradural meningiomas that arise within
the bone [80]. Intraosseous meningiomas of the
skull base are extremely rare. Most are osteoblas-
tic or mixed osteoblastic–osteolytic lesions, while
purely lytic lesions are the least common
[1, 50, 82]. Such tumors may be purely intraosseous
without evidence of dural invasion or may have
additional soft tissue component [82]. In order to
classify a tumor with soft tissue component as a
primary extradural meningioma, its origin and
center should be located outside the dura [48]. Of
note, clear-cut criteria still do not exist [25].
Skull-base intraosseous meningiomas are gen-
erally slow-growing, painless tumors. Due to
their location, they cause cranial nerve deficits
and/or cosmetic deformation. On CT, the osteo-
blastic intraosseous meningioma-induced hyper-
ostosis is demonstrated as focally thickened
hyperdense lesion. The osteolytic lesions cause
thinning, expansion, and interruption of the inner
419
and outer cortical layers of the skull [1]. The MRI
findings of intraosseous are similar to those of
intradural meningiomas: they are typically
hypointense on T1-weighted images and hyper-
intense on T2-weighted images with intense
homogeneous enhancement after contrast appli-
cation. Although the classical dural tail sign is
not seen, contrast enhancement of the underlying
dura may be noted [5]. The differential diagnosis
of intraosseous meningiomas includes metasta-
sis, epidermoid tumor, multiple myeloma, eosino-
philic granuloma, fibrous dysplasia, chordoma,
chondrosarcoma, brown tumor, skull dermoid,
giant cell tumor, hemangiopericytoma, and
hemangioma [82].
Treatment of choice of such meningiomas is
their wide surgical excision. In the skull base,
however, this is rarely possible, and maximal
tumor removal with decompression of vital neu-
ral structures is performed (see Case Illustration
7.8). Tumor remnants are followed and in case of
biological activity are subjected to radiation
therapy.
420 7 Meningiomas

Case Illustration 7.8 nerve dysfunction with hoarseness and dys-

Intraosseal Meningioma
Twenty-seven-year-old male patient pre-
sented with a history of diplopia for more
than 9 years. In the last 3 years, progressive
swallowing difficulties, vertigo, and inter-
mittent headache developed. At presenta-
tion, the patient had trigeminal hypesthesia
(V2), abducens nerve palsy, lower cranial
phagia, weakness of the sternocleidomastoid
muscle, and hemitongue atrophy on the right
side. MRI showed a contrast-enhancing
lesion of the medial petrous bone, extending
medially to the clivus (a–d). The tumor had
a large soft tissue component in the lower
CP angle, causing compression of the lower
pons and of the medulla. On CT, the lesion

a b

c d

Preoperative MR images of a patient with an intraos-
seal petrous meningioma. (a) T2-weighted. (b–d)
T1-weighted sequences after contrast administration.
(e) Postoperative MRI. A small part of the menin-
gioma that was most adherent to surrounding struc-
tures was not extirpated in order to avoid major
deficits. (f) MR venography: occlusion of the lateral
transverse and of the sigmoid sinuses on the side of
surgery; patent contralateral sinus
Cerebellopontine Angle Meningiomas
e f
had a mixed osteoblastic and osteolytic
appearance.
The patient was operated via a transmastoid
presigmoid approach. Frozen section examina-
tion suggested a WHO grade I meningioma. The
tumor was partially hard and fibrous with ossified
parts. Initially the intraosseal tumor part was
removed; the dura was found to be infiltrated and
was opened to inspect the CP angle cistern; how-
ever, no signs of dural penetration were found.
Considering the extension of the lesion and the
dense adherence to surrounding structures, com-
plete tumor removal and preservation of the nor-
mal facial nerve function and hearing was deemed
impossible. Moreover, the petrous carotid was
encased by the tumor. The most densely adherent
tumor parts were, therefore, not removed (e).
421
The patient suffered an epileptic fit shortly
after extubation, and an early cranial CT scan
was performed. Thrombosis of the sigmoid
and of the lateral segment of transverse
sinuses was suspected and confirmed on the
MR venography (f). Obviously the contralat-
eral sinus was patent because no signs of
venous infarction or edema were detected.
Appropriate therapy was initiated, and the
patient recovered completely. Following sur-
gery, his hearing level remained unchanged,
but a facial palsy (HB grade IV) developed.
At discharge, the facial nerve function
improved to HB grade III, and the patient
noted an improvement in swallowing.
Histopathological diagnosis was WHO grade
I sclerosing meningioma.
422
Postoperative Management
The patients should emerge smoothly from anes-
thesia with minimal agitation, and coughing
should be prevented. Invasive arterial blood pres-
sure monitoring is performed for at least 24 h:
major elevation of blood pressure should be
avoided, especially during the early postopera-
tive period. In case the patient is expected to have
lower cranial nerve deficit after surgery, he
should be extubated only when fully awake and
cooperating (see Chap. 10). Patients that remain
intubated (preferably nasally) should be mini-
mally sedated, which allows estimation of their
level of consciousness and early detection of
possible intracranial complications. If deeper
sedation is required, the medication should be
decreased at regular intervals (e.g., every 2 h),
and the patient should be examined. CT is per-
formed routinely in all patients the day after
surgery.
We perform steroid treatment (dexametha-
sone) for at least 3 days after surgery. Steroids are
known to inhibit vasogenic edema, to reduce
cytokines expression by the tumor cell, and to
inhibit the edemogenic response of the brain
microvascular. Furthermore, they stabilize the
endothelial cell membranes [62].
Early MRI should be made to document the
extent of tumor removal. In case of incomplete
resection, follow-up MRI studies are performed
3–6 months after surgery, and radiosurgery/radio-
therapy or follow-up of the residual tumor is rec-
ommended accordingly.
Outcome
The postoperative cranial nerve outcome is better
in meningiomas than in VS because meningiomas
generally do not invade but rather compress the
seventh and eighth cranial nerves. Removal of
the tumor can even lead to improvement of cra-
nial nerve function [58]. Currently, facial nerve
function can be preserved in the majority of
patients [10, 11, 66, 101, 106]. Anatomic preser-
vation of the seventh and eighth cranial nerves
was reported in 94 %, and functional preserva-
tion of the seventh and eighth cranial nerves was
reported in 86 and 77 % of CP angle menin-
giomas, respectively [57, 66].
7 Meningiomas
The outcome of surgery depends mainly upon
the location and consistency of the tumor and to a
lesser extent upon its size [10, 90]. In premeatal
meningiomas, the preservation of facial and audi-
tory function is less likely. Two further important
factors are the extent of skull-base involvement
and the adherence of the tumor to brain stem, cra-
nial nerves, and/or vessels [98]. In the large recent
series, the rate of total removal is above 80 %,
and the mortality is below 5 %.
Nakamura et al. [66] evaluated 421 patients
with CP meningiomas operated by the senior
author. They found that the radicality of tumor
removal as well as the functional and general out-
come correlated with the topographic location of
these tumors. The overall rate of complete
removal (Simpson grades 1 and 2) was 86 %.
Considering the tumor subtypes, highest rate was
achieved in suprameatal meningiomas (90 %), in
retromeatal meningiomas (89 %), and in those
involving the IAM (88 %). The rate was lowest in
premeatal (83 %) and in inframeatal tumors
(78 %). The outcome was worse in cases that
arose in or at the IAC [66].
In the series, good facial nerve function
(House–Brackmann grades 1 or 2) was observed
in 89 % of the patients. The rate of preserved nor-
mal facial nerve function was highest in ret-
romeatal meningiomas (90 %) and lowest in
premeatal meningiomas (76 %). The rate of pres-
ervation of functional hearing could be increased
from 88 % (earlier experience [58]) to 91 % in the
recent series [66]. The rate of hearing preservation
was highest in patients in the suprameatal and ret-
romeatal subgroups (100 and 73 %, respectively)
and lowest in those in the premeatal subgroup
(43 %). Postoperative hearing improvement
occurred in 8 % of our patients. In 2 % even recov-
ery from preoperative deafness was observed [66].
Interestingly, none of the seven patients present-
ing with sudden hearing loss demonstrated post-
operative improvement. Bassiouni et al. [10]
reported on the positive effect of surgery on tinni-
tus. They found that tinnitus was a major com-
plaint in 18 % of the patients preoperatively but
was ameliorated in all of them postoperatively.
Lower cranial nerves, however, are more vulnera-
ble, and more aggressive dissection or manipulation
Cerebellopontine Angle Meningiomas
of the arachnoid layer might cause new postopera-
tive deficits [97].
Meningiomas are increasingly frequently
diagnosed in elderly patients. Our opinion is that
surgery is the most appropriate management
option even in this age group, provided that the
patient is in good general condition. Although the
growth rate of meningiomas in elderly is lower
when compared with younger patients, most of
them have a certain growth tendency [68]. In
medically unstable patients, radiotherapy (frac-
tionated radiotherapy or radiosurgery) is an
acceptable alternative. Incidentally detected
small tumors or tumors causing minimal symp-
toms may be followed with regular radiological
and clinical examinations. The Senior authors’
experience encompasses 25 operated patients
with CP angle meningiomas aged 70 or more.
The outcome was related mostly to the preopera-
tive neurological and clinical condition of the
patient. Complete resection (Simpson grade 1
and/or 2) was achieved in 84 % of all cases, and
the general and functional outcome was similar
to that in younger patients. Elderly patients were
more prone to have lung infections. Furthermore,
the CSF leak rate was higher than in younger
ones. Generally, the length of hospital stay was
longer in elderly patients, but the difference was
not significant [68].
Recurrence
CP angle meningiomas have higher tendency
toward recurrence if compared with VSs and may
recur even late after surgery [10, 90, 91, 98, 101].
Patients should be therefore regularly followed,
both clinically and radiologically. Radiologic
follow-up interval for MRI is set between 3
months and 1 year for malignant and benign
meningiomas, respectively. Recurrent menin-
giomas, especially those after radiotherapy, may
show a milder contrast enhancement than the pri-
mary lesion, probably due to increased fibrosis in
older tumors.
The likelihood of recurrence correlates with
meningioma grade, extent of resection (complete
vs. incomplete), and—according to some studies—
to the loss of progesterone receptor immunostaining
[39, 77]. For WHO grade I meningiomas, it varies
423
between 3.2 and 23 % among the different series
but is usually less than 10 % [6, 36, 91, 98, 101].
The extent of tumor removal is well known to cor-
relate to the risk of recurrence. Total tumor
removal, however, does not preclude recurrences
[6, 98].
If the recurrence shows a growth tendency or
becomes symptomatic, surgery should be consid-
ered as the primary management option. Complete
tumor removal should be the goal, if safely pos-
sible. Generally, the chances to remove the tumor
completely without causing new major deficits
are lower than in case of primary surgery. In
recurrent meningiomas, the surgery-related mor-
bidity risk is higher. An anatomical dissection
plane is usually absent; the tumor tends to be
more adherent to the brain and to encase nerves
and vessels. Radiotherapy is an alternative man-
agement option of large recurrences that are not
considered amenable to operative removal. Small
recurrent tumors can be followed and—in case of
further growth—may be treated with radiosur-
gery or fractionated radiotherapy [31, 95].
Atypical and Anaplastic Meningiomas
Atypical and anaplastic meningiomas comprise
approximately 5 % from all meningiomas and
have a slight male predominance [104]. They
are approximately 2 % of posterior fossa
and 18–31 % of all skull-base meningiomas.
Characteristic for this small subgroup is the
more aggressive biological behavior with a ten-
dency for early recurrence even after complete
tumor removal [35, 42, 45].
These tumors are believed to develop de novo
or progress from benign to atypical or anaplastic
variants. Tumor progression, however, is rarely
observed in skull-base meningiomas [3, 41].
Benign, atypical, and anaplastic meningiomas
cannot be reliably differentiated radiologically.
Some features, such as marked edema, heteroge-
neous appearance, irregular or nodular cerebral
surface, mushrooming on the outer edge of the
lesion, extensive bone destruction, and absence
of calcification, are more frequently seen in atyp-
ical and anaplastic meningiomas but are
nonspecific [41, 54, 108]. Recent studies indi-
cated that the diffusion-weighted MRI, perfusion
424
MRI, and magnetic resonance spectroscopy
findings might be used to distinguish menin-
gioma subtypes, but the results have been incon-
clusive [63].
Recurrence rates of atypical and anaplastic
meningiomas are very high, irrespective of their
management mode. The recurrence rate is high
even after radical tumor removal: depending on
the length of follow-up in different series, it is
between 50 and 95 % [55]. The 5-year local
tumor control rate following radiosurgery in the
study of Stafford et al. [95] was 68 % in atypical
and 0 % in anaplastic meningiomas, treated with
radiosurgery. In another study, the 3-year survival
rate associated with linear accelerator-based
radiosurgery of atypical and anaplastic menin-
giomas was 24 and 14 months, respectively [38].
Best results are achieved with a multimodality
management, including radical surgery and
adjunctive radiation therapy either with fraction-
ated radiotherapy, stereotactic radiosurgery, or
proton beam irradiation [63, 71, 75]. The primary
treatment of such tumors should be their com-
plete operative removal, including the infiltrated
bone and/or soft tissue. Every patient with ana-
plastic meningioma, regardless of the degree of
resection, should receive adjuvant fractionated
radiotherapy soon after resection. Management
of atypical meningiomas is related to the extent
of their resection: follow-up is recommended in
case of complete resection, while early adjuvant
fractionated radiotherapy is indicated in subto-
tally resected cases. Modha et al. [63] recom-
mend postoperative fractionated radiotherapy
even in case of complete removal of atypical
meningioma if the tumor was found to invade the
brain. In both subtypes, radiosurgery may be con-
sidered as an alternative to fractionated radiother-
apy in case of a focal or nodular remnant.
Radical surgery is the primary management
option for recurrences, provided the tumor is
resectable without very high operative risks.
Fractionated radiotherapy should be performed
in patients who have not received it before.
Currently, effective chemotherapy is not avail-
able, and its role in the management of atypical
and anaplastic meningiomas remains unclear
[45, 63].
7 Meningiomas
Multiple Meningiomas
Patients with NF-2, in addition to the characteris-
tic bilateral VSs, often have other tumors, such as
cranial nerve schwannomas, spinal schwanno-
mas, and meningiomas [60] (see section
“Neurofibromatosis Type 2”). Meningiomas, fre-
quently multiple, are the second most frequent
tumor type in NF-2. They occur in 38–58 % of
these patients [29, 34, 76].
The term “multiple meningiomas” denotes the
clinical entity, in which the patient has two or
more independently situated meningiomas, not
necessarily of the same pathologic subtype
[12, 26]. Patients with multiple meningiomas
may not have (or still not have) other features of
NF-2, such as multiple schwannomas. The
reported incidence of patients harboring multiple
meningiomas not related to NF-2 varies: 1–12 %
in surgical series, 8–16 % in autopsy studies of
meningiomas, and 1–9 % in radiological series
[4, 27, 34]. As noted by Goutagny and Kalamarides
[34], however, NF-2 patients are difficult to seg-
regate from other multiple meningioma patients,
and multiple meningiomas as an entity largely
overlap with NF-2. In the population study of
Antinheimo et al. [4] in the absence of classic
NF-2, 4 % of all patients with meningiomas had
multiple meningiomas. Somatic mosaicism, fre-
quently seen in NF-2, is the proposed explanation
for developing of multiple meningiomas.
Meningiomatosis is a less clearly defined term; it
is used either to denote all patients with multiple
meningiomas or only those with more pro-
nounced clinical syndrome.
In some patients, especially in children,
meningiomas may be the first sign of NF-2
[84]. Patients with multiple meningiomas
should be hence investigated to exclude NF-2:
detailed family history analysis, dermatologi-
cal, ENT and ophthalmological examinations,
as well as MRI of the whole neuraxis should be
performed.
Meningiomas in NF-2 patients develop less
frequently in the skull base and, respectively, in
the CP angle [4]. Their management poses
significant problems. Major issue is the lifelong
propensity for development of new neoplasms
and the respective impossibility for definitive
Cerebellopontine Angle Meningiomas
healing. Treatment is focused on life prolonga-
tion, preservation of cranial nerve function, and
maintenance of quality of life. Symptomatic
meningiomas, as well as progressively growing
tumors, are best treated surgically. The goal is to
achieve decompression of essential neural
425
structures and—if possible—to remove the tumor
completely. Preservation of neural function has a
priority: in case the tumor infiltrates or is very
adherent to a cranial nerve, no attempt should be
made to remove it completely. See: Case
Illustrations 7.9 and 7.10.
426
Case Illustration 7.9
Multiple Meningiomas
Fifty-year-old female patient presented with
MRI findings of an extensive meningioma of
the anterior and middle cranial fossae (right >
left) with engulfing of both optic nerves and
the optic chiasm (a–c). The parasellar tumor
on the right side engulfed the carotid artery
(a–c) Preoperative MRI (T1-weighted sequence after
contrast administration), demonstrating the size and
extension of the tumor. (d–f) Postoperative MRI: com-
plete removal of the skull-base meningioma. Note the
convexity meningioma on the left side. Two more con-
vexity meningiomas of similar size were also detected.
7 Meningiomas
and the anterior and the middle cranial arteries
and extended toward the tentorial incisura,
compressing the right oculomotor nerve. The
patient had visual field defect and severe
visual loss (visual acuity on the right side, 0.3;
left side, 0.9), as well as partial oculomotor
nerve palsy. She was operated via a frontolat-
eral (pterional) craniotomy, and complete
(g–i) Bone-window CT scan and MRI, demonstrating
the petroclival meningioma, involving the cavernous
sinus. A hyperostotic/calcified part is seen on the
bone-window CT and on some MR sequences
(arrows). (j–l) MR images after the removal of the
right-sided petroclival meningioma
Cerebellopontine Angle Meningiomas
removal of the tumor was achieved with pres-
ervation of all neurovascular structures (d–f).
The patient presented 3 years later with
complaints of progressive hearing loss on the
right side. Neurological examination revealed
trigeminal hypesthesia (V1,2,3) and hearing
loss (still functional hearing). Visual acuity on
the right side was 3/10 and on the left side was
9/10. The MR examination demonstrated that
the known convexity meningiomas were
unchanged; however, a new tumor in the right
petroclival space, reaching the IAC and com-
pressing the seventh-eighth cranial nerves,
was detected (g–i). The tumor was approached
b
427
via the RS route. It compressed the trigemi-
nal, the seventh-eighth, and the lower cranial
nerves. The abducens nerve was completely
engulfed by the tumor. Initial removal was
started along the lower cranial nerves until the
basilar artery was reached and dissected free
from the tumor. Then, the space between the
trigeminal and the seventh-eighth nerves was
used to remove the meningioma further. The
abducens nerve was followed from its exit
zone to the Dorello’s canal. Despite the
adherence to the cranial nerves and basilar
artery perforators, the tumor mass could be
removed, and all neurovascular structures
428 7 Meningiomas

were preserved. The tumor matrix in the gothelial meningioma, WHO grade I. The last
petrous apex area was thoroughly coagulated. postoperative MR control is presented on
Histopathological tumor type was menin- figures (j–l).

c
Cerebellopontine Angle Meningiomas 429

d e

f
430 7 Meningiomas

g h

i
Cerebellopontine Angle Meningiomas 431

j k

l
432
Case Illustration 7.10
Two Tentorial Meningiomas Removed via
One Approach
The patient presented with headache and tran-
sient double vision. The MRI examination
demonstrated two separate tumors with imag-
ing characteristics compatible with tentorial
meningiomas: a small one arising from the
free tentorial edge in the area of the tentorial
incisura and a larger one, arising from the
middle tentorial part. The larger meningioma
had both a supra- and infratentorial extension.
Considering the location of the tumors, it was
7 Meningiomas
decided to remove the larger tumor via the
infratentorial supracerebellar approach and to
attempt removal of the smaller tumor via the
same route. The craniotomy was approxi-
mately 4 × 2.5 cm, extending from the sigmoid
sinus to 1 cm to the midline. Both tumors
could be adequately accessed via this route
and removed without any complications.
Histological diagnosis was WHO grade 1
meningioma. The patient recovered quickly
from surgery and was discharged without any
neurological deficits.
Cerebellopontine Angle Meningiomas 433

a b

c d

e f
434 7 Meningiomas

g h

i j

(a–f) Preoperative MRI study of the patient with 2 ten-
torial meningiomas. (a–e) T1-weighted sequence after
contrast application. (f) T2-CISS sequence. (g–j)
Intraoperative images. (g) The craniotomy extends from
the sigmoid sinus (SS) laterally to approximately 1 cm
from the midline. The edge of the transverse sinus (TS)
is exposed. The dura is incised along the transverse
sinus, which facilitates its later watertight closure, and
is slightly retracted with 2 sutures. The infratentorial
part of the large tentorial meningioma is visible (g, h).
Initially this tumor part was removed; then the attach-
ments of the tumor to the falx were incised, which
allowed us to mobilize the remaining supratentorial
tumor part. This part was carefully pulled down and
removed completely. A retractor was then placed, and
the second meningioma (arrow) could be visualized
from a more lateral perspective (i). The trochlear nerve
was compressed and adherent to the anterior tumor
surface (j). Nevertheless, the tumor could be com-
pletely removed, and the structures were preserved
Petroclival Meningiomas
Hormonal Therapy and Chemotherapy
of Meningiomas
Patients with meningiomas of any histological
grade that recur or regrow after surgery and radi-
ation therapy, as well as high-grade tumors, are
potential candidates for hormonal or chemother-
apy. Up to know, however, the role of chemother-
apy is very restricted because the currently
available chemotherapeutic agents have minimal
activity against meningiomas [17, 61]. Hormonal
therapies are also largely ineffective in patients
within this subset of meningiomas. Studies with
estrogen inhibitors did not show any efficacy,
which probably is due to the low concentration of
estrogen receptors: they are present at low levels
in approximately 10 % of meningiomas [33].
Progesterone and androgen and their receptors
are present in approximately two-thirds of men-
ingiomas [47, 87] . Still, studies with the applica-
tion of their antagonists failed to demonstrate any
treatment effect [37]. As Norden et al. [72] point,
the lack of efficacy may be explained in part by
the loss of progesterone expression in menin-
giomas with increased proliferation index and
higher histological grade. Inhibition of the growth
hormone receptors or their direct blockade and
the use of somatostatin agonist have been also
utilized, but the effect was minimal [23].
Potential molecular targets for targeted ther-
apy in the future may be the various growth fac-
tors and their receptors that are overexpressed
in some meningiomas: the platelet-derived
growth factor, the epidermal growth factor, and
the vascular endothelial growth factor, as well
as some steps of the signal transduction path-
ways [72].
Petroclival Meningiomas
Petroclival meningiomas account for 3–10 % of all
posterior fossa meningiomas and for approximately
2 % of all meningiomas [112, 114, 116, 119, 138].
They arise in the area of the petroclival junction
[150]. Included in the group are meningiomas
located medial to the trigeminal nerve (Fig. 7.21a):
originating from the clivus, from the petroclival
junction, from the petrous apex, or from the
435
Meckel’s cave. The location and the intimate rela-
tion of these tumors to important neural and vascu-
lar structures determine the complexity of their
surgery and the high risk of postoperative morbid-
ity [115]. Most challenging are meningiomas with
extensive supratentorial parasellar extension and
involvement of the cavernous sinus, sella turcica,
and sphenoid sinus.
Petroclival meningiomas show various exten-
sion patterns. In the senior authors’ series, purely
infratentorial were just 5 % of all petroclival
meningiomas; 15 % of them extended to the level
of tentorial notch, and 80 % had supratentorial
extension [115]. Medial tumor growth causing
brain stem compression was seen in 92 % of all
operated cases. Thirty-three percent of menin-
giomas with anteromedial extension reached the
clivus, and 67 % extended up to the parasellar
space. Extension into the cavernous sinus was
observed in 48 % of the cases: petroclival tumors
involved the posterior part of the sinus
(Fig. 7.21b), while the sphenopetroclival menin-
giomas occupied the whole sinus (Fig. 7.21c).
The latter involve in addition the anterior cavern-
ous sinus, sella turcica, and/or sphenoid sinus.
Lateral growth is defined in relation to the IAC:
31 % of the tumors remained medial to IAC (pre-
meatal), and 69 % had more lateral extension
(retromeatal). Anterolateral growth leads to inva-
sion of the Meckel’s cave (Fig. 7.21d).
Occasionally the tumor originates inside or at the
mouth of Meckel’s cave. Such tumors may grow
further in the middle fossa between the two leaves
of tentorium.
Fifty-five percent of all tumors extended infe-
rior to the level of IAC, while 24 % reached the
jugular foramen, and 21 % reached the foramen
magnum. Three percent of the latter extended
even down to the C1–C2 level. Very large and
giant meningiomas (approximately 15 % of all
patients) extend from the foramen magnum level
to the supratentorial region.
General Features
The clinical presentation of petroclival menin-
giomas is often of insidious onset; patients tend to
436 7 Meningiomas

a b

c d

Fig. 7.21 MRI studies of various petroclival menin- of the posterior cavernous sinus. (c) Sphenopetroclival
giomas. (a) Petroclival meningioma: the tumor originates meningioma with a large suprameatal part. The whole
medial to the trigeminal nerve (arrow). (b) Petroclival cavernous sinus is involved by the tumor. (d) Petroclival
meningioma with supratentorial extension and involvement meningioma with extension into the Meckel’s cave

have a longer history of symptoms prior to diag-
nosis than those with CP angle meningiomas: on
average 30–60 months [112, 114, 136, 144, 153].
The clinical picture is determined by the size,
location, and extension of the tumor and is quite
variable. It does not, however, always correlate
with tumor size: some large tumors may cause
relatively mild symptoms. Gait ataxia, trigeminal
nerve sensory loss, and headache are the most fre-
quent initial complaints. Most common present-
ing signs/symptoms are related to cranial nerve
palsies. Typical for meningiomas with this loca-
tion is the more severe involvement of the trigem-
inal nerve. The most frequent neurological deficit
in our series was trigeminal nerve dysfunction in
43 % of the patients, hearing loss in 43 %, ataxia
in 30 %, and abducens nerve palsy in 23 %.
Oculomotor and trochlear nerve symptoms had
3 % each. Understandably, patients with spheno-
petroclival meningiomas and petroclival menin-
giomas extending into the cavernous sinus have
more frequently extraocular motor dysfunction.
Petroclival Meningiomas
Later symptoms are gait ataxia, dysphagia,
and long-tract signs [114]. Due to the frequent
compression of the brain stem, long-tract signs
occur more frequently in petroclival than in CP
angle meningiomas: motor deficits have been
reported in 15–57 % and sensory deficits in
15–20 % of the patients with petroclival tumors
[112, 114, 136, 152, 159].
Management
A detailed radiological examination of the tumor,
including high-resolution thin-slice bone-window
CT, native and contrast-enhanced MRI, and MR
angio- and venography, should be performed
before surgery. Hearing evaluation is essential in
all patients. In extensive tumors with para- or
suprasellar extension, the hormonal status should
be evaluated with attention to the thyroid and cor-
tisol levels. Ophthalmologic examinations should
be also performed. In case of more caudal tumor
extension or existing swallowing dysfunction,
direct endoscopic examination of the vocal cords
and swallowing test are necessary.
DSA is generally substituted for by MR angio-
and venography. Vascular supply to petroclival
meningiomas is from arterial branches of the
internal carotid artery at the siphon and from the
external carotid artery via the ascending pharyn-
geal and middle meningeal arteries [126, 142].
These meningiomas displace typically the basilar
artery laterally away from the side of the tumor.
Rarely, the artery may be displaced dorsally or
ventrally—depending on the exact tumor origin.
In case the artery is encased within the tumor, no
displacement is visualized. Preoperative embo-
lization of the feeding vessels is rarely needed
with the exception of very large hypervascular
tumors.
Petroclival meningiomas are located at the cen-
ter of the skull base, adjacent to multiple cranial
nerves and vessels, which render their surgical
treatment with acceptable mortality and morbidity
rates extremely difficult. These tumors are
regarded as one of the most formidable challenges
in skull-base surgery. On the other hand, their
natural history is unpredictable and frequently
437
characterized by progressive growth and neuro-
logical deterioration [116, 157]. An attempt for
surgical removal is therefore justified. Van
Havenbergh et al. [157] studied the natural history
of 21 patients who were followed conservatively
by the senior author for a minimum of 4 years.
Radiological growth was demonstrated in 76 % of
the tumors, and in 63 % of them, neurologic dete-
rioration occurred.
The management of petroclival meningiomas
should be individualized, taking into account the
patient’s biological age, general medical condi-
tion, neurological symptoms and expectations, as
well as the tumor size and type. Treatment goal
should be complete resection of these benign
tumors, which is the only potentially curative
option. In case of large symptomatic tumors,
there are no alternatives to surgery. An inciden-
tally found small tumor in an older or medically
unstable patient, however, can be initially fol-
lowed clinically and radiologically. Patients with
contraindications for surgery or those refusing
operative treatment may be treated with primary
stereotactic radiosurgery or fractionated radio-
therapy [129, 141, 155, 158].
The extent of tumor removal is the most
important predictor of recurrences, and every
effort should be made to remove the tumor com-
pletely at initial surgery. Still, even after complete
tumor removal, the recurrence rates can be as
high as 4–26 % [114, 118, 132, 133, 152]. In one
large study of 150 patients with petroclival men-
ingiomas, the recurrence-free survival rate after
complete tumor removal was 100 % at 3 years,
but declined to 93 % at 7 years and to 85 % at 12
years [138]. Preservation of neurological func-
tions and quality of life after surgery has, there-
fore, the highest priority. Moreover, small
residuals are rarely biologically active, and their
progression course is often benign. In the study
of Natarajan et al. [138], further growth of the
remnant was observed in 5 % of the patients with
incomplete resection, compared to 4 % recur-
rence rate after complete tumor removal. Leaving
a small part of the tumor or of its capsule is con-
sequently reasonable if the attempt to remove it
completely would cause injury to essential neural
and vascular structures.
438 7 Meningiomas

This consideration is especially important in
case of small petroclival meningiomas (<3 cm):
their complete resection is potentially curable but
should not cause any major morbidity [139] (see
Fig. 7.22 and Case Illustration 7.11). In case the
risk of morbidity is higher, for example, in neuro-
surgical settings with less experience, stereotac-
tic radiosurgery, which leads to tumor control in
91 % in 5 and 86 % at 10 years [123], may be the
better option.

a b

c d

Fig. 7.22 Small petrous apex meningioma (53-year-old
female patient, no preoperative deficits). (a) T1-weighted
MR image following contrast administration. (b) Exposure
of the tumor via the retrosigmoid approach (Tu tumor, t
tentorium, SMT suprameatal tubercle). (c–e) Resection of
the suprameatal tubercle, complete removal of the tumor
and coagulation of its dural attachment (arrow trochlear
nerve, double arrows dura attachment, TS transverse
sinus, SS sigmoid sinus). (f) Postoperative MRI. The
patient had no new neurological deficits
Petroclival Meningiomas 439

e f

Fig. 7.22 (continued)

440
Case Illustration 7.11
Small Petroclival Meningioma
Fifty-seven-year-old male patient presented to
our outpatient department with complaints of
increasing double vision to the right side for 3
months. Neurological examination revealed
right-sided abducens nerve palsy. The MRI
study demonstrated a right-sided petroclival
meningioma, which caused slight brain stem
compression (a–c). The tumor occupied the
a b
(a–c) Small petroclival meningioma. Preoperative
T1-weighted MR images after contrast administration.
(d–i) Intraoperative images, illustrating the consecutive
steps of tumor removal. See the text for descriptions. (5
trigeminal nerve, 7–8 facial and vestibulocochlear
7 Meningiomas
upper 2/3 of the clivus and medially reached
the basilar artery.
Due to the progressive nature of the
abducens palsy, caused presumably by direct
tumor compression, a decision was taken for
operative management via the RS route. At
surgery, a large loop of the AICA was seen that
obstructed the approach to the trigeminal nerve
and to the tumor. The artery was extensively
dissected from the arachnoidea and displaced
nerve complex, * tumor, arrow abducens nerve, BA
basilar artery). Note the preserved petrous vein (arrow-
head). (j) Follow-up MRI 6 months after the surgery
(T1-weighted axial sequence after contrast
administration)
Petroclival Meningiomas
caudally, thereby opening enough working
space between the trigeminal and the seventh-
eighth cranial nerves (d). At the beginning, the
abducens nerve was identified—it was actu-
ally encased by the meningioma (e). The distal
part of the nerve at the Dorello’s canal and the
basilar artery were also identified (f). The
trigeminal nerve was slightly elevated by the
tumor, which had a small extension into the
Meckel’s cave. Once the relations of the men-
ingioma to all important surrounding struc-
tures were revealed, internal debulking was
initiated, followed by dissection of the capsule
d e
5
*
7-8
f g
5
BA
7-8
441
from the abducens nerve up to its
dural entry point (g, h). The meningioma
was removed completely, and its matrix,
located in the area of Dorello’s canal, was
coagulated (i).
The patient had no new deficits after sur-
gery. Histopathological diagnosis was menin-
gothelial meningioma WHO grade I. At
3-month follow-up examination, he reported a
significant improvement of the double vision,
and at 6 months, the function of the abducens
nerve was completely normal. The follow-up
MRI is presented on figure j.
5
*
7-8
BA
7-8
442 7 Meningiomas

h 5 i

7-8

j
Petroclival Meningiomas
Surgical Management
The history of petroclival meningioma surgery
reflects to a large extent the evolution of skull-
base surgery [113, 147]. Complete visualization
of the tumor with all surrounding neural and vas-
cular structures was regarded initially as a pre-
requisite for more radical and safer tumor
resection. Different extensive cranial base
approaches were elaborated. Malis [134] and
Mayberg and Symon [136] described the com-
bined supra-infra-transtentorial approach with
ligation of the transverse sigmoid sinus. Hakuba
[124] first described in the neurosurgical litera-
ture the surgical technique and results of the
transpetrosal–transtentorial route. In 1988, he
described his experience using the transpetrosal
approach for surgery of clivus meningiomas
[125]. The combined retrosigmoid–subtemporal–
transtentorial approach with or without ligation
of the sigmoid sinus was introduced later on
[112, 143, 154]. Complete or partial removal of
the petrous bone, which is the main anatomical
obstacle to good exposure of the petroclival area,
allows exposing widely the area of interest.
Transpetrosal techniques are directed either
through the surface of the temporal bone (supe-
rior approaches) or through the mastoid process
(lateral approaches) [110, 121, 156]. The senior
author introduced the intradural subtemporal
approach with resection of the petrous apex. The
epidural subtemporal approach was developed by
Kawase et al. and applied initially for accessing
the lower basilar artery and then for petroclival
and sphenopetroclival tumors [128, 131, 132].
The technique includes resection of the anterior
petrous bone medial to the IAC, behind the hori-
zontal segment of the petrous carotid artery and
lateral to the trigeminal nerve in Meckel’s cave.
Thus, the dura mater down to the level of inferior
443
petrosal sinus is exposed. Its opening and the
incision of tentorium to the tentorial incisura pro-
vide access to the petroclival region to the mid-
clival level. The surgeon’s field of view is crossed
only by the fourth cranial nerve medially and the
fifth nerve laterally.
The modifications of lateral cranial base
approaches—the presigmoid, retrolabyrinthine,
translabyrinthine, and transcochlear ones—differ
mainly in the amount of temporal bone removal
[121, 127, 137, 140, 148] . Their main advantages
are the shorter distance to tumor and surrounding
neurovascular structures; improved visualization,
especially of the anterior aspect of the brain stem;
and less brain retraction [121]. The presigmoid
approach combines a supra- and infratentorial
exposure with less amount of presigmoid petrous
bone resection. With the retrolabyrinthine
approach, the bone anterior to the sigmoid sinus
and posterior to the semicircular canals is drilled
off. The semicircular canals are skeletonized but
not opened, and hearing is preserved. The trans-
labyrinthine approach implies a complete mas-
toidectomy and labyrinthectomy, which increases,
respectively, the area of exposure. The transco-
chlear approach includes further resection of the
whole labyrinth and requires transposition of the
facial nerve, thereby allowing better visualization
of the structures anterior to the IAC. The trans-
petrosal approach with complete petrosectomy
was intended for large tumors in patients with
complete hearing loss. Other more extensive and
rarely applied approaches to the clival region
include the extended transbasal approach, the
midline approaches (transsphenoidal, transoral,
and transcervical), the infratemporal approach,
the facial degloving approach, and the anterior
transethmoid epidural petrous apex resection
[120, 122, 130].
444
Case Illustration 7.12
Presigmoid Approach
This is a case of a large petroclival meningioma,
causing severe compression of the brain stem,
operated in the late 1980s. The tumor extended
into the dilated Meckel’s cave and had a large
7 Meningiomas
supratentorial part (a). At that time, the senior
author favored the presigmoid approach with-
out ligation of sigmoid sinus for such tumors
(b). The tumor could be removed completely,
and all cranial nerve functions, including func-
tional hearing (c–e), were preserved.
Petroclival Meningiomas
a b
(a–e) Intraoperative images. (a) Preoperative MRI
demonstrating the size and extension pattern of this
meningioma. (b) The approach consists of a temporal
and retrosigmoid suboccipital craniotomies plus addi-
tional mastoid and partial pyramid resection (SS sig-
moid sinus, TS transverse sinus, T temporal lobe), (c)
Two retractors (R) are used to elevate the temporal
lobe and the cerebellar hemisphere thus affording a
wide exposure of the tumor (Tu). (d) Complete tumor
removal and preservation of all neurovascular
445
structures (5 trigeminal nerve, 7–8 facial and vestibu-
locochlear nerve complex, BS brain stem).
(e) Watertight reconstruction of the dura. Note the
intact transverse and sigmoid sinuses. (f) The postop-
erative bone-window CT demonstrates the extent of
bone resection. (g) Audiogram, showing that preserved
hearing (single arrowhead hearing before surgery,
double arrowhead hearing level after tumor removal).
(h) The patient 1 month after surgery
446 7 Meningiomas

e f

g
0.125 0.25 0.5 1 1.5 2 3 4 6 8 12
-10
-0
10
20
30
40
50
60
70
80
90
100
110
120
Petroclival Meningiomas
Growing experience with these extended
approaches showed, however, that they are
related to unacceptably high approach-related
mortality, mainly high rate of facial nerve palsy,
hearing loss, and CSF leaks. Another main dis-
advantage is the risk of injury to the highly
variable venous structures in the area [114, 117,
149, 151]. The interruption or even prolonged
stretching of the vein of Labbé, tentorial sinuses,
or any of the major draining veins may have
unpredictable and devastating consequences
(Fig. 7.23). On the other hand, as pointed out
earlier, even radiologically confirmed complete
resection does not always prevent recurrences.
Complete resection should be attempted,
447
therefore, only if the expected long-term mor-
bidity is minimal and the quality of life is pre-
served [135, 151, 159].
The senior author’s experience encompasses
more than 200 surgically treated patients with
petroclival meningiomas. Initially, he used the
frontotemporal or subtemporal approaches with
resection of the tentorium and petrous apex
(1970s). Later on, he introduced the combined
infratentorial supratentorial presigmoid approach
(see Case Illustration 7.12). The aforementioned
risks of venous injury or other morbidity were
considered unacceptably high and forced him to
change the operative concept and to abandon this
approach (see Case Illustration 7.13).
448 7 Meningiomas

a b

c d

Fig. 7.23 Venous phase of a DSA, showing the great variability of the venous system, in particular the vein of Labbé
Petroclival Meningiomas
Case Illustration 7.13
Massive Venous Infarction Following
Presigmoid Transtentorial Approach
This case presents a key experience that ulti-
mately led to change of the senior authors’
concept of operative management of petro-
clival meningiomas. The patient was 33 years
a b
(a, b) Preoperative MRI, demonstrating the size and
growth pattern of the tumor. (c) CT performed 2 days
after surgery when the first signs of neurological dete-
449
old and had a large right petroclival menin-
gioma with supratentorial extension and
growth into the Meckel’s cave (a, b).
Considerable compression of the brain stem
was evident on the MR images. The presig-
moid transtentorial approach was used to
remove the tumor completely. Despite the
rioration appeared; CT performed 1 week. (d) 4 weeks.
(e) After surgery
450
d e
adhesion of part of the tumor capsule to the
neural structures, they could be preserved. All
vascular channels were also intact at the end
of surgery. The patient awoke rapidly and was
extubated. In the first 24 h, she did not have
any deficits.
On the second postoperative day, she lost
consciousness and developed left-sided central
hemiplegia and facial palsy. CT demonstrated
7 Meningiomas
massive venous infarction with intracerebral
hemorrhage in the temporal lobe, presumably
due to thrombosis of the vein of Labbè. The
patient was re-intubated, and appropriate treat-
ment was initiated. Over the following 4
weeks, she recovered completely and at dis-
charge had no neurological deficits. The CTs,
shown on figures c–e, demonstrate the pro-
gressive recovery from the venous infarction.
Petroclival Meningiomas
Tumors that are confined to the petroclival
area are removed via the simple RS approach.
Large meningiomas have already displaced the
brain stem, so that the RS approach provides ade-
quate access even to the contralateral side or
supratentorially (Figs. 7.24 and 7.25). Resection
a b
Fig. 7.24 Petroclival meningioma, operated in the 1980s
by MS via the retrosigmoid suprameatal approach. (a) CT
of the tumor. (b) Initial view of the tumor, dislocating the
seventh-eighth nerves laterally and the trigeminal nerve
451
of the tentorium provides access to meningiomas
with supratentorial extension that are limited to
the middle cranial fossa and do not involve the
Meckel’s cave or the cavernous sinus. If addi-
tional exposure is required, however, the petrous
apex may be resected. In 1982, the senior author
superiorly. * suprameatal tubercle. (c) Complete tumor
removal. Note the extent of removal of the suprameatal
tubercle and the wide exposure of the trigeminal nerve
into the Meckel’s cave
452 7 Meningiomas

introduced the technique of the intradural resec- Currently, this is our favored approach in petro-
tion of the petrous apex via the retrosigmoid clival meningiomas. Endoscopic inspection is
route—the so-called retrosigmoid suprameatal increasingly being used to inspect hidden areas
approach (RSMA) or Samii approach (Fig. 7.24). for tumor remnants (see Case Illustration 7.17).

a b

c d

Fig. 7.25 Giant petroclival meningioma with very large (c, d). The early postoperative MRI control image is
supratentorial part (a, b), removed via the simple retrosig- shown on figures e, f
moid approach with additional resection of the tentorium
Petroclival Meningiomas
e f
Fig. 7.25 (continued)
Thereby, the amount of bone resection or the
need for retraction of neural structures to visual-
ize a given area can be reduced.
In particularly large tumors extending into the
suprasellar and parasellar areas that engulf the
optic nerve and carotid artery, we prefer operat-
ing in stages using two simple approaches [146]
(Figs. 7.26 and 7.27). Initially, the infratentorial
tumor part in CP angle, clivus, and/or Meckel’s
cave is removed via a retrosigmoid suprameatal
approach, and the brain stem is decompressed.
Thus, the risk of severe neurological deteriora-
tion is prevented. The remaining supratentorial
component is removed via the frontotemporal
approach at a second stage. Planned two-staged
meningioma removal was performed in 17 % of
the recently operated patients. The two-stage sur-
gery affords more radical tumor removal and
allows adequate decompression of the optic
nerve. Furthermore, it is associated with less
approach-related morbidity than the alternative
extended cranial base approaches. Removal of
the cavernous sinus component of such tumors
has never been the goal in patients with intact
oculomotor function in order to prevent func-
tional deficits. Therefore, total resection was
453
achieved in 3.4 % of those meningiomas with
cavernous sinus infiltration [147].
Retrosigmoid Suprameatal (Samii)
Approach: Technique
The retrosigmoid suprameatal approach (RSMA)
consists of the simple RS craniotomy and addi-
tional intradural resection of the petrous apex
[145, 149]. The drawbacks related to the alterna-
tive approaches to petrous apex, such as exten-
sive petrous bone resection or retraction of the
temporal lobe with the associated risks of dam-
age to neural and vascular structures, are thus
avoided. Initially the tumor bulk is removed via
one of the CP angle levels: the upper level
between the tentorium and the trigeminal nerve,
the second level between the trigeminal and the
facial-cochlear nerves, the third level between
the facial-cochlear nerves and the lower nerves,
and/or the lowest level, defined by the lower cra-
nial nerves and foramen magnum. Although ana-
tomically these spaces may be relatively narrow,
the meningioma usually widens some of them
and provides adequate working corridor. Tumor
removal should start first at the most expanded
level with the widest distance between the
454 7 Meningiomas

respective structures. Initial internal decompres-
sion is performed using an ultrasonic surgical
aspirator at the appropriate levels. In petroclival
meningiomas, as in almost all other CP angle
tumors, the principle that the dissection of tumor
capsule should be performed only after sufficient
internal decompression is achieved must be
always followed.
Once the accessible tumor portion is removed,
the bone located above and anterior to the IAC—
the suprameatal tubercle—is drilled off, provid-
ing access to the petroclival area and Meckel’s
cave (see Case Illustrations 7.14 and 7.15). The
dura in the area is incised circumferentially and
excised. Bone removal is performed with high-
speed drill with diamond spurs of decreasing

a b

c d

Fig. 7.26 Two-staged removal: retrosigmoid suprameatal
and frontotemporal approach. This meningioma (a, b) was
removed in two stages: at the first stage, the CP angle part
was resected, and the brain stem was decompressed (c, d).
The supratentorial part, which compressed the optic nerve
and partially encased the ICA, was resected at a second sur-
gery via the frontotemporal approach (e, f). The cavernous
sinus tumor part was not removed but at follow-up showed
no growth tendency (Reprinted from: Samii et al. [146].
Copyright © 2010, with permission from Springer-Verlag)
Petroclival Meningiomas
e f
Fig. 7.26 (continued)
size, similar to the technique used to open the
IAC. Generally, the amount of bone resection
depends on the individual anatomical character-
istics of the petrous bone and the tumor exten-
sion. It has been estimated that drilling of the
suprameatal tubercle increases the exposure up to
13.0 mm [117]. Opening the Meckel’s cave and
mobilizing the trigeminal nerve increase the
working angle further. Structures at risk during
the petrous apex resection are the petrosal seg-
ment of the internal carotid artery anterolaterally,
the superior petrosal sinus, and the superior semi-
circular canal laterally. The exposure of the ros-
tral tumor portion can be further enlarged by
incision and retraction of the tentorium. Care
should be taken to avoid injury to the trochlear
nerve: it should be identified prior to the incision
of tentorium.
The abducens nerve is located typically within
the tumor or is stretched anteriorly. With the
RSMA, the abducens nerve can be identified
early at its brain stem exit zone and followed dur-
ing tumor removal up to Dorello’s canal, thus
reducing the risk of its inadvertent injury. From a
455
lateral perspective, such as the one provided by
the Kawase approach, the nerve is hidden by the
tumor and is visualized at a later stage, which
increases the risk of its iatrogenic injury. The
RSMA provides access to Meckel’s cave, the pet-
roclival area, and the middle fossa including the
posterior cavernous sinus. Following tumor
removal and thorough hemostasis, the drilled
area of the petrous apex is sealed with multiple
pieces of fat tissue and fibrin glue.
Notably, 33 % of all petroclival meningiomas
in our series presented with signs of peritumoral
edema, which—as discussed previously—fre-
quently indicates invasion of the pial sheet [115].
Such tumors should be operated with utmost
care: in case a dissection plane does not exist,
small part of the tumor or tumor layer might be
left to preserve the microvasculature and/or to
avoid direct injury to the brain stem or cranial
nerves (10–20 % of all cases). Importantly, peri-
tumoral edema seen on the MRI does not always
correlate to infiltrative or invasive tumor growth:
this issue can be determined only at surgery
(Figs. 7.28 and 7.29).
456 7 Meningiomas

a b

c d

Fig. 7.27 This 41-year-old man presented with a history
of progressive visual disturbance, episodic headache,
chronic fatigue, and polyuria. The MRI (a–c) showed a
large meningioma of the upper clivus, extending retro-
and suprasellar. Initially, the lower tumor part was
removed via a retrosigmoid approach (d, e), and the brain
stem was decompressed. The immediate postoperative
images are shown on figures f, g. One week later, the sec-
ond stage was performed: the remaining tumor was
removed completely via a right frontolateral craniotomy
(h, i). Immediate postoperative MRI (j, k) and photo of
the patient (l). The patient had minimal functional impair-
ment of the right oculomotor nerve, which improved sub-
sequently later on
Petroclival Meningiomas 457

e f

Fig. 7.27 (continued)

458 7 Meningiomas

j k

Fig. 7.27 (continued)

Petroclival Meningiomas 459

a b

c d

Fig. 7.28 The presence of brain stem and/or cerebellar
edema does not necessarily mean that the meningioma
invades the pia mater. On this figure, we present a patient
with a petroclival meningioma, causing extensive brain
stem edema (a, b) that is best seen on the T2-weighted
sequences (c, d) and on the FLAIR sequence (e).
Nevertheless, a good arachnoid dissection plane could be
identified, and the tumor was removed completely without
any injury to the neural and vascular structures. The
patient did not have any new neurological deficits after
surgery. The postoperative CT examination, performed 10
days after surgery, is shown on figure f
460 7 Meningiomas

e f

Fig. 7.28 (continued)

Petroclival Meningiomas
a b
Fig. 7.29 Patient with a large petroclival meningioma,
extending into the Meckel’s cave and posterior cavernous
sinus. The patient presented with signs of intracranial
pressure due to obstructive hydrocephalus and was stupor-
ous, and an EVD was immediately placed. The CT and
MRI images at admission are presented on figures a–f.
Note the extensive perifocal edema and the occlusion of
the fourth ventricle. The patient was operated 1 day later,
461
and the infratentorial tumor part could be removed com-
pletely via a retrosigmoid suprameatal approach. All cra-
nial nerves were preserved. The early postoperative CT
(g) shows minimal blood collection in the tumor bed and
opened fourth ventricle. (h) CT at discharge. The patient
was discharged 14 days after surgery in a very good con-
dition without neurological deficits
462 7 Meningiomas

e f

g h

Fig. 7.29 (continued)

Petroclival Meningiomas
Case Illustration 7.14
Retrosigmoid Suprameatal Approach 1
This 48-year-old female patient reported on
an episode of hearing loss on the right side
that occurred 7 years earlier. Hearing then
recovered completely. Two months later,
however, she had a further similar episode.
MRI of the head was performed and demon-
strated a large CP angle tumor. At admission,
the patient did not have neurological deficits
besides slight imbalance and numbness of the
right half of the face. Audiogram surprisingly
revealed normal hearing levels on both sides.
MRI examination demonstrated a relatively
large tumor with broad base to the posterior
463
pyramid surface and extension deep into the
IAC and into the Meckel’s cave (a–d).
The patient was operated via the retrosig-
moid suprameatal approach—all essential
steps are illustrated on figures e–v. The men-
ingioma, which was found to originate from
the dura medial to the trigeminal nerve, could
be removed completely, and all neural and
vascular structures were preserved. After sur-
gery, the patient had an incomplete abducens
nerve palsy and facial nerve palsy HB grade
IV on the right side, which showed a gradual
tendency to improve and recovered completely
within 12 months.
464
a b
T1-weighted contrast-enhanced MR images of the
meningioma (a, b). T2-weighted sequences demon-
strating clearly the extent of intracanalicular tumor
growth (c, d). Initial view of the tumor, as seen via
the RS exposure (e). The transverse and sigmoid
sinuses are the limits of the craniotomy. CSF has
been drained, and once the tension decreased
sufficiently, the cerebellum was slightly retracted.
(R retractor, T tentorium, p posterior pyramid, Tu
tumor). (f) Gradual debulking of the tumor above
the seventh-eighth nerve complex. (g) The retro-
suprameatal tumor part has been removed. The
planned extent of opening of the IAC is indicated
with dotted dark line. (h) The posterior wall of the
IAC has been drilled off, and a part of the IAC is seen
in the middle. (i) In contrast to VS, the drilling is con-
tinued above the IAC, thereby exposing the hidden
premeatal tumor part. (j) The area of exposure gained
by the additional drilling of the suprameatal tubercle
is demarcated with a white dotted line. (k, l) The
trigeminal (5) and the trochlear (4) cranial nerves
were dissected from the tumor capsule. (m) The
7 Meningiomas
removal of the most medial tumor part allows visual-
ization even of the oculomotor nerve (3). (n) Further
opening of the IAC, up to its fundus. The main intra-
canalicular tumor bulk was anterior to the seventh-
eighth nerve complex. (o) The most lateral tumor part
was removed with an angled microhook with a tech-
nique similar to the one used in VS. (p) The abducens
nerve (6) was also dissected from the capsule—from
its exit zone to the Dorello’s canal. Tumor origin site
or matrix was found to be located medial to the IAC,
lateral to the trigeminal nerve (*). (q) Drilling off the
suprameatal tubercle allowed for access and removal
of the tumor from the Meckel’s cave (two arrow-
heads). (r) Removal of the blood cloths in the opera-
tive area and final hemostasis (BA basilar artery). (s)
Final view of the CPA, demonstrating the complete
tumor removal and preservation of all neurovascular
structures (LCN lower cranial nerves). (t) The drilled
area of the petrous bone is sealed off with fat pieces
and fibrin glue. (u) Dural closure—2 or 3 sutures are
placed to adapt the dura, and then an interrupted
suture is made to achieve a watertight closure (v)
Petroclival Meningiomas 465

e f

Tu

Tu
P

7-8

g h
T

Tu

7-8
466 7 Meningiomas

i j

Tu

IAC
7-8

k l

BS
Petroclival Meningiomas 467

m n

o p

6
468 7 Meningiomas

q r

BA

s t

7-8

LCN
Petroclival Meningiomas 469

u v
470 7 Meningiomas

Case Illustration 7.15 decompression and resection of the menin-

Retrosigmoid Suprameatal Approach 2
Fifty-four-year-old male patient presenting
with very mild symptoms: slight hearing loss
on the right side (35 dB). The preoperative
MRI is shown on figures a–f. Considering
the fact that the major tumor part was
infratentorial and caused severe compression
of the brain stem, the retrosigmoid approach
was selected. All vessels and cranial nerves
were hidden by the huge tumor and could
not be identified initially. Careful internal
gioma were started. The facial nerve was
identified by electrostimulation: it was dislo-
cated caudally. Therefore, the upper tumor
part was further removed, which required
resection of the suprameatal tubercle. When
the tumor was sufficiently decompressed
internally, indicated by the appearing brain
stem pulsations, careful dissection of its cap-
sule from the neural structures was per-
formed. The supratentorial tumor part could
be removed through the tentorial incisura.

a b

c d

(a–f) Preoperative MRI of a large petroclival menin- after contrast administration. (b) T2 sequence. (c, f)
gioma. The tumor extends into the IAC and partially TOFF sequence. (g–i) MRI control study 12 months
into the jugular foramen (d, f) and has a large supraten- after surgery
torial portion. (a, d, e) T1-weighted MR sequences
Petroclival Meningiomas 471

The patient recovered rapidly from surgery. leak occurred at the third postoperative day
He had no new neurological deficits; the hear- but was successfully treated with a lumbar
ing was preserved but nonfunctional. CSF drainage for 1 week.

g h

i
472
Outcome
Early postoperative MRI examination is war-
ranted to verify the amount of tumor resection.
This information is essential as a basis for further
patient management. The main reasons for subto-
tal removal of petroclival meningiomas are the
following: lack of dissection plane and/or en
plaque tumor growth with infiltration of cranial
nerves or brain stem; fibrous tumor type with
hard consistency; encasement or infiltration of
major vessels (in particular of the perforating
arteries); and cavernous sinus invasion [115, 133].
In case of incomplete resection, follow-up MRI
studies are performed 3–6 months after surgery,
and if the residual tumor demonstrates definitive
growth tendency, radiosurgery/fractionated radio-
therapy is recommended.
The outcome of surgery depends mainly upon
the location and consistency of the tumor and to a
lesser extent upon its size. As noted by different
authors, despite the modern cranial base
approaches, refined microsurgical techniques,
and reliable neurophysiological monitoring, the
surgical morbidity may still be high and is com-
monly related to cranial nerve deficits and pul-
monary embolisms [111, 114, 118, 152]. The
nerves at highest risk for iatrogenic injury, espe-
cially in meningiomas extending supratentorially,
are the trochlear nerve, followed by the cochlear,
7 Meningiomas
trigeminal, facial, and oculomotor nerves.
Meningiomas with major inferior growth toward
the jugular foramen and foramen magnum (see
Case Illustration 7.16) are associated with worse
postoperative outcome due to the involvement of
lower cranial nerves and the related neurological
dysfunction [115]. Best outcome, in respect to
radicality of tumor removal and avoidance of
neurological morbidity, can be achieved during
the first operation. Previous surgery or radiother-
apy, besides obliterating the arachnoid planes,
renders the neural and vascular structures more
vulnerable to iatrogenic injury.
In the seniors’ author series, the preoperative
abducens palsy improved or resolved completely
in 29 % of the patients. The rate of recovery of
preoperative oculomotor or trochlear palsy was
much lower. The most frequent new postopera-
tive extraocular muscle impairment was double
vision due to trochlear nerve (7 %), abducens
nerve (3 %), or oculomotor nerve (3 %) dysfunc-
tion [115]. CSF leak that required surgical repair
occurred in one patient. In one patient, hydro-
cephalus developed on the tenth postoperative
day, and a VP shunt was implanted. The periop-
erative mortality in the group was 0 %.
The management of recurrences is discussed
in the appropriate section of the chapter on CP
angle meningiomas.
Petroclival Meningiomas 473

Case Illustration 7.16 years. The neurological examination at admis-

Large Petroclival Meningioma with Extension
into the Jugular Foramen
The patient (48-year-old female) presented to
our outpatient department with a history of
progressive hearing loss and sporadic swal-
lowing difficulties and double vision since 3
sion did not reveal any abnormalities, besides
a mild hearing deficit—PTA of 30 dB. The
MRI and CT are shown on figures a–e.
She was operated via the RS approach. The
meningioma was located medial to the
seventh-eighth nerves and the lower cranial

a b

c d

(a–e) T1-weighted MRI after gadolinium administra-
tion, showing the meningioma in the left CP angle. The
typical intense contrast enhancement is obvious. The
tumor extends inferior to the level of the foramen mag-
num and has a supratentorial extension in the cavern-
ous sinus. Small tumor extension in the jugular foramen
is well seen on figures c and d. (f–l) Intraoperative
images of different stages of tumor removal (see the
description in the text). Tu tumor, P pyramid, C cere-
bellar hemisphere, 5 trigeminal nerve, 7–8 facial and
vestibulocochlear nerve complex, LCN lower cranial
nerves, PV petrous vein, R retractor. (m, n) The patient
had relatively large pneumocephalus, visualized on the
early postoperative CT examination (m CT scout scan
of the head, n CT axial scan). (o–q) Postoperative
T1-weighted MR images (after contrast administra-
tion) showing complete removal of the CP angle tumor
part and the tumor remnant in cavernous sinus
474 7 Meningiomas

nerves and extended from the tentorium to the
base of the PCF, caudally to the lower cranial
nerves (f). The tumor had relatively firm con-
sistency, was well vascularized, and was
strongly bleeding. It was initially debulked via
the most expanded level—the one between the
seventh-eighth nerves and the lower cranial
nerves (g). The neural structures were not
infiltrated, and the lower cranial nerves could
be gradually dissected from the tumor. Then
the removal proceeded via the upper level—
above the seventh and eighth nerves (h–i). The
petrous vein determined the upper limit of the
level in this particular case. The abducens
nerve was engulfed by the tumor and dislo-
cated, so that it could be visualized through
the level between the seventh and eighth and
the lower cranial nerves (j). Despite the strong
bleeding and its consistency, the tumor could
be further removed until the abducens, the
trigeminal, and the seventh-eighth nerves were
dissected free. The tumor part in the jugular
foramen could be removed with angled micro-
instruments. The attachment of the tumor,
which was in the area of the jugular foramen,
was coagulated (k). All neural and vascular
structures, including the petrous vein, were
preserved (l).

e f

g h
Petroclival Meningiomas 475

The postoperative course was uneventful.
Although relatively large pneumocephalus was
seen on the early postoperative CT (m, n), the
patient had no major related symptoms besides
some headache and nausea. He was mobilized
and transferred to a normal ward in 48 h. MRI
examination was done later and proved the
complete removal of the CP angle tumor part
(o–q). At discharge, the only deficit she had
was a worsened hearing on the left side. A rec-
ommendation was given to follow the cavern-
ous tumor portion with an MRI in 6 months.

i j

k l
476 7 Meningiomas

m n

p q
Petroclival Meningiomas
Case Illustration 7.17
Endoscope-Assisted Removal of Petroclival
Meningiomas
Fifty-six-year-old female patient presented
with progressive headache, trigeminal nerve
sensory loss (V1,2), absent corneal reflex, and
hypacusis. The MRI findings are demonstrated
on figures a, b. The patient was operated via
the RS approach (see figures c–s). Endoscopic
control was used to verify the completeness of
477
tumor removal: a small remnant along the
abducens nerve was identified that was not
visualized with the microscope (l–q). In this
case, the remnant was removed with endo-
scopic guidance.
The postoperative period was completely
uneventful. A slight abducens palsy appeared
early after surgery but until discharge resolved
completely. Hearing level was similar to the
preoperative.
478
a b
c d
(a) Preoperative MR images demonstrating the loca-
tion, size, and extension of the tumor (T1-weighted
sequences after gadolinium administration). (b)
Intraoperative images. (c) Standard RS craniotomy
was performed exposing the edges of the transverse
and sigmoid sinuses. The dura was incised along the
sinuses. (d) Elevation of the cerebellar hemisphere and
draining of CSF from the lateral cerebellomedullary
cistern. (e) The retractor (R) is placed to support the
hemisphere, and the tumor (Tu), lying medially to the
fifth and seventh-eighth cranial nerves, is exposed (T
tentorium). (f) Tumor debulking performed initially
via the level determined by the tentorium and the
trigeminal nerve. (g, h) Further tumor removal via the
second level—between the fifth and the seventh-eighth
nerves. (i) Removal of the small intracanalicular tumor
part: in this particular case, there was no need of wide
7 Meningiomas
opening of the IAC. (j, k) Presumed complete tumor
removal. The basilar artery (BA), the abducens nerve
(6), and the preserved petrous vein (arrowhead) are
well seen. (l) Endoscopic inspection demonstrating
the whole course of the trigeminal nerve. (m) The
endoscope is advanced above the trigeminal nerve: the
petrous apex area (PA), oculomotor nerve (3), and
brain stem (B) are visualized. No residual tumor is
seen in this area. (n) The tumor in the area of the IAC
(double small arrows) has been also removed. (o)
Then, the endoscope was directed to the lower level:
between the seventh-eighth and the lower cranial
nerves (LCN). (p) A small remnant surrounding the
distal part of the abducens nerve (6) in the area of
Dorello’s canal was visualized and removed under
direct endoscopic control (q). (r) Fat plug in the drilled
area of the petrous bone. (s) Watertight dural closure
Petroclival Meningiomas 479

e f

g h
480 7 Meningiomas

i j

k l

m
Petroclival Meningiomas 481

o p

q r

s
482
Foramen Magnum Meningiomas
Meningiomas are the most common benign
tumors of the foramen magnum. Foramen mag-
num meningiomas account for 1.8–3.2 % of all
meningiomas and for 6–7 % of all posterior fossa
meningiomas [160, 161, 166, 172]. They arise
primarily within the foramen magnum area or
involve it secondarily. Most frequently—in
52–4 % of the cases—the tumor occupies the
anterolateral part of the foramen. Anteriorly
located are 4–32 % of the tumors, posterolateral
are 10–20 %, and posterior are 5–7 % [161, 165,
167, 168].
The foramen magnum is a component of the
occipitocervical junction—a complex biome-
chanical entity of ligaments, joints, and muscles
connecting the atlas, axis, and occipital bone
[176]. Its limits are the following: lower third of
the clivus and upper margin of the C2 vertebral
body anteriorly, jugular tubercle down to the
upper margin of the C2 lamina laterally, and ante-
rior edge of the squamous occipital bone to the
spinous process of C2 posteriorly [169]. Relevant
neural structures in the area are the following:
lower cranial nerves (9–12th), cerebellar tonsils,
inferior vermis, fourth ventricle, caudal aspect of
brain stem, rostral aspect of spinal cord, and
upper cervical nerve roots (C1 and C2) [165].
The most important vascular structures in the
area are: the vertebral arteries (VA), the posterior
inferior cerebellar arteries (PICAs), the anterior
and posterior spinal arteries, the meningeal
branches, and the veins and dural sinuses at the
occipitocervical junction. Detailed knowledge of
the microanatomical relations between these
structures and of their physiological significance
is of enormous importance for planning and per-
forming safe surgeries. Both the normal anatomy
and the possible variations should be considered.
An important landmark in the foramen magnum
area is the dentate ligament, whose sectioning
increases the exposure to the area anterior to the
spinal cord. This ligament is attached to the pia
mater between the dorsal and ventral rootlets
medially and to the dura mater laterally [175].
The dural blood supply to foramen magnum
meningiomas is usually from branches of the
7 Meningiomas
posterior and anterior meningeal arteries, as well
as from meningeal branches of the ascending
pharyngeal and occipital arteries.
General Features
The clinical presentation of foramen magnum
meningiomas is nonspecific, and the mean dura-
tion of symptoms prior to diagnosis is usually
more than 2 years. Frequent initial complaints are
occipital headache and upper cervical pain, which
may be exacerbated by neck movements or
Valsalva maneuvers. Meningiomas that extend to
the foramen magnum secondarily with a major
intracranial portion cause brain stem, cerebellar,
and lower cranial nerve signs and symptoms.
High cervical meningiomas cause typically
symptoms and signs related to the upper cervical
spinal cord and roots. More extensive tumors
may present with a mixed clinical picture [165].
The typical “foramen magnum syndrome” is
rarely seen in its full form: patients present gen-
erally with varying degrees of sensory and motor
loss. Long-tract signs with spastic gait are fre-
quent findings. Atrophy of the intrinsic hand
muscles may be found on the side of the lesion,
probably as a result of vascular changes (com-
pression of the anterior spinal artery or venous
obstruction) or syringohydromyelia. In later
stages, the attempt to avoid pain by holding the
head in fixed position leads to a clinical picture
resembling torticollis.
Management
Preoperative Examinations
The preoperative neuroradiological examinations
are similar to those in other CP angle menin-
giomas. The stability of the occipitocervical
junction should be studied on dynamic X-rays
(flexion and extension) of cervical spine. The
vascular anatomy, which is surgically relevant, is
appreciated on MR angiography and MR venog-
raphy. Encasement and narrowing of the verte-
bral artery suggest that the adventitia of the artery
may be invaded. Encasement of the artery is
Foramen Magnum Meningiomas
found intraoperatively in 40–48 % of cases in
different series. Superselective embolization of
arterial feeders may be useful in highly vascular
meningiomas. The function of the lower cranial
nerves should be examined with direct endo-
scopic examination of the vocal cords and swal-
lowing test.
Principles of Surgery
Surgery in the foramen magnum area can cause
significant functional impairment and may be
related to a prolonged recovery period. The deci-
sion on the optimal management should be taken
together with the patients and their families.
Important factors to consider are the following:
patients’ expectations, psychological and social
needs, neurological status, and tumor size, exten-
sion, and relation to adjacent anatomical struc-
tures. The optimal management is complete
tumor removal with preservation of neurological
functions and avoidance of craniocervical insta-
bility. Radiotherapy/radiosurgery may be consid-
ered in patients with small tumors and severe
somatic diseases, precluding prolonged surgery
in general anesthesia or in those unwilling sur-
gery. In incidentally discovered small tumors, the
option of initial observation period has to be dis-
cussed with the patient.
Posterior-located meningiomas are approached
via a medial suboccipital craniectomy and—if
necessary—a C1/C2 laminectomy. The approach
affords bilateral access to approximately 120° of
the foramen magnum [174]. Anterior, anterolateral,
and posterolateral meningiomas are approached
via posterolateral approaches, which differ in the
degree of bone removal. Our favorite lateral sub-
occipital approach consists of a retrosigmoid
craniectomy and a C1 hemilaminectomy/lamine-
ctomy [178, 179]. More extensive approaches
have been developed to provide wider surgical
exposure and to enhance the feeling of safety to
the surgeon. Their common features are the
483
following: wide exposure and even transposition
of the extradural vertebral artery, exposure of the
transverse process of C1 and of the atlantooc-
cipital joint, and varying degrees of occipital
condyle removal. The occipital condyle may be
resected partially or entirely and may be com-
bined with removal of the lateral mass of the
atlas lateral to the dural entrance of vertebral
artery, partial mastoidectomy, or even sectioning
of the sigmoid sinus [163, 164, 171–173]. Wide
exposure of the tumor and its dural attachment is
thus achieved.
The benefits of such extensive approaches,
however, have to be carefully weighted against
the risk of higher morbidity, related to vascular
complications, injury to cranial nerves, prolonged
surgery, or CSF leak. Another drawback is the
potential risk of craniocervical instability that
may require an additional fusion procedure, espe-
cially in case of resection of more than 75 % of
the condyle [162, 180, 181].
The experience of the senior author with
almost all these approaches showed that in nearly
all foramen magnum meningiomas, such wide
exposure is not required [177, 179]. Notably, the
wide bone removal does not provide any addi-
tional advantages for the difficult operative steps,
such as dissection of the tumor from the brain
stem, lower cranial nerves, or vessels. Exceptions
are the rare strictly anterior located very small
meningiomas: resection of the posterior third of
the condyle may be needed to expose them ade-
quately. The lateral suboccipital approach, how-
ever, is sufficient in case of larger anterior foramen
magnum meningiomas (Figs. 7.30 and 7.31). The
rate of permanent surgical morbidity in series
when the lateral suboccipital approach was used
ranges from 0 to 8 %, and the mortality rate ranges
from 0 to 6.1 %, respectively [161, 170, 179]. In
case a transcondylar approach was used, the sur-
gical morbidity rate is 0–60 % and the mortality
rate is 0–29 % [160, 166, 169, 172].
484 7 Meningiomas

a b

c d

Fig. 7.30 Anterior foramen magnum meningioma, oper-
ated by MS more than 20 years ago. (a, b) Preoperative
MR images. (c) View of the tumor after sectioning of the
dentate ligament. The spinal part of the accessory nerve
and the C1 root is well visible. (d) Exposure of the verte-
bral artery and complete tumor removal (e). The CT scans
(f, g) demonstrate the size of the craniotomy and the open-
ing of the foramen magnum: the exposure was sufficient
to remove the tumor without undue risks
Foramen Magnum Meningiomas 485

e f

Fig. 7.30 (continued)

486 7 Meningiomas

a b

c d

Fig. 7.31 Large meningioma of the lower clivus extend- approach. (a, b) Preoperative MR images. (c, d) Post-
ing to the foramen magnum area. Complete tumor removal operative images: complete tumor removal was achieved,
could be safely achieved via the lateral suboccipital and all neurological functions were preserved
Foramen Magnum Meningiomas
Lateral Suboccipital Approach to the
Foramen Magnum Meningiomas
The approach is based on the retrosigmoid cran-
iotomy, as described in the corresponding chap-
ter. Here we will focus only on the differences
relevant for surgery of foramen magnum menin-
giomas (see Case Illustrations 7.18 and 7.19):
• The approach is performed on the side of
dominant tumor growth.
• A slightly curved skin incision medial to the
mastoid process is made, which has to be
extended caudally.
• The suboccipital muscles and fasciae are cut
in line with the skin incision, and the C1 arch
on the side of the tumor is exposed.
• The vertebral artery is identified and followed
to its entry point in the dura but is not
mobilized.
• Retrosigmoid craniectomy is performed
using bone rongeurs and high-speed drill,
exposing 1–2 mm from the sigmoid sinus.
The foramen magnum is opened widely.
Usually, in foramen magnum meningiomas,
there is no need to expose the transverse
sinus. Exceptions are meningiomas with a
large CP angle portion; in such cases, the
a b
Fig. 7.32 Extensive posterior fossa meningioma located
in the left CP angle extending caudally below the level of
foramen magnum. (a, b) Preoperative MR images. (c–e)
Intraoperative images. (c) Initial exposure of the tumor.
(d) Removal of the CP angle tumor portion. (e) Removal
487
superior extension of bone opening should
reach the sinus (Fig. 7.32).
• Depending on the tumor extension, the C1
lamina and even the C2 lamina have to be
removed laterally enough. Dissection or trans-
position of the vertebral artery in order to gain
more lateral exposure is unnecessary because
the opening of the dura is always medial to
the artery.
• The dura is incised in a curvilinear manner
just 1.5–2 mm medial to the sigmoid sinus,
and the incision is continued in the midline
down to C1 or C2. At the spinal level, stay
sutures are placed on both sides and fixed to
the muscles in order to open the dura.
Tumor Removal
The Tumor relation to the major neurovascular
structures—vertebral arteries, posterior spinal
arteries and PICA, Cl nerve root, and lower cra-
nial nerves—cannot be predicted before surgery.
Their early identification helps to avoid iatro-
genic injury but is possible only in smaller men-
ingiomas. Tumor removal starts always with
careful internal debulking, alternated with dissec-
tion of the capsule in the arachnoid plane. The
of the lower part of the tumor in the area of foramen mag-
num (Tu tumor, BS brain stem, 7–8 facial and vestibuloco-
chlear nerve complex, LCN lower cranial nerves, VA
vertebral artery). (f, g) Postoperative MR study, demon-
strating the complete tumor removal
488 7 Meningiomas

Fig. 7.32 (continued)

c

e
Foramen Magnum Meningiomas
f g
Fig. 7.32 (continued)
anterior exposure is increased by dividing the
dentate ligaments or even sectioning of the first
cervical nerve root distal to its connections with
the accessory nerve. Meningiomas located medial
to the glossopharyngeal, vagus, and accessory
nerves are accessed via the spaces between the
nerve fascicles (Figs. 7.33 and 7.34). These
spaces are frequently expanded by the tumor
itself. More rostral tumor parts may be accessed
via the space between the lower margin of the
vestibulocochlear/ facial nerves and the upper
roots of the glossopharyngeal nerve. Large men-
ingiomas usually cause posterior and contralat-
eral displacement of the brain stem and upper
cervical spine, creating sufficient surgical corri-
dor for their safe resection (Figs. 7.33 and 7.34).
During tumor removal, care should be taken not
to rupture the contralateral vertebral artery, which
is usually identified at a later stage (Fig. 7.35).
Radical excision of the dura in the area is impos-
sible; thorough coagulation of the tumor matrix/
attachment is therefore recommended.
Outcome
The experience of the senior author encompasses
more than 130 foramen magnum meningiomas.
Several parameters have been found to correlate
489
with the outcome and the possibility for complete
removal: tumor type (encapsulated or en plaque),
invasion of the pia of the brain stem/spinal cord
vs. of the cranial nerves or presence of a good
arachnoid plane, encasement of major artery, and
previous surgery or radiotherapy [179]. Complete
removal could be achieved in just 7 % of en
plaque meningiomas: in 86 % of them subtotal
removal, and in 7 %, only partial removal was
safely possible. In encapsulated meningiomas,
complete removal could be achieved in a much
higher rate—88 %. See Case Illustrations 7.18
and 7.19.
Best results are achieved at initial surgery:
complete removal was possible in 76 % of all pri-
mary surgeries and in just 46 % of the reopera-
tions. The encasement of a major artery was
another independent factor for the completeness
of tumor removal. In case such encasement
existed, complete tumor removal was feasible in
33 %, while if it was absent, the rate increased to
90 %. These parameters, especially en plaque
tumor type, surgery of recurrence, or after previ-
ous radiotherapy, were found to correlate also
with the complication rate. The complication rate
increased from 14 % in encapsulated menin-
giomas to 33 % in the en plaque type.
490 7 Meningiomas

a b

Fig. 7.33 Large foramen magnum meningioma (a–c), removal, the PICA is dissected free. (f) Complete tumor
removed completely via the lateral suboccipital approach. removal; all neurovascular structures are intact (BS brain
(d) initial tumor (Tu) exposure. (e) Following partial stem)
Foramen Magnum Meningiomas 491

d e

Fig. 7.33 (continued)

492 7 Meningiomas

a c

b d

Fig. 7.34 Patient with a large foramen magnum menin-
gioma, operated via the lateral suboccipital approach. The
tumor was located predominantly medial to the lower cra-
nial nerves; the facial-vestibulocochlear nerve complex
were dislocated medially. Tumor removal was accom-
plished between the fascicles of the lower cranial nerves.
(a, b) Preoperative MRI study. (c) Initial view of the tumor
(Tu). The cerebellum (C) is slightly elevated; the lower
cranial nerves (LCN) are spread over the tumors’ surface;
the facial-vestibulocochlear nerve complex is not visible.
(d) Complete tumor removal and preservation of all neu-
ral structures. (7–8 facial and vestibulocochlear nerve
complex, BS brain stem). (e) Postoperative CT, demon-
strating the extent of bone removal
Foramen Magnum Meningiomas
e Patients with small remnants should be observed
Fig. 7.34 (continued)
Although it is still impossible to predict the bio-
logical activity of small tumor remnants, most of
them remain rather inactive. We do not recom-
mend early adjuvant radiotherapy or radiosurgery.
493
closely, both clinically and radiologically, and
treated only if the remnant does show a growth ten-
dency. Considering the mode of treatment of such
remnants, very important issue is their location: in
case the tumor contacts the brain stem, the risk of
radiation injury and severe neurological deficits is
very high. Therefore, an attempt should be made to
remove the tumor surgically. Radiotherapy (radio-
surgery or fractionated radiotherapy) may be rec-
ommended only if the tumor is separated from the
radiosensitive neural structures or as ultima ratio.
Recurrence rate after complete removal
(Simpson grade 1) is low. Still, meningiomas
may recur even late after surgery. Follow-up with
regular MRI examinations is warranted at regular
intervals. In case of symptomatic or growing recur-
rences, active management is indicated. The pre-
ferred option is again operative. Radiotherapy
(radiosurgery or fractionated radiotherapy) has to
be considered as an adjunctive therapy, especially
in case of early recurrences. Primary radiotherapy
of the recurrence is an alternative option in elderly
somatically unstable patients.
494 7 Meningiomas

a b

c d

Fig. 7.35 Example of a foramen magnum meningioma is barely visible. (d) Elevation of the cerebellum and expo-
encompassing but not infiltrating the vertebral artery. (a, sure of the tumor. (e) Complete tumor removal. (f) The
b) Preoperative MR images. (c) Dura incision. The tumor patient 1 month later did not have neurological deficits
Foramen Magnum Meningiomas 495

Fig. 7.35 (continued)

496
Case Illustration 7.18
Foramen Magnum Meningioma 1
This 42-year-old female patient presented to
our outpatient department complaining of
headache and vertigo since 6 months. The ini-
tial complaint was strong pain in the right part
a b
c d
(a, b) Preoperative T1-weighted axial and sagittal
postcontrast MRI images showing an enhancing mass
in the foramen magnum area (arrowhead tumor exten-
sion in the jugular foramen). (c, d) T2-weighted MRI
images demonstrating an arachnoid plane between the
tumor and the brain stem. (e) RS craniotomy, exposing
the edges of the transverse and sigmoid sinuses. The
foramen magnum was also opened. The dura was
incised along the lower half of the sigmoid sinus (dot-
ted line), approximately 2 mm medial to its border. (f,
g) Initial view of the tumor, located inferior to the
jugular foramen (arrow ninth nerve, arrowheads
7 Meningiomas
of the neck, which appeared 1 year earlier and
gradually disappeared. The only neurological
deficits at examination were an ipsilateral
tongue atrophy and gait ataxia. MRI study
showed a relatively large tumor in the lower
CP angle with extension caudally to the
ramus spinalis of the 11th nerve); some nerve fibers
were engulfed by the tumor (small arrows), and the
10th and 12th nerve could not be visualized. (h) A
large calcification—appr.1 cm (asterisk)—was
removed from the area of the jugular foramen. (i) With
further tumor removal, the vertebral artery (VA) could
be identified. (j) The tumor from the JF could be
removed with an angled hook and platelet knife (small
arrows), without the need of extending the exposure.
(k) The tumor has been removed, and the vertebral
artery was freed. (l) Complete tumor removal. The
preserved lower cranial nerves are well seen
Foramen Magnum Meningiomas 497

foramen magnum level (a–d). The tumor
caused severe compression and dislocation of
the brain stem and had imaging characteris-
tics, compatible with meningioma. The typi-
cal dural tail sign was well visible. The tumor
had its bulk in the lower CP angle and fora-
men magnum area but extended also into the
jugular foramen (b-arrowhead).
The patient was operated via an RS
approach with opening of the foramen mag-
num to gain access to the most caudal tumor
parts (e). At surgery, the tumor turned out to
be moderately firm and well vascularized. The
9th–12th cranial nerves were tightly attached
to the tumor and dislocated (f, g). The menin-
gioma engulfed the ipsilateral vertebral artery
and extended into both the jugular foramen
and the hypoglossal canal. Following internal
decompression, its capsule could be dissected
from the surrounding structures. The tumor
part in the area of JF was strongly calcified.
A large calcified tumor part (h) had to be

e f

g h
498 7 Meningiomas

removed, which allowed further exposure of
the JF and enabled us to remove the tumor
portions, extending into the JF and into the
hypoglossal canal (i, j). Complete tumor
removal could be thus achieved (k, l). The
meningioma was found to originate from the
dura between the JF and the hypoglossal canal.
This part of the dura was coagulated and
excised. Pathological examination of the spec-
imen showed that the tumor was a secretory
WHO grade I meningioma. After surgery, a
transient lower cranial nerve deficit was regis-
tered that recovered almost completely until
discharge. The hypoglossal palsy was, how-
ever, more pronounced and showed minimal
recovery.

i j

k l
Foramen Magnum Meningiomas
Case Illustration 7.19
Foramen Magnum Meningioma 2
Sixty-five-year-old female patient complain-
ing of increasing occipital headache and gait
instability since 3 months and slight swallow-
ing difficulty since several weeks. Her neuro-
logical examination revealed gait ataxia, slight
sensorineural hearing deficit on both sides,
and decreased gag reflex on the right side. The
MR images, which demonstrated a large
a b
(a–c) Preoperative T1-weighted MRI scans showing
the foramen magnum meningioma, extending caudal to
the C1 level. (d) Retrosigmoid craniotomy with open-
ing of the foramen magnum and C1 laminectomy has
been performed, exposing the tumor (Tu), which
extended caudal to the level of C1 root (C1). (D dura,
499
meningioma in the area of foramen magnum,
engulfing the ipsilateral vertebral artery, are
presented on figures a–c.
The patient was operated via the lateral sub-
occipital approach (RS craniotomy, opening of
foramen magnum, and C1 laminectomy). The
vertebral artery was identified and followed to
its entry point into the dura but was not mobi-
lized. The consecutive steps of the surgery are
shown on figures d–i. Initially sufficient CSF
C cerebellum). (e) The tumor has been partially
removed, and the accessory nerve was identified (11).
(f, g) Gradually the ipsilateral vertebral artery (VA) was
exposed. (h, i) Complete removal of the tumor with
preservation of all neurovascular structures (BS brain
stem)
500 7 Meningiomas

was allowed to egress, and tumor debulking
was performed. Gradually, the lower cranial
nerves were identified, and after sufficient
internal decompression, the tumor capsule was
carefully dissected from the cranial nerves and
brain stem. The vertebral artery was engulfed
by the meningioma over a length of 2–2.5 cm
but could be completely freed. Complete tumor
was achieved with preservation of all neuro-
vascular structures. Then, the tumor matrix
was extensively coagulated.
No new neurological deficits developed after
surgery. The swallowing deficit was slightly
more pronounced in the first days but resolved
until discharge. Histopathological diagnosis
was WHO grade I secretory meningioma.

d e
C

11
D
Tu
D
Tu

C1

f g

Tu Tu

VA
VA

h i
C

BS

VA 11
References
References
Cerebellopontine Angle Meningiomas
1. Agrawal V, Ludwig N, Agrawal A, Bulsara KR (2007)
Intraosseous intracranial meningioma. AJNR Am J
Neuroradiol 28(2):314–315, 28/2/314 [pii]
2. Akeyson EW, McCutcheon IE (1996) Management of
benign and aggressive intracranial meningiomas.
Oncology (Williston Park) 10(5):747–756; discussion
756–759
3. Al-Mefty O, Kadri PA, Pravdenkova S, Sawyer JR,
Stangeby C, Husain M (2004) Malignant progression
in meningioma: documentation of a series and analy-
sis of cytogenetic findings. J Neurosurg 101(2):210–
218. doi:10.3171/jns.2004.101.2.0210
4. Antinheimo J, Sankila R, Carpen O, Pukkala E, Sainio
M, Jaaskelainen J (2000) Population-based analysis
of sporadic and type 2 neurofibromatosis-associated
meningiomas and schwannomas. Neurology 54(1):
71–76
5. Arana E, Diaz C, Latorre FF, Menor F, Revert A,
Beltran A, Navarro M (1996) Primary intraosseous
meningiomas. Acta Radiol 37(6):937–942
6. Arriaga M, Shelton C, Nassif P, Brackmann DE
(1992) Selection of surgical approaches for menin-
giomas affecting the temporal bone. Otolaryngol
Head Neck Surg 107(6 Pt 1):738–744
7. Asaoka K, Barrs DM, Sampson JH, McElveen JT Jr,
Tucci DL, Fukushima T (2002) Intracanalicular men-
ingioma mimicking vestibular schwannoma. AJNR
Am J Neuroradiol 23(9):1493–1496
8. Bacciu A, Piazza P, Di Lella F, Sanna M (2007)
Intracanalicular meningioma: clinical features, radio-
logic findings, and surgical management. Otol Neurotol
28(3):391–399. doi:10.1097/MAO.0b013e31803261b4
9. Baguley DM, Beynon GJ, Grey PL, Hardy DG, Moffat
DA (1997) Audio-vestibular findings in meningioma
of the cerebello-pontine angle: a retrospective review.
J Laryngol Otol 111(11):1022–1026
10. Bassiouni H, Hunold A, Asgari S (2004) Meningiomas
of the posterior petrous bone: functional outcome
after microsurgery. J Neurosurg 100:1014–1024
11. Batra PS, Dutra JC, Wiet RJ (2002) Auditory and
facial nerve function following surgery for cerebello-
pontine angle meningiomas. Arch Otolaryngol Head
Neck Surg 128(4):369–374, ooa00138 [pii]
12. Bonnal J, Born JD, Tremoulet M (1979) Multiple intrac-
ranial meningiomas (author’s transl). Neurochirurgie
25(2):78–83
13. Bonneville F, Savatovsky J, Chiras J (2007) Imaging
of cerebellopontine angle lesions: an update. Part 2:
intra-axial lesions, skull base lesions that may invade
the CPA region, and non-enhancing extra-axial
lesions. Eur Radiol 17(11):2908–2920. doi:10.1007/
s00330-007-0680-4
14. Carney AS, Ward V, Malluci CL, O’Donoghue GM,
Robertson I, Baldwin DL, Maw AR, Coakham HB
(1999) Meningiomas involving the internal auditory
501
canal: a diagnostic and surgical challenge. Skull Base
Surg 9(2):87–94
15. Carvalho GA, Matthies C, Tatagiba M, Eghbal R,
Samii M (2000) Impact of computed tomographic and
magnetic resonance imaging findings on surgical out-
come in petroclival meningiomas. Neurosurgery
47(6):1287–1294; discussion 1294–1295
16. Castellano F, Ruggiero G (1953) Meningiomas of the
posterior fossa. Acta Radiol Suppl 104:1–177
17. Chamberlain MC, Blumenthal DT (2004) Intracranial
meningiomas: diagnosis and treatment. Expert Rev
Neurother 4(4):641–648. doi:10.1586/14737175.4.4.641
18. Cho YD, Choi GH, Lee SP, Kim JK (2003) (1)
H-MRS metabolic patterns for distinguishing
between meningiomas and other brain tumors. Magn
Reson Imaging 21(6):663–672, S0730725X03000973
[pii]
19. Cushing H (1922) The meningiomas (dural endothe-
liomas): Their source and favoured seats of origin.
Brain 45:282–316
20. Cushing H, Eisenhardt L (1938) Meningiomas: Their
classification, regional behavior, life history and sur-
gical end results. Charles C Thomas, Springfield
21. De Monte F, Al-Mefty O (1993) Neoplasms and the
cranial nerves of the posterior fossa. In: Barrow DL
(ed) Surgery of the cranial nerves of the posterior
fossa. American Association of Neurological Surgeons,
Park Ridge, pp 253–274
22. Dezamis E, Sanson M (2003) The molecular genetics
of meningiomas and genotypic/phenotypic correla-
tions. Rev Neurol (Paris) 159(8–9):727–738, MDOI-
RN-09-2003-159-8-9-0035-3787-101019-ART1 [pii]
23. Drake WM, Grossman AB, Hutson RK (2005) Effect
of treatment with pegvisomant on meningioma growth
in vivo. Eur J Endocrinol 152(1):161–162
24. Drummond KJ, Zhu JJ, Black PM (2004)
Meningiomas: updating basic science, management,
and outcome. Neurologist 10(3):113–130, 00127893-
200405000-00001 [pii]
25. Elder JB, Atkinson R, Zee CS, Chen TC (2007)
Primary intraosseous meningioma. Neurosurg Focus
23(4):E13. doi:10.3171/FOC-07/10/E13
26. Eljamel MS (1990) Multiple meningiomas. J Neurosurg
72(5):834–835
27. Eljamel MS, Foy PM (1989) Multiple meningiomas
and their relation to neurofibromatosis. Review of the
literature and report of seven cases. Surg Neurol
32(2):131–136
28. Elster AD, Challa VR, Gilbert TH, Richardson DN,
Contento JC (1989) Meningiomas: MR and
histopathologic features. Radiology 170(3 Pt 1):
857–862
29. Evans DG, Huson SM, Donnai D, Neary W, Blair V,
Newton V, Harris R (1992) A clinical study of type 2
neurofibromatosis. Q J Med 84(304):603–618
30. Filippi CG, Edgar MA, Ulug AM, Prowda JC, Heier
LA, Zimmerman RD (2001) Appearance of menin-
giomas on diffusion-weighted images: correlating
diffusion constants with histopathologic findings.
AJNR Am J Neuroradiol 22(1):65–72
502
31. Flannery TJ, Kano H, Lunsford LD, Sirin S, Tormenti
M, Niranjan A, Flickinger JC, Kondziolka D (2010)
Long-term control of petroclival meningiomas
through radiosurgery. J Neurosurg 112(5):957–964.
doi:10.3171/2009.8.JNS09695
32. Go KG, Kamman RL, Wilmink JT, Mooyaart EL
(1994) A study on peritumoral brain edema around
meningiomas by MRI and contrast CT. Acta Neurochir
Suppl (Wien) 60:365–368
33. Goodwin JW, Crowley J, Eyre HJ, Stafford B, Jaeckle
KA, Townsend JJ (1993) A phase II evaluation of
tamoxifen in unresectable or refractory meningiomas:
a Southwest Oncology Group study. J Neurooncol
15(1):75–77
34. Goutagny S, Kalamarides M (2010) Meningiomas
and neurofibromatosis. J Neurooncol 99(3):341–347.
doi:10.1007/s11060-010-0339-x
35. Goyal LK, Suh JH, Mohan DS, Prayson RA, Lee J,
Barnett GH (2000) Local control and overall survival
in atypical meningioma: a retrospective study. Int J
Radiat Oncol Biol Phys 46(1):57–61, S0360-
3016(99)00349-1 [pii]
36. Grey PL, Baguley DM, Moffat DA, Hardy DG,
Beynon GJ (1996) Audiovestibular results after sur-
gery for cerebellopontine angle meningiomas. Am J
Otol 17(4):634–638
37. Grunberg SM, Weiss MH, Russell CA, Spitz IM,
Ahmadi J, Sadun A, Sitruk-Ware R (2006) Long-term
administration of mifepristone (RU486): clinical
tolerance during extended treatment of meningioma.
Cancer Invest 24(8):727–733. doi:10.1080/
07357900601062339, K6271K7768127257 [pii]
38. Hakim R, Alexander E 3rd, Loeffler JS, Shrieve DC,
Wen P, Fallon MP, Stieg PE, Black PM (1998) Results
of linear accelerator-based radiosurgery for intracra-
nial meningiomas. Neurosurgery 42(3):446–453; dis-
cussion 453–454
39. Hsu DW, Efird JT, Hedley-Whyte ET (1997)
Progesterone and estrogen receptors in meningiomas:
prognostic considerations. J Neurosurg 86(1):113–
120. doi:10.3171/jns.1997.86.1.0113
40. Ide M, Jimbo M, Yamamoto M, Umebara Y, Hagiwara
S, Kubo O (1996) MIB-1 staining index and peritu-
moral brain edema of meningiomas. Cancer 78(1):133–
143, 10.1002/(SICI)1097-0142(19960701)78:1<133::
AID-CNCR19>3.0.CO;2-0 [pii] 10.1002/(SICI)1097-
0142(19960701)78:1<133::AID-CNCR19>3.0.CO;2-0
41. Jaaskelainen J, Haltia M, Servo A (1986) Atypical and
anaplastic meningiomas: radiology, surgery, radiother-
apy, and outcome. Surg Neurol 25(3):233–242
42. Katz TS, Amdur RJ, Yachnis AT, Mendenhall WM,
Morris CG (2005) Pushing the limits of radiotherapy
for atypical and malignant meningioma. Am J Clin
Oncol 28(1):70–74, 00000421-200502000-00013
[pii]
43. Kleihues P, Louis DN, Scheithauer BW, Rorke LB,
Reifenberger G, Burger PC, Cavenee WK (2002) The
WHO classification of tumors of the nervous system.
J Neuropathol Exp Neurol 61(3):215–225; discussion
226–229
7 Meningiomas
44. Koerbel A, Gharabaghi A, Safavi-Abbasi S, Samii A,
Ebner FH, Samii M, Tatagiba M (2009) Venous com-
plications following petrosal vein sectioning in sur-
gery of petrous apex meningiomas. Eur J Surg Oncol
35(7):773–779. doi:10.1016/j.ejso.2008.02.015,
S0748-7983(08)00085-1 [pii]
45. Krayenbuhl N, Pravdenkova S, Al-Mefty O (2007) De
novo versus transformed atypical and anaplastic
meningiomas: comparisons of clinical course, cytoge-
netics, cytokinetics, and outcome. Neurosurgery
61(3):495–503. doi:10.1227/01.NEU.0000290895.
92695.22, 00006123-200709000-00009 [pii]; discus-
sion 503–504
46. Lalwani AK (1992) Meningiomas, epidermoids, and
other nonacoustic tumors of the cerebellopontine
angle. Otolaryngol Clin North Am 25(3):707–728
47. Lamszus K (2004) Meningioma pathology, genetics,
and biology. J Neuropathol Exp Neurol 63(4):
275–286
48. Lang FF, Macdonald OK, Fuller GN, DeMonte F
(2000) Primary extradural meningiomas: a report on
nine cases and review of the literature from the era of
computerized tomography scanning. J Neurosurg
93(6):940–950. doi:10.3171/jns.2000.93.6.0940
49. Laudadio P, Canani FB, Cunsolo E (2004) Meningioma
of the internal auditory canal. Acta Otolaryngol
124(10):1231–1234
50. Levin M, Wertheim SE, Klein E, Lowry J (1995)
Unusual lytic intraosseous meningioma. J Neuroimaging
5(4):247–248
51. Liu JK, Gottfried ON, Couldwell WT (2003) Surgical
management of posterior petrous meningiomas.
Neurosurg Focus 14(6):e7, 140607 [pii]
52. Lomas J, Bello MJ, Alonso ME, Gonzalez-Gomez P,
Arjona D, Kusak ME, de Campos JM, Sarasa JL, Rey
JA (2002) Loss of chromosome 22 and absence of
NF2 gene mutation in a case of multiple meningiomas.
Hum Pathol 33(3):375–378, S004681770215324X
[pii]
53. Louis DNSB, Budka H, von Deimling A, Kepes JJ
(2000) Meningiomas. In: Kleihues PCW (ed)
Pathology and Genetics of Tumours of the Nervous
System: World Health Organization Classification of
Tumours. IARC Press, Lyon, pp 176–184
54. Mahmood A, Caccamo DV, Tomecek FJ, Malik GM
(1993) Atypical and malignant meningiomas: a clini-
copathological review. Neurosurgery 33(6):955–963
55. Maier H, Ofner D, Hittmair A, Kitz K, Budka H (1992)
Classic, atypical, and anaplastic meningioma: three his-
topathological subtypes of clinical relevance. J Neurosurg
77(4):616–623. doi:10.3171/jns.1992.77.4.0616
56. Mallucci CL, Ward V, Carney AS, O’Donoghue GM,
Robertson I (1999) Clinical features and outcomes in
patients with non-acoustic cerebellopontine angle
tumours. J Neurol Neurosurg Psychiatry 66(6):
768–771
57. Maniglia AJ, Fenstermaker RA, Ratcheson RA (1989)
Preservation of hearing in the surgical removal of
cerebellopontine angle tumors. Otolaryngol Clin
North Am 22(1):211–232
References
58. Matthies C, Carvalho G, Tatagiba M, Lima M, Samii
M (1996) Meningiomas of the cerebellopontine angle.
Acta Neurochir Suppl 65:86–91
59. Maurer PK, Okawara SH (1988) Restoration of hear-
ing after removal of cerebellopontine angle menin-
gioma: diagnostic and therapeutic implications.
Neurosurgery 22(3):573–575
60. Mautner VF, Lindenau M, Baser ME, Hazim W,
Tatagiba M, Haase W, Samii M, Wais R, Pulst SM
(1996) The neuroimaging and clinical spectrum of
neurofibromatosis 2. Neurosurgery 38(5):880–885;
discussion 885–886
61. McMullen KP, Stieber VW (2004) Meningioma: cur-
rent treatment options and future directions. Curr
Treat Options Oncol 5(6):499–509
62. Merrill MJ, Oldfield EH (2005) A reassessment of
vascular endothelial growth factor in central nervous
system pathology. J Neurosurg 103(5):853–868.
doi:10.3171/jns.2005.103.5.0853
63. Modha A, Gutin PH (2005) Diagnosis and treatment
of atypical and anaplastic meningiomas: a review.
Neurosurgery 57(3):538–550, 00006123-200509000-
00017 [pii]; discussion 538–550
64. Mukhopadhyay D, Carrabba G, Guha A (2009)
Meningiomas and Brain Edema. In: Pamir N BP,
Fahlbusch R (eds) Meningiomas: a comprehensive
text. Saunders Elsevier, Philadelphia, pp 135–145
65. Nager GT, Masica DN (1970) Meningiomas of the
cerebello-pontine angle and their relation to the tem-
poral bone. Laryngoscope 80(6):863–895
66. Nakamura M, Roser F, Dormiani M, Matthies C,
Vorkapic P, Samii M (2005) Facial and cochlear nerve
function after surgery of cerebellopontine angle menin-
giomas. Neurosurgery 57(1):77–90; discussion 77–90
67. Nakamura M, Roser F, Dormiani M, Samii M,
Matthies C (2005) Intraoperative auditory brainstem
responses in patients with cerebellopontine angle
meningiomas involving the inner auditory canal: anal-
ysis of the predictive value of the responses.
J Neurosurg 102(4):637–642. doi:10.3171/jns.2005.
102.4.0637
68. Nakamura M, Roser F, Dormiani M, Vorkapic P,
Samii M (2005) Surgical treatment of cerebellopon-
tine angle meningiomas in elderly patients. Acta
Neurochir (Wien) 147(6):603–609. doi:10.1007/
s00701-005-0517-3; discussion 609–610
69. Nakamura M, Roser F, Mirzai S, Matthies C, Vorkapic
P, Samii M (2004) Meningiomas of the internal audi-
tory canal. Neurosurgery 55(1):119–127; discussion
127–128
70. Nakamura M, Samii M (2003) Surgical management
of a meningioma in the retrosellar region. Acta
Neurochir (Wien) 145(3):215–219
71. Noel G, Bollet MA, Calugaru V, Feuvret L, Haie-
Meder C, Dhermain F, Ferrand R, Boisserie G,
Beaudre A, Mazeron JJ, Habrand JL (2005) Functional
outcome of patients with benign meningioma treated
by 3D conformal irradiation with a combination of
photons and protons. Int J Radiat Oncol Biol Phys
62(5):1412–1422, S0360-3016(04)03155-4 [pii]
503
72. Norden AD, Drappatz J, Wen PY (2009) Advances in
meningioma therapy. Curr Neurol Neurosci Rep
9(3):231–240
73. Oguz KK, Cila A (2003) Rim enhancement of menin-
giomas on fast FLAIR imaging. Neuroradiology
45(2):78–81. doi:10.1007/s00234-002-0914-8
74. Ojemann R (1990) Meningiomas. Neurosurg Clin N
Am 1(1):181–197
75. Palma L, Celli P, Franco C, Cervoni L, Cantore G
(1997) Long-term prognosis for atypical and malig-
nant meningiomas: a study of 71 surgical cases.
Neurosurg Focus 2(4):e3, 020403 [pii]
76. Parry DM, Eldridge R, Kaiser-Kupfer MI, Bouzas EA,
Pikus A, Patronas N (1994) Neurofibromatosis 2 (NF2):
clinical characteristics of 63 affected individuals and
clinical evidence for heterogeneity. Am J Med Genet
52(4):450–461. doi:10.1002/ajmg.1320520411
77. Perry A, Cai DX, Scheithauer BW, Swanson PE,
Lohse CM, Newsham IF, Weaver A, Gutmann DH
(2000) Merlin, DAL-1, and progesterone receptor
expression in clinicopathologic subsets of menin-
gioma: a correlative immunohistochemical study of
175 cases. J Neuropathol Exp Neurol 59(10):872–879
78. Perry A, Scheithauer BW, Stafford SL, Lohse CM,
Wollan PC (1999) “Malignancy” in meningiomas:
a clinicopathologic study of 116 patients, with grad-
ing implications. Cancer 85(9):2046–2056, 10.1002/
(SICI)1097-0142(19990501)85:9<2046::AID-
CNCR23>3.0.CO;2-M [pii]
79. Perry A, Stafford SL, Scheithauer BW, Suman VJ, Lohse
CM (1997) Meningioma grading: an analysis of histo-
logic parameters. Am J Surg Pathol 21(12):1455–1465
80. Politi M, Romeike BF, Papanagiotou P, Nabhan A,
Struffert T, Feiden W, Reith W (2005) Intraosseous
hemangioma of the skull with dural tail sign: radio-
logic features with pathologic correlation. AJNR Am
J Neuroradiol 26(8):2049–2052, 26/8/2049 [pii]
81. Rokitansky C (1856) Lehrbuch der pathologischen
Anatomie. Wilhelm Braumüller, Wien
82. Rosahl SK, Mirzayan MJ, Samii M (2004) Osteolytic
intra-osseous meningiomas: illustrated review. Acta
Neurochir (Wien) 146(11):1245–1249. doi:10.1007/
s00701-004-0380-7
83. Roser F, Nakamura M, Dormiani M, Matthies C,
Vorkapic P, Samii M (2005) Meningiomas of the cer-
ebellopontine angle with extension into the internal
auditory canal. J Neurosurg 102(1):17–23.
doi:10.3171.jns.2005.102.1.0017
84. Ruggieri M, Iannetti P, Polizzi A, La Mantia I,
Spalice A, Giliberto O, Platania N, Gabriele AL,
Albanese V, Pavone L (2005) Earliest clinical mani-
festations and natural history of neurofibromatosis
type 2 (NF2) in childhood: a study of 24
patients. Neuropediatrics 36(1):21–34. doi:10.1055/
s-2005-837581
85. Samii M, Gerganov VM (2008) Surgery of extra-
axial tumors of the cerebral base. Neurosurgery
62(6 Suppl 3):1153–1166. doi:10.1227/01.
neu.0000333782.19682.76, 00006123-200806001-
00024 [pii]; discussion 1166–1168
504
86. Samii MAM, Ammirati M (1991) Cerebellopontine
angle meningiomas (Posterior pyramid menin-
giomas). In: Al-Mefty O (ed) Meningiomas. Raven,
New York, pp 503–515
87. Sanson M, Cornu P (2000) Biology of meningiomas.
Acta Neurochir (Wien) 142(5):493–505
88. Sawaya RE, Ligon BL (1994) Thromboembolic
complications associated with brain tumors.
J Neurooncol 22(2):173–181
89. Schaller B, Heilbronner R, Pfaltz CR, Probst RR,
Gratzl O (1995) Preoperative and postoperative
auditory and facial nerve function in cerebellopon-
tine angle meningiomas. Otolaryngol Head
Neck Surg 112(2):228–234, S0194-5998(95)70241-5
[pii]
90. Schaller B, Merlo A, Gratzl O, Probst R (1999)
Premeatal and retromeatal cerebellopontine angle
meningioma. Two distinct clinical entities. Acta
Neurochir (Wien) 141(5):465–471
91. Sekhar LN, Jannetta PJ (1984) Cerebellopontine
angle meningiomas. Microsurgical excision and fol-
low-up results. J Neurosurg 60(3):500–505.
doi:10.3171/jns.1984.60.3.0500
92. Sekhar LN, Swamy NK, Jaiswal V, Rubinstein E,
Hirsch WE Jr, Wright DC (1994) Surgical excision
of meningiomas involving the clivus: preoperative
and intraoperative features as predictors of postop-
erative functional deterioration. J Neurosurg
81(6):860–868. doi:10.3171/jns.1994.81.6.0860
93. Shapiro WR, Hiesiger EM, Cooney GA, Basler GA,
Lipschutz LE, Posner JB (1990) Temporal effects of
dexamethasone on blood-to-brain and blood-to-
tumor transport of 14C-alpha-aminoisobutyric acid
in rat C6 glioma. J Neurooncol 8(3):197–204
94. Simon MBJ, Hartmann C (2007) Molecular genetics
of meningiomas: from basic research to potential
clinical applications. Neurosurgery 60:787–798
95. Stafford SLPB, Foote RL et al (2001) Meningioma
radiosurgery: tumor control, outcomes, and complica-
tions among 190 consecutive patients. Neurosurgery
49:1029–1038
96. Tamiya T, Ono Y, Matsumoto K, Ohmoto T (2001)
Peritumoral brain edema in intracranial menin-
giomas: effects of radiological and histological fac-
tors. Neurosurgery 49(5):1046–1051; discussion
1051–1052
97. Tekkok IH, Suzer T, Erbengi A (1992) Non-acoustic
tumors of the cerebellopontine angle. Neurosurg
Rev 15(2):117–123
98. Thomas NW, King TT (1996) Meningiomas of the
cerebellopontine angle. A report of 41 cases. Br J
Neurosurg 10(1):59–68
99. Vellutini EA, Cruz OL, Velasco OP, Miniti A,
Almeida GM (1991) Reversible hearing loss from
cerebellopontine angle tumors. Neurosurgery
28(2):310–312; discussion 312–313
100. Virchow R (1863) Die krankhaften Geschwülste:
Dreissig Vorlesungen gehalten während des
Wintersemesters 1862–1863 an der Universität zu
Berlin. A. Hirschwald, Berlin
7 Meningiomas
101. Voss NF, Vrionis FD, Heilman CB, Robertson JH
(2000) Meningiomas of the cerebellopontine angle.
Surg Neurol 53(5):439–446, S0090-3019(00)00195-6
[pii]; discussion 446–447
102. Wallace EW (2004) The dural tail sign. Radiology
233(1):56–57. doi:10.1148/radiol.2331021332,
233/1/56 [pii]
103. Wellenreuther R, Kraus JA, Lenartz D, Menon AG,
Schramm J, Louis DN, Ramesh V, Gusella JF,
Wiestler OD, von Deimling A (1995) Analysis of the
neurofibromatosis 2 gene reveals molecular variants
of meningioma. Am J Pathol 146(4):827–832
104. Wiemels J, Wrensch M, Claus EB (2010) Epidemiology
and etiology of meningioma. J Neurooncol 99(3):307–
314. doi:10.1007/s11060-010-0386-3
105. Wilms G, Plets C, Goossens L, Goffin J, Vanwambeke
K (1992) The radiological differentiation of acoustic
neuroma and meningioma occurring together in the
cerebellopontine angle. Neurosurgery 30(3):443–
445; discussion 445–446
106. Wu ZB, Yu CJ, Guan SS (2005) Posterior petrous
meningiomas: 82 cases. J Neurosurg 102(2):284–
289. doi:10.3171/jns.2005.102.2.0284
107. Yamaguchi N, Kawase T, Sagoh M, Ohira T, Shiga
H, Toya S (1997) Prediction of consistency of men-
ingiomas with preoperative magnetic resonance
imaging. Surg Neurol 48(6):579–583, S0090-
3019(96)00439-9 [pii]
108. Younis GA, Sawaya R, DeMonte F, Hess KR,
Albrecht S, Bruner JM (1995) Aggressive meningeal
tumors: review of a series. J Neurosurg 82(1):17–27.
doi:10.3171/jns.1995.82.1.0017
109. Zamani AA (2000) Cerebellopontine angle tumors:
role of magnetic resonance imaging. Top Magn
Reson Imaging 11(2):98–107
Petroclival Meningiomas
110. Abdel Aziz KM, Sanan A, van Loveren HR, Tew JM
Jr, Keller JT, Pensak ML (2000) Petroclival
meningiomas: predictive parameters for transpetro-
sal approaches. Neurosurgery 47(1):139–150;
discussion 150–152
111. Al-Mefty O, Ayoubi S, Smith RR (1991) The petro-
sal approach: indications, technique, and results.
Acta Neurochir Suppl (Wien) 53:166–170
112. Al-Mefty O, Fox JL, Smith RR (1988) Petrosal
approach for petroclival meningiomas. Neurosurgery
22(3):510–517
113. Bambakidis NC, Kakarla UK, Kim LJ, Nakaji P,
Porter RW, Daspit CP, Spetzler RF (2007) Evolution
of surgical approaches in the treatment of petroclival
meningiomas: a retrospective review. Neurosurgery
61(5 Suppl 2):202–209. doi:10.1227/01.
neu.0000303218.61230.39; discussion 209–211
114. Bricolo AP, Turazzi S, Talacchi A, Cristofori L
(1992) Microsurgical removal of petroclival menin-
References
giomas: a report of 33 patients. Neurosurgery
31(5):813–828; discussion 828
115. Carvalho GA, Matthies C, Tatagiba M, Eghbal R,
Samii M (2000) Impact of computed tomographic
and magnetic resonance imaging findings on surgical
outcome in petroclival meningiomas. Neurosurgery
47(6):1287–1294; discussion 1294–1295
116. Castellano F, Ruggiero G (1953) Meningiomas of
the posterior fossa. Acta Radiol Suppl 104:1–177
117. Chanda A, Nanda A (2006) Retrosigmoid intradural
suprameatal approach: advantages and disadvan-
tages from an anatomical perspective. Neurosurgery
59(1 Suppl 1):ONS1–ONS6. doi:10.1227/01.
NEU.0000220673.79877.30; discussion ONS1–ONS6
118. Couldwell WT, Fukushima T, Giannotta SL, Weiss
MH (1996) Petroclival meningiomas: surgical expe-
rience in 109 cases. J Neurosurg 84(1):20–28.
doi:10.3171/jns.1996.84.1.0020
119. Cushing H, Eisenberg L (1938) Meningiomas: Their
classification, regional behavior, life history and sur-
gical end results. Charles C Thomas, Springfield
120. Decker RE, Malis LI (1970) Surgical approaches to
midline lesions at the base of the skull: a review. Mt
Sinai J Med 37(2):84–102
121. Erkmen K, Pravdenkova S, Al-Mefty O (2005)
Surgical management of petroclival meningiomas:
factors determining the choice of approach.
Neurosurg Focus 19(2):E7, 190207 [pii]
122. Fisch U (1978) Infratemporal fossa approach to
tumours of the temporal bone and base of the skull.
J Laryngol Otol 92(11):949–967
123. Flannery TJ, Kano H, Lunsford LD, Sirin S, Tormenti
M, Niranjan A, Flickinger JC, Kondziolka D (2010)
Long-term control of petroclival meningiomas
through radiosurgery. J Neurosurg 112(5):957–964.
doi:10.3171/2009.8.JNS09695
124. Hakuba A (1978) Total removal of cerebellopontine
angle tumors with a combined transpetrosal-tran-
stentorial approach (author’s transl). No Shinkei
Geka 6(4):347–354
125. Hakuba A, Nishimura S, Jang BJ (1988) A combined
retroauricular and preauricular transpetrosal-tran-
stentorial approach to clivus meningiomas. Surg
Neurol 30(2):108–116
126. Hakuba A, Nishimura S, Tanaka K, Kishi H,
Nakamura T (1977) Clivus meningioma: six cases of
total removal. Neurol Med Chir (Tokyo) 17(1 Pt 1):
63–77
127. House WF, De la Cruz A, Hitselberger WE (1978)
Surgery of the skull base: transcochlear approach to
the petrous apex and clivus. Otolaryngology
86(5):ORL-770–ORL-779
128. Ichimura S, Kawase T, Onozuka S, Yoshida K, Ohira
T (2008) Four subtypes of petroclival meningiomas:
differences in symptoms and operative findings
using the anterior transpetrosal approach. Acta
Neurochir (Wien) 150(7):637–645. doi:10.1007/
s00701-008-1586-x
129. Iwai Y, Yamanaka K, Nakajima H (2001) Two-
staged gamma knife radiosurgery for the treatment
505
of large petroclival and cavernous sinus menin-
giomas. Surg Neurol 56(5):308–314, S0090-
3019(01)00622-X [pii]
130. Janecka IP, Sen CN, Sekhar LN, Arriaga M (1990)
Facial translocation: a new approach to the cranial
base. Otolaryngol Head Neck Surg 103(3):413–419
131. Kawase T, Shiobara R, Toya S (1991) Anterior trans-
petrosal-transtentorial approach for sphenopetro-
clival meningiomas: surgical method and results in
10 patients. Neurosurgery 28(6):869–875; discus-
sion 875–876
132. Kawase T, Shiobara R, Toya S (1994) Middle fossa
transpetrosal-transtentorial approaches for petroclival
meningiomas. Selective pyramid resection and radi-
cality. Acta Neurochir (Wien) 129(3–4):113–120
133. Little KM, Friedman AH, Sampson JH, Wanibuchi
M, Fukushima T (2005) Surgical management of
petroclival meningiomas: defining resection goals
based on risk of neurological morbidity and tumor
recurrence rates in 137 patients. Neurosurgery
56(3):546–559; discussion 546–559
134. Malis L (1991) The petrosal approach. Clin
Neurosurg 37:528–540
135. Mathiesen T, Gerlich A, Kihlstrom L, Svensson M,
Bagger-Sjoback D (2008) Effects of using combined
transpetrosal surgical approaches to treat petroclival
meningiomas. Neurosurgery 62(6 Suppl 3):1213–
1223. doi:10.1227/01.neu.0000333787.06221.e5
136. Mayberg MR, Symon L (1986) Meningiomas of the
clivus and apical petrous bone. Report of 35 cases.
J Neurosurg 65(2):160–167. doi:10.3171/jns.1986.
65.2.0160
137. Morrison AW, King TT (1973) Experiences with a
translabyrinthine-transtentorial approach to the cer-
ebellopontine angle. Technical note. J Neurosurg
38(3):382–390. doi:10.3171/jns.1973.38.3.0382
138. Natarajan SK, Sekhar LN, Schessel D, Morita A
(2007) Petroclival meningiomas: multimodality
treatment and outcomes at long-term follow-up.
Neurosurgery 60(6):965–979. doi:10.1227/01.
NEU.0000255472.52882.D6; discussion 979–981
139. Ramina R, Neto MC, Fernandes YB, Silva EB,
Mattei TA, Aguiar PH (2008) Surgical removal of
small petroclival meningiomas. Acta Neurochir
(Wien) 150(5):431–438. doi:10.1007/s00701-007-
1403-y; discussion 438–439
140. Rhoton AL Jr (2000) The temporal bone and tran-
stemporal approaches. Neurosurgery 47(3
Suppl):S211–S265
141. Roche PH, Pellet W, Fuentes S, Thomassin JM, Regis
J (2003) Gamma knife radiosurgical management of
petroclival meningiomas results and indications. Acta
Neurochir (Wien) 145(10):883–888. doi:10.1007/
s00701-003-0123-1; discussion 888
142. Salamon GM, Combalbert A, Raybaud C, Gonzalez
J (1971) An angiographic study of meningiomas of
the posterior fossa. J Neurosurg 35(6):731–741.
doi:10.3171/jns.1971.35.6.0731
143. Samii M, Ammirati M (1988) The combined supra-
infratentorial pre-sigmoid sinus avenue to the
506
petro-clival region Surgical technique and clinical
applications. Acta Neurochir (Wien) 95(1–2):
6–12
144. Samii M, Ammirati M, Mahran A, Bini W, Sepehrnia
A (1989) Surgery of petroclival meningiomas: report
of 24 cases. Neurosurgery 24(1):12–17
145. Samii M, Carvalho GA, Tatagiba M, Matthies C
(1997) Surgical management of meningiomas origi-
nating in Meckel’s cave. Neurosurgery 41(4):767–
774; discussion 774–775
146. Samii M, Gerganov V, Giordano M, Samii A (2010)
Two step approach for surgical removal of petro-
clival meningiomas with large supratentorial exten-
sion. Neurosurg Rev 34(2):173–179. doi:10.1007/
s10143-010-0299-9
147. Samii M, Gerganov VM (2008) Surgery of extra-
axial tumors of the cerebral base. Neurosurgery 62(6
Suppl 3):1153–1166. doi:10.1227/01.
neu.0000333782.19682.76, 00006123-200806001-
00024 [pii]; discussion 1166–1168
148. Samii M, Tatagiba M (1992) Experience with 36 sur-
gical cases of petroclival meningiomas. Acta
Neurochir (Wien) 118(1–2):27–32
149. Samii M, Tatagiba M, Carvalho GA (2000)
Retrosigmoid intradural suprameatal approach to
Meckel’s cave and the middle fossa: surgical tech-
nique and outcome. J Neurosurg 92(2):235–241.
doi:10.3171/jns.2000.92.2.0235
150. Samii M, Ammirati M (1991) Cerebellopontine
angle meningiomas (Posterior pyramid menin-
giomas). In: Al-Mefty O (ed) Meningiomas. Raven,
New York, pp 503–515
151. Seifert V (2010) Clinical management of petroclival
meningiomas and the eternal quest for preservation
of quality of life: personal experiences over a period
of 20 years. Acta Neurochir (Wien) 152(7):1099–
1116. doi:10.1007/s00701-010-0633-6
152. Sekhar LN, Wright DC, Richardson R, Monacci W
(1996) Petroclival and foramen magnum menin-
giomas: surgical approaches and pitfalls. J Neurooncol
29(3):249–259
153. Spallone A, Makhmudov UB, Mukhamedjanov DJ,
Tcherekajev VA (1999) Petroclival meningioma. An
attempt to define the role of skull base approaches in
their surgical management. Surg Neurol 51(4):412–
419, S0090-3019(98)00100-1 [pii]; discussion
419–420
154. Spetzler RF, Daspit CP, Pappas CT (1992) The com-
bined supra- and infratentorial approach for lesions
of the petrous and clival regions: experience with 46
cases. J Neurosurg 76(4):588–599. doi:10.3171/
jns.1992.76.4.0588
155. Stafford SL, Pollock BE, Foote RL, Link MJ,
Gorman DA, Schomberg PJ, Leavitt JA (2001)
Meningioma radiosurgery: tumor control, outcomes,
and complications among 190 consecutive patients.
Neurosurgery 49:1029–1038
156. Tedeschi H, Rhoton AL Jr (1994) Lateral approaches
to the petroclival region. Surg Neurol 41(3):180–
216, 0090-3019(94)90123-6 [pii]
7 Meningiomas
157. Van Havenbergh T, Carvalho G, Tatagiba M, Plets C,
Samii M (2003) Natural history of petroclival menin-
giomas. Neurosurgery 52(1):55–62; discussion 62–64
158. Zachenhofer I, Wolfsberger S, Aichholzer M,
Bertalanffy A, Roessler K, Kitz K, Knosp E (2006)
Gamma-knife radiosurgery for cranial base menin-
giomas: experience of tumor control, clinical course,
and morbidity in a follow-up of more than 8 years.
Neurosurgery 58(1):28–36, 00006123-200601000-
00003 [pii]; discussion 28–36
159. Zentner J, Meyer B, Vieweg U, Herberhold C,
Schramm J (1997) Petroclival meningiomas: is radi-
cal resection always the best option? J Neurol
Neurosurg Psychiatry 62(4):341–345
Foramen Magnum Meningiomas
160. Arnautovic KI, Al-Mefty O, Husain M (2000)
Ventral foramen magnum meningiomas. J Neurosurg
92(1 Suppl):71–80
161. Bassiouni H, Ntoukas V, Asgari S, Sandalcioglu EI,
Stolke D, Seifert V (2006) Foramen magnum menin-
giomas: clinical outcome after microsurgical resec-
tion via a posterolateral suboccipital retrocondylar
approach. Neurosurgery 59(6):1177–1185.
doi:10.1227/01.NEU.0000245629.77968.37; discus-
sion 1185–1187
162. Bejjani GK, Sekhar LN, Riedel CJ (2000)
Occipitocervical fusion following the extreme lateral
transcondylar approach. Surg Neurol 54(2):109–115,
S0090-3019(00)00255-X [pii]; discussion 115–106
163. Bertalanffy H, Seeger W (1991) The dorsolateral,
suboccipital, transcondylar approach to the lower
clivus and anterior portion of the craniocervical
junction. Neurosurgery 29(6):815–821
164. Borba LA, de Oliveira JG, Giudicissi-Filho M, Colli
BO (2009) Surgical management of foramen mag-
num meningiomas. Neurosurg Rev 32(1):49–58.
doi:10.1007/s10143-008-0161-5; discussion 59–60
165. Boulton MR, Cusimano MD (2003) Foramen mag-
num meningiomas: concepts, classifications, and
nuances. Neurosurg Focus 14(6):e10, 140610 [pii]
166. Bruneau M, George B (2008) Foramen magnum
meningiomas: detailed surgical approaches and
technical aspects at Lariboisiere Hospital and review
of the literature. Neurosurg Rev 31(1):19–32.
doi:10.1007/s10143-007-0097-1; discussion 32–33
167. David CA, Spetzler RF (1997) Foramen magnum
meningiomas. Clin Neurosurg 44:467–489
168. George B (1991) Meningiomas of the foramen mag-
num. In: Schmidek HH (ed) Meningiomas and Their
Surgical Management. WB Saunders, Philadelphia,
pp 459–470
169. George B, Lot G, Boissonnet H (1997) Meningioma
of the foramen magnum: a series of 40 cases. Surg
Neurol 47(4):371–379, S0090-3019(96)00204-2 [pii]
170. Goel A, Desai K, Muzumdar D (2001) Surgery on
anterior foramen magnum meningiomas using a con-
References
ventional posterior suboccipital approach: a report
on an experience with 17 cases. Neurosurgery
49(1):102–106; discussion 106–107
171. Heros RC (1986) Lateral suboccipital approach for
vertebral and vertebrobasilar artery lesions.
J Neurosurg 64(4):559–562. doi:10.3171/jns.1986.
64.4.0559
172. Margalit NS, Lesser JB, Singer M, Sen C (2005)
Lateral approach to anterolateral tumors at the fora-
men magnum: factors determining surgical proce-
dure. Neurosurgery 56(2 Suppl):324–336; discussion
324–336
173. Menezes AH (2008) Surgical approaches: postoper-
ative care and complications “posterolateral-far
lateral transcondylar approach to the ventral fora-
men magnum and upper cervical spinal canal”.
Childs Nerv Syst 24(10):1203–1207. doi:10.1007/
s00381-008-0597-5
174. Menezes AH, Traynelis VC, Gantz BJ (1994)
Surgical approaches to the craniovertebral junction.
Clin Neurosurg 41:187–203
175. Rhoton AL Jr (1994) Meningiomas of the cerebel-
lopontine angle and foramen magnum. Neurosurg
Clin N Am 5(2):349–377
507
176. Rhoton AL Jr (2000) The foramen magnum.
Neurosurgery 47(3 Suppl):S155–S193
177. Samii M, Gerganov VM (2008) Surgery of extra-
axial tumors of the cerebral base. Neurosurgery 62(6
Suppl 3):1153–1166. doi:10.1227/01.neu.
0000333782.19682.76; discussion 1166–1168
178. Samii M, Gerganov V (2008) Surgery of extraaxial
tumors of the skull base. Neurosurgery 62(6):
1153–1168
179. Samii M, Klekamp J, Carvalho G (1996) Surgical
results for meningiomas of the craniocervical junc-
tion. Neurosurgery 39(6):1086–1094; discussion
1094–1095
180. Shin H, Barrenechea IJ, Lesser J, Sen C, Perin
NI (2006) Occipitocervical fusion after resection of
craniovertebral junction tumors. J Neurosurg
Spine 4(2):137–144. doi:10.3171/spi.2006.4.2.137
181. Vishteh AG, Crawford NR, Melton MS, Spetzler RF,
Sonntag VK, Dickman CA (1999) Stability of the
craniovertebral junction after unilateral occipital
condyle resection: a biomechanical study. J Neurosurg
90(1 Suppl):91–98
 Cerebellopontine Angle
Epidermoids
General Features
Epidermoids or epidermoid cysts are congenital
lesions that arise from ectodermal cells, mis-
placed during an aberrant closure of the neural
tube [25, 30]. According to the most popular
theory, the epithelial cell rests are transplanted
to the CP angle by the laterally migrating otic
capsule or by the developing neurovasculature.
Epidermoids are the most common intracranial
embryonal tumor and are most frequently located
in the CP angle, in which they comprise 4–7 %
of all lesions [3, 10, 29]. They are the third most
common CP angle tumor [15, 25, 29].
The epidermoid cyst wall is composed of
stratified squamous epithelium, while the central
core contains solid keratin protein—a pearly
blistering material. The tumors are hence
referred to as “pearly tumors.” They grow from
the slow desquamation of the stratified kerati-
nized epithelium, which lines the cyst and spread
along pathways of least resistance into the pos-
terior fossa cisterns [15, 23]. Epidermoids cause
gradual displacement of neighboring neural
structures and encase cranial nerves and vessels
(Fig. 8.1). Over the time, dense adhesions to cra-
nial nerves or to brain stem may develop.
Extension into the Meckels’ cave, IAC, petrous
bone, or jugular foramen is occasionally seen
(Figs. 8.2 and 8.3).
CP angle epidermoids, if untreated, tend to
extend through the tentorial incisura into the mid-
dle cranial fossa toward the contralateral CP angle
or toward the foramen magnum (Table 8.1).
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_8, © Springer-Verlag Berlin Heidelberg 2013
8
Neuroimaging
Radiologically, epidermoids are well-delineated
cystic lesions that have irregular lobulated or
scalloped margins. On CT, they are hypodense;
rim or mural calcification is rarely observed
(Fig. 8.3) [9, 22].
On T1-weighted MR images, epidermoids
are characteristically heterogeneous lesions
with low signal intensity, usually isointense to
CSF (Fig. 8.3). T2-weighted MR images typi-
cally demonstrate a nonhomogeneous lesion
with high signal intensity [11, 13, 31]. The
intensity of the signal on MRI is related to the
cyst contents: in case cholesterol predominates,
the cysts are hyperintense on T1-weighted
images and hypointense on T2-weighted images.
The main differential diagnosis of epidermoids
is arachnoid cysts. Their radiologic characteris-
tics on routine CT and MRI are similar [11].
Arachnoid cysts have characteristically less
mass effect and are more homogeneous on
T2-weighted images. In contrast to arachnoid
cysts, epidermoids cause no adjacent bony reac-
tion on bone-window CT [6]. Fluid-attenuated
inversion recovery imaging (FLAIR), diffusion-
weighted imaging, and CISS spin-echo MR
images are more accurate imaging modalities
that allow for definitive differentiation of these
entities [13]. On the FLAIR sequence, epider-
moid cysts can be easily differentiated from
arachnoid cysts because the former show mixed
iso- to hypersignal intensities but with poor
demarcation, while the signal of the latter is
509
510 8 Cerebellopontine Angle Epidermoids

a b

c
d

Fig. 8.1 CT and MRI images of a large CP epidermoid, extending into the prepontine cisterns and even to the
contralateral CP angle
Management
a
b double vision [3, 5, 9, 10, 24]. Vestibular symp-
Fig. 8.2 CISS MR sequence, clearly demonstrating the
epidermoid in the IAC and a smaller part protruding into
the CP angle
suppressed, like the signal of CSF [16]. The
presence of residual tumor is also well visual-
ized on these later sequences [27].
MR cisternography, by means of heavily
T2-weighted 3D sequence, demonstrates the epi-
dermoid cyst signal hypointense to CSF, reveals
the lobulated margins of the tumor, and clearly
depicts the anatomical relation to neighboring
nerves and vessels, allowing precise surgical
planning [16, 21]. DWI offers a finding specific
for extra-axial epidermoid cysts by showing a
very high signal. Restricted ADC compared to
CSF, almost comparable to that of the brain, and
T2 shine-through effect both play an important
role in the high signal intensity of epidermoid
cyst at DWI (Figs. 8.3 and 8.4) [4, 16]. MR spec-
troscopy might be helpful, as it shows only ele-
vated lactate peaks.
Epidermoids are nonenhancing lesions. In
case an enhancing component is visualized, a
squamous carcinoma arising from an epidermoid
should be suspected [19].
511
Clinical Presentation
The very slow growth of CP epidermoids causes
insidious and protracted course of symptoms and
signs. The typical patient is usually in the third to
fifth decades of life and has a long-standing
history of tinnitus and hearing loss [18, 25].
Symptoms are caused by compression of neuro-
vascular structures or by irritation due to the
adhesion of cysts’ capsule. The most common
symptoms are hearing impairment and trigeminal
neuralgia. Other common signs/symptoms are
headache, cerebellar and pyramidal signs, facial
weakness, hemifacial spasm, glossopharyngeal
neuralgia, lower cranial nerve dysfunction, and
toms are rarely seen. Epidermoids cause trigemi-
nal neuralgia and facial nerve signs more
frequently than other CP angle tumor [25].
Patients may present with classical trigeminal
neuralgia or with constant neuralgic facial pain
[14]. The cranial nerves hyperactive dysfunction
syndrome may be caused by several mechanisms
or combination thereof: direct compressive effect,
“strangle” of the nerve caused by the growing
tumor with consequent reduction of its blood
supply, pushing the nerve against a pulsating
blood vessel, or local irritation from cholesterol
oozing [9, 14, 17, 25]. Cyst rupture—although
rare—may cause recurrent episodes of aseptic
meningitis and lead to hydrocephalus [1, 7].
Management
The treatment of choice of CP epidermoids is
surgical, and the preferred approach is the ret-
rosigmoid [14, 15, 20, 25, 28, 30]—see Case
Illustrations 8.1, 8.2, 8.3, and 8.4. The approach
allows for removal of epidermoids of any
size, even those extending beyond the CP angle
[12, 25, 30]. We discourage from utilization of
translabyrinthine or any other hearing destructive
approach because after tumor removal, hearing
can improve or even recover.
For detailed description of the RS approach,
please refer to the appropriate chapter. The cra-
nial nerves are usually engulfed by the tumor
512 8 Cerebellopontine Angle Epidermoids

and usually not significantly dislocated. Due to
the long-standing compression, they might
have altered morphology and be very vulnera-
ble, in particular the cochlear nerve. Large epi-
dermoids with considerable extension are
removed consequently at each of the CP angle
levels—see Case Illustration 8.3. A lateral-to-
medial direction of removal is followed, which
allows for earlier identification of the cranial
nerves near their bony or dural entrance. The
tumor is removed completely in one region
before moving to the next one, thereby

a b

c d

Fig. 8.3 CT and MRI of a patient with a CP angle epider-
moid tumor with extension into the Meckels’ cave and
erosion of the petrous apex. (a, b) Native (a) and bone-
window (b, c) CT scans. The hyperdense signal on the
native CT is due to multiple calcifications, as seen on the
bone-window scan b. Note the erosion of the left petrous
apex (b). (d) T1-weighted sequence after gadolinium
administration. (e) FLAIR sequence. (f, g) T2-CISS
sequences. (h) ADC. (i) Diffusion MRI. (j–l) Proton den-
sity sequences
Management 513

e f

g h

Fig. 8.3 (continued)

514 8 Cerebellopontine Angle Epidermoids

i j

k l

Fig. 8.3 (continued)

minimizing the risk of overlooking a remnant.
The powerful ultrasonic aspiration can easily
cause injury to neural structures and is not rec-
ommendable. Simple suction, tumor forceps,
microdissector, and platelet knife are usually
sufficient. A helpful maneuver to avoid iatro-
genic injury is to decrease the suction power
while introducing or removing the suction tip
and increase it, while working under direct
microscope control. When a smaller tumor part
remains, it is hold with one hand and the suc-
tion with the other hand. Thus, inadvertent tear
or suction-induced injury of the cranial nerves
can be avoided. Very important consideration
in epidermoids is that major vessels may be
embedded in the tumor mass, in contrast to VS,
in which the large vessels are on the tumor
capsule.
Management
Table 8.1 Tumor location and extension (n = 60)
Location/extension of CP angle
epidermoids Rate (%)
CP angle alone 37
CP angle + transtentorial extension 8 removed in two steps (Figs. 8.6, 8.7, and 8.8).
CP angle + parasellar extension 5 Tumors that involve the contralateral CP angle
CP angle + middle fossa extension 10
CP angle + foramen magnum extension 18
CP angle + transtentorial and foramen 17
magnum
Tumor reaches midline 55
Contralateral CP angle 26
Modified from Samii et al. [25]
The removal of tumor capsule is performed
only when sufficient debulking has been achieved,
similar to the case of all other CP angle tumors.
Capsule dissection is performed under optimal
visualization and illumination with the two-hand
technique. In order to prevent additional neuro-
logical deficits, small remnants of the capsule
that are firmly attached to important structures
should not be removed.
The endoscope is an extremely useful tool in
case of CP angle epidermoids and should be
always available—see Case Illustration 8.3 and
8.4. We use straight and angled scopes—usually
30° and exceptionally 75° (Karl Storz GmbH). It
is used after the microscopically visible tumor
part has been removed to inspect for hidden tumor
remnants. All corners of the CP angle should be
systematically inspected. Any remaining tumor
can be removed either under microscopic or
endoscopic control.
Epidermoids located purely in the petrous
bone are exceptionally rare. Their removal
requires different approaches, depending on the
exact extension of the tumor (Fig. 8.5).
Large epidermoids, extending into adjacent
cranial base spaces, pose specific management
problems. Tumors with transtentorial extension
or growth toward the foramen magnum might be
removed via the RS approach with the additional
use of angled endoscopes. The additional inci-
sion of the tentorium and use of endoscope, how-
ever, does not always allow complete removal of
the remaining portion. Actually, tumor rests are
frequently well visualized, but the restricted
515
access to a narrow deep space, as well as the lack
of appropriate instruments, prohibits its safe
removal. Therefore, very large epidermoids with
considerable supratentorial part have to be
are also removed in two separate steps. The
symptomatic side is approached initially. Notably,
the time between the two procedures should be
sufficient to allow recovery or improvement of
potential neurological deficits, especially of the
lower cranial nerve dysfunction. The contralat-
eral tumor is approached more conservatively to
avoid bilateral cranial nerve deficits.
During surgery, spillage of the epidermoid
contents should be avoided in order to prevent
aseptic meningitis or later recurrences.
Furthermore, communicating hydrocephalus can
develop in a delayed fashion due to leakage of
cyst contents into the operative field [2]. Following
tumor removal and hemostasis, the surgical field
should be thoroughly irrigated to ensure that all
particles are eliminated.
Outcome
The rate of radical removal in different published
series ranges from 38 to 75 % [20, 24–26, 28].
The senior authors’ experience encompasses
more than 110 CP angle epidermoids, removed
via the RS approach. Complete removal was
achieved in 75 % of the cases. Surgical morbidity
was related mainly to cranial nerve dysfunction.
Possible complication after surgery is aseptic
meningitis, which is reported to vary between 2.5
and 40 % [25]. Its occurrence might be prevented
by careful operative technique and application of
dexamethasone, 16–24 mg/day, for 1–2 weeks
after surgery.
Recurrences
Epidermoids tend to recur even decades after pri-
mary surgery. In subtotally resected epidermoids,
recurrences can develop even after 20 or 30 years.
Close follow-up of such patients is therefore
516 8 Cerebellopontine Angle Epidermoids

mandatory. The radiological detection and differ-
entiation of small recurrent epidermoids from
CSF may be difficult [24]. Their diagnosis is
based on specific MR sequences: the fast fluid-
attenuated inversion recovery (fast-FLAIR) MR
imaging and the echo-planar diffusion-weighted
imaging [8]. Dechambre et al. [8] demonstrated
the usefulness of DWI for postoperative follow-
up as it allows confirmation of the presence of a
possible residual or recurrent tumor. Considering
the very slow growth rate of small remnants,
observation might be the most adequate initial
strategy. Repeated operative removal is indicated
in case the recurrence becomes symptomatic or
reaches considerable size and a steady growth
tendency is observed. Such surgeries are more
difficult and risky due to the development of scar
tissue and adhesions.

a b

c d

Fig. 8.4 Postoperative imaging of the patient from Fig. 8.3—(a) Native CT scan. (b) T1-weighted native sequence.
(c) T1-weighted sequence after gadolinium. (d) FLAIR sequence. (e) T2-CISS sequence. (f) ADC. (g) Diffusion MRI
Management 517

e f

Fig. 8.4 (continued)

518 8 Cerebellopontine Angle Epidermoids

a b

c d

Fig. 8.5 Petrous bone epidermoid. The patient had been
operated for the first time 12 years earlier in another facil-
ity, and complete removal of a medial petrous epidermoid
had been achieved. Five years later, a recurrence was
detected and was initially observed. It showed, however,
growth tendency. At presentation, the patient had trigemi-
nal hypesthesia and dysesthesia, as well as anacusis on the
left side. (a, b) Bone-window CT scans, demonstrating
the bone remodeling. (c–f) MR images. We decided in
favor of the subtemporal epidural approach and succeeded
to remove the tumor completely utilizing endoscope-
assisted microsurgical technique. The patient did not have
new neurological deficits following surgery
Management
e f
Fig. 8.5 (continued)
a b
Fig. 8.6 Initial MR images, showing a giant epidermoid
with massive supra- and infratentorial extension. (a, b)
T1-weighted sequences after gadolinium administration.
(c, d) FLAIR sequences. (e–g) T2-sequences. (h–j) CISS
sequences. This 32-year-old female patient presented
with decreased bilateral vision, mainly on the left side
(she could not count fingers >20 cm distance) and right-
sided homonymous hemianopia. She has been diagnosed
of having an epidermoid incidentally 6 years earlier at
another institution and has been followed conservatively
with irregular MRI scans. Considering the size and exten-
sion pattern of the lesion, a decision in favor of two-step
tumor removal—via a frontotemporal and retrosigmoid
519
approaches—was taken. Due to the severe visual deficit,
the supratentorial part was approached initially. The
tumor was removed microsurgically with endoscopic
assistance, and decompression of the olfactory, optic,
oculomotor, and abducens nerves, as well as of the pitu-
itary stalk and the internal carotid and basilar artery and
their branches, was achieved. The patient recovered well
after surgery but on the seventh post-op day, developed
severe hyponatremia due to a SIADH. The endocrino-
logical examination revealed a slight hypocortisolism
and hypothyroidism, necessitating substitution. After
regulation of the electrolyte homeostasis, she was
discharged
520 8 Cerebellopontine Angle Epidermoids

c d

e f

Fig. 8.6 (continued)

Management 521

g h

i j

Fig. 8.6 (continued)

522 8 Cerebellopontine Angle Epidermoids

a b

c d

f
Management 523

a b

Fig. 8.8 CT scan before (a) and after (b) the second patient recovered rapidly and was discharged without new
surgery: removal of the remaining infratentorial tumor via neurological deficits
a retrosigmoid approach with endoscopic assistance. The

Fig. 8.7 MR images prior to the second surgery. The homonymous hemianopia persisted. No double vision or
patient was admitted again in 6 months for the planned other symptoms were noted. The hormonal levels were
second stage. Her vision was significantly better, but the normal besides the hypothyroidism
524
Case Illustration 8.1
CP Angle Epidermoid 1
This 43-year-old male patient presented with
long-standing history of hearing loss on the
right side. MRI was performed 2 months prior
to admission and showed an epidermoid tumor
in the right CP angle. At admission, the patient
had right-sided hypacusis—approximately
40 dB hearing loss in the main speech area. He
had no further neurological deficits or com-
plaints. The MRI study demonstrated the
medium-sized epidermoid that was embedded
in the medial part of the lower CP angle in the
space between the brain stem and cerebellum
(the cerebellopontine fissure). It looked inti-
mately related to the most medial parts of the
seventh and eighth cranial nerves, in particular
to their exit/entry zones. On T1- and
T2-weighted images, the lesions were isoin-
tense to the CSF (a, c, d). No contrast uptake
was demonstrated. The epidermoid, as well as
its relations to the cranial nerves, was clearly
seen on the CISS study (e).
The patient was operated via the standard
RS approach. The CP angle was exposed, and
the epidermoid located at the level between
the seventh and eighth and the lower cranial
nerves (*) was seen (f). It was medial to the
lower cranial nerves and had a small exten-
sion into the jugular foramen. The epidermoid
was debulked using microdissector (g) and
tumor forceps (h). The arrow points at the
tumor part entering the jugular foramen (i).
8 Cerebellopontine Angle Epidermoids
A microhook was used to remove the tumor
from jugular foramen (j); the tumor mass
could be then mobilized from the lower cra-
nial nerves (k). Further removal allowed visu-
alization of the choroid plexus of the fourth
ventricle, which was densely adherent to the
epidermoid capsule (l). The PICA was gradu-
ally dissected from the capsule. Extreme care
was paid to preserve all tiny arterial branches
(m). The tumor capsule was dissected from
the brain stem with the two-hand technique
(BS—brain stem). In the depth of the opera-
tive field, the vertebral artery (VA) was then
seen. The epidermoid (n) was actually embed-
ded in the brain stem between the exit/entry
zone of the facial and cochlear nerves. These
nerves were preserved anatomically; however,
the cochlear nerve was severely changed by the
long-standing compression, and during its dis-
section, the ABR wave V was lost. Although
it was extremely adherent to the brain stem,
the last tumor part was then also removed (o).
All neural and vascular structures of the CP
angle were preserved, including the petrous
vein (p, q—arrow). In this case, the endo-
scopic inspection confirmed the complete-
ness of tumor removal—no residual tumor
was found. Additional advantage of the endo-
scope was that it allowed directly excluding
bleeding from infratentorial bridging veins
(r–t: arrowhead, trochlear nerve; 5, trigemi-
nal nerve; 7, facial nerve; BS, brain stem; P,
posterior pyramid).
Management 525

a b

c d

MRI of the patient demonstrating the epidermoid cyst trating the consecutive steps of the epidermoid removal
in the right CP angle (a–e). Intraoperative images illus- (f–t). See the text for explanations
526 8 Cerebellopontine Angle Epidermoids

e f

g h
Management 527

i j

k l
528 8 Cerebellopontine Angle Epidermoids

m n

o p
Management 529

q r

s t
530
Case Illustration 8.2
CP Angle Epidermoid 2
This 31-year-old female patient presented
with left-sided facial hypesthesia (V1,2), pro-
gressive facial weakness (HB grade IV), and
hearing loss (PTA 20 dB). The diagnosis of a
CP angle cystic tumor was made 1 year earlier
at another facility, but the patient was preg-
nant, and follow-up was recommended.
During the last several months, however, the
hearing deteriorated, and a new MRI, pre-
sented on figures a–g, was made.
The patient was operated via the RS
approach (h–s). The dura was incised along
the transverse and sigmoid sinuses, and the
posterior aspect of the tumor was seen. The
seventh–eighth nerve complex was dislocated
8 Cerebellopontine Angle Epidermoids
caudally. Tumor removal was performed ini-
tially purely endoscopically. Later on, in
order to dissect the tightly attached tumor
capsule from the brain stem, we switched to
microscopic visualization. The angled endo-
scope was used to inspect those parts of the
operative field that remained hidden when the
microscope was used, and a remnant at
the brain stem, medial to the exit zone of the
facial nerve, was identified and removed.
Complete removal of the epidermoid was thus
achieved.
The patient had an uneventful postoperative
period. Her hearing level remained unchanged.
Intensive facial training was started, and the
facial nerve function slightly improved. The
postoperative MRI is presented on figures T–U.
Management 531

a b

c d

(a–g) MRI showed a lesion in the left CP angle with
characteristics, compatible with an epidermoid. (a, b)
T1-weighted sequence after gadolinium enhancement.
(c, d) T2-weighted sequence. (e) Proton density sequence.
(f) T2-CISS sequence. (g) FLAIR sequence. (h–s)
Intraoperative images: (h) initial microscopic view of the
tumor, located superior to the seventh–eighth cranial
nerves (7–8), reaching superior the tentorium (T).
P petrous bone, R retractor. (i) Corresponding endo-
scopic view. (j) Tumor removal under pure endoscopic
observation. (k) Gradually the brain stem came into
view. Note the preserved vessels within the tumor and
the petrous vein. (l) Further bimanual removal and dis-
section of the epidermoid capsule. (m) The major part of
the tumor is removed; the trigeminal (5) and the seventh–
eighth cranial nerves are free from tumor. Piece of the
tumor capsule was densely adherent to the brain stem
(m, n—asterisk; BS brain stem). (o) This part was
removed under microscopic visualization. (p–r)
Endoscopic control at the end of surgery and the corre-
sponding microscopic view (s). (t, u) Postoperative MR
images, demonstrating the completeness of removal
532 8 Cerebellopontine Angle Epidermoids

e f

h
g

i
j
Management 533

k l

m n

o p
534 8 Cerebellopontine Angle Epidermoids

q r

s t

u
Management
Case Illustration 8.3
CP Angle Epidermoid 3
One year prior to presentation, this 55-year-old
male patient had a transient ischemic attack.
MR examination of the brain was performed,
and a cystic lesion in the right CP angle was
visualized. Radiological follow-up was rec-
ommended. Since several months, however,
he noticed some hearing deterioration and
numbness of the right half of the face. At
admission, he had a trigeminal hypesthesia
and paresthesia (V1–3) and hearing loss on
the right side (audiogram: hearing loss of
35 dB in the main speech area). MRI revealed
a cystic lesion in the upper right CP angle
(a–e). The characteristics of the lesion were
compatible to those of an epidermoid.
The patient was operated via the RS
approach. Initially, only the epidermoid,
extending lateral to the IAC, could be seen.
The cranial nerves were hidden—they were
either embedded in the tumor or compressed
medially. An extremely careful tumor debulk-
ing was started. The fifth cranial nerve—and
later the seventh–eighth nerve complex—
could be identified using the tentorium and
the porus acusticus internus as landmarks.
535
Tumor removal continued medially along
these nerves up to the brain stem. This part
of the epidermoids’ capsule was very adher-
ent to the pia of brain stem. Still, using the
two-hand dissection technique, the capsule
could be completely dissected, and all small
vessels were preserved. As noted earlier in
epidermoids—in contrast to VS—major ves-
sels can be embedded in the tumor mass. In
the current case, a complex arterial network
was found deep in the lower tumor part. The
tumor had to be removed between the ves-
sels, and its capsule had to be dissected from
the vessel wall.
The inspection with angled endoscope dem-
onstrated complete removal of the epidermoid
in the CP angle. A remnant was seen, however,
in the IAC and removed with a microhook
under microscopic control. The endoscope was
used again at the end to confirm the complete-
ness of tumor removal.
The patient recovered rapidly from surgery
and had no new neurological deficits. Hearing
was preserved at the preoperative level. At the
sixth postoperative day, a subcutaneous CSF
collection appeared and was treated with a
lumbar drainage for 6 days.
536 8 Cerebellopontine Angle Epidermoids

a b

c d

(a–d) Preoperative MRI—(a, b): T1-weighetd
sequence after contrast administration. (c, d) Native
T1-weighted sequence. (e) T2-weighted sequence.
(f–q) Intraoperative endoscopic and microscopic
images. (f) Initial view of the CP angle and of the epi-
dermoid. (g) Tumor removal and visualization of the
trigeminal nerve and upper pons (h). Dissection of
the tumor from the multiple arteries embedded in the
tumor (i–k) and from the brain stem (l). (m) Endoscopic
examination demonstrating small remaining tumor in
the IAC (arrow). (n, o) Removal of the remnant under
microscopic control. (p, q) Complete removal of the
epidermoid: endoscopic panoramic view at the end of
surgery. The arrow points at the IAC, free form tumor;
arrowhead—Dorello’s canal and abducens nerve
Management 537

e f

g h
538 8 Cerebellopontine Angle Epidermoids

i j

k l
Management 539

m n

q
540
Case Illustration 8.4
CP Angle Epidermoid 4
Fifty-one-year-old female with a history of
headache and facial pain on the right side
since 2 years. MR examination was performed
due to exacerbation of her complaints, and a
right-sided CP angle mass was visualized.
Neurological examination at presentation
revealed no abnormalities, besides the strong
trigeminal pain (V1,2) on the right side. Her
MRI is presented on figures a–c.
The tumor was exposed via the RS avenue
(d–q): it was found to be located medial to the
fifth and seventh–eighth cranial nerves.
Therefore, it was approached initially via the
upper CP angle level (between the tentorium
and the trigeminal nerve), taking care not to
injure the petrosal vein. Later on tumor, removal
was continued via the level between the trigem-
inal and the vestibulocochlear nerves. Gradually,
the basilar artery could be visualized in the
depth of the operative field. Then, the remaining
8 Cerebellopontine Angle Epidermoids
tumor was removed via the level defined by the
fifth and seventh–eighth cranial nerves.
Although a complete removal was presumed,
the inspection with an angled endoscope (30°)
revealed tumor remnant at the brain stem, ante-
rior to the REZ of trigeminal nerve. This part
was removed under microscopic control and
verified with the endoscope. By pointing the
endoscope more anteriorly, a small tumor rem-
nant, localized just lateral to the lower basilar
artery, was seen and removed under direct
endoscopic control. The last three figures dem-
onstrate the complete tumor removal and the
preservation of all neural and vascular struc-
tures of the CP angle. A rare anatomical variant
was seen: a small vessel passed through the
split abducens nerve.
The postoperative period was uneventful,
and the patient had no neurological deficits.
The pain syndrome was initially controlled
with medications. At 6 months follow-up, the
pain had completely disappeared.
Management
a b
c
Epidermoid tumor, located in the medial CP angle on
the right side. Preoperative T1-weighted MR images:
native (a) and after gadolinium administration (b). (c)
FLAIR sequence. Intraoperative images, illustrating
the consecutive steps of tumor removal. (d) Initial
microscopic view of the CP angle and the epidermoid
(arrows), medial to the trigeminal (5) and vestibuloco-
chlear (7–8) nerves. (e, f) The tumor was approached
initially via the superior CP angle level, above the
trigeminal nerve, taking care not to injure the petrous
vein (asterisk). (g) Further tumor removal via the level
between the trigeminal and the vestibulocochlear
541
d
nerves. (h, i) Gradually, the basilar artery was visual-
ized (arrowheads). (j) The endoscope allowed to
detect a small remnant at the brain stem, which was
hidden by the trigeminal nerve. (k) Removal of the
remnant under microscopic control. (l, m) Small piece
of the tumor (arrow) was seen at the clivus, close to
the basilar artery and removed under endoscopic con-
trol (n). (o) The abducens nerve was split, and a vessel
was passing through it. Endoscopic (p) and micro-
scopic (q) view of the CP angle at the end of surgery
(CP—choroid plexus). (r, s) Postoperative T1- and
T2-weighted MRI—complete tumor removal
542 8 Cerebellopontine Angle Epidermoids

e f

g h
Management 543

i j

m n
544 8 Cerebellopontine Angle Epidermoids

o p

q r

s
References 545

References 15. Lalwani AK (1992) Meningiomas, epidermoids, and

other nonacoustic tumors of the cerebellopontine
angle. Otolaryngol Clin North Am 25(3):707–728
1. Abramson RC, Morawetz RB, Schlitt M (1989)
16. Liu P, Saida Y, Yoshioka H, Itai Y (2003) MR imaging
Multiple complications from an intracranial epider-
of epidermoids at the cerebellopontine angle. Magn
moid cyst: case report and literature review.
Reson Med Sci 2(3):109–115. doi:JST.JSTAGE/
Neurosurgery 24(4):574–578
mrms/2.109 [pii]
2. Ahmed I, Auguste KI, Vachhrajani S, Dirks PB, Drake
17. Maccarty CS, Leavens ME, Love JG, Kernohan JW
JM, Rutka JT (2009) Neurosurgical management of
(1959) Dermoid and epidermoid tumors in the central
intracranial epidermoid tumors in children. Clinical
nervous system of adults. Surg Gynecol Obstet 108(2):
article. J Neurosurg Pediatr 4(2):91–96.
191–198
doi:10.3171/2009.4.PEDS08489 [pii]
18. Mallucci CL, Ward V, Carney AS, O’Donoghue GM,
3. Altschuler EM, Jungreis CA, Sekhar LN, Jannetta PJ,
Robertson I (1999) Clinical features and outcomes in
Sheptak PE (1990) Operative treatment of intracranial
patients with non-acoustic cerebellopontine angle
epidermoid cysts and cholesterol granulomas: report of
tumours. J Neurol Neurosurg Psychiatry 66(6):768–771
21 cases. Neurosurgery 26(4):606–613; discussion 614
19. Mohanty A, Kolluri VR, Santosh V (1996) Squamous
4. Annet L, Duprez T, Grandin C, Dooms G, Collard A,
cell carcinomatous change in a posterior fossa epider-
Cosnard G (2002) Apparent diffusion coefficient
moid: case report with a review of the literature. Br J
measurements within intracranial epidermoid cysts in
Neurosurg 10(5):493–495
six patients. Neuroradiology 44(4):326–328.
20. Mohanty A, Venkatrama SK, Rao BR, Chandramouli
doi:10.1007/s00234-001-0726-2
BA, Jayakumar PN, Das BS (1997) Experience with
5. Berger MS, Wilson CB (1985) Epidermoid cysts of
cerebellopontine angle epidermoids. Neurosurgery
the posterior fossa. J Neurosurg 62(2):214–219.
40(1):24–29; discussion 29–30
doi:10.3171/jns.1985.62.2.0214
21. Murakami N, Matsushima T, Kuba H, Ikezaki K,
6. Bonneville F, Sarrazin JL, Marsot-Dupuch K,
Morioka T, Mihara F, Inamura T, Fukui M (1999)
Iffenecker C, Cordoliani YS, Doyon D, Bonneville JF
Combining steady-state constructive interference and
(2001) Unusual lesions of the cerebellopontine angle:
diffusion-weighted magnetic resonance imaging in
a segmental approach. Radiographics 21(2):419–438
the surgical treatment of epidermoid tumors.
7. Cantu RC, Wright RL (1968) Aseptic meningitic syn-
Neurosurg Rev 22(2–3):159–162
drome with cauda equina epidermoid tumor. J Pediatr
22. Nagashima C, Takahama M, Sakaguchi A (1982)
73(1):114–116
Dense cerebellopontine epidermoid cyst. Surg Neurol
8. Dechambre S, Duprez T, Lecouvet F, Raftopoulos C,
17(3):172–177
Gosnard G (1999) Diffusion-weighted MRI postop-
23. Osborn AG, Preece MT (2006) Intracranial cysts: radio-
erative assessment of an epidermoid tumour in the
logic-pathologic correlation and imaging approach.
cerebellopontine angle. Neuroradiology 41(11):
Radiology 239(3):650–664. doi:239/3/650 [pii]
829–831. doi:90410829.234 [pii]
24. Safavi-Abbasi S, Di Rocco F, Bambakidis N, Talley
9. Dee RH, Kishore PR, Young HF (1980) Radiological
MC, Gharabaghi A, Luedemann W, Samii M, Samii A
evaluation of cerebello-pontine angle epidermoid
(2008) Has management of epidermoid tumors of the
tumor. Surg Neurol 13(4):293–296
cerebellopontine angle improved? A surgical synopsis
10. deSouza CE, deSouza R, da Costa S, Sperling N,
of the past and present. Skull Base 18(2):85–98.
Yoon TH, Abdelhamid MM, Sharma RR, Goel A
doi:10.1055/s-2007-991108
(1989) Cerebellopontine angle epidermoid cysts:
25. Samii M, Tatagiba M, Piquer J, Carvalho GA (1996)
a report on 30 cases. J Neurol Neurosurg Psychiatry
Surgical treatment of epidermoid cysts of the cerebel-
52(8):986–990
lopontine angle. J Neurosurg 84(1):14–19.
11. Dutt SN, Mirza S, Chavda SV, Irving RM (2002)
doi:10.3171/jns.1996.84.1.0014
Radiologic differentiation of intracranial epidermoids
26. Schroeder HW, Oertel J, Gaab MR (2004) Endoscope-
from arachnoid cysts. Otol Neurotol 23(1):84–92
assisted microsurgical resection of epidermoid tumors
12. Goel A, Muzumdar D, Desai K (2006) Anterior tento-
of the cerebellopontine angle. J Neurosurg 101(2):
rium-based epidermoid tumours: results of radical
227–232. doi:10.3171/jns.2004.101.2.0227
surgical treatment in 96 cases. Br J Neurosurg
27. Sirin S, Gonul E, Kahraman S, Timurkaynak E (2005)
20(3):139–145. doi:XQ74738410212029 [pii]
Imaging of posterior fossa epidermoid tumors. Clin
13. Kallmes DF, Provenzale JM, Cloft HJ, McClendon RE
Neurol Neurosurg 107(6):461–467. doi:S0303-
(1997) Typical and atypical MR imaging features of
8467(04)00184-2 [pii]
intracranial epidermoid tumors. AJR Am J Roentgenol
28. Talacchi A, Sala F, Alessandrini F, Turazzi S, Bricolo
169(3):883–887
A (1998) Assessment and surgical management of
14. Kobata H, Kondo A, Iwasaki K (2002) Cerebellopontine
posterior fossa epidermoid tumors: report of 28 cases.
angle epidermoids presenting with cranial nerve
Neurosurgery 42(2):242–251; discussion 251–242
hyperactive dysfunction: pathogenesis and long-term
29. Yamakawa K, Shitara N, Genka S, Manaka S,
surgical results in 30 patients. Neurosurgery
Takakura K (1989) Clinical course and surgical
50(2):276–285; discussion 285–276
546
prognosis of 33 cases of intracranial epidermoid
tumors. Neurosurgery 24(4):568–573
30. Yasargil MG, Abernathey CD, Sarioglu AC (1989)
Microneurosurgical treatment of intracranial dermoid
and epidermoid tumors. Neurosurgery 24(4):561–567
8 Cerebellopontine Angle Epidermoids
31. Zamani AA (2000) Cerebellopontine angle tumors:
role of magnetic resonance imaging. Top Magn Reson
Imaging 11(2):98–107
 Nonvestibular Schwannomas
Schwannomas originate most frequently from the
sensory cranial nerves. In the absence of
neurofibromatosis, the motor nerves are involved
extremely rare [8, 73]. The most frequent nonves-
tibular schwannomas are those originating from
the trigeminal nerve, facial nerve, and lower cra-
nial nerves. Nonvestibular schwannomas consti-
tute 2–3 % of all CP angle tumors and 8–10 % of
all intracranial schwannomas [26, 74].
Trigeminal Schwannomas
General Features
Trigeminal schwannomas have been described
for the first time by Dixon in 1846 [3]. In 1918,
Frazier reported the first successful removal
of a primary trigeminal (gasserian) ganglion
neurinoma. Trigeminal schwannomas are the
second most frequent intracranial schwannoma,
comprising 0.8–8 % of all intracranial schwan-
nomas and 0.07–0.5 % of all intracranial tumors
[9, 10, 31, 43, 50, 65, 84].
These slow-growing tumors may arise from
the trigeminal nerve root, the trigeminal gan-
glion, or its peripheral branches. In 1955,
Jefferson [31] proposed a classification scheme
for trigeminal schwannomas that—with some
modifications—is currently generally applied [3,
13, 23, 43]. According to tumor location, three
distinct types were identified (Fig. 9.1a–c). Type
A tumors arise from the trigeminal ganglion
(gasserian ganglion) and are located mainly in
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_9, © Springer-Verlag Berlin Heidelberg 2013
9
the middle fossa; type B tumors arise from the
root of the trigeminal nerve and are located pre-
dominately in the posterior fossa; and type C
tumors have significant components in both mid-
dle and posterior fossae (hourglass or dumbbell
type). A fourth category—type D—was added
later on (Fig. 9.1d); it includes tumors originat-
ing more peripherally from the distal branches of
the trigeminal nerve. They have a smaller intrac-
ranial part and a predominant extracranial exten-
sion to the orbit, infratemporal fossa, or
pterygopalatine fossa [6, 43, 50].
Type A tumors occur in 24–50 % of the
patients, type B occur in 8–30 %, and type C in
20–76 % [31, 43, 65]. Type D tumors are
extremely rare—less than 50 cases have been
reported in the literature [2, 61]. This classification
is very useful for the selection of surgical
approach and for predicting the outcome.
Neuroimaging
The diagnosis of trigeminal schwannomas is
based on CT scan setting and on MRI, both native
and after gadolinium application. On CT, the
tumor generally appears isodense or slightly
hyperdense and enhances uniformly after admin-
istration of contrast material [47, 50, 77].
In type A tumors, sharp-edged bone defects in
the middle fossa floor, involving the foramen
ovale and the foramen spinosum, might be seen.
In case of significant medial and anterior exten-
sion, the lateral aspect of sella turcica, anterior
547
548 9 Nonvestibular Schwannomas

a b

c d

Fig. 9.1 (a) Type A schwannomas, arising from the fossa. (c) Type C schwannomas—hourglass or dumbbell
trigeminal ganglion and are located mainly in the middle type with middle and posterior fossae parts. (d) Type D
fossa. (b) Type B schwannomas, arising from the trigeminal schwannomas, arising from the distal branches of the
nerve root and are located predominately in the posterior trigeminal nerve with a predominant extracranial extension

clinoid process, dorsum sellae, and superior
orbital fissure may be eroded [15, 31, 52]. In type
B and C schwannomas, erosion of the petrous
apex and of the inferomedial surface of the
petrous bone might be seen.
On MRI, trigeminal schwannomas appear as
well as circumscribed lesions, isointense or
hypointense on T1-weighted images and hyper-
intense on T2-weighted sequences. They
enhance homogeneously after the administra-
tion of gadolinium [61, 65, 86]. Cystic compo-
nents have been identified in up to 26 % of the
cases in some series [3]. Axial, sagittal,
and coronal views are essential for precise
Trigeminal Schwannomas
evaluation of the tumor extension and for plan-
ning the operative approach.
Conventional cerebral angiography currently
is seldom required. Trigeminal schwannomas are
generally less vascular tumors, and embolization
is needed in exceptional cases. Magnetic reso-
nance angiography demonstrates reliably the dis-
placement of major vessels, for example,
displacement of the intracavernous and petrosal
segments of the internal carotid artery and stretch-
ing of the carotid siphon in anteriorly situated
tumors [23, 76]. Rarely, the carotid artery may be
engulfed by the tumor. Posterior fossa (type B)
and dumbbell-shaped (type C) tumors frequently
elevate and displace medially the posterior cere-
bral and the superior cerebellar arteries. These
tumors can appear as encircled by the posterior
cerebral artery in the superior aspect and by the
superior cerebellar artery inferior [59]. The basi-
lar artery is displaced usually in medial and pos-
terior direction.
Tumors that grow anterior may penetrate the
cavernous sinus wall and expand within the sinus,
leading to characteristic displacement of the
carotid artery, described above [50, 65]. In rare
cases, the tumor may penetrate the temporal dura
and expand intradurally. Type B and C trigeminal
schwannomas extend out of the porus trigeminus
or through the eroded medial petrous bone intra-
durally into the posterior fossa.
The differential diagnosis of trigeminal
schwannomas includes meningiomas, epider-
moids, vestibular schwannomas, chordomas,
chondrosarcomas, lymphomas, and skull-base
metastases.
Clinical Presentation
The average age at diagnosis is similar to that in
patients with other intracranial schwannomas—
typically the fourth to fifth decades of life.
A characteristic clinical syndrome caused by
trigeminal schwannomas does not exist: clinical
presentation is related rather to their location and
size. Most common symptoms and signs are
trigeminal sensory deficit—paresthesia or numb-
ness (74–96 %), often in more than one division
549
of the nerve; trigeminal motor deficit (33–60 %);
facial pain (15–34 %); headache (20–44 %);
diplopia (15–52 %); and—more rarely—hearing
loss, ataxia, and various cranial nerve palsies [13,
23, 50, 76]. Interestingly, up to 10 % of patients
may not have trigeminal dysfunction as a pre-
senting feature [13, 43, 50].
Wasting of the temporal and pterygoid mus-
cles is a relatively common finding and is
observed in 24–60 % of patients. The corneal
reflex is depressed or absent in 36–82 % of
patients. Very large tumors cause symptoms of
increased intracranial pressure, papilledema, and/
or contralateral pyramidal signs. Cranial nerve
palsies occur usually secondary to tumor com-
pression. In some series, as many as 50–52 % of
the patients had oculomotor nerve palsies by the
time of diagnosis [3, 38, 43]. Dysfunction of the
abducens nerve is seen in up to 40 %; this nerve
is the second most frequently affected cranial
nerve after the trigeminal one.
The facial pain in patients with trigeminal
schwannomas can vary in intensity and type—
from dull constant to severe lancinating pain. It
differs from the pain in patients suffering from
trigeminal neuralgia, and the two conditions should
not be confused. These tumors very rarely cause
typical trigeminal neuralgia. Most patients com-
plain of constant pain or of paroxysmal lancinat-
ing facial pain with attacks that last longer and do
not always have specific triggering mechanisms.
In contrast to trigeminal neuralgia, the pain related
to trigeminal schwannomas does not respond well
to medications, such as carbamazepine [13, 85].
Although trigeminal schwannomas are typi-
cally benign lesions, histological malignancy has
been reported with varying frequency—from 0 to
7.9 % [13, 17, 23, 50].
Management
In the last decades, remarkable improvement in
the surgical management of trigeminal schwan-
nomas has been achieved in terms of complete-
ness of removal, avoidance of major morbidity,
and preservation of neural functions [3, 13, 17,
23, 65, 76]. The reported rate of total or near-total
550
removal in largest series is above 70 %. Complete
tumor removal is possible even in case of large
schwannomas because they most frequently dis-
place surrounding neurovascular structures rather
than engulf them.
The optimal management of patients with
trigeminal schwannomas, according to our experi-
ence, is their complete surgical removal, which
leads to definitive cure of the patient. The preserva-
tion of neurological functions, however, has always
a priority. Therefore, exceptions to complete
removal are those cases in which the tumor infiltrates
the neurovascular structures or a clear dissection
plane to the brain stem is absent. Patients in bad
general condition, who cannot tolerate prolonged
surgical procedures, as well as those unwilling sur-
gery, may be followed (small tumors, stable minor
neurological deficit) or treated with radiosurgery.
Surgical Treatment
Inadequate tumor exposure has been long regarded
as a major reason for incomplete tumor removal.
Various skull-base techniques and extensive
approaches, which were supposed to facilitate
tumor removal, have been therefore introduced.
Some early reports suggested some surgical benefit
when using a “skull-base approach.” Taha et al.
[76] compared their experience with the conven-
tional approaches, such as frontotemporal
transsylvian, subtemporal–intradural, subtempo-
ral–transtentorial, and suboccipital, and the
skull-base approaches (frontotemporal extradural–
intradural, frontoorbitozygomatic, subtemporal
anterior petrosal, and presigmoid posterior petro-
sal) for the excision of trigeminal schwannomas.
In a series of 15 patients published in 1995, they
found that tumor exposure, ease of dissection, and
radicality of removal were superior with skull-base
approaches. Residual or recurrent tumors were
found in 65 % of patients following conventional
approaches compared with 10 % of patients fol-
lowing skull-base approaches.
Trigeminal neurinomas of the middle
fossa have been operated via the frontotem-
poral transsylvian, cranioorbitozygomatic,
subtemporal–intradural, zygomatic middle fossa,
or the combined subtemporal–intradural and extra-
dural approaches [3, 20, 76]. The extradural ante-
rior temporopolar approach and the subtemporal
9 Nonvestibular Schwannomas
extradural–intradural approach, combined with
drilling the bone margins of the superior orbital
fissure, lateral wall of the cavernous sinus, and
Meckels’ cave, are recommended for intracav-
ernous lesions [14, 16, 25]. Dumbbell tumors
are operated via the petrosal, extended petrosal,
or combined petrosal approaches [3, 13, 72].
The subtemporal (subtemporal–transtentorial)
approach with incision of the tentorium has been
recommended for tumors extending through the
porus trigeminus into the posterior fossa, which
remain cephalad to the seventh–eighth nerve
complex [28, 76, 82]. The anterior petrosal or
Kawase approach, which includes resecting the
pyramidal apex, is proposed for better expos-
ing the tumor and the petroclinoid region down
to the inferior petrosal sinus [33]. Some authors
even recommend the subtemporal extradural–
intradural approach combined with presigmoid
posterior petrosectomy or with an anterior petro-
sectomy [4, 51, 68, 69] as the optimal approach,
which provides excellent exposure of the tumor
with minimal brain retraction.
With increasing experience, however, it became
obvious that the goals of surgery—complete tumor
removal and preservation of neurological func-
tions—could be well achieved using more simple
and safe approaches. The senior author favored the
combined presigmoid–subtemporal approach for
type C tumors previously (Case Illustration 9.2).
In an early series, published in 1995 [65], this
approach was used in 42 % of the cases, while the
retrosigmoid was used in just 8 %. Nowadays,
however, this approach is not considered suitable
for trigeminal schwannomas removal because of
the high risk of approach-related morbidity. The
retrosigmoid suprameatal approach is a safer and
simpler alternative, allowing sufficient exposure
of the tumor. The main restrictions to complete
removal are the tumors’ biological characteristics
and surgeons’ experience rather than the extent of
the approach or of the tumor exposure.
Type A Trigeminal Schwannomas
(Predominant Location in the Middle
Cranial Fossa)
(a) Frontotemporal (pterional) transsylvian
approach. This flexible approach provides
adequate exposure to most type A tumors.
Trigeminal Schwannomas
A low temporal craniotomy and early opening
of the basal cisterns and of the Sylvian fissure
to allow sufficient egress of CSF are manda-
tory to avoid excessive brain retraction. Unless
the tumor extends into the infratemporal fossa,
an additional zygomatic osteotomy is not
required.
a b
Fig. 9.2 Type A trigeminal schwannoma, located mainly
in the Meckels’ cave (a). The bone-window CT demon-
strated clearly the extent of erosion of the petrous apex
(b). Using the BrainLAB software, the relation of the
tumor to the petrous and the cavernous segments of the
551
(b) Infratemporal extradural technique. Trigeminal
schwannomas located within the Meckels’
cave or in the posterior cavernous sinus can
be approached via the infratemporal extradural
technique (Fig. 9.2). In case the tumor has lim-
ited extension into the posterior fossa, it can be
removed by incising the tentorium.
carotid artery could be appreciated (c). The subtemporal
extradural approach was chosen and performed under
navigational guidance (d). The postoperative MRI (e, f)
confirmed the completeness of tumor removal
552 9 Nonvestibular Schwannomas

Fig. 9.2 (continued)

Trigeminal Schwannomas
e f
Fig. 9.2 (continued)
Type B Trigeminal Schwannomas
(Predominant Posterior Fossa Location)
(a) Retrosigmoid approach. The standard RS
approach, as described in the book, is
sufficient for tumors, located within the pos-
terior fossa or those having minimal exten-
sion into the Meckels’ cave.
(b) Retrosigmoid suprameatal approach. The
additional removal of the suprameatal tuber-
cle exposes the Meckels’ cave tumor portion
553
(see chapter Retrosigmoid Suprameatal
Approach and Case Illustration 9.1).
Trigeminal schwannomas are located
typically medial to the seventh–eighth cra-
nial nerve complex. The working corridor is
between these nerves and the tentorium.
Extreme care should be therefore applied
not to injure the nerves during tumor
removal or when inserting/removing the
instruments.
554
Case Illustration 9.1
Retrosigmoid Suprameatal Approach
Twenty-seven-year-old female patient pre-
sented with MR images showing a polycyclic
trigeminal schwannoma on the right side with
a predominant CP angle tumor and smaller
portions in the Meckels’ cave (a–f). The
examination was performed because of
unspecific complaints of slight vertigo and
numbness in the left hand. Her neurological
status was normal at admission. Still, because
of the tumor large size and the severe brain
stem compression, surgery was the only man-
agement option. The retrosigmoid suprameatal
approach was selected.
The tumor was exposed between the sev-
enth and eighth cranial nerves and tentorium
(g), and internal decompression with CUSA
and suction was done (h). Then, the capsule
was separated carefully from the seventh–
eighth cranial nerve complex and from the
brain stem. This dissection was performed
using the 2-hand technique, as shown on (i):
the surgeon holds the tumor with one hand and
exerts slight traction (in the present case with
a microdissector), while the other hand is used
to peel the arachnoidea of the capsule. Despite
the very intense adhesions of the capsule to
the brain stem, the CP angle tumor part could
be completely removed. Then, the suprameatal
tubercle was drilled off with a diamond drill
9 Nonvestibular Schwannomas
(j, k), exposing the contents of the Meckels’
cave (l—arrows). This allowed mobilization
of the remaining tumor part, its dissection
from the surrounding structures—including
the oculomotor and abducens nerves—and
complete tumor removal (m–o). The trigemi-
nal nerve was splayed by the tumor, as seen on
(m–o), but could be preserved. Only the fas-
cicles from which the tumor originated had to
be sacrificed. Importantly, after tumor resec-
tion, all blood clots in the CP angle have to be
thoroughly removed. Thus, a hidden bleeding
point/vessel can be identified and coagulated;
secondly, a potential obstruction to the CSF
flow is avoided. After the removal of a large
clot through the level between the seventh and
eighth and the lower cranial nerves, the clivus
and the basilar artery could be visualized
(p, q).
The routinely performed CT on the day
after surgery (r) did not reveal any pathologi-
cal findings. In the first several days after sur-
gery, the patient had severe nausea and
vomiting that had to be managed with antie-
metics. Within 1 week, these complaints sub-
sided. She had trigeminal hypesthesia, mainly
in the V2 area; slight hearing loss; and HB
grade II facial palsy that improved during the
duration of the hospital stay. At follow-up, the
patient complained of slight trigeminal hypes-
thesia and decreased but functional hearing.
Trigeminal Schwannomas
a b
c
Preoperative MR study of the patient. (a–c) T1-weighted
sequence after contrast administration. (d, e) T2-weighted
sequences. (f) CISS sequence. (g–r) Intraoperative
images, illustrating the steps of surgery. (g) Initial view
of the tumor in the CP angle (Tu tumor, T tentorium, R
retractor, P pyramid, 7 seventh–eighth cranial nerve
complex). (h) Initial internal debulking with the ultra-
sonic aspirator (CUSA) and the suction (S). (i) Once
sufficient internal decompression was achieved, the cap-
sule was dissected from the surrounding structures. (j, k)
555
d
The suprameatal tubercle (dotted line) was removed to
gain access to the middle fossa tumor (l, arrows). (m)
Thus, the trigeminal nerve fibers could be better mobi-
lized and dissected from the tumor (asterisk (clivus)).
(n, o) Most of the fibers of the splayed trigeminal
nerve were not involved by the tumor. (p, q) Complete
tumor removal; preservation of all neurovascular struc-
tures (asterisk (basilar artery)). The basilar artery could
be visualized only after removal of the blood coagulum
(C). (r) Postoperative control CT scan
556 9 Nonvestibular Schwannomas

e f

g h

i
j
Trigeminal Schwannomas 557

k l

m
n

o p
558 9 Nonvestibular Schwannomas

q r
Trigeminal Schwannomas
Type C Trigeminal Schwannoma
(Dumbbell Tumors)
(a) Retrosigmoid suprameatal approach. The
approach is usually sufficient for tumors with
smaller middle fossa part.
(b) Retrosigmoid suprameatal approach with/
without additional resection of the tento-
rium and use of angled endoscope (Figs. 9.3,
9.4, and 9.5). The approach allows for safe
removal of the most challenging posterior
fossa tumor part and its dissection form the
a b
c d
Fig. 9.3 Type C trigeminal schwannoma with a very large middle cranial fossa part. (a, b) T1-images after contrast
administration and (c, d) CISS images
559
brain stem and cranial nerves under direct
visual control. Thereafter, the petrous apex
is resected intradurally by drilling off the
suprameatal tubercle. The Meckels’ cave
can be thus opened as widely as necessary,
according to the tumor expansion and the
individual anatomical characteristics. An
important advantage of the approach is the
enhanced possibility to preserve the
abducens nerve, which is frequently com-
pressed/dislocated by the tumor. In contrast
560 9 Nonvestibular Schwannomas

a b

c d

Fig. 9.4 Removal of the tumor presented on the previous
figure via the retrosigmoid suprameatal endoscope-
assisted approach. The tumor is seen in the depth of the
surgical field, hidden by the suprameatal tubercle (a). The
dura of the tubercle was excised (b), and it was drilled
off (c). Thus, sufficient exposure of the tumor, including
its Meckels’ cave part, was gained, and it could be deb-
ulked (d, e) and removed (f). The endoscopic inspection
did not reveal any tumor remnants (g, h)
Trigeminal Schwannomas
ee
g h
Fig. 9.4 (continued)
to the approaches from superior and lateral
(e.g., the anterior transpetrosal approach or
the subtemporal transtentorial approach),
the nerve can be identified early at its brain
stem exit zone and can be followed up to
Dorello’s canal during tumor removal. The
exposure of the supratentorial tumor part
can be further enlarged by resection of the
tentorium. Care should be taken not to
injure the superior cerebellar artery, which
is in close contact with the tumor, and of
the trochlear nerve.
561
f
Very important advancement in the last two
decades was the introduction of the
endoscope-assisted technique. The use of angled
endoscopes allows for reliable visualization of the
whole tumor, including its middle fossa part, and
its removal under microscopic or endoscopic guid-
ance (see Case Illustration 9.3). Even tumors with
considerable extension in the middle cranial fossa
can be thus removed (Figs. 9.3, 9.4, and 9.5). The
transpetrosal approaches, although used previously
by the senior author (see Case Illustrations 9.2 and
9.4), are currently rarely indicated.
562 9 Nonvestibular Schwannomas

a b

c d

Fig. 9.5 Postoperative control images of the patient presented on Figs. 9.3 and 9.4. (a) CT scan and (b–d) MR
images
Trigeminal Schwannomas
Case Illustration 9.2
Presigmoid–Subtemporal Approach
This is a case operated by the senior author in
the early 1990s. The patient had a dumbbell
type C tumor with large tumor portions in both
the middle and posterior cranial fossae. The
combined presigmoid–subtemporal approach
(b, c) was selected that allowed for perfect
exposure of the whole tumor and its
a b
(a) Preoperative MRI, demonstrating the extension of
the tumor. (b) Skin incision. (c) One-piece bone flap
was made. (d) Extent of bone removal. (e) Elevation of
the temporal lobe and lateral retraction of the cerebel-
563
surrounding neural structures (d). The tumor
could be removed completely (e). The patient
did not have new neurological deficits and
made an uneventful recovery. Nevertheless,
nowadays this tumor would be operated via
the much safer retrosigmoid suprameatal
approach with endoscopic assistance to ensure
the completeness of tumor removal, in partic-
ular in the area of the Meckels’ cave.
lum afforded a perfect visualization of the tumor and
of all surrounding structures. (f) Tumor removal. (g)
The tumor has been completely removed, and most of
the trigeminal nerve fibers have been preserved
564 9 Nonvestibular Schwannomas

c d

f g
Trigeminal Schwannomas
Case Illustration 9.3
Retrosigmoid Suprameatal Approach with
Endoscope Assistance
This 36-year-old female patient presented to
our department with a history of headache
since many years and facial pain in the area of
V2,3. A dumbbell trigeminal schwannoma
was diagnosed, and she had been operated in
another institution—the tumor part in the CP
angle was removed partially. After surgery,
the patient had trigeminal nerve hypesthesia,
vertigo, hearing loss, as well as gait instabil-
ity. Due to the high-dose steroid treatment, she
developed iatrogenic diabetes mellitus. At
presentation, she had V1 and V2 anesthesia
and V3 hypesthesia, atrophy of the mastica-
tory muscles, and hearing loss (20 dB PTA).
The MRI (a, b) showed a type C partially cys-
tic trigeminal schwannoma, located in the
Meckels’ cave and in the posterior cranial
fossa. A decision was taken to approach the
tumor via the retrosigmoid suprameatal route.
A retrosigmoid recraniotomy was per-
formed (c–r). Tumor exposure in the CP angle
proved to be extremely difficult due to the
a b
(a, b) Preoperative MRI showing a type C trigeminal
schwannoma, located in the Meckels’ cave and in the
posterior cranial fossa. (c–r) Intraoperative images.
565
intense adhesions after the first surgery (c).
The anatomical structures could not be recog-
nized initially, and the tumor was approached
along the posterior pyramid with very careful
blunt and sharp dissection and frequent use of
electrical stimulation (d). Then, the cystic
tumor contents were evacuated, internal
decompression was performed and the CP
angle tumor part was removed (e–g). Using
angled endoscope (30°), the remaining tumor
portion in the Meckels’ cave could be seen (h)
but could not be safely removed. Therefore,
the suprameatal tubercle (dotted line on figure
i) was drilled off (j, k). Using angled microin-
struments, it could be dissected from the sur-
rounding structures and from the trigeminal
nerve fascicles and removed as a large piece
under pure endoscopic guidance (l–o). The
remaining smaller tumor pieces in the anterior
part of the Meckels’ cave (p—asterisk) were
then removed with an endoscopic guidance (q)
and complete tumor resection achieved (r).
After surgery, the only new neurological
deficit was a transient diplopia due to a partial
abducens nerve dysfunction.
See the text for explanations. (5 trigeminal nerve P
pyramid, T tentorium, R retractor, Tu tumor, MC
Meckels’ cave, * small tumor remnants)
566 9 Nonvestibular Schwannomas

c d

e f

g h
Trigeminal Schwannomas 567

i j

k l

m n
568 9 Nonvestibular Schwannomas

o p

q r
Trigeminal Schwannomas
Type D Trigeminal Schwannomas
(Predominant Infratemporal
Extracranial Location)
These schwannomas usually originate from the
mandibular or maxillary divisions of the trigem-
inal nerve and extend into the infratemporal
fossa through the foramen ovale or foramen
rotundum. We favor the extradural subtempo-
ral–infratemporal approach with additional
removal of the zygomatic arch (Fig. 9.6) or the
frontotemporal intradural approach with endo-
scopic assistance (Fig. 9.7). In case the tumor
grows intradurally and compresses the tempo-
ral lobe, an additional temporal or frontotempo-
ral craniotomy is necessary. On Figs. 9.8 and
9.9, a rare trigeminal schwannoma, arising from
the intraorbital fascicles of the nerve, is
presented.
a b
c d
Fig. 9.6 Trigeminal schwannoma, extending into fossa
infratemporalis (surgery in the early 1990s). (a, b)
CT and T2-weighted MR images of a type D trigeminal
569
General Operative Technique
Trigeminal schwannomas are typically well encap-
sulated; have a smooth, lobulated appearance; and
are typically yellowish. Although relatively avas-
cular schwannomas with soft consistency predom-
inate, some tumors are firm and/or well
vascularized. Once the tumor is exposed, its
removal follows the standard scheme: initial intra-
capsular debulking and then dissection of the cap-
sule from adjacent neural structures in the
arachnoid plane. Most schwannomas have a good
arachnoid plane and can be dissected even from
the healthy fascicles of the trigeminal nerve. In
contrast to neurofibromas, which cause fusiform
enlargement of the whole nerve, schwannomas do
not involve all fascicles of the nerve and tend to
grow eccentrically. Hence, only the fascicles from
which the tumor originates should be sacrificed
avoiding severe trigeminal deficits—anesthesia,
masticatory problems, or disabling keratitis.
schwannoma. The tumor was exposed and removed
via the transzygomatic subtemporal extradural approach
(c, d). 5 trigeminal nerve; arrowheads tumor
570 9 Nonvestibular Schwannomas

a b

c d

Fig. 9.7 Type D trigeminal schwannoma with extracra-
nial extension into the infratemporal fossa via the enlarged
oval foramen. (a–c) T1-weighted sequences MR images
following contrast administration. (d, e) T2-weighted
sequence. (f, g) CISS sequence. (h) Preoperative bone-
window CT, demonstrating the enlarged oval foramen.
The tumor could be removed completely without causing
additional neurological deficits via the frontotemporal
intradural approach
Trigeminal Schwannomas 571

e f

g h

Fig. 9.7 (continued)

572
a b
Fig. 9.8 Trigeminal schwannoma type D, arising from
the intraorbital fascicles of the nerve (the patient was
operated in the 1980s). (a, b) CT demonstrating the loca-
a b
Fig. 9.9 Trigeminal schwannoma type D (continuation
of the previous figure). (a) Preoperative photo of the
patient. (b) Skin incision, marked with arrowheads and
exposure of the tumor (Tu) (c). Of note, identical tech-
nique is applied, regardless of the tumor origin/location:
9 Nonvestibular Schwannomas
tion of the tumor lateral and inferior the optic nerve and
bulb. The steps of its removal are presented in the next
figure
initially, the tumor is debulked, and only afterward, the
capsule is dissected and removed. Image (d) shows the
debulked tumor (arrows—tumor capsule). (e, f) Complete
tumor removal (R—retractor)
Trigeminal Schwannomas 573

c d

e f

Fig. 9.9 (continued)

574
Case Illustration 9.4
Melanotic Trigeminal Schwannoma
The CT and MRI images (a, b) of this patient
show a type C trigeminal schwannoma with
characteristics that do not differ from those of
classical tumors. The patient was operated via
the presigmoid subtemporal approach. On
figure (c), a microscopic photograph of the
exposed tumor, medial to the main trunk of
the trigeminal nerve, is presented. The tumor
a b
Preoperative CT and MRI, demonstrating the tumor and its extension (a, b). Intraoperative microscopic view of
the tumor (c). (d) Complete tumor removal
9 Nonvestibular Schwannomas
was highly pigmented and originated from the
fascicles of the nerve within the Meckels’
cave. It could be removed completely with
preservation of the adjacent cranial nerves,
including most fascicles of the trigeminal
nerve (d). Histopathological diagnosis was
WHO grade I melanotic schwannoma. The
follow-up of the patient is more than 10 years,
and up to now, no recurrences have been
detected.
Trigeminal Schwannomas 575

c d
576
Tumors involving the cavernous sinus can be
dissected from its wall and removed safely using
the current operative techniques [9, 17, 84]. The
experience of the senior author has shown, how-
ever, that very tinny intracavernous residual parts
might be indiscernible and could lead to tumor
recurrence. As reported previously in all eight
patients with tumor invading the cavernous sinus,
complete tumor removal was achieved [65]. At
follow-up MRI, however, in two cases, tumor
recurrence was detected. The risk of recurrence is
highest in trigeminal schwannomas located in the
cavernous sinus, followed by those located in
Meckels’ cave and the porus trigeminus, and is
least in posterior fossa tumors [76].
It has been pointed out that in case of incom-
plete resection, the recurrence rate is higher in
trigeminal schwannomas than in vestibular
schwannomas [22]. Still, the natural history of
tumor remnants is not known: some authors
found a high incidence of symptomatic tumor
growth, while other report on long periods of
remission [50, 63].
Pigmented or melanotic trigeminal schwanno-
mas are exceedingly rare (see Case Illustration
9.4). Melanotic schwannomas are very rare
peripheral nerve tumors with a predilection for
spinal nerve involvement. Trigeminal nerve ori-
gin of the tumor has been described in a few
cases. Characteristic is their heavy pigmentation,
as evident in the presented case, which is due to
deposit of melanin pigment in the cytoplasm.
Melanotic schwannomas might be a component
of the autosomal dominant Carney syndrome that
includes spotted mucocutaneous pigmentation,
myxomas (cardial, cutaneous, and mammary),
and endocrine hyperactivity [11]. A prevalence
of growth hormone producing pituitary ade-
nomas, causing acromegaly, has been described
in such patients. Importantly, a higher rate of
malignant features has been reported in melan-
otic schwannomas.
Outcome
Neurological symptoms, caused by trigeminal
schwannomas, are related generally to their com-
pression rather than infiltration. Surgery, there-
fore, may lead to relieve of these symptoms, for
9 Nonvestibular Schwannomas
example, diplopia, facial pain, hypacusis, or
ataxia. In respect to trigeminal nerve sensory
function, a transient postoperative deterioration
may be observed that tends to improve in approx-
imately half of the cases.
Surgery-related cranial nerve morbidity—
most frequently of the ocular motor, cochlear
nerves, and facial nerves—is usually caused by
operative manipulations and/or tumor dissection.
It is related mostly to the operative approach and
to the surgeons’ experience.
Radiotherapy
Stereotactic radiosurgery has been promoted as
an alternative primary or adjuvant management
option that leads to a high rate of tumor growth
control. It is reported to be an effective treatment
modality for small- and medium-size trigeminal
schwannomas [29, 60]. In one large prospective
study, presented by Pan et al. [60], good response
to the treatment—tumor shrinkage more than
one-third in its greater diameter—was noted in
88.5 % of tumors smaller than 30 mm. The review
of the relevant literature, however, shows that the
rate of tumor progression, despite the radiosur-
gery, is >9 %. The probability for improvement
of existing neurological symptoms is less in case
of radiosurgery when compared to surgery.
Furthermore, the rate of cranial nerve morbidity
is higher when radiosurgery is applied.
Kano et al. [32] evaluated 33 consecutive
patients with trigeminal schwannomas treated via
gamma knife surgery and found that at an average
of 6 years, the rate of progression-free survival at
1, 5, and 10 years after SRS was 97.0, 82.0, and
82.0 %, respectively. Three patients (9.1 %) had
symptomatic disease progression. Phi et al. [62]
achieved tumor growth control in 95 % of their
patients. Twenty-seven percent experienced new
or worsening cranial neuropathies, and in 46 %,
tumor expansion after radiosurgery was observed.
The authors conclude that cranial neuropathies
are bothersome complications of radiosurgery.
Worsening of the disease was observed in 12 % of
the patients treated by Sheehan et al. [70].
Due to these considerations, we regard complete
tumor resection with preservation of brain and cra-
nial nerve function the ideal treatment. Radiosurgery
Oculomotor Nerve Schwannoma
may be an alternative in case of small tumors in
elderly, somatically unstable patients.
Oculomotor Nerve Schwannoma
In 1927, Kovacs described an isolated oculomotor
nerve schwannoma observed at autopsy [37].
Since then, 35 cases have been reported in the lit-
erature [27, 78]. Schwannomas can arise from any
segment of the oculomotor nerve. Celli et al. [12]
categorized them accordingly into cisternal, cav-
ernous, and cisternocavernous types. These tumors
are located most frequently within the interpedun-
cular cistern and cavernous sinus. Large tumors
may have parasellar and suprasellar extension.
Cisternal tumors expand in the prepontine cistern
and may compress the brain stem [44, 66].
Patients with oculomotor schwannomas have
almost always a certain degree of diplopia due to
oculomotor palsy. Other symptoms are related to
compression of neighboring cranial nerves or—in
case of large lesions—to intracranial hyperten-
sion [56, 78].
The still-limited experience with surgery of
these tumors has shown that cisternal lesions can
be removed while preserving the anatomical con-
tinuity of the nerve with good chances for func-
tional recovery. Complete resection of the
cavernous schwannomas, however, might be
impossible without nerve sacrifice. Therefore,
the management plan should be discussed
together with the patient, considering his neuro-
logical and general condition, as well as tumor
size, location, and growth tendency.
577
Trochlear Schwannomas
King JS published the first case of surgical treat-
ment of trigeminal schwannoma in 1976 [35].
Less than 30 cases of pathologically verified tro-
chlear nerve schwannomas have been reported up
to now in the literature [21, 36, 57]. They origi-
nate usually from the intradural cisternal part of
the nerve and more rarely from its extradural cav-
ernous part and are subdivided into cisternal, cav-
ernous, and cisternocavernous groups [18]. The
tumor expands in the ambient and interpeduncu-
lar cisterns or grows toward the CP angle.
Anterior extension into the parasellar space has
been also observed [81].
Patients with trochlear schwannomas usually
present with trochlear nerve palsy, trigeminal
nerve signs/symptoms, headache, long-tract
signs, ataxia, or symptoms from other cranial
nerves [1, 18, 21, 35]. Interestingly, only 50 %
of the patients have trochlear nerve palsy at pre-
sentation, which can be explained by the devel-
opment of compensatory mechanisms for the
lost function during the slow tumor growth
[18, 49]. The nerve of origin is determined reli-
ably only intraoperatively, but the possibility of
trochlear schwannomas should be at least
considered.
Trochlear schwannomas can be accessed via
different approaches, according to their exten-
sion, clinical symptoms, and surgeons’ prefer-
ence [21]. (See Case Illustration 9.5). The
trochlear nerve palsy persists after surgery in
more than 40 % after surgery, but a gradual reso-
lution/compensation usually occurs.
578
Case Illustration 9.5
Trochlear Nerve Schwannoma
This 52-year-old female patient presented
with trigeminal hypesthesia, double vision
due to trochlear nerve palsy, facial nerve palsy
(House–Brackmann grade III), decreased
hearing on the left side (40 dB at 1.5 Hz), and
gait instability for 10 months. She had long-
tract signs and a grade 4/5 central right-sided
hemiparesis. The patient underwent an open
biopsy at another institution with the presum-
able diagnosis of pilocytic astrocytoma. The
MRI showed a lesion with a cystic and com-
ponents (a). The 2.5-cm large solid tumor part
was isointense on T1-weighted images and
hypointense on T2-weighted images with
intense contrast enhancement and was well
circumscribed. The cystic part was located
dorsally.
The tumor was approached via the RS ave-
nue. Intense adhesions following the previous
surgery were encountered that precluded
opening of the cerebellomedullary cistern.
Furthermore, the attempt to retract the cere-
bellar hemisphere led to significant
(a) Preoperative imaging. (b) Intraoperative view of the tumor (T tentorium; C cerebellum; P pyramid).
(c) Postoperative follow-up MRI (From Gerganov et al. [21]. Copyright © 2007, with permission from Elsevier)
9 Nonvestibular Schwannomas
bradycardia. Therefore, the cyst was drained
initially, which lead to relaxation of the cere-
bellum (b). The solid tumor portion with the
typical appearance of schwannoma was
located in the ambient, crural, and upper CP
cisterns. The tumor originated from the cister-
nal part of the trochlear nerve just before its
entrance in the free edge of the tentorium.
Initial debulking using an ultrasound aspirator
and platelet knife was performed, and—fol-
lowing relieve of the tension—the capsule was
dissected in the arachnoid plane from the sur-
rounding structures. The trochlear nerve was
completely destroyed by the tumor, and its
distal segment could not be identified. Total
tumor removal was achieved, and the histo-
logical examination revealed a WHO grade I
schwannoma.
After surgery, the patient did not have new
neurological deficits and had an uneventful
recovery (c). At follow-up, the trigeminal
nerve dysfunction, the facial nerve palsy,
ataxia, and long-tract signs recovered com-
pletely. However, the right-sided trochlear
palsy and the hypacusis persisted.
Trochlear Schwannomas 579

c
580
Abducens Nerve Schwannoma
Since the first description of an abducens nerve
schwannoma by Bing-huan in 1981, 18 such
cases have been presented in the literature.
According to the classification of Tung et al. [79],
based on the tumor origin, the abducens schwan-
nomas are two types: arising within the cavern-
ous sinus or arising along the course of the nerve
in the prepontine area. Most tumors belong to the
first group and involve the cavernous sinus.
Abducens schwannomas, in contrast to all other
cranial nerve schwannomas, originate away from
the glial–Schwann sheath junction [80]. Patients
present typically with double vision due to
abducens nerve palsy. In case of large tumors,
symptoms from compression of adjacent neural
structures and/or intracranial hypertension
develop [58]. In some rare cases [54], no obvious
9 Nonvestibular Schwannomas
functional disturbance of the abducens nerve was
seen, which has been explained by the develop-
ment of compensatory mechanisms.
The neuroradiological features of these
tumors are similar to those of other schwanno-
mas. Therefore, preoperative differentiation
between oculomotor, trochlear, trigeminal, and
abducens nerve schwannomas is generally not
possible. A variety of operative approaches have
been applied. In some of the reported cases, the
tumor could be removed from the nerve fasci-
cles of origin, preserving the main trunk. Such
favorable outcome could be explained by the
presence of anatomical variations, such as split-
ting of the nerve or the existence of two separate
nerve trunks [30, 55]. In 3 reported cases, the
abducens nerve could be preserved, and at fol-
low-up, complete recovery of its function was
achieved [53, 54, 80].
Abducens Nerve Schwannoma
Case Illustration 9.6
Abducens Nerve Schwannoma
This 42-year-old man presented with a history
of headache and episodes of dizziness and
vertigo since several months. His neurological
examination revealed only a slight left-sided
hypacusis. The MRI (a–c) showed a solid
lesion with intense contrast enhancement that
caused significant compression of the brain
stem at the level of the lower pons and upper
medulla oblongata.
The patient was operated via the retrosig-
moid approach. The tumor was found to
a b
(a–c) Preoperative MRI study, demonstrating the con-
trast-enhancing tumor in the prepontine space. (d, e)
Intraoperative images. (d) Initial exposure of the tumor
and its relations to the facial and vestibulocochlear
nerve complex (VII–VIII) and the lower cranial nerves
581
compress the lower cranial nerves inferolater-
ally and the seventh–eighth cranial nerve com-
plex laterally (d, e). It was debulked, and
despite the strong adhesion of its capsule to
the abducens nerve, it could be completely
removed, preserving the anatomical integrity
of the nerve (f, g). The only neurological
deficit that appeared after surgery was a par-
tial abducens nerve paresis on the left side. At
1-year follow-up, however, the function of the
nerve recovered completely.
(IX–XI) and (e) complete tumor removal. All anatomi-
cal structures have been preserved. (f, g) Postoperative
MRI, proving the completeness of tumor removal
(Reprinted from Nakamura et al. [54]. Copyright ©
2002, with permission, Elsevier)
582 9 Nonvestibular Schwannomas

d e

f g
Facial Schwannomas 583

Facial Schwannomas ganglion), and meatal segment lesions. A more

In 1930, Schmidt described for the first time a
schwannoma originating from the facial nerve
[24]. Facial schwannomas are slow-growing
tumors that may originate from any part of the
nerve—from the CP angle to its extracranial
peripheral portion (see figures and the case
illustrations)—but have a predilection for the
geniculate segment [41, 75, 83]. Lipkin et al. [45]
subdivided facial schwannomas into tympanic,
vertical, labyrinthine (including the geniculate
practically oriented classification is that of Sarma
et al. [66], which is based on their surgical ana-
tomic features: (1) cerebellopontine angle, (2)
geniculate, and (3) tympanomastoidal lesions
(Figs. 9.10, 9.11, and 9.12). Frequently, however,
the tumor involves more than one segment of the
nerve. Facial nerve schwannomas account for
less than 2 % of all schwannomas [75]. Twenty
percent of them are located within the CP angle
and comprise approximately 3 % of all CP angle
tumors [34].

a b

Fig. 9.10 Facial schwannoma, located predominantly in the middle cranial fossa with a smaller component in the IAC,
operated by the senior author in the early 1990s
584 9 Nonvestibular Schwannomas

a b

Fig. 9.11 Facial schwannoma in the mastoid segment of
the nerve. (a) CT scan demonstrating the smooth bone
widening (arrow). (b, c) MRI images demonstrating the
contrast-enhancing lesion in the area (arrow). (d) The
approach was performed under neuronavigational guid-
ance. (e, f) Intraoperative images demonstrating the
tumor-involved segment of the facial nerve and its com-
plete removal. (g, h) Sural nerve graft was adjusted at the
healthy proximal and distal edges of the nerve (g, arrows)
and fixed with fibrin glue (h, asterisks). (i, j) Photos of the
patient 1 year later
Facial Schwannomas 585

e f

g h

Fig. 9.11 (continued)

586 9 Nonvestibular Schwannomas

i j

Fig. 9.11 (continued)

Facial Schwannomas 587

a b

c d

Fig. 9.12 Facial schwannoma arising in the area of the the CT images. The tumor was removed completely via
ganglion geniculi and extending to the middle cranial the middle fossa approach. (a, b) Bone-window CT scans.
fossa and into the IAC. Note the destruction of the middle (c, b) T1-weighted contrast-enhanced MR images. (e, h)
cranial fossa floor and the communication to the IAC on T2-weighted images
588 9 Nonvestibular Schwannomas

e f

g h

Fig. 9.12 (continued)

Facial Schwannomas
Case Illustration 9.7
Facial Schwannoma in the IAC
This 56-year-old male patient presented with
a history of hearing loss on the right side and
vertigo. His neurological examination
revealed slight HB grade 2 facial nerve palsy.
He had functional hearing with a decrease of
25 dB in the main speech area. MRI showed a
contrast-enhancing lesion in the right IAC
(a), which was slightly dilated. Presentation
with facial nerve palsy is rather untypical for
VS, especially of this size. The possibility
Facial schwannoma located in the right IAC. (Aa, Ab)
T1-weighted contrast-enhanced MRI of the tumor,
appearing as a vestibular schwannoma. No radiologi-
cal clues on its eventual pathological type. (b) Initial
exposure of the CP angle (arrowhead (seventh–eighth
nerves); r (retractor); arrows (incision of the dura
around the posterior lip of the IAC)). (c) The dura
around the posterior lip has been stripped off and
incised. (d) The IAC has been widely opened. The ves-
tibular nerves (8) and the tumor (tu) are seen. A small
part of the dura, covering the medial part of IAC, has
not been still incised (*). Its excision revealed the
whole intracanalicular and part of the CP angle tumor
part (e). (f–h) Dissection of the tumor from the ves-
tibular and cochlear nerves and its gradual removal.
589
that it could be a case of facial nerve schwan-
noma was discussed with the patient before
surgery. The tumor was approached via the
retrosigmoid route (b–o). It originated from
the CP angle segment of the facial nerve. In
sake of radicality, the nerve was transected in
its healthy parts. Nerve reconstruction was
then performed with interpositional sural
nerve graft. The vestibular and the cochlear
nerves were preserved, and after surgery,
hearing level was unchanged. The postopera-
tive period was uneventful.
The origin of the tumor from the facial nerve (7) is
well seen on image. (h, i) Complete tumor removal
with preservation of the vestibular and the cochlear
nerves. The distal facial nerve stump is seen in the lat-
eral part of the IAC. (j) In order to identify a healthy
proximal stump, the facial nerve exit zone at the brain
stem had to be dissected (LCN—lower cranial nerves).
The intact vestibulocochlear nerve complex was used
as a guide. (k) Proximal facial nerve stump. (l) Sural
nerve graft of appropriate length has been approxi-
mated between the proximal and distal facial stumps
(small arrows) and fixed with fibrin glue (m, n). (o)
The IAC is filled with small fat pieces, sealed with
fibrin glue. During this procedure, great care had to be
exerted not to dislocate the graft
590 9 Nonvestibular Schwannomas

b c

d e

Tu Tu

f g

Tu

8
Facial Schwannomas 591

h i

j k

LCN

l
592 9 Nonvestibular Schwannomas

m n

o
Facial Schwannomas 593

The preoperative neuroradiological work-up is
similar to that for other CP angle tumors: bone-
window thin-slice CT, native and contrast-
enhanced MRI, as well as flexion/extension X-ray
of the spine (semi-sitting position!). Several radio-
logical patterns of facial schwannomas have been
reported, but none of them are pathognomonic
(Fig. 9.13). The only radiological clue to differen-
tiate a facial from a vestibular schwannoma may
be tumor location (Figs. 9.10, 9.11, and 9.12). An
example for this is the widening of the fallopian
canal seen on bone-window CT.
The clinical presentation of patients with such
tumors is variable and is related to tumor location
and size. Patients with facial schwannomas, in
contrast to those with VSs, typically have some
degree of facial palsy at presentation. Therefore, in
every patient with a CP angle tumor and facial

a b

c d

Fig. 9.13 Patient with a facial schwannoma on the left
side, located in the CP angle and IAC. The findings are not
specific, and a differential diagnosis to vestibular schwan-
noma, based on imaging, cannot be made. (a) Bone-window
CT scan; note the widened IAC and the high location of the
jugular bulb. (b) T1-weighted native MRI. (c, d)
T1-weighted sequences after gadolinium application. (e)
T2-weighted sequence. (f, g) T2-CISS sequences. At sur-
gery, the tumor turned out to originate from the facial nerve.
It was removed completely, and the nerve was reconstructed
with an interposition graft. The postoperative CT—the day
after surgery—is shown on (h) and (i)
594 9 Nonvestibular Schwannomas

e f

g h

Fig. 9.13 (continued)

palsy, an origin of the schwannoma from the facial facial nerve sacrifice and reconstruction with a
nerve should be suspected, especially if the palsy sural nerve graft should be considered. Normal
is progressive in nature. The patient should be facial function, however, does not exclude a diag-
counseled accordingly, and the option of eventual nosis of facial schwannoma—27–54 % of the
Facial Schwannomas
patients do not have facial palsy at presentation
[64, 67]. Hearing loss, vertigo, or tinnitus are the
other common initial symptoms [24, 40]. Rarely
patients present with hemifacial spasm [66]. The
interval between onset of symptoms and diagnosis
is usually long. Sherman et al. [71] pointed that the
reason for delayed diagnosis might be initial con-
fusion with Bell’s palsy. These authors recom-
mend MRI examination in all patients with
progressive weakness of the facial nerve, as well
as in patients with suspected Bell’s palsy in case of
progressive worsening of the facial function or in
case no improvement after 6 months is observed.
In case of pronounced facial palsy (HB grade
III or worse) or enlarging tumors, tumor removal
with nerve reconstruction is the only reasonable
option [48]. The issue if and when to operate a
patient with small tumor and normal facial nerve
function or with mild symptoms is discussed con-
troversially in the literature. Some authors recom-
mend an initial wait-and-see strategy until the loss
of facial function develops [19, 46]. The rationale
for this strategy is the fact that these tumors may
grow quite slowly. If the facial palsy is less
a b
Fig. 9.14 Giant facial nerve schwannoma in a 53-year-
old female patient, presenting with facial hypesthesia,
anacusis, HB grade II facial nerve weakness, swallowing
difficulty, severe gait ataxia, and headache. Preoperative
images (a–f): note the extension of the tumor in the
595
pronounced, some surgeons recommend conser-
vative surgery with the hope of preserving the
remaining facial function as long as possible. The
proposed surgical options are tumor stripping with
at least partial anatomical preservation of the facial
nerve or—if this is impossible—only decompres-
sion/tumor debulking [5]. Other surgeons recom-
mend immediate surgery and tumor removal after
the diagnosis because of the increased risk to hear-
ing loss related to further tumor growth [66].
Our opinion is that the best treatment of facial
nerve schwannomas is complete tumor removal
with resection of the involved facial nerve seg-
ment and restoration of the facial function (see
Figs. 9.14 and 9.15 and Case Illustration 9.7).
Earlier reconstruction of the facial nerve might
lead to better recovery (see Case Illustration 9.8).
Preservation of hearing is mandatory. In some
patients without facial nerve deficits before sur-
gery, it may be possible to resect only the
involved portion of the nerve and to preserve
some of the fibers. Thereby, the facial nerve
function can be preserved. In case a facial palsy
exists preoperatively, however, more extensive
petrous bone and its massive destruction. The patient has
been operated via the RS approach, and the tumor could
be removed completely; the origin of the schwannoma
was the facial nerve, which had to be therefore sacrificed
and then reconstructed
596 9 Nonvestibular Schwannomas

c d

e f

Fig. 9.14 (continued)

involvement of the nerve fibers is likely. Radical
surgery in such case necessitates nerve transec-
tion and reconstruction. The healthy proximal
and distal segments of the nerve have to be
exposed, even by performing partial mastoidec-
tomy. Tensionless end-to-end anastomosis usu-
ally requires the use of a nerve graft from the
sural nerve (see chapter on Facial Nerve
Reconstruction).
Rare finding is the more diffuse infiltration of
the nerve, usually appearing as pearl-like clusters
along its course. In such case, healthy nerve
stumps do not exist, and primary nerve recon-
struction is impossible. In these patients, decom-
pressive surgery to retain residual function is an
option. Once the functional loss becomes more
severe, complete tumor removal and hypoglos-
sal–facial anastomosis are performed.
Facial Schwannomas 597

a b

c d

Fig. 9.15 Postoperative images of the patient from tracheostoma and PEG were placed. Within 3 weeks, she
Fig. 9.14. (a) CT scan 1 day after surgery. (b) CT scan 4 could resume oral feeding and at discharge could walk
days later. (c, d) MRI 4 months after surgery. Immediately without help. Four months later, a hypoglossal–facial
after tumor removal, she had worsening of swallowing; a nerve anastomosis was performed
598
Case Illustration 9.8
Giant Facial Nerve Schwannoma
Nine-year-old girl complaining of progres-
sively deteriorating hearing since 2 years.
Neurological examination revealed anacusis
and gaze nystagmus on the right side and severe
gait ataxia. The initial CT and MR findings
are presented on (a–f): giant homogeneously
enhancing CP angle tumor with massive
destruction of the petrous bone and extension
in an anterosuperior direction up to the dura of
the middle fossa, in anteromedial direction to
the petrous segment of the carotid artery, and
caudally to the jugular foramen and significant
brain stem compression but no hydrocephalus.
No clinical or radiological signs of NF-2 have
been detected, and the presumptive diagnosis
was VS, despite the very young patients’ age.
Surgical removal of the tumor was planned via
the RS approach in the semi-sitting position.
Transesophageal echocardiography revealed a
hemodynamically insignificant opened fora-
men ovale, which did not hinder the planned
surgery.
The tumor was exposed via the typical RS
approach (g–q). The petrous tumor part was
covered by a very thin layer of bone and dura.
They were removed widely exposing this
tumor portion. Caudally, the tumor capsule
was attached to the jugular bulb and had to be
dissected from it. It was debulked, and a small
segment of the facial nerve could be identified
in the fundus area of the IAC. Medially, it dis-
appeared in the tumor mass. Therefore, we
9 Nonvestibular Schwannomas
continued with internal debulking of the CP
angle tumor part. The tumor had a hard consis-
tency, and only in the depth, close to the brain
stem, was somewhat softer. The tumor capsule
was gradually dissected from the compressed
lower cranial nerves, and the facial nerve was
visualized at the brain stem. It could not be,
however, recognized anatomically in its more
lateral segment: it practically disappeared in
the tumor. Electrical stimulation of the whole
medial tumor part provoked vivid facial EMG
response, although nerve fibers could not be
identified, which confirmed the diagnosis of
facial nerve schwannoma. Taking into account
the patients’ age, it was decided to proceed
with tumor removal and to sacrifice the nerve.
The tumor was removed completely; then
healthy medial and lateral stumps of the facial
nerve were prepared, and a 2-cm long sural
nerve graft was used to bridge them.
The postoperative period was uneventful,
and the patient was discharged 13 days after
surgery. Her facial nerve showed significant
recovery at the follow-up examination 12
months later (t–v).
Summary: The clinical presentation of this
young patient and the neuroradiological exam-
inations did not give any clues to the patho-
logical tumor type. Complete tumor removal,
which was the goal of surgery, could be
achieved only if the facial nerve was sacrificed.
In young patients, the degree of functional
facial recovery after direct end-to-end nerve
reconstruction in the CP angle is very high.
Facial Schwannomas 599

a b

c d

(a–f) Preoperative images, demonstrating a giant
intensely enhancing tumor in the right CP angle with
large intrapetrous part. The extensive destruction of
the petrous bone is well visible on the bone-window
high-resolution CT scan (a, b). (c–e) T1-weighted MR
sequence after contrast administration and (f) CISS
sequence. (g–q) Intraoperative images, illustrating the
consecutive steps of tumor removal. (g) RS approach
with exposure of the edges of the sigmoid (SS) and
transverse sinuses (TS). (h) The tumor portion in the
pyramid was covered by a very thin bone lamina,
which was drilled off. The dotted line shows the
amount of bone removal. This part was removed
initially. (i) Internal decompression of the CP angle
tumor portion followed. (j) The tumor has been
removed completely and the brain stem—decom-
pressed. (k, l) The facial nerve was infiltrated by the
tumor. The proximal stump is seen with some attached
tumor (arrows) on the images; preparation of the prox-
imal (m) and of the distal (n) nerve stumps (PS proxi-
mal stump, DS distal stump); a graft of appropriate
length is prepared (o) and fixed to the stumps with
fibrin glue (p). (q) Fat is used to prevent CSF leak.
Photo of the patient immediately after surgery (r, s)
and 12 months later (t–v)
600 9 Nonvestibular Schwannomas

e f

g h
TS P

SS

i j
Facial Schwannomas 601

k l

m n
PS

DS
602 9 Nonvestibular Schwannomas

o p

q
Facial Schwannomas 603

r s

t u v
604
Cochlear Schwannomas
Schwannomas arising from the cochlear nerve
are extremely rare. They are discovered usually
at surgery, but high-resolution MRI, in particular
the 3D-CISS mode, showing enhancement of the
cochlear division of the eighth cranial nerve or
cochlear tumors in the inferior compartment of
the anterior wall of the IAC with or without
extension into the cochlea, may suggest such ori-
gin [39, 42].
Patients with cochlear schwannomas usually
present with profound hearing loss and tinnitus.
Complaints of vertigo are less typical. Some
authors even point that in case of rapidly progres-
sive hearing loss and absence of vertigo, the diag-
nosis cochlear nerve tumor has to be considered
[7]. Factors guiding the management of such
patients are their general condition, hearing level
on the side of the tumor, and on the healthy side,
as well as tumor size. While in case of profound
hearing loss surgery is the best option, in case of
less severe loss, especially if the hearing on the
other side is also compromised, a period of obser-
vation might be warranted.
Lower Cranial Nerve Schwannomas
This topic is discussed in the chapter tumors of
the jugular foramen.
References
1. Abe T, Iwata T, Shimazu M, Matsumoto K (1994)
Trochlear nerve neurinoma associated with a giant
thrombosed dissecting aneurysm of the contralateral
vertebral artery. Surg Neurol 42(5):438–441
2. Akhaddar A, El-Mostarchid B, Zrara I, Boucetta M
(2002) Intracranial trigeminal neuroma involving the
infratemporal fossa: case report and review of the litera-
ture. Neurosurgery 50(3):633–637; discussion 637–638
3. Al-Mefty O, Ayoubi S, Gaber E (2002) Trigeminal
schwannomas: removal of dumbbell-shaped tumors
through the expanded Meckel cave and outcomes of
cranial nerve function. J Neurosurg 96(3):453–463.
doi:10.3171/jns.2002.96.3.0453
4. Al-Mefty O, Fox JL, Smith RR (1988) Petrosal
approach for petroclival meningiomas. Neurosurgery
22(3):510–517
9 Nonvestibular Schwannomas
5. Angeli SI, Brackmann DE (1997) Is surgical excision
of facial nerve schwannomas always indicated?
Otolaryngol Head Neck Surg 117(6):S144–S147.
doi:S0194-5998(97)70084-6 [pii]
6. Arena S, Hilal EY (1976) Neurilemomas of the
infratemporal space: report of a case and review of the
literature. Arch Otolaryngol 102(3):180–184
7. Barbieri M, Bruzzo M, Mora R, Meller R, Chays A,
Magnan J (2001) Cochlear schwannomas. Skull Base
11(4):241–244
8. Berger MS, Edwards MS, Bingham WG (1982)
Hypoglossal neurilemmoma: case report and review
of the literature. Neurosurgery 10(5):617–620
9. Bordi L, Compton J, Symon L (1989) Trigeminal neu-
roma. A report of eleven cases. Surg Neurol
31(4):272–276
10. Cantini R, Giorgetti W, Valleriani AM, Burchianti M
(1987) Trigeminal schwannomas in adolescence.
Pediatr Neurosci 13(4):198–201
11. Carrasco CA, Rojas-Salazar D, Chiorino R, Venega
JC, Wohllk N (2006) Melanotic nonpsammomatous
trigeminal schwannoma as the first manifestation of
Carney complex: case report. Neurosurgery
59(6):E1334–E1335. doi:10.1227/01.NEU.0000
245608.07570.D2; discussion E1335
12. Celli P, Ferrante L, Acqui M, Mastronardi L,
Fortuna A, Palma L (1992) Neurinoma of the third,
fourth, and sixth cranial nerves: a survey and report
of a new fourth nerve case. Surg Neurol 38(3):
216–224
13. Day JD, Fukushima T (1998) The surgical manage-
ment of trigeminal neuromas. Neurosurgery 42(2):
233–240; discussion 240–231
14. Day JD, Fukushima T, Giannotta SL (1994)
Microanatomical study of the extradural middle
fossa approach to the petroclival and posterior cavern-
ous sinus region: description of the rhomboid con-
struct. Neurosurgery 34(6):1009–1016; discussion
1016
15. de Benedittis G, Bernasconi V, Ettorre G (1977)
Tumours of the fifth cranial nerve. Acta Neurochir
(Wien) 38(1–2):37–64
16. Dolenc VV (1985) A combined epi- and subdural
direct approach to carotid-ophthalmic artery aneu-
rysms. J Neurosurg 62(5):667–672. doi:10.3171/
jns.1985.62.5.0667
17. Dolenc VV (1994) Frontotemporal epidural approach
to trigeminal neurinomas. Acta Neurochir (Wien)
130(1–4):55–65
18. Du R, Dhoot J, McDermott MW, Gupta N (2003)
Cystic schwannoma of the anterior tentorial hiatus.
Case report and review of the literature. Pediatr
Neurosurg 38(4):167–173. doi:10.1159/000069094
19. Fenton JE, Chin RY, Fagan PA, Sterkers O, Sterkers
JM (2004) Facial nerve outcome in non-vestibular
schwannoma tumour surgery. Acta Otorhinolaryngol
Belg 58(2):103–107
20. Fukaya R, Yoshida K, Ohira T, Kawase T (2010)
Trigeminal schwannomas: experience with 57 cases
and a review of the literature. Neurosurg Rev
34(2):159–171. doi:10.1007/s10143-010-0289-y
References
21. Gerganov V, Amir S, Koerbel A, Brandes A, Stan
A, Madjid S (2007) Cystic trochlear nerve schwan-
noma. Case report. Surg Neurol 68(2):221–225.
doi:S0090-3019(06)00963-3 [pii]
22. Goel A (1995) Infratemporal fossa interdural approach
for trigeminal neurinomas. Acta Neurochir (Wien)
136(1–2):99–102
23. Goel A, Muzumdar D, Raman C (2003) Trigeminal
neuroma: analysis of surgical experience with 73
cases. Neurosurgery 52(4):783–790; discussion 790
24. Hajjaj M, Linthicum FH Jr (1996) Facial nerve
schwannoma: nerve fibre dissemination. J Laryngol
Otol 110(7):632–633
25. Hakuba A, Tanaka K, Suzuki T, Nishimura S (1989)
A combined orbitozygomatic infratemporal epidural
and subdural approach for lesions involving the entire
cavernous sinus. J Neurosurg 71(5 Pt 1):699–704.
doi:10.3171/jns.1989.71.5.0699
26. Hamm KD, Gross MW, Fahrig A, Surber G, Henzel
M, Kleinert G, Grabenbauer GG, Engenhart-Cabillic
R (2008) Stereotactic radiotherapy for the treatment
of nonacoustic schwannomas. Neurosurgery 62(5
Suppl):A29–A36. doi:10.1227/01.neu.0000325934.
16229.03; discussion A36
27. Hironaka Y, Nakase H, Motoyama Y, Mishima H,
Park YS, Hirabayashi H, Sakaki T (2010) Orbital
schwannoma extending to the lateral wall of the cav-
ernous sinus through the superior orbital fissure – case
report. Neurol Med Chir (Tokyo) 50(2):154–157.
doi:JST.JSTAGE/nmc/50.154 [pii]
28. House WF, Hitselberger WE, Horn KL (1986) The
middle fossa transpetrous approach to the anterior-
superior cerebellopontine angle. Am J Otol 7(1):1–4
29. Huang CF, Kondziolka D, Flickinger JC, Lunsford LD
(1999) Stereotactic radiosurgery for trigeminal schwan-
nomas. Neurosurgery 45(1):11–16; discussion 16
30. Iaconetta G, Fusco M, Cavallo LM, Cappabianca
P, Samii M, Tschabitscher M (2007) The abducens
nerve: microanatomic and endoscopic study.
Neurosurgery 61(3 Suppl):7–14. doi:10.1227/01.neu.
0000289706.42061.19; discussion 14
31. Jefferson G (1953) The trigeminal neurinomas with
some remarks on malignant invasion of the gasserian
ganglion. Clin Neurosurg 1:11–54
32. Kano H, Niranjan A, Kondziolka D, Flickinger JC,
Dade Lunsford L (2009) Stereotactic radiosurgery for
trigeminal schwannoma: tumor control and functional
preservation Clinical article. J Neurosurg 110(3):
553–558
33. Kawase T, Shiobara R, Toya S (1991) Anterior transpet-
rosal-transtentorial approach for sphenopetroclival
meningiomas: surgical method and results in 10 patients.
Neurosurgery 28(6):869–875; discussion 875–866
34. Kertesz TR, Shelton C, Wiggins RH, Salzman KL,
Glastonbury CM, Harnsberger R (2001) Intratemporal
facial nerve neuroma: anatomical location and radio-
logical features. Laryngoscope 111(7):1250–1256.
doi:10.1097/00005537-200107000-00020
35. King JS (1976) Trochlear nerve sheath tumor; case
report. J Neurosurg 44(2):245–247. doi:10.3171/jns.
1976.44.2.0245
605
36. Kohama M, Murakami K, Endo T, Watanabe M,
Tominaga T (2009) Surgical and histological observa-
tions of trochlear neurinoma: case report. Neurol Med
Chir (Tokyo) 49(5):217–220. doi:JST.JSTAGE/
nmc/49.217 [pii]
37. Kovacs W (1927) Ueber ein solitares Neurinoma des
Nervus oculomotorius. Zentralbl Allgemeine Pathol
Patholog Anat 40:518–522
38. Krayenbuhl H (1959) Neurinoma of trigeminal nerve.
Bull Schweiz Akad Med Wiss 15(1):89–100
39. Krueger WW, Kemper JV (2000) Cochlear schwan-
nomas. Skull Base Surg 10(2):87–88
40. Lalwani AK (1992) Meningiomas, epidermoids,
and other nonacoustic tumors of the cerebellopontine
angle. Otolaryngol Clin North Am 25(3):707–728
41. Latack JT, Gabrielsen TO, Knake JE, Kemink JL,
Graham MD, Gebarski SS, Yang PJ (1983) Facial
nerve neuromas: radiologic evaluation. Radiology
149(3):731–739
42. Leonetti JP (1998) Cochlear neuromas. Am J Otol
19(4):499–502
43. Lesoin F, Rousseaux M, Villette L, Autricque A,
Dhellemmes P, Pellerin P, Vaneecloo JM, Leys D,
Jomin M (1986) Neurinomas of the trigeminal nerve.
Acta Neurochir (Wien) 82(3–4):118–122
44. Leunda G, Vaquero J, Cabezudo J, Garcia-Uria J,
Bravo G (1982) Schwannoma of the oculomotor
nerves. Report of four cases. J Neurosurg 57(4):563–
565. doi:10.3171/jns.1982.57.4.0563
45. Lipkin AF, Coker NJ, Jenkins HA, Alford BR (1987)
Intracranial and intratemporal facial neuroma.
Otolaryngol Head Neck Surg 96(1):71–79
46. Liu R, Fagan P (2001) Facial nerve schwannoma: sur-
gical excision versus conservative management. Ann
Otol Rhinol Laryngol 110(11):1025–1029
47. Lunardi P, Missori P, Gagliardi FM, Fraioli B
(1989) Trigeminal schwannoma with infratemporal
extension. Case report. J Neurosurg Sci 33(3):
293–295
48. Marzo SJ, Zender CA, Leonetti JP (2009) Facial nerve
schwannoma. Curr Opin Otolaryngol Head Neck Surg
17(5):346–350. doi:10.1097/MOO.0b013e32832ea999
49. Matsui T, Morikawa E, Morimoto T, Asano T (2002)
Presigmoid transpetrosal approach for the treatment
of a large trochlear nerve schwannoma–case report.
Neurol Med Chir (Tokyo) 42(1):31–35. doi:JST.
JSTAGE/nmc/42.31 [pii]
50. McCormick PC, Bello JA, Post KD (1988) Trigeminal
schwannoma. Surgical series of 14 cases with review
of the literature. J Neurosurg 69(6):850–860.
doi:10.3171/jns.1988.69.6.0850
51. McElveen JT Jr, Dorfman BE, Fukushima T (2001)
Petroclival tumors: a synthesis. Otolaryngol Clin
North Am 34(6):1219–1230, x
52. Mello LR, Tanzer A (1972) Some aspects of trigemi-
nal neurinomas. Neuroradiology 4(4):215–221
53. Nakagawa T, Uchida K, Ozveren MF, Kawase T
(2004) Abducens schwannoma inside the cavernous
sinus proper: case report. Surg Neurol 61(6):559–
563. doi:10.1016/S0090-3019(03)00580-9; discus-
sion 563
606
54. Nakamura M, Carvalho GA, Samii M (2002)
Abducens nerve schwannoma: a case report and
review of the literature. Surg Neurol 57(3):183–188.
doi:S009030190100670X [pii]; discussion 188–189
55. Nathan H, Ouaknine G, Kosary IZ (1974) The abducens
nerve. Anatomical variations in its course. J Neurosurg
41(5):561–566. doi:10.3171/jns.1974.41.5.0561
56. Netuka D, Benes V (2003) Oculomotor nerve schwan-
noma. Br J Neurosurg 17(2):168–173
57. Ohba S, Miwa T, Kawase T (2006) Trochlear nerve
schwannoma with intratumoral hemorrhage: case
report. Neurosurgery 58(4):E791. doi:10.1227/01.
NEU.0000204307.99246.17; discussion E791
58. Okada Y, Shima T, Nishida M, Okita S (1997) Large
sixth nerve neuroma involving the prepontine region:
case report. Neurosurgery 40(3):608–610
59. Paillas JE, Grisoli F, Farnarier P (1974) Trigeminal
nerve neurinomas. Apropos of 8 cases. Neurochirurgie
20(1):41–54
60. Pan L, Wang EM, Zhang N, Zhou LF, Wang BJ, Dong
YF, Dai JZ, Cai PW (2005) Long-term results of
Leksell gamma knife surgery for trigeminal schwan-
nomas. J Neurosurg 102(Suppl):220–224
61. Paquis P, Castillo L, Lonjon M, Santini J, Grellier P
(1998) Extracranial trigeminal schwannomas with
middle temporal fossa development. Neurochirurgie
44(1):38–45. doi:MDOI-JNG-03-1998-44-1-0028-
3770-101019-ART70 [pii]
62. Phi JH, Kim DG, Chung HT, Lee J, Paek SH, Jung
HW (2009) Radiosurgical treatment of vestibular
schwannomas in patients with neurofibromatosis type
2: tumor control and hearing preservation. Cancer
115(2):390–398. doi:10.1002/cncr.24036
63. Pollock BE, Kondziolka D, Flickinger JC, Maitz A,
Lunsford LD (1993) Preservation of cranial nerve
function after radiosurgery for nonacoustic schwan-
nomas. Neurosurgery 33(4):597–601
64. Saleh E, Achilli V, Naguib M, Taibah AK, Russo A,
Sanna M, Mazzoni A (1995) Facial nerve neuromas:
diagnosis and management. Am J Otol 16(4):
521–526
65. Samii M, Migliori MM, Tatagiba M, Babu R (1995)
Surgical treatment of trigeminal schwannomas.
J Neurosurg 82(5):711–718. doi:10.3171/jns.1995.82.
5.0711
66. Sarma S, Sekhar LN, Schessel DA (2002)
Nonvestibular schwannomas of the brain: a 7-year
experience. Neurosurgery 50(3):437–448; discussion
438–439
67. Sataloff RT, Frattali MA, Myers DL (1995) Intracranial
facial neuromas: total tumor removal with facial nerve
preservation: a new surgical technique. Ear Nose
Throat J 74(4):244–246, 248–256
68. Sekhar LN, Pomeranz S, Sen CN (1991) Extradural
petrous bone and petroclival neoplasms. Acta
Neurochir Suppl (Wien) 53:183–192
69. Sekhar LN, Schessel DA, Bucur SD, Raso JL, Wright
DC (1999) Partial labyrinthectomy petrous apicec-
tomy approach to neoplastic and vascular lesions of
the petroclival area. Neurosurgery 44(3):537–550;
discussion 550–532
9 Nonvestibular Schwannomas
70. Sheehan J, Yen CP, Arkha Y, Schlesinger D, Steiner L
(2007) Gamma knife surgery for trigeminal schwan-
noma. J Neurosurg 106(5):839–845. doi:10.3171/
jns.2007.106.5.839
71. Sherman JD, Dagnew E, Pensak ML, van Loveren
HR, Tew JM Jr (2002) Facial nerve neuromas: report
of 10 cases and review of the literature. Neurosurgery
50(3):450–456
72. Spetzler RF, Daspit CP, Pappas CT (1992) The com-
bined supra- and infratentorial approach for lesions of
the petrous and clival regions: experience with 46
cases. J Neurosurg 76(4):588–599. doi:10.3171/
jns.1992.76.4.0588
73. Spinnato S, Talacchi A, Musumeci A, Turazzi S,
Bricolo A (1998) Dumbbell-shaped hypoglossal
neurinoma: surgical removal via a dorsolateral
transcondylar approach. A case report and review of
the literature. Acta Neurochir (Wien) 140(8):827–832
74. Springborg JB, Poulsgaard L, Thomsen J (2008)
Nonvestibular schwannoma tumors in the cerebello-
pontine angle: a structured approach and management
guidelines. Skull Base 18(4):217–227. doi:10.1055/s-
2007-1016959
75. Symon L, Cheesman AD, Kawauchi M, Bordi L
(1993) Neuromas of the facial nerve: a report of 12
cases. Br J Neurosurg 7(1):13–22
76. Taha JM, Tew JM Jr, van Loveren HR, Keller JT, el-
Kalliny M (1995) Comparison of conventional and
skull base surgical approaches for the excision of
trigeminal neurinomas. J Neurosurg 82(5):719–725.
doi:10.3171/jns.1995.82.5.0719
77. Tancioni F, Gaetani P, Villani L, Zappoli F, Rodriguez
Y, Baena R (1995) Neurinoma of the trigeminal root
and atypical trigeminal neuralgia: case report and
review of the literature. Surg Neurol 44(1):36–42.
doi:0090301995001735 [pii]
78. Tanriover N, Kemerdere R, Kafadar AM, Muham-
medrezai S, Akar Z (2007) Oculomotor nerve schwan-
noma located in the oculomotor cistern. Surg Neurol
67(1):83–88. doi:S0090-3019(06)00432-0 [pii]; dis-
cussion 88
79. Tung H, Chen T, Weiss MH (1991) Sixth nerve
schwannomas. Report of two cases. J Neurosurg
75(4):638–641. doi:10.3171/jns.1991.75.4.0638
80. Vachata P, Sames M (2009) Abducens nerve schwan-
noma mimicking intrinsic brainstem tumor. Acta
Neurochir (Wien) 151(10):1281–1287. doi:10.1007/
s00701-009-0302-9
81. Veshchev I, Spektor S (2002) Trochlear nerve neu-
roma manifested with intractable atypical facial pain:
case report. Neurosurgery 50(4):889–891; discussion
891–882
82. Visot A, Derome P, De Leon JL (1992) Spheno-
cavernous and infratemporal trigeminal neurinomas:
surgical series of 15 cases. Skull Base Surg 2(3):
142–149
83. Yamaki T, Morimoto S, Ohtaki M, Sakatani K, Sakai
J, Himi T, Harabuchi Y, Tanabe S, Hashi K (1998)
Intracranial facial nerve neurinoma: surgical strategy
of tumor removal and functional reconstruction. Surg
Neurol 49(5):538–546. doi:S0090301997000256 [pii]
References 607

84. Yasui T, Hakuba A, Kim SH, Nishimura S (1989) 86. Yoshida K, Kawase T (1999) Trigeminal neurinomas
Trigeminal neurinomas: operative approach in eight extending into multiple fossae: surgical methods and
cases. J Neurosurg 71(4):506–511. doi:10.3171/ review of the literature. J Neurosurg 91(2):202–211.
jns.1989.71.4.0506 doi:10.3171/jns.1999.91.2.0202
85. Yonas H, Jannetta PJ (1980) Neurinoma of the trigem-
inal root and atypical trigeminal neuralgia: their com-
monality. Neurosurgery 6(3):273–277
 Jugular Foramen Tumors
General Features
The management of jugular foramen (JF) tumors
remains a challenge despite the considerable
progress over the last decades. Their location
deep in a confined space of the skull base and the
close proximity or even involvement of vital neu-
ral structures and/or major vessels complicates
surgical removal. The access to the JF area is
restricted by the surrounding structures: the
carotid artery anterior, the vertebral artery infe-
rior, the hypoglossal nerve medially and the mas-
toid, styloid process, transverse process of C1,
and facial nerve laterally [116]. Interdisciplinary
cooperation offers the possibility to select the
best treatment option for each individual patient.
Jugular foramen tumors arise either primary
within the foramen—glomus jugulare tumors and
schwannomas of the 9th–11th cranial nerves—or
from neighboring structures and involve the fora-
men secondarily: meningiomas, chordomas, chon-
drosarcomas, cholesteatomas, endolymphatic sac
tumor, chondroblastoma, lymphangioma, temporal
bone carcinoma, sarcoma, and metastases. Primary
bone tumors may also present as JF lesions: giant-
cell tumor, aneurismal bone cysts, osteoblas-
toma, plasmacytomas, ossifying fibroma, osteoid
osteoma, and fibrous dysplasia [63, 64]. The most
common primary tumors are glomus jugulare, fol-
lowed by schwannomas and meningiomas [28, 38,
84]—these three types compose more than 80 % of
all JF tumors [95]. The most common primary ori-
gin of JF metastases is the head and neck, followed
by breast, lung, and prostate tumors [41].
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_10, © Springer-Verlag Berlin Heidelberg 2013
10
JF tumors tend to extend through the path of
least resistance. They may extend within the CP
angle and involve the petrous bone, may grow
anterior to the pons with or without involvement
of the clivus, or expand inferior even below the
foramen magnum. Some dumbbell-shaped
tumors, usually meningiomas and schwannomas,
have both intra- and extracranial parts [76, 77].
Neuroimaging
The radiological assessment confirms the pres-
ence of a jugular foramen mass and—in most
cases—allows for precise preoperative assess-
ment of the tumor type. Accurate preoperative
diagnosis is of great value for the decision-making
process and for patient counseling (Table 10.1).
High-resolution CT and MRI scans should
be obtained in each patient. Thin-slice (1 mm)
bone-window CT allows to evaluate the tumor
for calcifications and the bone structures for
hyperostosis or bone erosion. Tumors in the area
causing significant bone changes are schwanno-
mas, glomus tumors, meningiomas, aneurysmal
bone cysts, chondrosarcomas, endolymphatic
sac tumors, giant-cell tumors, and metastases
[63]. Native and contrast-enhanced MRI provides
information regarding not only tumor features but
also on its extension and precise relationship with
neighboring structures. MR angiography permits
to evaluate tumor vascularization; MR venogra-
phy depicts the venous circulation and patency/
occlusion of the sigmoid sinus and jugular vein.
609
610 10 Jugular Foramen Tumors

Table. 10.1 Typical radiological characteristics of the most frequent JF tumors

CT Bone-window CT MRI Angiography
Schwannoma Isodense or slightly Enlargement of JF, smooth T1: hypo–isointense Mild/moderate
hyperdense well-defined bony margins vascularity
No evidence of bone T2: hyperintense
infiltration Strong contrast
enhancement
Glomus tumor Isodense or slightly Permeative “moth-eaten” T1: salt-and-pepper Extreme vascularity
hyperdense erosion of JF appearance
Irregular bony margins T2: hyperintense Prolonged vascular
blush
Loss of density in the Intense contrast AV shunting
affected bone enhancement
Intratumoral flow voids
Meningioma Slightly Maintained architecture T1: iso–hypointense Mild to moderate
hyperintense vascularity
Hyperostosis/sclerotic T2: iso–hyperintense No arteriovenous
changes of the JF Uniform contrast shunting
enhancement
Absence of vascular
flow voids
Dural tail sign
Modified from Löwenheim et al. [63]

Turbulent flow in a hypertrophic jugular bulb or
slow/absent flow in case of jugular bulb thrombo-
sis must be distinguished from JF tumors. The dif-
ferential diagnosis is based on evaluation of MR
signal characteristics and application of vascular
flow-sensitive techniques, such as phase-contrast
MR venography [20, 84]. Fat saturation technique
allows improved detection of marrow involve-
ment and has been found useful for evaluation of
skull-base abnormalities [38].
The degree of tumor vascularity and the nature
of the tumor should guide the need for preopera-
tive selective angiography and embolization.
Intratumoral flow voids on MRI indicate high vas-
cularity. In such cases, digital subtraction angiog-
raphy may be done—for diagnostic purposes and
as a guide for preoperative embolization [85].
Preoperative embolization might be extremely use-
ful in cases of highly vascularized tumors [74, 85].
The precise knowledge of the tumor-feeding ves-
sels and their relationship to the carotid and verte-
bral arteries, as well as to venous structures, are
important for the approach planning. Further fac-
tors to consider are the tumor relation to brain
stem, lower cranial nerves, middle ear, petrous
apex, and clivus. In case the internal carotid artery
is involved or encased by the tumor, balloon
occlusion test might be required.
Clinical Presentation and Diagnosis
The diagnosis of JF tumors is made usually by
ENT surgeons: the most frequent initial com-
plaints of these patients are related to cochlear
nerve dysfunction (hearing loss or tinnitus) and
lower cranial nerves dysfunction (swallowing
difficulty, hoarseness). Frequently, the clinical
picture is variable due to extension into or com-
pression of surrounding structures: CP angle,
brain stem, middle ear, or para-/retropharyngeal
space. The lesion can differentially affect any of
the low cranial nerves and cause a number of JF
syndromes, which are rarely seen isolated in
patients with JF tumors. The three typical syn-
dromes are JF or Vernet’ syndrome (unilateral
9th + 10th + 11th cranial nerve involvement), pos-
terior laterocondylar or Collet–Sicard syndrome
(unilateral 9th + 10th + 11th + 12th cranial nerve),
and posterior retropharyngeal or Villaret’s syn-
drome (involvement of 9th + 10th + 11th + 12th
cranial nerves and Horner’s syndrome).
Management
The most frequent initial symptoms are pulsa-
tile tinnitus (25–84 %), conductive hearing loss
(66–77 %), headache (20–66 %), hoarseness
(35–38 %), facial paralysis (15–25 %), vertigo
(37 %), hoarseness (25–44 %), swallowing
difficulty (37–50 %), shoulder weakness (22 %),
paralysis of the tongue (19–31 %), trigeminal
hypesthesia (13 %), and—in more advanced
cases—cerebellar (up to 44 %) and long-tract
symptoms [28, 84, 94, 98].
Although most authors report that the clinical
presentation of various JF lesions is similar [28, 38],
other found some differences. Ramina et al.[85]
reported that in glomus jugulare tumors the ini-
tial symptoms are hearing loss and tinnitus, while
schwannomas and meningiomas may present
with lower cranial nerves deficit. The function of
the lower cranial nerves is affected in 20–27 % of
glomus jugulare tumors, in 35–50 % of menin-
giomas, and in 44–50 % of schwannomas [28,
64]. One finding that might be important in the
differential diagnosis is the retrotympanic mass:
it is found in 91 % of glomus jugulare tumors, in
43 % of meningiomas, and rarely (11 %) in
schwannomas [28].
Management
Management options of JF tumors include obser-
vation, radiation therapy and surgery, or combi-
nation thereof. The ideal one is complete surgical
excision with preservation of all neurological
functions, which leads to definitive healing of the
patients, provided the tumor is benign [1, 2, 30,
40, 53, 84]. In malignant tumors and in those
infiltrating essential structures, subtotal removal
with adjuvant radiotherapy may be the most rea-
sonable option. Subtotal tumor removal is pre-
ferred also in cases in which radical surgery
carries high risk of new neurologic deficits:
extensive infiltration of the skull base or of the
cavernous sinus, absence of a cleavage plane
between the tumor and the brain stem or its
infiltration, extreme adherence to cranial nerves
in patients with normal preoperative function, or
encasement of neurovascular structures. Normal
cranial nerve function before surgery does not
611
exclude the possibility that they are actually
involved by the tumor. Further important issues
are tumor type and size, patients’ general and
neurological status, as well as his expectations.
Thus, in case of normally functioning lower cra-
nial nerves, a tightly adherent tumor part, for
example, in the jugular foramen, might be left.
Contrary, if a severe lower cranial nerve dysfunc-
tion is already present, complete tumor removal
should be favored.
External beam radiotherapy and radiosurgery
are primary management options in the elderly
medically unstable patients and in those unwill-
ing to have surgery. They are used as an adjunc-
tive treatment in case of unresectable tumor
remnants or recurrences [37].
Operative Approaches
A good knowledge of the normal and pathologi-
cal microanatomy of the JF area is a major pre-
requisite for successful surgical management.
According to the classical concept, the jugular
foramen consists of two parts: pars nervosa or
nervous part and pars venosa or venous part. Pars
nervosa is located anteromedially and contains
the inferior petrosal sinus, meningeal branches of
the ascending pharyngeal artery, vena canaliculi
cochlea, and glossopharyngeal nerve, while the
pars venosa, located posterolaterally, contains the
jugular bulb and vagus and accessory nerves [5,
7, 26]. The vertical portion of the facial nerve
straddles the jugular bulb. Recent microanatomi-
cal studies have shown, however, that this
simplified concept lacks practical significance.
The JF consists actually of three compartments,
defined by dural septations. The third one is
located between the two venous compartments at
the level of the interjugular process [57, 88]. This
interjugular compartment contains the 9th, 10th,
and 11th cranial nerves, running through two
dural perforations: the glossopharyngeal meatus
(9th nerve) and the vagal meatus (10th and 11th
nerves). The great interindividual variability, in
regard to the course of the cranial nerves within
the JF and venous anatomy, should be considered
by the surgeon [103].
612
The operative approach to the jugular fora-
men should provide adequate access to the area
and enable the surgeon to perform safe tumor
removal. Generous exposure of all surrounding
structures, however, is not a prerequisite for suc-
cessful surgery. The approach should be tailored to
the individual patients and tumor characteristics.
Importantly, it should not cause additional mor-
bidity or major discomfort to the patient. Multiple
approaches to the area of the jugular foramen,
depending on the tumor extension pattern, have
been developed and are still used: infratemporal
fossa, modified infratemporal fossa, transmas-
toid, widened transcochlear, translabyrinthine, far
lateral, trans-sigmoid, transjugular, suboccipital–
retromastoid, petro-occipital–trans-sigmoid, jux-
tacondylar, mastoid, and lateral neck approaches
[1, 9, 24, 30, 31, 36, 77, 81, 94, 98].
The infratemporal approach, which is a
refinement of the cervicomastoid approach of
Gallard and Rebattu [94], was described by Fisch
in 1978 [29]. It allows direct view of the jugular
foramen and wide exposure of the tumor. The
approach was modified later to enable resection of
both tumor components in a single stage [56, 81].
This approach is regarded by some surgeons as
the optimal one for resection of more vascular
tumors, such as glomus jugulare tumors [99].
Drawbacks of this technique is that it necessitates
sacrifice of the middle ear structures, closure of
the ear canal, which can cause conductive hear-
ing loss and anterior rerouting of the descending
facial nerve, related to a certain risk of functional
deficits [13, 77]. With some later technical
improvements, the risks for permanent facial
palsy have been considerably reduced. Leonetti
and Brackmann [62] developed a technique for
mobilizing the facial nerve during infratemporal
fossa surgery and preserving its vasculature at the
stylomastoid foramen. Pensak and Jackler [82]
described the fallopian bridge technique, which
allows tumor removal without mobilization or
transposition of the facial nerve. In their series,
normal facial function at 6 months follow-up had
92 % of the cases. In case of smaller jugular fora-
men schwannomas and meningiomas with lim-
ited extension, more conservative approaches
have been proposed.
10 Jugular Foramen Tumors
The main principle that we follow when select-
ing the operative approach is that it should be tai-
lored to the specific characteristics of each patient in
terms of tumor location, type, size and extension
pattern, involvement and degree of erosion/destruc-
tion of the bone structures, and patients’ neurologi-
cal status and expectations. Moreover, surgery
should be regarded as part of the whole manage-
ment—in some tumors it can heal the patient, while
in others, it is a component of the multimodal treat-
ment plan. The approach should allow achieving
the goals of surgery but—on the other hand—should
not be related to high procedure-related morbidity.
Electrophysiological monitoring of the 9th,
11th, and 12th cranial nerves is performed in addi-
tion to the routine monitoring of SSEP, facial nerve
EMG, and BAEP. The electrodes are placed in the soft
palate, trapezius muscle, and tongue, respectively.
Endotracheal tube electrodes are utilized in rare cases
to map and monitor the function of vagus nerve.
Lateral Suboccipital (Retrosigmoid)
Approach
Tumors confined to the posterior cranial fossa, as
well as most of those located primarily in the
jugular foramen with a smaller intracranial part,
are approached intradurally via the lateral suboc-
cipital route. The technique of the approach is
described in the chapter on RS approach. In case
of jugular foramen tumors, in contrast to VS, the
dural incision should not be continued necessar-
ily to the level of the transverse sinus. The main
anatomical landmarks and neural structures are
identified, and the intracranial tumor portion is
approached initially. In a manner similar to the
one applied in VS, the tumor is debulked with
ultrasonic aspiration or with suction and platelet
knife. The dissection of the tumor capsule from
the cranial nerves is performed only when
sufficient internal decompression has been
achieved and is performed always in the arach-
noid plane. The two-hand technique is used—the
tumor capsule is held with one hand and the dis-
section is performed with the other hand (usually
the dominant one). The intraforaminal part of the
tumor is approached afterward. If it is small, it
can be gently pulled out of the foramen into the
CP angle and removed. Opening its dorsal wall,
Management
in a technique analogous to the one used to open
the IAC in case of VS removal, can extend the
approach to the jugular foramen (see Case
Illustration 10.1). The senior author has applied
this technique since the 1980s. The amount of
safe bone removal is evaluated on the bone-
window thin-slice CT and tailored to the particu-
lar tumor extension. The dura overlying the
posterosuperior part of the foramen is stripped
off and cut away. High-speed drill is used to
remove the bone in 0.1-mm increments under
direct visual control. Drilling starts usually at the
superior margin of the foramen and proceeds
posteriorly. Large-sized diamond burr (4–5 mm
in diameter) are used initially and changed to
613
smaller burrs in the depth of the foramen. The
last bone layer or lamina is removed with a small
platelet knife in order to avoid damage to under-
lying structures. The intraforaminal tumor part is
debulked initially, and then its capsule is dis-
sected from the cranial nerves and/or vessels. The
utilization of angled rigid endoscopes (since 2005
at the INI) allowed for removal of tumors with
even larger intraforaminal extension (see below).
Following tumor removal, in case the jugular
foramen has been opened, it should also be sealed
with multiple small fat pieces fixed with fibrin
glue. The dura is closed in a watertight fashion,
and the opened mastoid air cells are also sealed
with fat pieces and fibrin glue.
614
Case Illustration 10.1
Cystic Jugular Foramen Schwannoma
Removal (Drilling of the dorsal Wall of the
jugular foramen)
This patient—operated in 1988—was one of
the first cases in which the technique of open-
ing the dorsal jugular foramen wall was applied.
He presented with swallowing deficits and
hoarseness. The preoperative MR images,
showing the cystic tumor with extension in the
a b
(a–c) Preoperative MR images. (d–j) Intraoperative
images. (d) Initial view of the tumor (Tu) in the CP
angle. (e–f) Removal of the CP angle tumor part, visu-
alization of the seventh–eighth cranial nerve complex
10 Jugular Foramen Tumors
foramen jugulare, are presented on figures a–c.
The patient was operated in the semi-sitting
position: a retrosigmoid craniotomy was
performed, and the CP angle tumor portion was
removed. Then, the dura dorsal to the jugular
foramen was excised, and the underlying bone
drilled off. Thus, the intraforaminal tumor por-
tion was well exposed and completely removed.
The patient recovered rapidly from surgery and
did not have new neurological deficits.
(7–8). (g) The arrowheads delineate the jugular fora-
men. (h) Drilling of the dorsal foramen wall and expo-
sure of the intraforaminal tumor portion (small arrows
on i). (j) Complete tumor removal
Management 615

d e

f g

h i

j
616
Combined Cervical and Lateral
Suboccipital Intra–extradural
Transmastoid Infralabyrinthine Approach
The approach is used in tumors with cervical
extension or very large inaccessible intraforam-
inal part. It consists of several steps:
1. Skin incision and identification of the anterior
border of the sternocleidomastoid muscle
2. Exposure of the mastoid
3. Neck dissection and exposure of the cervical
region, vessels, and nerves
4. Exposure of the facial nerve
5. Retrosigmoid craniectomy and mastoidectomy
6. Exposure of the sigmoid sinus
7. Intradural exposure
8. Extracranial tumor removal
9. Intracranial tumor removal
10. Reconstruction of the dura and skull base
Neck Dissection and Exposure of the Cervical
Region, Vessels, and Nerves
The patient is placed supine on the operating
table. The head is fixed with a Mayfield head
holder and turned 60° to the contralateral side. A
slightly curved retroauricular skin incision is per-
formed, starting in the temporal region and reach-
ing the anterior border of sternocleidomastoid
muscle. A dissection plane is created between the
parotid gland and the anterior border of the ster-
nocleidomastoid muscle, preserving the greater
auricular nerve. The facial nerve is identified
between the mastoid process and angle of man-
dible, ventral to the stylomastoid foramen.
Helpful landmark for its identification is the
“pointer” of the cartilaginous auditory canal. The
sternocleidomastoid muscle is detached from the
mastoid and reflected inferiorly. The posterior
belly of the digastrics muscle is also cut at the
skull base and reflected inferiorly.
The hypoglossal nerve is located inside the
carotid triangle, medially to the posterior belly of
the digastric muscle. The caudal cranial nerves
are exposed in the neck, along with the internal
jugular vein, the common carotid artery with the
external and internal carotid arteries, and fol-
lowed to the skull base.
10 Jugular Foramen Tumors
Craniectomy, Mastoidectomy/Petrosectomy
A retromastoid craniectomy is made, as described
earlier. Additional mastoidectomy is performed
with a diamond drill to expose the sigmoid sinus
from the superior petrosal sinus to the jugular
bulb. The jugular foramen can be further exposed
dorsolaterally by drilling the posterior part of the
occipital condyle.
The tumor extension and the degree of involve-
ment of the pyramid, middle, and/or inner ear
define the extent of bone removal. Whenever
needed, a radical mastoidectomy is performed,
and the sigmoid sinus is exposed widely, down to
the jugular foramen. The pyramid is drilled fur-
ther anteromedial to the fallopian canal, superior
to the styloid process and inferior to the laby-
rinth. The antrum, the lateral semicircular canal,
and the vertical portion of the facial nerve are
exposed. Mobilization and rerouting of the facial
nerve are necessary only if the tumor extends to
the middle ear cavity or into the carotid canal. In
order to avoid severe facial nerve palsy, care is
taken to preserve the vascularized fascia and soft
tissue attached to the nerve at the stylomastoid
foramen.
In deaf patients with extensive tumors, the
posterior wall of the external auditory meatus is
drilled, and the external auditory canal is cut at
the osteocartilaginous junction, thereby gaining
access to the tumor portion in the ear.
Intradural Exposure
The dura might be incised posterior or anterior to
the sigmoid sinus; the incision is then extended to
the jugular foramen. The dura, transverse and
sigmoid sinuses, and the jugular bulb are retracted
anteriorly. The cerebellopontine cistern is then
opened to release the CSF, and the cerebellum is
retracted gently, exposing the intracranial tumor
portion.
Tumor Removal
The extracranial portion of the tumor is usually
resected first, following the identification of the
crucial neck structures. The varying direction of
displacement and/or involvement of the major
Management
vessels in different tumors should be consid-
ered. Glomus jugulare tumors may surround or
even infiltrate the walls of the internal carotid
arteries, while schwannomas frequently displace
it anteriorly.
The intracranial portion of the tumor is deb-
ulked with the ultrasonic aspiration and dissected
from the cranial nerves and vessels. With this
technique, a wide exposure of the jugular fora-
men is achieved, allowing removal of the whole
intraforaminal tumor portion. The most lateral
parts might be visualized and removed with endo-
scopic control, as described earlier. Schwannomas
and meningiomas of the jugular foramen usually
displace the nerves ventrally and the jugular bulb
dorsally and inferiorly, while in case of glomus
tumors, the relations between the tumor and sur-
rounding structures cannot be predicted. Tumors
arising from the dome of the jugular bulb can
extend intraluminally—in the sigmoid sinus and
internal jugular vein. In such cases, the sigmoid
sinus and the internal jugular vein have to be
ligated and opened in order to achieve complete
tumor removal. The anterior jugular bulb wall
helps to preserve the lower cranial nerves.
In case of benign noninvasive lesions,
complete removal of the tumor and infiltrated
bone should be attempted. Contrary, in case
of malignant tumors or if a cleavage plane is
absent, complete “oncological” removal is fre-
quently not possible without the risk of severe
neurological deficits and the amount of resec-
tion should be carefully planned. If the facial
nerve has been destroyed by the tumor, healthy
proximal and distal ends are identified and an
interposition sural nerve graft is used for its
reconstruction.
Reconstruction of the Dura
and Skull Base
After the tumor has been resected, the jugular
foramen is sealed with several fat pieces and
fibrin glue. It can be further reinforced from out-
side with the posterior belly of the digastric
muscle. The dura is closed in a watertight fash-
ion, and the posterior fossa is reconstructed with
617
methyl methacrylate (Palacos). The sterno-
cleidomastoid muscle is sutured back at its
insertion on the occipital bone, the wound cav-
ity is filled with hemostatic sponges and/or fat
pieces, and the wound is closed in layers. In
case the external auditory canal had been cut, it
is closed in two layers.
Endoscope-Assisted or Pure Endoscopic
Tumor Removal
An important advancement in the recent years
was the introduction of the rod lens endoscopes
and the development of appropriate instrumen-
tation. Endoscopy is currently an integral part
of the neurosurgical armamentarium in case of
jugular foramen tumors. Angled endoscopes
allow to visualize and remove, whether under
microscopic or under pure endoscopic control,
tumor parts, extending or located “behind the
corner.” Importantly, the microscope and the
endoscope should not be considered as mutu-
ally excluding tools but rather should be used
judiciously to achieve the goals of surgery.
Complete resection of such tumors required
previously complex combined approaches or
two separate operative steps, which were related
to certain risk of approach-related morbidity.
Currently, the extracranial and the jugular fora-
men parts of the tumors can be removed via the
standard cervical transmastoid approach, while
the intracranial part can be removed with an
endoscope-assisted/pure endoscopic technique:
the “outside–inside technique.” Thus, the neces-
sity of a retrosigmoid approach to access this
part is obviated (see Case Illustration 10.2).
Tumors with a major intracranial intradural
part, especially if they cause brain stem com-
pression, should be approached and removed
via the retrosigmoid route. The intraforaminal
tumor extension in such cases can be resected
with an endoscope-assisted/pure endoscopic
technique: the “inside–outside technique” (see
Case Illustration: Endoscope-Assisted transcer-
vical Tumor Removal)
618
Case Illustration 10.2
Endoscope-Assisted Transcervical Tumor
Removal
The patient presented with a large recurrent
chondrosarcoma in the left CP angle and jug-
ular foramen areas with a major extracranial
extension along the internal jugular vein (a–l).
She has been operated three times previously:
24 years, 10 years, and 4 years ago via differ-
ent approaches, including a retrosigmoid one.
At presentation, she had an HB grade IV facial
nerve palsy (since the last surgery), trigemi-
nal nerve hypesthesia (V1,V2), swallowing
difficulty, and dysphonia.
Considering the size of the tumor and the
growth tendency of its intracranial part, a deci-
sion in favor of surgery was taken. The surgical
strategy was to approach initially the extracra-
nial tumor part transcervically and follow it
proximally through the bone corridor created
by the growing tumor (see the bone-window
CT scans on figure a). In case the intradural
part could not be safely removed, a lateral
suboccipital craniotomy could be additionally
made. The internal carotid artery and the lower
cranial nerves were exposed at the beginning.
10 Jugular Foramen Tumors
The tumor was seen encroaching the digastric
muscle from above (m, n). Its capsule was
then incised, and intracapsular removal under
microscopic control was performed (o, p). In
order to visualize and reach the more rostral
parts, an angled endoscope was utilized. Using
angled instruments and endoscopic visualiza-
tion, the whole tumor, including its intradural
part in the CP angle, could be completely
removed (q, r). Enlargement of the bone open-
ing was not necessary. The integrity of the
lower cranial nerves and of seventh–eighth
nerves was preserved. Fat pieces and fibrin
glue were used to seal the dural opening.
Thus, the tumor could be removed completely
in one stage, and a transcranial approach was
not necessary.
After the surgery, temporary worsening of
the swallowing deficit was noted. Still, the
function recovered rapidly, and in 3 weeks,
the oral feeding could be started. At 3 months
follow-up examination, the patient reported
that swallowing is even better than prior to
surgery. The facial nerve palsy remained
unchanged. The postoperative MRI is pre-
sented on figures s–x.
Management 619

a b

c d

Preoperative CT (a) and MR images (b–l) of the
patient, demonstrating the extension of the tumor. See
the text for details. Intraoperative images: (m, n) expo-
sure of the tumor and incision of its capsule. (o) Gradual
internal decompression. (p) Complete removal of the
tumor part, visible with the microscope. (q)
Visualization of tumor remnants with the angled endo-
scope and their removal with angled instruments (r).
(s–x) MRI performed 3 weeks after surgery. The tumor
cavity is filled with fat tissue
620 10 Jugular Foramen Tumors

e f

g h
Management 621

i j

k l
622 10 Jugular Foramen Tumors

m n

p
o

q r
Management 623

s t

u v
624 10 Jugular Foramen Tumors

w x
Postoperative Care and Outcome
Extended Endoscopic Endonasal
Approach
The extended endoscopic endonasal approach to
the JF has been developed in the recent years as
an alternative to the microsurgical technique. Its
theoretical advantage is reduction of the risks
related to manipulation of neurovascular struc-
tures [108, 125]. The approach is regarded as
especially useful in case of caudal and medial
tumor extension. Early experience has shown that
the technique is feasible and does not restrict the
access and—respectively—the possibility to
remove the tumor. The direct access to the carotid
artery in its subpetrous and retropharyngeal parts
allows for early devascularization of the tumor.
The experience with this approach is still limited
and insufficient to prove its supremacy to micro-
surgical or endoscope-assisted tumor removal in
terms of neurological outcome and radicality of
tumor removal. A major problem that remains is
the difficult reconstruction of the dura and the
skull base in tumors with intracranial extension
and the respectively high CSF leak rate [125].
Postoperative Care and Outcome
Lower Cranial Nerves Dysfunction
The most frequent and dangerous operative com-
plication is the occurrence of new lower cranial
nerve deficit. Injuries to these nerves lead to
dysphagia and/or dysphonia (9th, 10th cranial
nerves), scapular winging and shoulder droop
(11th nerve), and weakness and atrophy of the
tongue (12th nerve). These nerves are vulnerable
to injury along their intracranial, extracranial,
and foraminal segments. The glossopharyngeal
nerve is regarded as the most vulnerable to sur-
gical injury due to its medial position within
the jugular foramen. Its most important func-
tion is sensory innervation of the upper phar-
ynx: it triggers the swallow reflex. In case the
vagus nerve is intact, however, it acts as a sec-
ond trigger of the swallow reflex. Injury to the
vagus nerve disrupts the peristaltic contractions
625
of the esophageal muscles. On the other hand,
laryngeal closure during swallowing, which in
healthy persons prevents aspiration, is inhibited.
Especially endangered for aspiration are patients
with palsy of both ninth and tenth nerves.
Bilateral vagus nerve injuries lead additionally
to stridor and respiratory insufficiency due to the
inability to abduct the vocal cords and, hence,
to open the larynx. The hypoglossal nerve has
the lowest incidence of iatrogenic injury. Its uni-
lateral dysfunction causes dysarthria and swal-
lowing difficulties. Bilateral dysfunction causes
significant swallowing dysfunction due to dis-
turbed swallowing initiation, which is mediated
by the hypoglossal nerve.
Lower cranial nerve dysfunction severely
impacts the patients’ quality of life and might
increase the risk of other, potentially life-threatening,
complications. In order to avoid such outcome,
every effort should be made to preserve the
nerves at surgery and to manage the patients
with neurological deficits properly. The main
prerequisites for preservation of lower cranial
nerves are the following: careful selection of
surgical indications and goals; choice of most
appropriate operative approach; detailed knowl-
edge of lower cranial nerve microanatomy;
early identification of all crucial structures,
using the anatomical landmarks; and constant
intraoperative nerve monitoring. Dissection of
the tumor capsule should be performed only
after sufficient internal tumor decompression
has been achieved and should always proceed in
the correct arachnoid plane.
Postoperative Care
Slowly developing swallowing dysfunction dur-
ing tumor growth can be generally well compen-
sated for by the healthy contralateral side. New
postoperative lower cranial nerve dysfunction,
however, is related to a very high risk of compli-
cations, such as aspiration pneumonia. The swal-
lowing difficulty and the impaired ability to
cough and expectorate promote frequent aspira-
tions with the related risk of aspiration pneumo-
nia and other pulmonary complications.
626
Patients operated for jugular foramen tumors
are usually intubated nasally. The risk of aspira-
tion is related to the patients’ level of conscious-
ness, and too early extubation after surgery
should be avoided. Nasal tubes are well tolerated
and should be removed only when the patient is
really awake and cooperative. In case of swal-
lowing dysfunction, oral feeding is prohibited.
The reason for the swallowing dysfunction
should be identified and specific examina-
tions carried out: endoscopic and/or radiologi-
cal evaluation of the swallowing act. The later
study provides information on the mechanics
of swallowing, the laryngeal function, and the
risk of aspiration. Nasogastric tube feeding is
continued—even for up to several weeks—
until swallowing recovers, and resumption of
adequate oral intake is safe. Tracheostomy
should be considered in patients with swallow-
ing difficulty and recurrent aspiration. It should
be performed as early as possible if clinical
signs of persistent aspiration are present. Early
vocal cord medialization may be an alternative
to tracheostomy. If prolonged recovery is antici-
pated, a gastrostomy is necessary as a long-term
means for providing nutrition. Currently, it is
performed as a percutaneous endoscopic proce-
dure. Speech–language (logopedic) therapy is
helpful to promote velopharyngeal function and
teach compensatory articulation techniques and
should be initiated early after surgery.
Tumor type has been found to correlate with
the risk of developing lower cranial nerve dys-
function. Glomus jugulare tumors often compress
the lower cranial nerves, without necessarily
infiltrating them. Schwannomas originate from
one of the lower cranial nerves, and preop-
erative palsy is often present. The risk for new
neurological deficits is highest in case of menin-
giomas. In the series of Lustig and Jackler [64],
new deficit was registered in approximately 60 %
of the meningiomas, 30 % of glomus jugulare
tumors, and in 15 % of schwannomas. Oghalai
et al. [77] presented a series of 28 patients with
jugular foramen tumors with intracranial exten-
sion: new postoperative lower cranial palsy was
found only in patients with meningiomas (40 %)
and schwannomas (36 %).
10 Jugular Foramen Tumors
Fayad et al. [28] presented their experience
with 83 patients with jugular foramen tumors.
Total tumor removal was achieved in 81 % of the
cases. The occurrence of new lower cranial nerve
deficits was highest in meningiomas (50 %),
followed by schwannoma (22 %) and glomus
jugulare tumors (19 %). Normal or near-normal
facial function at last follow-up had 88 % of the
patients. Although the conclusion of the authors
is that total resection of jugular foramen tumors
can be a curative treatment, subtotal removal
may be required to preserve neural functions.
Ramina et al. [85] reviewed retrospectively 102
patients with jugular foramen tumors. Complete
tumor removal was achieved in 77.5 % of the
patients with paragangliomas and in all patients
with schwannomas. Lower cranial nerve deficit
occurred in 10 % but was transient in 3.8 %.
The mortality rate in the series was 4.2 %. In the
series of Lustig and Jackler [64], new postopera-
tive CN dysfunction occurred most frequently
in patients with meningiomas (55 %), followed
by those with glomus tumors (35 %) and with
schwannomas (15 %).
Another common complication, related mostly
to the technique used to reconstruct the dura and
the skull base, is the CSF leak and the related
infectious complications. The incidence of CSF
leak in the literature varies from 3.9 to 13.3 %
[52, 85, 98].
Radiotherapy/Radiosurgery
Radiosurgery and stereotactic radiotherapy are
alternative treatment options for patients with
smaller JF tumors. The experience is expanding,
and many groups report good results. Compared
to surgery in the JF area, good standards of radio-
surgical treatment are much earlier achieved and
are not so directly related to the individual expe-
rience. The outcome after radiosurgery, espe-
cially at long-term follow-up, however, is not
superior to that of surgical resection, performed
by experienced surgeon. Furthermore, the goal of
radiation therapy is tumor control, not cure.
Additional drawbacks of radiation therapy are the
following: lack of tissue diagnosis, delay from
Jugular Foramen Schwannomas
treatment to its effect, and the risks of radiation,
especially in case of younger patients. Therefore,
we consider primary radiosurgery/radiotherapy
as an option only for those unwilling or unable to
undergo surgery.
Jugular Foramen Schwannomas
JF schwannomas originate either from the
glossopharyngeal, vagus or accessory nerves, or
from the cervical sympathetic chain within the
jugular foramen [50, 58, 109]. They are relatively
uncommon and in the absence of neurofibromatosis
comprise 2.9–4 % of all intracranial schwannomas
[94, 96, 98, 113]. In the senior authors’ series,
the ratio of JF to vestibular schwannomas was
1:24 [94].
JF schwannomas originate most frequently
from the 9th cranial nerve and most rarely from
the 11th nerve. As noted by some authors, how-
ever, the exact nerve of origin is often difficult to
determine and is not always reported [23, 44,
113]. Song et al. [109] estimated that up to 90 %
of the cases reported in the literature are identified
to originate from the glossopharyngeal or vagus
nerves (Figs. 10.1 and 10.2). The reason for this
predilection is still unknown. According to one
hypothesis, Schwann cells in the vicinity of the
respective ganglia are more prone to tumor devel-
opment. The superior and inferior ganglia of the
glossopharyngeal nerve are both located within
the JF; the superior ganglion of the vagus nerve is
located within the JF, while its inferior ganglion
is located below the extracranial orifice of the
foramen [64, 109]. On the other hand, the acces-
sory nerve has no ganglia within the JF.
JF schwannomas are more common in females
and are diagnosed usually in the fourth to sixth
decade of life [23, 31, 58]. They are slow-growing
noninfiltrative lesions. Malignant schwannomas
in this region are rare but have been reported [8].
JF schwannomas comprise a highly variable
group not only in terms of nerve of origin. The
tumor itself may have intracranial, intraforaminal,
and/or extracranial components or combination
thereof. The variable site of origin along the nerve
could explain these differences, for example,
627
Fig. 10.1 Small schwannoma in the area of the right JF
(T2-weighted MR image). The diagnosis was made
relatively early due to the patients’ complaints of swal-
lowing difficulty
proximal or distal within the jugular foramen.
More proximal origin along the nerve usually
leads to predominant intracranial tumor exten-
sion, while tumors with more distal sites of origin
tend to grow intraforaminally and extracranially
[23]. Song et al. [109] pointed that the tumor
grows in the direction of least pressure or
resistance, but the expansion can be modified by
specific anatomical structures acting as a barrier
to tumor growth, for example, the inferior
petrosal sinus.
Classification
Several classifications of JF schwannomas related
to tumor location and extension pattern have been
introduced and are currently used by different
surgeons [19, 31, 58, 69]. We present the one that
we use in our practice [94], which is useful for
approach selection and outcome prediction:
• Type A—the tumor is located primarily intracra-
nially with minimal enlargement of the JF.
• Type B—the tumor is primarily in the JF with
a smaller intracranial portion.
• Type C—the tumor is primarily extracranial.
• Type D—“dumbbell”-shaped tumor with
extra- and intracranial components.
628
a b
d e
g h
Fig. 10.2 MRI of a patient with a vagus nerve schwan-
noma. The preoperative images (a–e) provide no clue on
the possible nerve of origin. (a–c) T1-weighted contrast-
enhanced sequences. (d) T2-weighted sequence. (e) T2
dark fluid sequence. Imaging does not allow to distinguish
Clinical Presentation
The early clinical symptoms in patients with
JF schwannomas are related to the tumors’ ori-
gin, while in later stages they are determined
10 Jugular Foramen Tumors
c
f
it from other JF schwannomas. The tumor was removed
completely via the retrosigmoid approach. At surgery, it
was found that it originated from the fibers of the vagus
nerve. The control MR images 12 months later are shown
on figure (f–h)
by the tumors’ size, location, and effect (e.g.,
compression) on the surrounding structures
[63]. In the senior authors’ series, the most fre-
quent initial symptom was headache (66 %),
Jugular Foramen Schwannomas
followed by hearing loss (50 %), hoarseness
(38 %), and dysphagia (38 %). At presentation,
81 % of the patients had lower cranial nerve
deficit, 75 % had hearing loss, 44 % had cer-
ebellar symptoms, 31 % had tongue hemiat-
rophy, 25 % had facial palsy, and 13 % had
altered facial sensation [94]. The lower cranial
nerve dysfunction does not necessarily cor-
relate with the nerve of origin because the JF
compartment is so small that all cranial nerves
may be affected at the same time.
JF schwannomas should be differentiated
preoperatively from VS and glomus jugulare
tumors. Type A tumors tend to present as a
CP angle mass and cause hearing loss, tinni-
tus, vertigo, and ataxia, thus mimicking a ves-
tibular schwannoma [94]. Preoperative facial
nerve weakness, however, has been reported in
20–25 % of patients with JF schwannomas [69, 94],
while in VS, such presentation is not typical.
Patients with type B tumors present with tinni-
tus, deafness, middle ear mass, and lower cra-
nial nerve and/or 12th nerve dysfunction, while
patients with type C tumors present with a pal-
pable neck mass, mass of the lateral pharyngeal
wall, or with a variety of JF syndromes [67, 105].
Dumbbell-shaped tumors frequently present as
a neck mass or dysphagia/dysphonia.
a b
Fig. 10.3 Type A JF schwannoma with a large intracranial part and a smaller JF part. (T1-weighted MRI after gado-
linium application)
629
Neuroimaging
On native CT scans, JF schwannomas appear
isodense or slightly hyperdense and show moder-
ate enhancement after administration of contrast
medium. Bone-window thin-slice CT demonstrates
the enlargement of JF with smooth well-defined
bony margins and no evidence of bone infiltration
(see the case illustrations to the chapter) [7].
JF schwannomas are iso- to slightly hypoin-
tense on T1-weighted MRI and have a mild or
moderate hyperintensity on T2-weighted MRI.
They show dense enhancement after contrast
administration. The tumor is well circumscribed
and has a round or elongated configuration that
follows the course of the respective cranial nerves
[27, 65, 96]. The lack of IAC involvement differ-
entiates them from VS. The “dumbbell” shape—
if present—is almost pathognomonic [63]. Based
on the preoperative neuroimaging data, it is, how-
ever, impossible to determine the nerve of origin
of the schwannoma, even if the tumor is of small
size (Figs. 10.1, 10.2, 10.3, 10.4, 10.5, and 10.6).
MR angiography is generally sufficient to present
the vasculature of and around the tumor. Highly
vascularized schwannomas are exceptional, and
preoperative conventional angiography with or
without embolization is generally not required.
630 10 Jugular Foramen Tumors

a b

Fig. 10.4 Axial and coronal T1-weighted MRI after gadolinium application, demonstrating a type B JF schwannoma,
located predominantly within the jugular foramen (arrow)

a b

Fig. 10.5 T1-weighted contrast-enhanced and T2-weighted MRI contrast application of a large type C schwannoma
Jugular Foramen Schwannomas 631

a b

c d

Fig. 10.6 Type D schwannoma—“dumbbell”-shaped tumor (T1-weighted MRI after gadolinium application)
632
Case Illustration 10.3
JF Schwannoma with a Predominant
Intracranial Growth
This 53-year-old male patient presented with
complaints of headache, hearing loss on the
right side, and dizziness since 12 months.
Neurological examination revealed a slight
abducens nerve palsy and complete hearing
loss on the right side, as well as gait ataxia.
Bone-window CT showed an area of erosion
of the petrous bone with smooth borders, as
usually seen in case of slow-growing schwan-
nomas (a, b). MRI demonstrated a large tumor
in the right jugular foramen area with a pre-
dominant intradural intracranial extension,
irregular shape, and inhomogeneous contrast
uptake (c–e).
Preoperative CT (a, b) and T1-weighted MRI after
contrast application (c–e), demonstrating a large tumor
in the right jugular foramen area. The tumor has an
inhomogeneous signal on the MR images; it is predom-
inantly located intracranially intradurally and has a
smaller extension into the jugular foramen and the
petrous bone. Severe compression of the brain stem
and of the cerebellum is also visible. The bone erosion,
well seen on the bone-window CT, has the typical for
lower cranial nerve schwannoma’s smooth margins.
Intraoperative images. (f) The dura has been incised
along the sigmoid sinus. (g) The cerebellum is retracted
very slightly to expose only a small part of the tumor
(Tu). (C cerebellum, D dura). Internal decompression
was performed to decrease the pressure, and only after-
ward, a larger part of the tumor was exposed. Further
internal decompression and dissection of the tumor
10 Jugular Foramen Tumors
Considering the fact that the major tumor
part was located intradurally in the CP angle
and caused brain stem and cerebellar com-
pression with respective neurological signs, a
decision in favor of the RS approach was
taken. Initially, the CP angle tumor part was
debulked, and its capsule was dissected from
all surrounding structures in the arachnoid
plane. Then, the jugular foramen was opened,
and the intraforaminal tumor part was removed
completely. The anatomical integrity of the
lower cranial nerves was preserved.
After the surgery, the patient did not have
new neurological deficits (o, p: postoperative
CT scan early after surgery). He recovered
rapidly and at discharge had just slight gait
instability and hearing loss.
capsule were performed intermittently, and gradually
the facial nerve (asterisks) was seen and dissected from
the tumor (h). Gradually, the whole CP angle tumor
portion was removed. (i) The brain stem, free of tumor,
and almost the entire abducens nerve up to the Dorello’s
canal (6) are visible. (j) Tumor removal around the
basilar artery. (k) The dorsal wall of the foramen has
been removed. (Tu tumor, protruding from the jugular
foramen); the small arrowheads indicate the amount of
bone drilling. (l) The intraforaminal tumor part was
then removed. (m, n) The jugular foramen, free from
tumor, prior to the placement of fat pieces to prevent
CSF leak. (o, p) Postoperative CT. The schwannoma
has been removed completely via the retrosigmoid
approach. The hyperdense signal on the postoperative
CT is caused by the fat pieces, placed to prevent CSF
leak through the petrous bone
Jugular Foramen Schwannomas 633

a b

c d

e
634 10 Jugular Foramen Tumors

f g

h i
Jugular Foramen Schwannomas 635

j k

l m
636 10 Jugular Foramen Tumors

n o

p
Jugular Foramen Schwannomas
Histologically, JF schwannomas are similar to
other intracranial schwannomas; variable nerves
of origin or tumor location do not modify their
biological characteristics. Most JF schwannomas
are solid. Approximately 15–24 % may have cys-
tic degeneration or be primarily cystic [23, 58].
Patients with such tumors are usually younger
and present with hearing loss, ataxia, headache,
and minor dysphagia.
Radiologically, cystic schwannomas are sub-
divided into type 1 lesions (single large cysts with
thin ringlike enhancement of the tumor wall) and
type 2 lesions (multiple cysts with very irregular,
thick enhancement of the tumor walls). Such
tumors generally extend into the CP angle and
rarely cause bony enlargement of the JF [23].
Management
The goal of surgery of JF schwannomas is their
complete excision—this can be achieved in
most cases without producing new neurological
deficits. Reoperations are related to higher risks
of injury to the lower cranial nerves second-
ary to scarring. Therefore, exceptions to com-
plete removal should be made only in case of
infiltration of a given cranial nerve or absence of
arachnoid dissection plane.
Type A schwannomas are approached via
a RS craniectomy (see Case Illustrations 10.3,
10.4, and 10.6), while in types B to D tumors,
a single-stage combined cervical and lateral sub-
occipital intra–extradural transmastoid infral-
abyrinthine approach is used (see Fig. 10.7 and
Case Illustrations 10.5 and 10.7). In recent years,
such tumors are removed via an endoscope-
assisted intracranial or transcervical approaches
(see Case Illustration 10.9). In the JF, the lower
cranial nerves and jugular bulb are typically dis-
placed caudally. Infiltration of the cranial nerves
is exceptional, and with meticulous microsur-
gical technique, radical tumor removal can be
achieved with minimal or no morbidity. Despite
their benign histological structure, some schwan-
nomas tend to cause extensive bone destruc-
tion and to adhere intensely to surrounding
structures. In such cases, especially if the tumor is
637
hypervascular, a staged removal via two different
complimentary approaches is justified (see Case
Illustration 10.10). Preoperative embolization of
the arterial feeders in such extensive tumors is of
great help and should be an integral part of the
management.
In our series, all tumors were removed com-
pletely [94] without operative mortality. Transient
worsening of the facial nerve function was seen
in 19 % of patients, and transient vagus and
hypoglossal nerve dysfunction were each seen
in 6 % of patients. All cranial nerve dysfunction
had improved considerably at the last follow-up
examination.
The intraoperative management of cystic JF
schwannomas is somewhat different. If the cyst
is opened and decompressed, it may be difficult
to recognize the plane between the tumor capsule
and the arachnoid membrane. The initial step of
surgery is therefore identification of the interface
between the tumor and the surrounding struc-
tures (see Case Illustration 10.8). The arachnoid
plane in cystic schwannomas, which have thick
tumor capsules, can be recognized by pulling
slightly the tumor capsule with a microdissector.
Of note, the brain stem surface may appear very
fragile after stripping off the tumor, increasing
the risk of postoperative bleeding or neurologi-
cal deficit. Patients with cystic JF schwannomas,
in contrast to those with cystic VS, have a bet-
ter outcome, in terms of neurological function-
ing. We have noted even hearing improvement
in more than half of the operated patients with
preoperative hearing loss.
Patients with JF schwannomas can be treated
with radiosurgery or with stereotactic radiother-
apy—either as a primary treatment modality.
Although VS and JF are histologically identical,
they have different location and originate from dif-
ferent nerves. Therefore, the results of radiosurgery
of VS cannot be generalized to JF schwannomas.
The experience with JF schwannomas is relatively
limited, and the follow-up is not sufficient to make
definite recommendations. Zhang et al. [123,
124] presented their experience with 27 patients
with JF schwannomas treated with gamma knife
radiosurgery. Their mean tumor volume was
13.5 cm3; the mean marginal dose was 14.6 Gy
638 10 Jugular Foramen Tumors

a b

c d

e f

Fig. 10.7 This patient with a JF schwannoma type D transmastoid infralabyrinthine approach. (c) Skin incision,
(a, b: preoperative CT and MRI) was operated by the exposure (d) Complete tumor removal. (e) Reconstruction of
senior author in the late 1980s. The tumor was removed the dura (f). Postoperative CT (g)
completely using the combined cervical and extradural
Jugular Foramen Schwannomas
g
Fig. 10.7 (continued)
and was prescribed to the 35–55 % isodose line.
The patients were followed for a mean of 38.7
months: 44 % decreased in size, 52 % remained
unchanged, and in 4 % tumor growth was observed.
Muthukumar et al. [75] reported on a series of 17
639
patients with JF schwannomas treated with gamma
knife radiosurgery over a period of 10 years.
During a mean follow-up of 3.5 years, the tumors
decreased in 8/17 patients, remained unchanged in
8/17 patients, and increased in one patient. Martin
et al. [68] presented a large series of 34 patients
with 35 tumors that underwent radiosurgery. The
mean duration of follow-up was 83 months, dur-
ing which the tumors regressed in 17 patients,
remained stable in 16, and progressed in two. The
5- and 10-year actuarial control rates were 97 and
94 %, respectively. Although the long-term tumor
control rates were favorable, the study has a more
limited value in regard to neurological outcome, in
particular the risk of new cranial nerve deficits: 22
patients (65 %) had previous microsurgical resec-
tion, and all patients experienced various cranial
neuropathies.
We reserve primary radiosurgery for elderly
medically unstable patients that are unable to
undergo a complex surgery due to their somatic
condition and for patients unwilling to surgical
tumor removal.
640
Case Illustration 10.4
JF Schwannoma Type A (RS Approach and
Opening of the JF)
This 64-year-old patient presented with
severe right-sided hearing loss, facial nerve
palsy (HB grade III), and slight swallow-
ing difficulty. He was operated via the RS
approach. The CP angle tumor part was
first debulked and then dissected from the
brain stem and from the seventh–eighth
(a) CT of the PCF, demonstrating the enlarged JF on the
right side. The foramen is with smooth well-defined
bony margins; the petrous bone is not infiltrated. (b–f)
MRI of the same patient, showing a partially cystic
lesion in the JF with the imaging characteristics of a
schwannoma. (b) Native T1-weighted sequence. (c, d)
T1-weighted sequences after gadolinium administra-
tion. (e, f) T2-weighted sequences. The tumor is located
predominantly in the JF and extends into the posterior
cranial fossa, compressing the cerebellum. (g–r)
Intraoperative images illustrating the consecutive steps
of surgery. (g) Initial view of the tumor (Tu), following
dural incision and slight superolateral retraction of the
cerebellum. (h) The tumor was debulked and gradually
dissected from the brain stem. The seventh–eighth nerve
10 Jugular Foramen Tumors
nerve complex. Afterward, the dorsal wall
of the JF was opened, and the intraforaminal
tumor part was removed under microscopic
and endoscopic visualization. Complete
tumor removal could be achieved, and the
lower cranial nerves were preserved. After
surgery a temporary deterioration of swal-
lowing function was noted. At discharge,
however, the swallowing recovered to the
preoperative level.
complex was found to be compressed and dislocated supe-
riorly. (i) The tumor portion in the JF area (arrow) was
then approached. (j, k) Gradually the lower cranial
nerves were freed (small arrow). (l) In order to gain
sufficient access to the intraforaminal part (arrow), the
dorsal wall of the JF was drilled off with diamond drill
(the drilled area is marked with the two arrows). (m)
Microscopic view of the JF with some remaining tumor
distally. (n) The use of an angled endoscope allowed to
extend the view into the lateral part of the foramen. (o)
The JF was further drilled off under direct endoscopic
control. (p) Endoscope-controlled tumor removal. (q)
Complete tumor removal, verified with the endoscope.
(r) Plugging of the opened JF with multiple fat pieces
and fibrin glue—microscopic view
Jugular Foramen Schwannomas 641

a b

c d

e f
642 10 Jugular Foramen Tumors

g h

i j
Jugular Foramen Schwannomas 643

k l

m n
644 10 Jugular Foramen Tumors

o p

q r
Jugular Foramen Schwannomas
Case Illustration 10.5
JF Schwannoma Type II Combined
Cervical and Extradural Transmastoid
Infralabyrinthine Approach
This 35-year-old male patient presented with
complete hearing loss and abducens nerve and
recurrent nerve palsies on the right side. He
had been operated initially at another institu-
tion: the intracranial part of the tumor (a
schwannoma) has been removed via the RS
approach. The MRI demonstrated a primarily
extracranial tumor remnant with imaging char-
acteristics compatible with a JF schwannoma
(a–c). The lesion was approached via the com-
bined cervical and extradural transmastoid
infralabyrinthine approach. The facial nerve
was exposed and dissected in its mastoid seg-
ment and dislocated slightly anteriorly. Once
a b
Remnant of a schwannoma located extradurally in the area of the JF. The relationship of the tumor to the petrous
carotid artery is well seen on the axial slice (a)
645
the JF was exposed sufficiently and the lower
cranial nerves were seen, the tumor capsule
was coagulated and internal decompression
was performed. The tumor capsule could be
then dissected from the surrounding structures
including the carotid artery, and complete
tumor removal was accomplished. A broad
scar tissue layer, a sequel of the initial intra-
dural surgery, was seen to separate the JF from
the CP angle. This layer was free from tumor
and was not opened in order to prevent CSF
leak or injury to the intracranial structures.
Furthermore, the need of complex reconstruc-
tion could be thus avoided. The patient made
an uneventful recovery and was discharged 2
weeks after surgery. The only new deficit was
slight facial nerve palsy—HB grade 3/6 at dis-
charge—recovered completely at follow-up.
646
Case Illustration 10.6
JF Schwannoma Type A (Pure Intracranial
Location)
This 55-year-old male patient presented with
an 18-month history of low back pain with
irradiation into the right leg. MRI of the lum-
bar spine showed a spinal tumor, most proba-
bly a schwannoma at L2 level. MRI of the
whole neuraxis was also performed to exclude
further lesions, and the CP angle lesion, pre-
sented on the figure, was detected. The lesion
had characteristics of a schwannoma located
in the caudal part of the CP angle without
obvious penetration into the IAC (a–d).
Therefore, the presumptive diagnosis was
schwannoma of the lower cranial nerves,
which was supported by the fact that the
cochlear function was normal. The neurologi-
cal examination was entirely negative.
The tumor was approached via the RS route
(e–l). It was located entirely in the CP angle
(a–d) JF schwannoma type A with pure intracranial loca-
tion. (e–g) Initial view of the tumor in the CP angle. Its
relation to the seventh to eighth cranial nerves and to
the lower cranial nerves (arrowhead) is well seen. (Tu
tumor, P pyramid). The tumor was debulked with the
CUSA (h) and dissected from the seventh to eighth
nerve complex. The facial nerve (FN) and the vestibu-
locochlear nerves (VC) can be well seen (i). The tumor
originated from the proximal cisternal segment of the
10 Jugular Foramen Tumors
and originated from the cisternal segment of
the glossopharyngeal nerve. The seventh–
eighth nerve complex was slightly dislocated
superiorly. Complete tumor removal with
preservation of the integrity of all structures
was achieved. A rare anatomical finding—
duplication of the abducens nerve—was seen.
The incidence of this variant is reported to be
5–28.6 % in cadaveric studies. In this case, the
nerve originated at the pontomedullary sulcus
as two independent trunks. A small arterial
branch of the AICA was found to pass between
the two nerve trunks in the vicinity of the canal
of Dorello. The possibility of this variant has
to be kept in mind when working in the area in
order to avoid nerve interruption/injury.
After surgery the patient had only slight
hearing deficit (PTA of 30 dB). The lumbar
tumor was removed uneventfully 10 days later.
The histopathological finding was schwan-
noma (WHO grade I).
glossopharyngeal nerve (black arrow). A rare anatomical
variant was seen (j, k)—a duplicated abducens nerve,
originating at the pontomedullary sulcus as two inde-
pendent trunks. A small arterial branch of the AICA
was found to pass between the two nerve trunks in the
vicinity of the canal of Dorello (j, white arrow).
*Vertebral artery. (l) Complete tumor removal with
preserved neurovascular structures (arrowheads:
glossopharyngeal nerve)
Jugular Foramen Schwannomas 647

a b

c d

e f
648 10 Jugular Foramen Tumors

g h

i j

k
l
Jugular Foramen Schwannomas
Case Illustration 10.7
Accessory Nerve Schwannoma
This 7-year-old boy presented with a diagnosis
of an accessory nerve schwannoma. He had
been operated twice 2 years ago elsewhere, and
later on a VP shunt has been implanted. The
tumor had been removed partially, but the rem-
nant showed a steady growth tendency (a–d).
At presentation the patient had slightly ataxic
gait, incomplete binasal hemianopsia, facial
nerve paresis (grade 4/6 HB), complete hearing
loss on the left side, and incomplete left-sided
accessory and hypoglossal nerve paresis.
649
The tumor could be removed completely
via the combined cervical and lateral suboc-
cipital intra–extradural transmastoid infral-
abyrinthine approach. The facial nerve was
so strongly adherent to the tumor that it had
to be resected and reconstructed with sural
nerve interposition graft. Besides that, the
patient did not have any new neurological
deficits. The postoperative MRI is demon-
strated on figures e and f: note the fat placed
in the resection cavity in order to prevent
CSF leak.
650 10 Jugular Foramen Tumors

a b

c d

(a–d) Preoperative contrast-enhanced T1-weighted
MRI of the patient. The MR venography (d) showed an
occlusion of the sigmoid sinus distal to its knee. (e, f)
Postoperative T1-weighted MRI demonstrating the
complete tumor removal. The high intensity signal in
the area of the pyramid corresponds to the fat tissue
used for the reconstruction and for preventing CSF
leak. The widened ventricular system had no clinical
significance and at follow-up proved to be stable
Jugular Foramen Schwannomas 651

e f
652
Case Illustration 10.8
Cystic JF Schwannoma
This 49-year-old male patient has been oper-
ated elsewhere 2 years ago for a tumor in the
jugular foramen area (histopathology: WHO
grade II ependymoma). The tumor was
removed partially, and radiotherapy was per-
formed. Due to radiological evidence of tumor
growth, he underwent subsequently radiosur-
gery. Nevertheless, the last follow-up MRI
showed further tumor growth, which corre-
lated to a progressive neurological deteriora-
tion. At presentation to our institute, he had
hearing loss (30 dB in the main speech area),
swallowing difficulty, severe gait ataxia, and a
slight right-sided central hemiparesis. MR
examination showed a cystic tumor exhibiting
a ring-enhancement pattern after contrast
administration (a–e). The tumor was located
medially at the brain stem in close proximity
to the lower cranial nerves entry/exit zones.
All potential risks, related to the previous
surgery and radiation therapy, were thoroughly
discussed with the patient and his family.
Considering the biological activity of the
tumor, its steady growth tendency, and the
corresponding neurological deterioration, a
decision for an operative tumor removal was
taken. The standard RS approach with addi-
tional opening of the foramen magnum was
selected (f–m).
10 Jugular Foramen Tumors
The tumor was partially cystic and located
medial to the lower cranial nerves. It was
accessed between the fibers of the individual
nerves and through the level below the
seventh–eighth nerve complex and the lower
cranial nerves. First, it was decompressed
internally, and then its capsule, which was
very tightly adherent to adjacent neural struc-
tures, was carefully dissected. As pointed ear-
lier, radiotherapy renders the cranial vessels
extremely vulnerable. Utmost care was taken
to preserve both the major arteries and the
small but vital perforators. The tumor part
attached to the brain stem could be also
removed without violating the pial layer.
Complete tumor removal was achieved, and
all neurovascular structures were preserved.
After surgery the dysphagia worsened, and
the patient had a protracted postoperative period
in the intensive care unit. A tracheotomy and
later on a percutaneous endoscopic gastros-
tomy were placed. The patient suffered severe
pulmonary embolism, as well as infectious
complications, and received appropriate ther-
apy. Gradually his condition began to improve,
and he could be transferred to a normal ward.
Swallowing improved, and the tracheostoma
could be closed. At discharge he could walk
and care of himself independently. Oral feed-
ing, however, was still impossible. The follow-
up period of this patient is less than 1 month.
Jugular Foramen Schwannomas
a b
c
(a–e) Preoperative MRI of the patient, demonstrat-
ing an intracranially located cystic lower cranial
nerve schwannoma. (a–c) T1-weighted sequences
after gadolinium administration. (d, e) T2-weigted
sequences. (f–m) Intraoperative images illustrating
the steps of tumor removal. (f) Initial view of the
tumor, located medially to the lower cranial nerves
Tu tumor; LCN lower cranial nerves; 11 abducens
nerve. The C1 nerve root is hidden by the tumor.
653
d
(g) The caudally located cystic portion was opened,
and the C1 root could be seen. (h, i) Internal decom-
pression of the tumor between the fibers of the lower
cranial nerves and above the C1 root. (j, k) The
tumor capsule was adherent to the vertebral artery
but could be dissected, and all perforators were pre-
served (l). Complete tumor removal with anatomical
preservation of all neurovascular structures in the
area (m)
654 10 Jugular Foramen Tumors

f g

h i
Jugular Foramen Schwannomas 655

j k

l
m
656
Case Illustration 10.9
JF Schwannoma Removed via a Retrosigmoid
Endoscope-Assisted Approach
Thirty-two-year-old female patient with right-
sided hearing loss and facial nerve palsy (HB
grade IV), swallowing difficulty, and gait insta-
bility presented with radiological evidence of a
large jugular foramen tumor. MRI demonstrated
a very large tumor in the area of the jugular
foramen with a major intradural part and with
significant extracranial extension (a–g). The
CT and bone-window CT (a–c) demonstrated
dilated jugular foramen and extensive erosion of
the adjacent petrous bone. The signal character-
istics of the tumor, the intense contrast uptake,
and the smooth bony margins were compatible
with those of schwannoma.
Considering the degree of intracranial tumor
growth and the compression of brain stem, the
RS approach was selected. Initially, the dura
dorsal to the jugular foramen was excised,
and the roof of the foramen was drilled off,
thereby exposing the intraforaminal tumor
part. Internal decompression and dissection of
the tumor capsule were performed intermit-
tently in all directions. The seventh–eighth
10 Jugular Foramen Tumors
cranial nerves could be dissected from the
tumor and preserved anatomically. Then, the
tumor capsule was dissected from the brain
stem, from the basilar, and the right vertebral
arteries. The intraforaminal tumor part could
be well demonstrated with the angled endo-
scope and removed under pure endoscopic
control with appropriate angled instruments.
Due to the wide dilatation of the jugular
foramen, the endoscope allowed not only to
visualize but also to remove the extracranial
tumor part completely. The tumor cavity was
initially coated with Gelfoams and then filled
with multiple fat pieces, which were sealed
with fibrin glue. The postoperative period
was unremarkable. The swallowing difficulty
persisted, and hoarseness appeared. A slight
tendency toward improvement of facial nerve
function was noted before discharge. At 1-year
follow-up examination, she had an HB grade
III facial nerve function; the gait stability
recovered completely. She could eat normally
but reported to have occasional swallowing
problems, especially with hard food. MRI
study was unremarkable and did not demon-
strate tumor residual/recurrence.
Jugular Foramen Schwannomas 657

a b

c d

(a–g) Preoperative CT and MRI of the patient: a large
jugular foramen tumor with intra- and extracranial exten-
sion is visible. Substantial erosion of the petrous bone
with smooth well-defined bony margins, and no evidence
of bone infiltration can be seen on the bone-window CT
images. The signal characteristics of the tumor and the
pattern of bone erosion are highly suggestive of schwan-
noma. (a–c) CT, including bone-window mode. (d, e)
T1-weighted MR image after contrast application. (f, g)
T2-weighted MR images. (h–n) Early postoperative MRI
performed 5 days after surgery. The heterogeneous signal
on the various sequences is caused by the Gelfoam, fat
tissue, and the typical radiological artifacts, appearing
early after surgery. (h, i) CT scan. (j–l) T1-weighted MR
sequence after gadolinium application. (m, n)
T2-weighted sequences. The ring enhancement does not
reflect tumor remnant, as evident when the corresponding
T2-weighted signal characteristics are evaluated
658 10 Jugular Foramen Tumors

e f

g h

i j
Jugular Foramen Schwannomas 659

k l

m n
660
Case Illustration 10.10
Giant JF Schwannoma Removed in Two
Steps
This 15-year-old male patient presented with a
history of hearing loss on the right side for 3
years and occasional swallowing difficulty.
Neurological examination revealed right-sided
hypacusis (20 dB in the main speech area), pare-
sis of the right hypoglossal nerve, and a slight
deviation of the soft palate to the left side with a
weak gag reflex. CT and MRI studies demon-
strated a huge skull-base lesion, extending from
the jugular foramen to the sphenoid sinus (5.3 ×
4.6 × 5.2 cm in diameter) with signal character-
istics of schwannoma (a–h). The tumor caused
extensive destruction of the skull base and had
large intradural and extracranial portions.
Considering the size and the extension of
the tumor, as well as the relatively good neuro-
logical condition of the patient, a decision was
taken for a staged tumor removal via two dif-
ferent approaches. At the first stage, the tumor
was approached via the transmastoid presig-
moid approach (i, j). A retroauricular retro-
mastoid skin incision was done and extended
caudally; the stylomastoid foramen and the
facial nerve were identified; a mastoidectomy
was performed, the proximal facial nerve seg-
ment was identified, and the sigmoid sinus was
exposed up to the jugular bulb in the jugular
foramen. Thus, sufficient portion of the tumor
was exposed to allow its safe removal. The
tumor turned out to be extremely and strongly
10 Jugular Foramen Tumors
vascularized. Still, it could be decompressed
internally, and its lower part could be removed.
The strong bleeding from the tumor prohibited
more radical surgery.
Prior to the second surgery, which was
done 2 weeks after the first one via the ret-
rosigmoid approach, superselective catheter-
ization and embolization of the tumor feeders
were performed (k–n). Despite the emboliza-
tion, the lesion was still strongly vascularized.
Following thorough internal debulking, the
intradural part could be dissected from the
brain stem and cranial nerves and removed
completely. Then, the exposure of the petrous
tumor part was enhanced by additional bone
removal around the IAC and by resection of
the petrous apex. Complete tumor removal
was achieved using microscopic and endo-
scopic technique, and all cranial nerves could
be preserved anatomically. The nerve of ori-
gin of this tumor could not be identified with
certainty. The exposed bone of the pyramid
was covered with fat pieces, fixed with fibrin
glue, in order to avoid CSF leak, while the
huge cavity was filled with multiple sponges
(Gelita-s) and Tabotamp (o, p).
Histopathological examination proved that
the tumor was a WHO grade I schwannoma.
After surgery the lower cranial nerve dysfunc-
tion was more pronounced but showed gradual
improvement, and within 1 week peroral feed-
ing was initiated. The hearing on the right side
was completely lost.
Jugular Foramen Schwannomas 661

a b

c d

(a–h) Giant skull-base tumor, extending in the posterior
and middle cranial fossae, with extensive bone destruc-
tion, as visible on the CT images (a, b). (c) T1-weighted
native image. (d, e) Axial T1-weighted images after
contrast application. (f) Axial T2-weighted image. (g)
Coronal and sagittal. (h) T1-weighted images after
contrast application. Note the severe brain stem and
temporal lobe compression. (i, j) Bone-window CT
demonstrating the amount of petrous bone resection
and the approach to the tumor. (k–n) Digital subtraction
angiography demonstrating the hypervascular tumor
and the feeders, coming from the ascending pharyngeal,
medial meningeal, and accessory meningeal arteries
(k, l). The petrous segment of the ICA was narrowed and
dislocated. The occlusion test showed sufficient blood
supply from the contralateral anterior and posterior com-
municating arteries. (m, n) Only partial embolization of
the feeding arteries was possible. (o, p) CT scans, per-
formed 1 day after the last surgery. The tumor cavity is
filled with multiple sponges (Gelita-s) and Tabotamp
662 10 Jugular Foramen Tumors

e f

g h

i j
Jugular Foramen Schwannomas 663

k l

m n

o p
664
Hypoglossal Nerve Schwannomas
In 1933, De Martel et al. [111] first reported a
hypoglossal nerve schwannoma. These tumors
can be completely intracranial, intracranial–
extracranial, or completely extracranial [112].
Symptoms differ depending on the location
of the tumor. Tongue wasting is present in the
majority of patients at the time of presentation.
Suboccipital headaches, exacerbated by neck
movements, are early symptoms of intracranial
tumors [11]. Patients with intracranial tumors
may also exhibit jugular foramen syndrome or
symptoms of brain stem compression. Patients
with extracranial tumors may present with a
mass in the upper neck or parapharyngeal area, in
addition to wasting of the tongue [112]. Surgical
strategies depend on tumor location. Preoperative
angiography is important to establish the position
and dominance of the vertebral arteries and the
anatomic features of the venous sinuses. In the
immediate postoperative period, the possibility
of aspiration attributable to paresis of CNs IX
and X must be considered [117].
Glomus Jugulare Tumors
Glomus jugulare tumors have long fascinated
surgeons and are still regarded as one of the most
challenging skull-base lesions. The advancement
in microsurgical techniques and intensive care
management; the improvements in neuroradiol-
ogy, in particular the use of selective preoperative
embolization; and the team approach allowed to
reduce significantly the operative morbidity and
to achieve good long-term outcome.
General Features
The first descriptions of glomus tissue are credited
to Valentin in 1840 and Krause in 1878 [12]. The
carotid body-like structures in the temporal bone
were described in 1941 by Guild who named them
“glomus jugulare bodies” [42]. Three years later—
in 1945—Rosenwasser published the first descrip-
tion of a middle ear paraganglioma and found a
10 Jugular Foramen Tumors
correlation between these tumors and the para-
ganglion tissue described by Guild [90]. In 1962,
Alford and Guilford classified these lesions as glo-
mus tympanicum and glomus jugulare tumors [3].
Glomus tumors, which are also called paragan-
gliomas, chemodectomas, and nonchromaffin
tumors, are relatively rare: they represent 0.012 %
of all tumors diagnosed in humans [60]. Four typ-
ical primary locations are known in the head and
neck region: middle ear cavity (glomus tympani-
cum), dome of the jugular bulb (glomus jugulare),
ganglion nodosum of the vagus nerve (glomus
vagale), and the carotid body near the carotid
bifurcation (carotid body tumor). Glomus tumors
are the second most common temporal bone tumor
after VS. Glomus jugulare tumors comprise
60–80 % of all primary JF tumors [10, 71].
Females are affected more frequently than male
persons [3, 110] with peak age of presentation
being the fourth–fifth decade. It has been esti-
mated that up to 20 % of glomus tumors are famil-
ial. Familial tumors are multicentric or bilateral in
approximately 25–78 % of the cases [119].
The glomus jugulare tumors are histologically
benign lesions, which rarely grow rapidly and are
usually encapsulated. Still, if untreated, they are
related to high morbidity and mortality rates.
Pathogenesis and Classification
Glomus jugulare tumors arise from paraganglia
cells situated near the jugular foramen. The para-
ganglion tissue is regarded as part of the extra-
adrenal neuroendocrine system. In the temporal
bone, the vast majority of paraganglia are found
within the adventitia of the dome of jugular bulb
(glomus jugulare) and over the promontory in the
middle ear (glomus tympanicum). They can be also
found along the course of the glossopharyngeal
nerve, the Jacobson nerve (the tympanic branch of
the glossopharyngeal nerve), or the Arnold nerve
(the auricular branch of the vagus nerve) [18, 43].
Histologically, paragangliomas in all locations are
similar.
Familial glomus tumors show an autosomal
dominant inheritance pattern with maternal
imprinting effect: the trait is transmitted from the
Glomus Jugulare Tumors
father to his offspring. Two putative regions on
chromosome 11—PGL1 at 11q23.1 and PGL2 at
11q13.1—contain the candidate genes [47]. It
has been shown that both of these loci are associ-
ated with maternal imprinting [46]. Genetic
screening of high-risk individuals in affected
families allows early detection and management
of multifocal or bilateral disease [101].
Glomus jugulare tumors arise in the lateral
skull base and expand along the paths of least
resistance. Although histologically benign, they
are locally invasive and may invade adjacent bone,
blood vessels, dura mater, and cranial nerves.
They may cause destruction of the pyramid,
including the labyrinth into the hypotympanum,
mesotympanum and sinus tympani, the juguloca-
rotid spine, and the hypoglossal canal [87]. These
tumors may grow intracranially and penetrate the
dura of the posterior cranial fossa or extend along
the cranial nerves around the jugular bulb, infe-
rior petrosal sinus, and carotid artery. More rarely,
they penetrate the dura of the middle cranial fossa
[55, 99]. Extracranial extension into and/or along
the jugular vein may cause obstruction of the
venous drainage or produce a pharyngeal or neck
mass.
This intimate association of glomus jugulare
tumors to the lower cranial nerves and inner ear
structures, the involvement of major vessels, the
hypervascularity of the tumor, as well as its pro-
pensity for intracranial extension, are the main
factors complicating operative removal [29, 47].
The currently most widely applied classifi-
cation schemes reflect this complex extension
patterns (Tables 10.2 and 10.3).
Neuroimaging
The bone-window CT (Fig. 10.8a) shows skull-
base infiltration and destruction that cause an
irregular “moth-eaten”-like erosion pattern and
enlargement of the JF [63, 79, 100]. On
T1-weighted MR images, a typical salt-and-pep-
per appearance is seen with hyperintense foci,
which represents serpentine flow void pattern or
hemorrhagic foci (Fig. 10.8b–d) [63, 78, 85]. On
T2-weighted sequences, the tumor is hyperin-
tense and shows flow voids within the mass, cor-
responding to regions of high blood flow within
665
Table. 10.2 Fisch classification of glomus tumors of the
temporal region
Class Description
A Tumors limited to middle ear space
B Tumors limited to middle ear space or mastoid
w/o involvement of the infralabyrinthine space
of the temporal bone
C Tumors involving the infralabyrinthine and
apical spaces, w/ extension into the apex
D1 Tumors w/ intracranial extension < 2 cm
D2 Tumors w/ intracranial extension > 2 cm
Table 10.3 Glasscock–Jackson classification of glomus
jugulare tumors [54]
Grade Description
I Small tumor involving jugular bulb,
middle ear, and mastoid
II Tumor extending under internal
auditory canal; may have intracranial
extension
III Tumor extending into petrous apex;
may have intracranial extension
IV Tumor extending beyond petrous
bone into clivus or infratemporal
fossa; may have intracranial extension
the tumor (Fig. 10.8e). The tumor enhances
intensely after gadolinium administration and
lacks dural tail sign.
Glomus jugulare tumors are predominantly
supplied by the external carotid artery system,
mainly by the ascending pharyngeal artery, fol-
lowed by the posterior auricular, stylomastoid,
and occipital branches. Less frequently, it has
feeders from the internal carotid artery (caroti-
cotympanic branches of intrapetrous carotid) or
the vertebrobasilary system (posteroinferior cere-
bellar artery) [85]. The conventional angiographic
appearance is of a hypervascular mass with an
intense characteristic tumor “blush,” which is
coarser and less pronounced than in meningiomas.
Early draining veins, representing early arterio-
venous shunting, are seen. Angiography demon-
strates the relationship of the tumor to the carotid
artery, its vascularity, and feeding vessels. The
carotid artery may be encased, narrowed, or even
occluded. The venous phase study provides infor-
mation regarding the patency of the sigmoid sinus,
jugular bulb, and jugular vein, as well as on the
666 10 Jugular Foramen Tumors

size and dominance of the contralateral trans- Clinical Presentation

verse–sigmoid sinuses.
MR angiography and MR venography that
have been implemented are less invasive tools to
study the vascular supply of the tumor and the
jugular vein intraluminal patency [79]. In one
study, 3D time-of-flight MR angiography showed
a sensitivity and specificity of 90 and 92 %,
respectively, when compared with the digital
substraction angiography [118].
The clinical presentation of glomus jugulare
tumors is related to their compressive effect, local
invasiveness, and high vascularity. Typical signs
and symptoms are hearing loss, pulsatile tinnitus,
and lower cranial nerve palsies. The most com-
mon presenting sign is unilateral conductive hear-
ing loss, typically caused by invasion of the middle
ear, followed by pulsatile tinnitus secondary to the

a b

c d

Fig. 10.8 Glomus jugulare tumor. CT images. Native T1-weighted contrast-enhanced images. (i) T2-weighted
(a). After contrast administration (b). Bone-window scan image. (j, k) MR angiography
(c). MR imaging. (d, e) T1-weighted images. (f–h)
Glomus Jugulare Tumors 667

e f

g h

i j k

Fig. 10.8 (continued)

668
tumor’s rich vascularity. Lower cranial nerve dys-
function is seen in patients with large tumors.
Notably, slowly developing palsies can be well
compensated by the patients. Other symptoms are
determined by the tumor growth pattern and the
direction of extension. The facial nerve is affected
most commonly (in up to 33 %), followed by the
vagus, glossopharyngeal, hypoglossal, and spinal
accessory nerves [66]. Compression of surround-
ing neural structures may cause nystagmus, ataxia,
gait instability, and—in more advanced cases—
hemiparesis or symptoms of increased intracra-
nial pressure. Large tumors with anterior extension
may encase or even invade the walls of the carotid
artery, causing Horner’s syndrome.
Glomus tumors are known to produce vasoac-
tive substances. Clinically significant hormonal
secretion occurs in 1–4 % of the patients [18, 52].
These could lead to potentially serious complica-
tions, such as severe hypertension crisis. If the
patient presents with signs suggestive of a hor-
monally active tumor, such as labile hyperten-
sion, tachycardia, and facial flushing, a thorough
evaluation is mandatory. In case the level of
secretion of vasoactive substances is too low, it
may not be recognized clinically. Therefore,
some authors propose screening for excess cate-
cholamine secretion in all patients with glomus
jugulare tumors [2]. Twenty-four-hour urine
sample and the plasma should be analyzed for
catecholamines, metanephrines, and normeta-
nephrines. Patients in whom the catecholamine
level is four times higher than normal require pre-
operative prophylaxis, usually with an alpha and
beta catecholamine blocker. Adrenal imaging
should be performed in patients with hyperse-
creting tumors in order to exclude coexisting
adrenal norepinephrine-secreting tumors.
Management
General Management Principles
The optimal treatment of glomus jugulare tumors
is still a matter of debate. The options are microsur-
gical resection, tumor embolization, radiosurgery/
stereotactic radiotherapy, conventional fractionated
external beam radiotherapy, or a combination of
10 Jugular Foramen Tumors
these modalities. Initial observation or “watchful
waiting” may be another option. The known pro-
pensity of glomus tumors to grow and cause symp-
toms, however, restricts its application to some
selected cases, for example, moribund asymptom-
atic patients. A more proactive approach is gener-
ally favored, and surgery remains the mainstay of
treatment [2, 28, 85, 100].
The current multimodal management of glo-
mus jugulare tumors is aimed at improving local
tumor control and minimizing the resultant mor-
bidity. It includes preoperative embolization of
feeding arteries and judicious microsurgical tumor
resection with attempted preservation of neuro-
logical functions. In most patients with confined
glomus jugulare tumors, the goal of complete
tumor resection with preservation of function is
feasible. In case of infiltration of the cranial nerves
or brain stem, subtotal tumor resection is per-
formed. Similarly, in case the carotid artery is
infiltrated, subtotal resection is preferred to carotid
resection and reconstruction. Tumor remnants
that have a propensity to grow at follow-up are
subjected to radiosurgery/radiotherapy.
Patients with more extensive glomus jugulare
tumors pose specific challenges. In such cases,
the neurological condition of the patient at pre-
sentation is of paramount importance for the
decision-making process. The attempt of radical
surgery is inevitably related to a very high risk of
injury to the lower cranial nerves. Surgery is
therefore recommended only in patients present-
ing with compromised lower cranial nerve func-
tion. Due to the phenomenon of brain plasticity,
during the gradual loss of ipsilateral function, the
contralateral nerves take over the control of swal-
lowing. Such patients are not so endangered from
aspiration and the related complications. In con-
trast, an acute lower cranial nerve dysfunction,
for example, occurring at surgery, can be gener-
ally poorly compensated for. Surgery in patients
with normal swallowing function should be per-
formed only in case of significant brain stem
compression, and its goal should be merely
decompression of neural structures. We believe
that the treatment of choice of such patients is
embolization, which generally ensures tumor
growth control or even leads to tumor shrinkage.
Glomus Jugulare Tumors
If the tumor demonstrates further growth later on,
the procedure may be repeated. Glomus jugulare
tumors that cannot be controlled with this method
should be treated with radiosurgery or radiother-
apy, depending on tumor size and extension.
Radiation therapy causes slow worsening of
lower cranial nerve function over many month or
years that enables the development of compensa-
tory mechanisms.
Surgery of Glomus Jugulare Tumors
The first exploration of the jugular bulb was
reported by Seiffert in 1934. Early attempts for a
systematic surgical approach to glomus jugulare
tumors were made in 1941–1945 by Guild and
Rosenwasser [91]. In 1951, Semmes performed
the first successful neurosurgical removal of a
glomus jugulare tumor via a suboccipital approach
and reported it in 1953 [102]. Capps reported on
five cases of successfully operated tumors and
described some important technical advances in
1952 [21]. Due to the high morbidity/mortality
rate of these early surgical attempts, alternative
management options have been developed in the
1950s. Williams et al. [122] reported on the Mayo
Clinic experience with radiotherapy of glomus
tumors in 1955, and this method became tempo-
rarily the mainstay of treatment. In the same
period, neuroradiology made considerable prog-
ress, mainly due to the work of Valvassori. In
1960, Meacham and Capps [70] and Thoms et al.
[115] described their experience with removing
glomus jugulare tumors via a suboccipital route,
which actually revived the surgical management
of these tumors. They used the suboccipital
approach and found that tumor extension deter-
mines the radicality of surgery. Since then, multi-
ple surgical approaches to the JF have been
developed and refined with the goal of more com-
plete and safe tumor resection [2, 14, 36, 62, 100].
In 1964, Shapiro and Neues [104] reported on the
surgical resection of a recurrent glomus jugulare
tumor with minimal blood loss and good neuro-
logical outcome. Complete tumor resection was
possible by removing the jugular bulb and trans-
location of the facial nerve. One year later, Gejrot
669
presented a series of four patients operated with a
similar technique [35]. In 1966, Denecke reported
on the further improvement of the surgical tech-
nique, allowing its application in more extensive
lesions [25]. In 1968, House introduced the com-
bined neck and simple mastoidectomy technique
[51], while Portmann advocated the team approach
(ENT and neurosurgeon) and surgery in two
stages [61]. Kempe et al. described the successful
use of suboccipital craniectomy and standard
mastoidectomy in tumors involving both the tem-
poral bone and the posterior fossa [59]. Similar
technique with additional exposure of the internal
carotid artery via the glenoid fossa was reported
by Hilding and Greenberg [49]. Glasscock et al.
[39], in 1974, described an approach which com-
bined the wide exposure of the skull base of
Shapiro and Neues and the use of the extended
facial recess, described by House. Gardner et al.
[34] presented their concept of multidisciplinary
cooperation in 1977. Their technique consisted of
three steps: exposure of the skull base through the
neck, bone removal within the temporal bone and
jugular fossa, and tumor removal/reconstruction
of the wound.
In the late 1970s and early 1980s, new
approaches have been described by Graham,
Gardner, Fish, Menzel, Draf, and the senior author
[93, 95]. In 1978, U. Fisch described an approach
that allowed accessing and controlling the inter-
nal carotid artery within the temporal bone—the
infratemporal approach [29]. Thus, even larger
glomus jugulare tumors could be safely resected.
The classic infratemporal fossa type A approach
[30] affords an excellent exposure of the jugular
bulb area. It consists of a cervical approach with
control of the carotid artery and jugular vein, a
mastoidectomy with skeletonization of the jugular
bulb, closure of the external auditory canal, and
anterior rerouting of the facial nerve. Later on—in
1987—Al Mefty et al. [1] described a combined
infratemporal and posterior fossa approach for
the removal of giant glomus jugulare tumors that
have a large intracranial component.
The anterior transposition of the facial nerve,
as described by Capps [22] and refined later by
670
several authors [16, 30, 54], provides wide expo-
sure and early control of the internal carotid
artery; however, the cost is temporary or even per-
manent facial weakness. A major issue related to
these extended lateral approaches is the safe man-
agement of the facial nerve and of the lower cra-
nial nerves during the surgical procedure without
compromising the exposure of the surgical mass.
Glomus jugulare tumors with intracranial exten-
sion have been resected via the MFA, subtempo-
ral–infratemporal transmastoid, retrolabyrinthine,
translabyrinthine, transcochlear, transcondylar,
juxtacondylar, or combined approaches [14, 28,
55, 80, 85, 100]. The reported rate of gross total
tumor removal in large clinical series is 77–92 %,
depending on the tumor’ stage. The main source
of morbidity after glomus jugulare surgery is
lower cranial nerve dysfunction [2].
The ideal operative technique should allow for
control of the carotid artery, coagulation or liga-
tion of the afferent vessels, proximal control of
the sigmoid sinus and internal jugular vein,
identification and preservation of the lower cra-
nial nerves, and possibility for reliable dural clo-
sure and reconstruction of the operative area
[48]—Fig. 10.9. On the other hand, of paramount
importance is the avoidance of significant
approach-related morbidity. With increasing
experience, it became obvious that the achieve-
ment of best outcome requires individually tar-
geted approach selection.
In complex glomus jugulare tumors with cra-
nial and cervical extension, we favor the combined
cervical and lateral suboccipital intra–extradural
transmastoid infralabyrinthine approach, as descri-
bed earlier (Figs. 10.10 and 10.12).
A major recent advancement was the intro-
duction of the endoscope as an adjuvant visual-
ization tool. With the endoscope-assisted
technique or with pure endoscopy, the tumor por-
tion in the jugular foramen can be removed under
direct visual control. Thus, in tumors with lim-
ited intracranial extension and no adhesion to
neural/vascular structures, the need of additional
craniotomy can be avoided. In tumors with pre-
dominant intracranial extension, the retrosigmoid
10 Jugular Foramen Tumors
suboccipital approach may be sufficient: the
intraforaminal tumor part can be removed by
drilling the wall of the jugular foramen or by
applying the angled endoscope. In case of tumors
with predominant extracranial extension, the cer-
vical approach is usually sufficient. The naviga-
tional guidance may be of great help, especially
if the tumor has destroyed the normal anatomical
landmarks (Fig. 10.11).
Embolization
Parallel to the advancement of surgery, major
progress has occurred in the endovascular embo-
lization techniques of glomus jugulare tumors,
promoted mainly by Hilal, Michelsen, and
Lajaunas. Hilal and Michelsen [48] and Brismar
and Cronqvist [17] first described the preopera-
tive superselective embolization of major feeding
vessels of glomus jugulare tumors in separate
publications. In 1979, Simpson et al. [107]
reported the use of preoperative embolization in
glomus jugulare tumors in an effort to reduce
intraoperative blood loss. Since then, its efficiency
in reducing intraoperative bleeding, facilitating
complete surgical resection, and shortening oper-
ative time has been well established (see Case
Illustration 10.11) [84]. Embolization is an essen-
tial component of the current management of
large and complex glomus jugulare tumors (see
Case Illustration 10.11). The recommended delay
between embolization and surgery should be at
least 2–3 days to allow embolization-related
edema to subside but no longer than 2–4 weeks
because of the gradual recanalization of feeding
vessels. Of note, some adverse effects of embo-
lization on the complexity of surgery have been
reported: the vessels might be more susceptible
to injury, and the tumor might be hardened and
therefore more difficult to remove.
Radiotherapy/Radiosurgery
Glomus jugulare tumors have been increasingly
frequently treated in the last decades with frac-
tionated radiotherapy, radiosurgery, or stereotac-
tic radiotherapy. These treatment modes have
been applied as primary management, as an
Glomus Jugulare Tumors
a b
c
Fig. 10.9 Giant glomus jugulare tumor, operated by the
senior author in 1980s. The CT scan (a) shows the extent
of petrous bone erosion. (b, c) Preoperative angiography.
The tumor was visible at pharyngoscopy (d) and otoscopy
(e). At that time, embolization of the tumor was not fea-
sible, what necessitated wide neck dissection to access the
671
d
tumor feeders and to have proximal control of the carotid
artery (g, h). (i) Complete tumor removal with preserva-
tion of the carotid artery. (j) Skin closure. (k) Postoperative
angiography—intact carotid artery. (l) The patient has
been followed up for almost three decades and had no
signs of recurrence
672 10 Jugular Foramen Tumors

e h

Fig. 10.9 (continued)

Glomus Jugulare Tumors 673

adjunct to surgery, or as a salvage therapy in case

k
of recurrences [121]. The goal of radiotherapy/
radiosurgery is obtaining tumor growth control
and providing symptom relief. Glomus tumors
are regarded as radioresistant, and the radiation
is supposed to control tumor growth by
influencing the highly developed vasculature of
the tumors.
Multiple clinical studies on glomus tumors
treated primarily with radiosurgery described
high rates of tumor control with only rare cases
of tumor progression [15, 33, 72]. Stereotactic
radiation for glomus jugulare tumors is generally
well tolerated. In contrast to VS and menin-
giomas, in case of glomus tumors, a symptomatic
improvement, especially of tinnitus and otalgia,
l can be achieved [28]. The reported tumor control
rates vary between 71 and 100 %, and the symp-
tom control rate is 80–100 %.
No study reported, however, long-term out-
come results—the mean follow-up period was
up to 60 months [32, 72, 83, 106]. Recurrences
of glomus jugulare tumors can arise up to 25
years after the initial treatment. Radiotherapy,
as stated previously in the text, aims at achiev-
ing tumor growth control. It does not affect the
“chief” cells, which keep the tumor alive [45].
Further drawback is that the catecholamine
secretion is not affected by the application of
radiation. Therefore, we consider radiosurgery
as a primary management option in patients
with advanced age and with contraindication
for other therapy.
Radiation therapy may be an adjunctive
option in the rare aggressive glomus jugulare
tumors. Its role in patients with extensive tumors
and normal lower cranial nerve function has
been described earlier in this chapter. As salvage
therapy, radiosurgery/radiotherapy is an accept-
able option in case surgery of the recurrence is
deemed too risky.
Fig. 10.9 (continued)
674 10 Jugular Foramen Tumors

a b

Fig. 10.10 Glomus jugulare tumor (a, b), removed com- (c) Cervical exposure. (d) Cranial part of the approach. (e)
pletely via the combined cervical and lateral suboccipital The tumor has been removed. (f) Tumor specimen. (g)
intra–extradural transmastoid infralabyrinthine approach. Postoperative audiogram
Glomus Jugulare Tumors 675

c d

e f

Fig. 10.10 (continued)

676 10 Jugular Foramen Tumors

a b

Fig. 10.11 Neuronavigation screenshots made during the resection of a glomus jugulare tumor. The position of the
pointer tip demonstrates the extent of tumor removal
Glomus Jugulare Tumors 677

a b

c d

Fig. 10.12 Large glomus jugulare tumor, operated by the
senior author in 1992 (a) CT scan. (b) MR image after con-
trast administration. Initially, tumor embolization was per-
formed (angiography before (c) and after the embolization
(d)), and then the combined cervical and lateral suboccipi-
tal intra–extradural transmastoid infralabyrinthine approach
was used to expose and remove the tumor completely. (e)
Skin incision along the anterior border of the sternocleido-
mastoid muscle and posterior to the ear. (f) Exposure of the
mastoid, neck dissection, and exposure of the cervical ves-
sels and nerves. (g) Retrosigmoid craniectomy and mas-
toidectomy have been performed, the sigmoid sinus are
well visible. (h) The tumor is exposed intradurally. (i)
Resection of the jugular vein with the tumor. (j) Complete
tumor removal. (k) Watertight reconstruction of the dura.
Postoperative imaging: (l–o) CT scans. (p) MR image
678 10 Jugular Foramen Tumors

e f

g h

i j

Fig. 10.12 (continued)

Glomus Jugulare Tumors 679

k n

m
p

Fig. 10.12 (continued)

680
Case Illustration 10.11
Glomus Jugulare Tumor (Embolization
and Surgery)
This 42-year-old male patient presented with
a history of pulsatile tinnitus in the left ear
since 20 months and retroauricular pain since
3–4 months. One month earlier, spontaneous
subcutaneous emphysema behind his left ear
appeared. Neurological examination revealed
slight ataxia and decreased hearing on the left
side—more than 60-dB loss in the main speech
area. CT and MR examinations demonstrated
a tumor with irregular shape in the area of the
left jugular foramen and bone destruction of
the adjacent petrous bone (a–j). The epidural
emphysema was clearly visible. The tumor
showed strong and homogeneous contrast
uptake after administration of gadolinium (a,
b, e, f). Interestingly, neither the MRI nor the
angiographic characteristics (j) of the tumor
did correspond to that of a typical glomus
tumor.
Superselective embolization of tumor feed-
ers was performed (k–p), and 1 week later, the
tumor was approached via a transmastoid
approach with the goal of obtaining a tissue
sample and—respectively—of a definitive
histopathological diagnosis (q). The lesion
proved to be a glomus jugulare tumor.
(a–j) Preoperative CT and MR images showing the
jugular foramen tumor. The main tumor part is extra-
dural, but an intradural part and an extracranial
parapharyngeal extension are visible. Note the tumor
relation to the carotid artery (g–j). (a, b) Bone-window
CT demonstrating the extensive petrous bone destruc-
tion and the air collection. (c–i) T1-weighted sequences
after gadolinium administration. (j) MR angiography.
(k–p) Preoperative DSA demonstrating the highly
vascular tumor and its feeders. Main vascular supply
comes from the meningeal branch of the ascending
pharyngeal artery, from the stylomastoid branch of the
10 Jugular Foramen Tumors
A decision was then taken to attempt tumor
resection via the RS route. Once the skin was
incised, the subcutaneous air collection was
encountered—the air penetrated through
multiple small holes of the underlying bone.
Typical retrosigmoid approach was per-
formed, and a jugular foramen tumor that
extended in the CP angle and in the IAC was
seen. Taking care not to injure the com-
pressed seventh and eighth cranial nerves,
internal debulking was performed. Then, the
dura of the pyramid was excised, and the pet-
rosal tumor part, extending from the jugular
foramen inferior to the petrous carotid artery
anterolaterally and to the petrous apex medi-
ally, was exposed and removed via an
inframeatal approach (described in the chap-
ter). The posterior and inferior walls of the
IAC were then also drilled off, and the intra-
canalicular tumor was dissected from the
nerves and removed. Complete tumor
removal was thus achieved, and the bone
opening was filled with multiple fat pieces
sealed with fibrin glue (r–u: postoperative
MRI control).
After surgery the patient had a swallowing
difficulty that, however, improved within 3
days, and normal peroral feeding was started.
Hearing level remained unchanged.
occipital artery, from the petrosal branch of the middle
meningeal artery, and from the posterior auricular
artery. These vessels were selectively catheterized
with microcatheters, and the tumor was embolized
with polyvinyl alcohol. (m–p) Control angiography
after the embolization showing substantial devascular-
ization of the tumor. (f) Venous phase. (q) Bone-
window CT, performed after the transmastoid biopsy
approach. (r–u) Postoperative MR images demon-
strating the complete removal of both the intra- and of
the extracranial tumor parts. (r–t) T1-weighted
sequences. (u) T2-weighted sequence
Glomus Jugulare Tumors 681

a b

c
d

e
f
682 10 Jugular Foramen Tumors

g h

i j
Glomus Jugulare Tumors 683

k l

m n
684 10 Jugular Foramen Tumors

o p

q
Glomus Jugulare Tumors 685

r s

t u
686
Meningiomas of the Jugular Foramen
General Features
Meningiomas of JF are either primary or
secondary—extending to the JF from adjacent
areas. Primary jugular foramen meningiomas are
very rare and are estimated to comprise 4–9 % of all
posterior fossa meningiomas [4, 89, 97]. They are
considered to arise from the arachnoid-lining cells
of the jugular bulb within the jugular fossa [73, 97].
JF meningiomas are usually benign and have a
slow insidious growth. These tumors occasionally
are locally aggressive and infiltrate the temporal
bone and nerve tissue [6, 73]. From the JF area, the
tumor tends to extend superior into the CP angle,
inferior toward the foramen magnum or medially,
causing brain stem compression. It may further
invade the temporal bone and middle ear or extend
extracranially to the neck region. The extracranial
extension is usually limited to the carotid space,
and carotid encasement and/or jugular vein occlu-
sion may occur [38]. The classification, presented
by Bakar [6], which is based on the degree of
tumor extension, clearly illustrates the great vari-
ability of JF meningiomas:
• Type I—primarily within the JF
• Type II—primarily extension to the CP angle/
intracranially
• Type IIa—extension to the CP angle/intracranially
and middle ear
• Type III—primarily extension to the neck
• Type IIIa—extension to neck and middle ear
• Type IV—extension to the CP angle/intracra-
nial region and neck (dumbbell shape)
• Type IVa—extension to the CP angle/intracranial
region, neck, and middle ear
Neuroimaging
The comprehensive evaluation of patients with JF
meningiomas includes thin-slice bone-window
10 Jugular Foramen Tumors
CT, MRI, and MR angiography, including the
MR venography. On CT, meningiomas appear as
slightly hyperintense masses. The surrounding
bone structures have relatively preserved archi-
tecture, but occasionally hyperostosis/sclerotic
changes of the JF are seen [38]. Meningiomas, in
contrast to schwannomas and glomus tumors,
may have calcifications [63].
JF meningiomas are typically iso- to hypoin-
tense on T1-weighted MRI and have intermediate
to increased signal intensity on T2-weighted images,
depending on histology composition [65, 97]. No
flow voids are noted. The tumor enhances strongly
after contrast application. Meningiomas may have
a dura tail, which is key feature for their differen-
tial diagnosis [38, 65].
The venous flow and the patency of the sig-
moid sinus and jugular vein can be reliably evalu-
ated on MR venography. Meningiomas, as well
as glomus jugulare tumors, tend to infiltrate the
jugular vein; in contrast, schwannomas usually
only compress it [73, 89, 114]. DSA and embo-
lization of the arterial feeders might be consid-
ered in selected highly vascular tumors.
Primary and secondary JF meningiomas have
different growth pattern that is reflected by the neu-
roimaging findings. Those with secondary exten-
sion into the JF are usually solid, well-circumscribed
sessile lesions with a broad dural base. Their gross
appearance is en plaque or globular (Fig. 10.13).
Meningiomas arising primarily within the JF have a
more infiltrative growth pattern with extension into
the surrounding skull base and/or protrusion into
the skull-base foramina (see Case Illustration 10.12)
[38, 73]. In large tumors, however, it is impossible
to differentiate between primary and secondary JF
meningiomas (Fig. 10.14). Some meningiomas
tend to infiltrate centrifugally the surrounding skull
base in all directions [65]. MacDonald et al. [65]
presented five patients with late diagnosis: in all of
them, the posterior fossa, middle ear cavity, inter-
nal auditory canal, hypoglossal canal, and carotid
space were involved.
Meningiomas of the Jugular Foramen 687

a b

c d

e f

Fig. 10.13 Preoperative CT (a, b) and MRI (c, d) images
of a patient with a left-sided jugular foramen meningioma
with a large intra- and extracranial parts. Considering the
absence of hearing on the tumor side and the growth pattern
of the tumor, the transmastoid–transpetrous approach was
selected. Initially, the intradural tumor was exposed (e) and
removed (f). Then the pyramid was drilled (g), and the
remaining tumor removed completely. The postoperative
CT scans (h, i) demonstrate the extent of bone removal
688 10 Jugular Foramen Tumors

g h

Fig. 10.13 (continued)

Fig. 10.14 Meningioma extending from the JF (arrow)

to the foramen magnum area
Meningiomas of the Jugular Foramen
Case Illustration 10.12
Primary JF Meningioma
This 44-year-old patient had been operated 8
years prior to presentation at another facility—
a partial removal of his jugular foramen men-
ingioma had been performed. Four years later,
he had been treated with radiosurgery because
of growth of the tumor remnant. The follow-
up MRIs 2 and 3 years later showed a slow
but constant growth tendency of the extrafo-
raminal tumor part. At presentation, he had
severe accessory nerve palsy on the left side
with atrophy of the trapezius muscle and slight
hearing loss. He reported occasional swallow-
ing disturbance, but the examination, includ-
ing fiber-optic endoscopy, did not reveal any
obvious dysfunction of the ninth and tenth
cranial nerves. The possible management
options were discussed with the patient and
his family thoroughly, and—considering the
steady growth tendency—a decision was taken
to attempt removal of the tumor. The patient
689
was unwilling, however, to accept any risk of
new neurological dysfunction. The standard
RS approach was used to expose the area of
the jugular foramen. A relatively small tumor
bulging from the foramen into the CP angle was
seen and removed. The 10th and 11th cranial
nerves were severely adherent to the tumor and
the scar tissue, formed after the previous sur-
gery and radiosurgery. All lower cranial nerves
showed good response to electrical stimula-
tion. The jugular foramen could be entered
via a small corridor between the nerve fibers,
and a part of the tumor could be removed.
Considering the very high risk of damaging
the nerves and the related risk of severe dys-
function, it was decided not to proceed with
tumor removal. After the surgery, a transient
swallowing difficulty was observed, but within
4 days, normal feeding could be resumed. The
histological diagnosis was meningothelial
meningioma grade 1. At follow-up 12 months
later, the tumor remnant remained unchanged.
690 10 Jugular Foramen Tumors

a b

(a–c) Primary JF meningioma with a limited extension on T2-weighted images (b) and shows a strong homo-
into the CP angle. The tumor is isointense on geneous contrast uptake (c)
T1-weighted images (a) and isointense to hypointense
Meningiomas of the Jugular Foramen
Clinical Manifestation
The clinical manifestation of JF meningiomas is
related to their size and exact location and can
mimic the presentation of glomus jugulare tumors.
Initial symptoms are due to lower cranial nerves or
cochlear nerve dysfunction. At presentation the
most frequent symptoms/signs are hearing loss
(52–62 %), middle ear mass (23 %), dysphagia
(23–50 %), deficit of the accessory nerve (6–38 %),
tinnitus (17 %), dizziness (17 %), hoarseness
(14 %), tongue paresis (5–12 %), ataxia (12 %),
and headache (6–25 %) [6]. Middle ear mass may
be identified on otoscopy (in type IIa, IIIa, and
IVa). The clinical picture is similar to that of other
jugular foramen tumors and is hence not sufficient
for preoperative differential diagnosis [86].
Operative Treatment
The selection of an operative approach is based
on the following parameters: tumor size; loca-
tion; extension; patient’s presenting symptoms,
in particular hearing function; facial nerve and
lower cranial nerves function; and goal of sur-
gery. A wide variety of operative approaches has
been promoted in the literature: infratemporal
fossa type A approach; translabyrinthine; transco-
chlear; transcondylar–suprajugular, transcondy-
lar–retrojugular, or transcondylar–transjugular;
petro-occipital–transmastoid; and retrosigmoid
approach—alone or in combination.
The goal of surgery of JF meningiomas is their
complete removal and preservation of neurologi-
cal functions. We favor the RS as the optimal
operative approach that provides sufficient tumor
exposure in most cases (see chapters on RS
approach). The jugular foramen can be opened—
as described earlier—to access the intraforaminal
tumor part (see Case Illustration 10.13). The
involved bony structures, as well as the infiltrated
dura, should be removed as far as safely possible.
In case of extensive tumors, involving the pyra-
mid and middle ear or extending extracranially,
the approach should be modified accordingly.
Considering the typical for skull-base meningio-
ma’s propensity to persist or recur despite the
691
radicality of surgery, every effort should be made
to avoid new severe deficits and deterioration of
patient’s quality of life. In case of tumor
infiltration of the cranial nerves or brain stem or
in case clear dissection plane is absent, a less
than total resection is the most reasonable option.
The biological activity of small tumor remnants
cannot be reliably predicted. Frequently they do
not show growth tendency for a long time period.
Therefore, we do not recommend immediate
postoperative radiotherapy even in case of known
remnants. A follow-up MRI is performed in 6
months, and radiotherapy—usually stereotactic
radiosurgery—is applied in case the remnant
shows progressive growth.
Meningiomas on the side of a single ipsilat-
eral sigmoid sinus/bulb or even in case of domi-
nant ipsilateral sinus should be removed with
utmost care. Very important issue to consider is
the preoperative neurological status of the
patients. Thus, in case the tumor involves the
pyramid and the patient is deaf and has other cra-
nial nerve deficits, the transmastoid–transpetrous
approach might be most suitable to achieve the
goals of surgery (Fig. 10.13).
Outcome and Follow-Up
The most frequent new neurological deficits after
surgery are lower cranial nerve, hearing loss, and
facial nerve dysfunction. The facial nerve is espe-
cially endangered if a procedure involving its
wide dissection or transposition is performed.
Meningioma patients tend to have worse postop-
erative lower cranial nerve outcomes than patients
with glomus tumors or schwannomas [38, 64].
To avoid neurological disasters, tumor relation to
the brain stem should be assessed very carefully.
Meningiomas of the jugular foramen, similar to
all other skull-base meningiomas, may be either
infiltrative or have a well-developed and pre-
served arachnoid plane (see the discussion in the
chapter on CP angle meningiomas). In case the
pia covering is infiltrated, no attempt at complete
resection should be made.
The recurrence rate in case of JF meningiomas
is reported to lie between 12.5 and 25 % [4, 73,
86, 97] and is higher in case of incomplete tumor
removal. These tumors, however, tend to recur
692
despite of the extent of resection. Even after com-
plete tumor removal, Molony et al. [73] observed
recurrences in two of eight patients, Vrionis in
3/4 patients [120], Ramina in 1/5 patients [86],
and Arnautovic and Al Mefty [4] in 1/8 patients.
A judicious tumor removal and vigilant follow-
up with regular MR imaging is therefore abso-
lutely mandatory. The preferred treatment of
tumor recurrences is their surgical removal, pro-
vided it can be safely performed. Remnants con-
sidered high surgical risks are best managed with
radiotherapy/radiosurgery.
10 Jugular Foramen Tumors
The outcome in patients with malignant
meningiomas or with aggressive histological
tumor type (papillary, rhabdoid, microcys-
tic, and clear cells meningiomas) is poor: the
tumors tend to recur irrespective of the com-
pleteness of their resection. Early radiotherapy
is recommended because it could provide bet-
ter tumor control and can postpone the time to
recurrence [86, 92].
Primary radiosurgery is an option, acceptable
only in patients with small meningiomas, inca-
pable, or unwilling to undergo surgical removal.
Meningiomas of the Jugular Foramen
Case Illustration 10.13
JF Meningioma with a Large CP Angle
Part
This 60-year-old patient presented with a his-
tory of left-sided hearing loss since 12 months
and tinnitus since 7 months. His neurological
status was completely normal besides a
decreased hearing on the left side (40d PTA).
The MRI showed a contrast-enhancing tumor
in the left CP angle, located mainly inframeatal
and extending into the jugular foramen (a–f).
The lesion had imaging characteristics of a
meningioma. The widened jugular foramen
(CT scan) indicated that the tumor could be
originating in the area of the jugular foramen.
The RS approach was performed as usual,
exposing the edges of transverse and sig-
moid sinuses (g–n). The highly vascular-
ized meningioma was located inferior to the
693
seventh to eighth cranial nerves and supe-
rior to the lower cranial nerves, which were
severely compressed. The tumor capsule
was incised, and the tumor debulked using
alternately the CUSA, platelet knife, tumor
forceps, and suction–irrigation. Then, the
capsule was then dissected from the brain
stem and from the CP angle vessels. In order
to gain access to the intraforaminal tumor
part, the roof (dorsal wall) of the jugu-
lar foramen was widely opened. Thus, the
whole tumor could be removed completely
with preservation of all cranial nerves and
vessels. The histopathological diagnosis
was meningothelial meningioma (WHO
grade 1). The postoperative period was
uneventful. No new neurological deficits
appeared after surgery, and the hearing level
remained unchanged (40 dB PTA).
694 10 Jugular Foramen Tumors

a b

c d

CT (a, b) and MRI (c–f) images demonstrating a primary
JF meningioma on the right side. (g) RS craniotomy,
exposing the edges of the transverse and sigmoid sinuses
(asterisks). (h, i) Initial view of the highly vascularized
meningioma located inferior and lateral to the seventh to
eighth cranial nerve complex (not visible initially) and
superior to the lower cranial nerves (arrows). Once the
tumor mass in the CP angle was removed, the jugular
foramen had to be opened to gain access to the intrafo-
raminal tumor part. (j) The dura overlying the dorsal wall
of the jugular foramen was incised (arrowheads). The
diamond drill head was used to open the foramen widely
(k) and thereby to expose the whole tumor—(l) (Tu). The
tumor was completely removed, and all neurovascular
structures were preserved. (m) The intact AICA and
abducens nerve, dissected from the tumor. (n) Final view
of the CP angle, demonstrating the complete tumor
removal, the preservation of the seventh to eighth cranial
nerves and of the lower cranial nerves (LCN). The opened
jugular foramen was afterward plugged with multiple fat
pieces to prevent CSF leak. (o) CT scan, performed on
the first postoperative day
Meningiomas of the Jugular Foramen 695

e f
696 10 Jugular Foramen Tumors

g h

i j
Meningiomas of the Jugular Foramen 697

k l

m
n

o
698 10 Jugular Foramen Tumors

References 18. Brown LA (1953) Glomus jugulare tumor of the mid-

dle ear; clinical aspects. Laryngoscope 63(4):281–
292. doi:10.1288/00005537-195304000-00001
1. Al-Mefty O, Fox JL, Rifai A, Smith RR (1987) A
19. Bulsara KR, Sameshima T, Friedman AH, Fukushima
combined infratemporal and posterior fossa approach
T (2008) Microsurgical management of 53 jugular
for the removal of giant glomus tumors and chondro-
foramen schwannomas: lessons learned incorporated
sarcomas. Surg Neurol 28(6):423–431
into a modified grading system. J Neurosurg
2. Al-Mefty O, Teixeira A (2002) Complex tumors of
109(5):794–803. doi:10.3171/JNS/2008/109/11/0794
the glomus jugulare: criteria, treatment, and outcome.
20. Caldemeyer KS, Mathews VP, Azzarelli B, Smith RR
J Neurosurg 97(6):1356–1366
(1997) The jugular foramen: a review of anatomy,
3. Alford BR, Guilford FR (1962) A comprehensive study
masses, and imaging characteristics. Radiographics
of tumors of the glomus jugulare. Laryngoscope
17(5):1123–1139
72:765–805. doi:10.1288/00005537-196206000-00006
21. Capps FC (1952) Glomus jugulare tumours of the
4. Arnautovic KI, Al-Mefty O (2002) Primary menin-
middle ear. J Laryngol Otol 66(7):302–314
giomas of the jugular fossa. J Neurosurg 97(1):12–20.
22. Capps FC (1958) Chemodectoma or tumor of the glo-
doi:10.3171/jns.2002.97.1.0012
mus jugulare and tympanic bodies. AMA Arch
5. Ayeni SA, Ohata K, Tanaka K, Hakuba A (1995) The
Otolaryngol 67(5):556–559
microsurgical anatomy of the jugular foramen. J
23. Carvalho GA, Tatagiba M, Samii M (2000) Cystic
Neurosurg 83(5):903–909. doi:10.3171/jns.1995.83.
schwannomas of the jugular foramen: clinical and
5.0903
surgical remarks. Neurosurgery 46(3):560–566
6. Bakar B (2010) Jugular foramen meningiomas: review
24. Clemis JD (1980) Neurogenic tumors of the skull
of the major surgical series. Neurol Med Chir (Tokyo)
base. Otolaryngol Head Neck Surg 88(5):511–518
50(2):89–96
25. Denecke HJ (1966) On the surgery of diffuse glomus
7. Bakar B (2008) The jugular foramen schwannomas:
tumors in the region of the foramen jugulare. Arch
review of the large surgical series. J Korean Neurosurg
Klin Exp Ohren Nasen Kehlkopfheilkd 187(2):
Soc 44(5):285–294. doi:10.3340/jkns.2008.44.5.285
656–662
8. Balasubramaniam C (1999) A case of malignant
26. Dichiro G, Fisher RL, Nelson KB (1964) The jugular
tumour of the jugular foramen in a young infant.
foramen. J Neurosurg 21:447–460. doi:10.3171/
Childs Nerv Syst 15(6–7):347–350
jns.1964.21.6.0447
9. Bartal AD, Djaldetti MM, Mandel EM, Lerner MA
27. Eldevik OP, Gabrielsen TO, Jacobsen EA (2000)
(1973) Dumb-bell neurinoma of the hypoglossal
Imaging findings in schwannomas of the jugular fora-
nerve. J Neurol Neurosurg Psychiatry 36(4):592–595
men. AJNR Am J Neuroradiol 21(6):1139–1144
10. Batsakis JG, Farber ER (1967) Chemodectomas of the
28. Fayad JN, Keles B, Brackmann DE (2009) Jugular
head and neck. Univ Mich Med Cent J
foramen tumors: clinical characteristics and treatment
33(3):123–130
outcomes. Otol Neurotol 31(2):299–305. doi:10.1097/
11. Berger MS, Edwards MS, Bingham WG (1982)
MAO.0b013e3181be6495
Hypoglossal neurilemmoma: case report and review
29. Fisch U (1978) Infratemporal fossa approach to
of the literature. Neurosurgery 10(5):617–620
tumours of the temporal bone and base of the skull.
12. Bickerstaff ER, Howell JS (1953) The neurological
J Laryngol Otol 92(11):949–967
importance of tumours of the glomus jugulare. Brain
30. Fisch U (1982) Infratemporal fossa approach for glo-
76(4):576–593
mus tumors of the temporal bone. Ann Otol Rhinol
13. Borba LA, Ale-Bark S, London C (2004) Surgical
Laryngol 91(5 Pt 1):474–479
treatment of glomus jugulare tumors without rerout-
31. Franklin DJ, Moore GF, Fisch U (1989) Jugular fora-
ing of the facial nerve: an infralabyrinthine approach.
men peripheral nerve sheath tumors. Laryngoscope
Neurosurg Focus 17(2):E8. doi:170208 [pii]
99(10 Pt 1):1081–1087. doi:10.1288/00005537-
14. Borba LA, Araujo JC, de Oliveira JG, Filho MG,
198210000-00020
Moro MS, Tirapelli LF, Colli BO (2010) Surgical
32. Galbraith JG, Butler JS, Harty JA (2011) Recurrent
management of glomus jugulare tumors: a proposal
spontaneous hip dislocation in a patient with
for approach selection based on tumor relationships
neurofibromatosis type 1: a case report. J Med Case
with the facial nerve. J Neurosurg 112(1):88–98.
Reports 5(1):106. doi:10.1186/1752-1947-5-106.
doi:10.3171/2008.10.JNS08612
1752-1947-5-106 [pii]
15. Boyle JO, Shimm DS, Coulthard SW (1990) Radiation
33. Ganz JC, Abdelkarim K (2009) Glomus jugulare
therapy for paragangliomas of the temporal
tumours: certain clinical and radiological aspects
bone. Laryngoscope 100(8):896–901. doi:10.1288/
observed following Gamma Knife radiosurgery. Acta
00005537-199008000-00018
Neurochir (Wien) 151(5):423–426. doi:10.1007/
16. Brackmann DE (1987) The facial nerve in the
s00701-009-0268-7
infratemporal approach. Otolaryngol Head Neck Surg
34. Gardner G, Cocke EW Jr, Robertson JT, Trumbull
97(1):15–17
ML, Palmer RE (1977) Combined approach surgery
17. Brismar J, Cronqvist S (1978) Therapeutic emboliza-
for removal of glomus jugulare tumors. Laryngoscope
tion in the external carotid artery region. Acta Radiol
87(5 Pt 1):665–688
Diagn (Stockh) 19(5):715–731
References
35. Gejrot T (1965) Surgical treatment of glomus jugulare
tumours with special reference to the diagnostic value
of retrograde jugularography. Acta Otolaryngol
60:150–168
36. George B, Lot G, Tran Ba Huy P (1995) The jux-
tacondylar approach to the jugular foramen (without
petrous bone drilling). Surg Neurol 44(3):279–284.
doi:0090-3019(95)00174-3 [pii]
37. Gerosa M, Visca A, Rizzo P, Foroni R, Nicolato A,
Bricolo A (2006) Glomus jugulare tumors: the option
of gamma knife radiosurgery. Neurosurgery 59(3):
561–569. doi:10.1227/01.NEU.0000228682.92552.
CA. 00006123-200609000-00008 [pii]; discussion
561–569
38. Gilbert ME, Shelton C, McDonald A, Salzman KL,
Harnsberger HR, Sharma PK, Jensen RL (2004)
Meningioma of the jugular foramen: glomus jugulare
mimic and surgical challenge. Laryngoscope 114(1):25–
32. doi:10.1097/00005537-200401000-00004
39. Glasscock ME 3rd, Harris PF, Newsome G (1974)
Glomus tumors: diagnosis and treatment. Laryngoscope
84(11):2006–2032
40. Green JD Jr, Brackmann DE, Nguyen CD, Arriaga MA,
Telischi FF, De la Cruz A (1994) Surgical management
of previously untreated glomus jugulare tumors.
Laryngoscope 104(8 Pt 1):917–921.
doi:10.1288/00005537-199408000-00001
41. Greenberg HS, Deck MD, Vikram B, Chu FC, Posner
JB (1981) Metastasis to the base of the skull: clinical
findings in 43 patients. Neurology 31(5):530–537
42. Guild S (1941) A hitherto unrecognized structure, the
glomus jugularis, in man. Anat Rec 79:28
43. Gulya AJ (1993) The glomus tumor and its biology.
Laryngoscope 103(11 Pt 2 Suppl 60):7–15
44. Hakuba A, Hashi K, Fujitani K, Ikuno H, Nakamura
T, Inoue Y (1979) Jugular foramen neurinomas. Surg
Neurol 11(2):83–94
45. Hawthorne MR, Makek MS, Harris JP, Fisch U (1988)
The histopathological and clinical features of irradi-
ated and nonirradiated temporal paragangliomas.
Laryngoscope 98(3):325–331. doi:10.1288/00005537-
198803000-00018
46. Heth J (2004) The basic science of glomus jugulare
tumors. Neurosurg Focus 17(2):E2. doi:170202 [pii]
47. Heutink P, van der Mey AG, Sandkuijl LA, van Gils
AP, Bardoel A, Breedveld GJ, van Vliet M, van
Ommen GJ, Cornelisse CJ, Oostra BA et al (1992) A
gene subject to genomic imprinting and responsible
for hereditary paragangliomas maps to chromosome
11q23-qter. Hum Mol Genet 1(1):7–10
48. Hilal SK, Michelsen JW (1975) Therapeutic percuta-
neous embolization for extra-axial vascular lesions of
the head, neck, and spine. J Neurosurg 43(3):275–287.
doi:10.3171/jns.1975.43.3.0275
49. Hilding DA, Greenberg A (1971) Surgery for large
glomus jugulare tumor. The combined suboccipital,
transtemporal approach. Arch Otolaryngol 93(3):
|227–231
50. Horn KL, House WF, Hitselberger WE (1985)
Schwannomas of the jugular foramen. Laryngoscope
95(7 Pt 1):761–765
699
51. House WF, Glasscock ME 3rd (1968) Glomus tym-
panicum tumors. Arch Otolaryngol 87(5):550–554
52. Jackson C (2001) Glomus tympanicum and glomus
jugulare tumors. Otolaryngol Clin North Am
34:941–970
53. Jackson CG (1993) Neurotologic skull base surgery
for glomus tumors. Diagnosis for treatment planning
and treatment options. Laryngoscope 103(11 Pt 2
Suppl 60):17–22
54. Jackson CG, Glasscock ME 3rd, Nissen AJ, Schwaber
MK (1982) Glomus tumor surgery: the approach,
results, and problems. Otolaryngol Clin North Am
15(4):897–916
55. Jackson CG, Kaylie DM, Coppit G, Gardner EK (2004)
Glomus jugulare tumors with intracranial extension.
Neurosurg Focus 17(2):E7. doi:170207 [pii]
56. Jenkins HA, Fisch U (1981) Glomus tumors of the
temporal region. Technique of surgical resection.
Arch Otolaryngol 107(4):209–214
57. Katsuta T, Rhoton AL Jr, Matsushima T (1997) The
jugular foramen: microsurgical anatomy and opera-
tive approaches. Neurosurgery 41(1):149–201; dis-
cussion 201–142
58. Kaye AH, Hahn JF, Kinney SE, Hardy RW Jr, Bay JW
(1984) Jugular foramen schwannomas. J Neurosurg
60(5):1045–1053. doi:10.3171/jns.1984.60.5.1045
59. Kempe LG, VanderArk GD, Smith DR (1971) The neuro-
surgical treatment of glomus jugulare tumors. J Neurosurg
35(1):59–64. doi:10.3171/jns.1971.35.1.0059
60. Lack EE, Cubilla AL, Woodruff JM, Farr HW (1977)
Paragangliomas of the head and neck region: a clini-
cal study of 69 patients. Cancer 39(2):397–409
61. Leman P, Portmann M, Cohadon F, Leifer C, Traissac,
Castel (1968) Oto-neurosurgical collaboration in the
total ablation of a tympano-jugular glomus tumor
with intracranial extension. Neurochirurgie 14(7):
828–830
62. Leonetti JP, Brackmann DE, Prass RL (1989)
Improved preservation of facial nerve function in the
infratemporal approach to the skull base. Otolaryngol
Head Neck Surg 101(1):74–78
63. Lowenheim H, Koerbel A, Ebner FH, Kumagami H,
Ernemann U, Tatagiba M (2006) Differentiating imaging
findings in primary and secondary tumors of the jugular
foramen. Neurosurg Rev 29(1):1–11. doi:10.1007/
s10143-005-0420-7; discussion 12–13
64. Lustig LR, Jackler RK (1996) The variable relation-
ship between the lower cranial nerves and jugular
foramen tumors: implications for neural preservation.
Am J Otol 17(4):658–668
65. Macdonald AJ, Salzman KL, Harnsberger HR, Gilbert
E, Shelton C (2004) Primary jugular foramen menin-
gioma: imaging appearance and differentiating fea-
tures. AJR Am J Roentgenol 182(2):373–377
66. Makek M, Franklin DJ, Zhao JC, Fisch U (1990)
Neural infiltration of glomus temporale tumors. Am J
Otol 11(1):1–5
67. Maniglia AJ, Chandler JR, Goodwin WJ Jr, Parker JC
Jr (1979) Schwannomas of the parapharyngeal space
and jugular foramen. Laryngoscope 89(9 Pt
1):1405–1414
700
68. Martin JJ, Kondziolka D, Flickinger JC, Mathieu D,
Niranjan A, Lunsford LD (2007) Cranial nerve preser-
vation and outcomes after stereotactic radiosurgery for
jugular foramen schwannomas. Neurosurgery 61(1):
76–81. doi:10.1227/01.neu.0000279726.90650.6d.
00006123-200707000-00008 [pii]; discussion 81
69. Mazzoni A, Sanna M, Saleh E, Achilli V (1997)
Lower cranial nerve schwannomas involving the jug-
ular foramen. Ann Otol Rhinol Laryngol 106(5):
370–379
70. Meacham WF, Capps JM (1960) Intracranial glomus-
jugulare tumor with successful surgical removal.
J Neurosurg 17:157–160
71. Megerian CA, McKenna MJ, Nadol JB Jr (1995)
Non-paraganglioma jugular foramen lesions mas-
querading as glomus jugulare tumors. Am J Otol
16(1):94–98
72. Miller JP, Semaan MT, Maciunas RJ, Einstein DB,
Megerian CA (2009) Radiosurgery for glomus jugu-
lare tumors. Otolaryngol Clin North Am 42(4):689–
706. doi:10.1016/j.otc.2009.04.004. S0030-6665
(09)00059-0 [pii]
73. Molony TB, Brackmann DE, Lo WW (1992)
Meningiomas of the jugular foramen. Otolaryngol
Head Neck Surg 106(2):128–136
74. Murphy TP, Brackmann DE (1989) Effects of preop-
erative embolization on glomus jugulare tumors.
Laryngoscope 99(12):1244–1247. doi:10.1288/
00005537-198912000-00007
75. Muthukumar N, Kondziolka D, Lunsford LD,
Flickinger JC (1999) Stereotactic radiosurgery for
jugular foramen schwannomas. Surg Neurol
52(2):172–179. doi:S0090-3019(99)00031-2 [pii]
76. Oghalai JS, Buxbaum JL, Jackler RK, McDermott MW
(2005) Skull base chondrosarcoma originating from the
petroclival junction. Otol Neurotol 26(5):1052–1060.
doi:00129492-200509000-00037 [pii]
77. Oghalai JS, Leung MK, Jackler RK, McDermott MW
(2004) Transjugular craniotomy for the management
of jugular foramen tumors with intracranial extension.
Otol Neurotol 25(4):570–579; discussion 579.
doi:00129492-200407000-00026 [pii]
78. Olsen WL, Dillon WP, Kelly WM, Norman D, Brant-
Zawadzki M, Newton TH (1987) MR imaging of para-
gangliomas. AJR Am J Roentgenol 148(1):201–204
79. Ong CK, Fook-Hin Chong V (2009) Imaging of jugu-
lar foramen. Neuroimaging Clin N Am 19(3):469–
482. doi:10.1016/j.nic.2009.06.007. S1052-5149
(09)00043-4 [pii]
80. Patel SJ, Sekhar LN, Cass SP, Hirsch BE (1994)
Combined approaches for resection of extensive glo-
mus jugulare tumors. A review of 12 cases. J Neurosurg
80(6):1026–1038. doi:10.3171/jns.1994.80.6.1026
81. Pellet W, Cannoni M, Pech A (1988) The widened
transcochlear approach to jugular foramen tumors.
J Neurosurg 69(6):887–894. doi:10.3171/jns.1988.69.
6.0887
82. Pensak ML, Jackler RK (1997) Removal of jugular
foramen tumors: the fallopian bridge technique.
Otolaryngol Head Neck Surg 117(6):586–591.
doi:S0194-5998(97)70037-8 [pii]
10 Jugular Foramen Tumors
83. Pollock BE (2004) Stereotactic radiosurgery in
patients with glomus jugulare tumors. Neurosurg
Focus 17(2):E10. doi:170210 [pii]
84. Ramina R, Maniglia JJ, Fernandes YB, Paschoal JR,
Pfeilsticker LN, CoelhoNeto M (2005) Tumors of the
jugular foramen: diagnosis and management.
Neurosurgery 57(1 Suppl):59–68; discussion
59–68
85. Ramina R, Maniglia JJ, Fernandes YB, Paschoal JR,
Pfeilsticker LN, Neto MC, Borges G (2004) Jugular
foramen tumors: diagnosis and treatment. Neurosurg
Focus 17(2):E5. doi:170205 [pii]
86. Ramina R, Neto MC, Fernandes YB, Aguiar PH, de
Meneses MS, Torres LF (2006) Meningiomas of the
jugular foramen. Neurosurg Rev 29(1):55–60.
doi:10.1007/s10143-005-0415-4
87. Rao AB, Koeller KK, Adair CF (1999) From the
archives of the AFIP. Paragangliomas of the head and
neck: radiologic-pathologic correlation. Armed Forces
Institute of Pathology. Radiographics
19(6):1605–1632
88. Rhoton AL Jr (2000) Jugular foramen. Neurosurgery
47(3 Suppl):S267–S285
89. Roberti F, Sekhar LN, Kalavakonda C, Wright DC
(2001) Posterior fossa meningiomas: surgical experi-
ence in 161 cases. Surg Neurol 56(1):8–20; discussion
20–21. doi:S0090-3019(01)00479-7 [pii]
90. Rosenwasser H (1945) Carotid body like tumor
involving the middle ear and mastoid bone. Arch
Otolaryngol 41:64–67
91. Ruben RJ (2007) The history of the glomus tumors –
nonchromaffim chemodectoma: a glimpse of biomed-
ical Camelot. Acta Otolaryngol 127(4):411–416.
doi:10.1080/00016480601002088. 777054949 [pii]
92. Salcman M (1991) Malignant meningiomas. In: A-M O
(ed) Meningiomas. Raven Press, New York, pp 75–85
93. Samii M (1989) Tumors of the internal auditory canal
and cerebellopontine angle. In: Samii M, Draf W
(eds) Surgery of the skull base: an interdisciplinary
approach. Springer, Berlin, pp 377–395
94. Samii M, Babu RP, Tatagiba M, Sepehrnia A
(1995) Surgical treatment of jugular foramen
schwannomas. J Neurosurg 82(6):924–932.
doi:10.3171/jns.1995.82.6.0924
95. Samii M, Bini W (1993) Surgical strategy for jugular
foramen tumors. In: Sekhar LN, Janecka IP (eds)
Surgery of cranial base tumors. Raven Press, New
York, pp 379–387
96. Sanna M, Bacciu A, Falcioni M, Taibah A (2006)
Surgical management of jugular foramen schwanno-
mas with hearing and facial nerve function preserva-
tion: a series of 23 cases and review of the literature.
Laryngoscope 116(12):2191–2204. doi:10.1097/01.
mlg.0000246193.84319.e5. 00005537-200612000-
00017 [pii]
97. Sanna M, Bacciu A, Falcioni M, Taibah A, Piazza P
(2007) Surgical management of jugular foramen
meningiomas: a series of 13 cases and review of
the literature. Laryngoscope 117(10):1710–1719.
doi:10.1097/MLG.0b013e3180cc20a3. 00005537-
200710000-00003 [pii]
References
98. Sanna M, De Donato G, Di Lella F, Falcioni M,
Aggrawal N, Romano G (2009) Nonvascular lesions
of the jugular foramen: the gruppo otologico experi-
ence. Skull Base 19(1):57–74. doi:10.1055/s-
0028-1103124
99. Sanna M, De Donato G, Taibah A, Russo A, Falcioni
M, Mancini F (1999) Infratemporal fossa approaches
to the lateral skull base. Keio J Med 48(4):189–200
100. Sanna M, Jain Y, De Donato G, Rohit, Lauda L,
Taibah A (2004) Management of jugular paragan-
gliomas: the Gruppo Otologico experience. Otol
Neurotol 25(5):797–804. doi:00129492-200409000-
00025 [pii]
101. Semaan MT, Megerian CA (2008) Current assess-
ment and management of glomus tumors. Curr Opin
Otolaryngol Head Neck Surg 16(5):420–426.
doi:10.1097/MOO.0b013e32830c4595. 00020840-
200810000-00006 [pii]
102. Semmes R (1951) Discussion of Alexander E Jr,
Beamer PR, Williams JO: Tumor of the glomus jug-
ulare with extension into the middle ear. J Neurosurg
8:515–523
103. Sen C, Hague K, Kacchara R, Jenkins A, Das S,
Catalano P (2001) Jugular foramen: microscopic
anatomic features and implications for neural preser-
vation with reference to glomus tumors involving the
temporal bone. Neurosurgery 48(4):838–847; dis-
cussion 847–838
104. Shapiro MJ, Neues DK (1964) Technique for
removal of glomus jugulare tumors. Arch Otolaryngol
79:219–224
105. Shearman CP, Pearman K, Hockley AD (1986)
Dumb-bell schwannoma of the jugular foramen.
Childs Nerv Syst 2(1):52–53
106. Sheehan J, Kondziolka D, Flickinger J, Lunsford LD
(2005) Gamma knife surgery for glomus jugulare
tumors: an intermediate report on efficacy and safety.
J Neurosurg 102(Suppl):241–246
107. Simpson GT 2nd, Konrad HR, Takahashi M, House J
(1979) Immediate postembolization excision of glo-
mus jugulare tumors: advantages of new combined
techniques. Arch Otolaryngol 105(11):639–643
108. Snyderman CH, Pant H, Carrau RL, Prevedello D,
Gardner P, Kassam AB (2009) What are the limits of
endoscopic sinus surgery? The expanded endonasal
approach to the skull base. Keio J Med 58(3):152–160
109. Song MH, Lee HY, Jeon JS, Lee JD, Lee HK, Lee
WS (2008) Jugular foramen schwannoma: analysis
on its origin and location. Otol Neurotol 29(3):387–
391. doi:10.1097/MAO.0b013e318164cb83
110. Spector GJ, Ciralsky RH, Ogura JH (1975) Glomus
tumors in the head and neck: III. Analysis of clinical
manifestations. Ann Otol Rhinol Laryngol 84(1 Pt
1):73–79
111. Spinnato S, Talacchi A, Musumeci A, Turazzi S,
Bricolo A (1998) Dumbbell-shaped hypoglossal
neurinoma: surgical removal via a dorsolateral
transcondylar approach. A case report and review of
the literature. Acta Neurochir (Wien) 140(8):827–832
701
112. Sutay S, Tekinsoy B, Ceryan K, Aksu Y (1993)
Submaxillary hypoglossal neurilemmoma.
J Laryngol Otol 107(10):953–954
113. Tan LC, Bordi L, Symon L, Cheesman AD (1990)
Jugular foramen neuromas: a review of 14 cases.
Surg Neurol 34(4):205–211. doi:0090-3019(90)
90130-H [pii]
114. Tekkok IH, Ozcan OE, Turan E, Onol B (1997)
Jugular foramen meningioma. Report of a case and
review of the literature. J Neurosurg Sci 41(3):
283–292
115. Thoms OJ, Shaw DT, Trowbridge WV (1960)
Glomus jugulare tumor: report of a case with surgi-
cal removal. J Neurosurg 17:500–504. doi:10.3171/
jns.1960.17.3.0500
116. Tummala RP, Coscarella E, Morcos JJ (2005)
Transpetrosal approaches to the posterior fossa.
Neurosurg Focus 19(2):E6. doi:190206 [pii]
117. Ulso C, Sehested P, Overgaard J (1981) Intracranial
hypoglossal neurinoma: diagnosis and postoperative
care. Surg Neurol 16(1):65–68
118. van den Berg R, Schepers A, de Bruine FT, Liauw L,
Mertens BJ, van der Mey AG, van Buchem MA
(2004) The value of MR angiography techniques in
the detection of head and neck paragangliomas. Eur
J Radiol 52(3):240–245. doi:10.1016/j.
ejrad.2003.12.002. S0720048X03003747 [pii]
119. van der Mey AG, Maaswinkel-Mooy PD, Cornelisse
CJ, Schmidt PH, van de Kamp JJ (1989) Genomic
imprinting in hereditary glomus tumours: evidence
for new genetic theory. Lancet 2(8675):1291–1294
120. Vrionis FD, Robertson JH, Gardner G, Heilman CB
(1999) Temporal bone meningiomas. Skull Base
Surg 9(2):127–139
121. Willen SN, Einstein DB, Maciunas RJ, Megerian
CA (2005) Treatment of glomus jugulare tumors in
patients with advanced age: planned limited surgical
resection followed by staged gamma knife radiosur-
gery: a preliminary report. Otol Neurotol 26(6):1229–
1234. doi:00129492-200511000-00026 [pii]
122. Williams HL, Childs DS Jr, Parkhill EM, Pugh DG
(1955) Chemodectomas of the glomus jugulare
(nonchromaffin paragangliomas) with especial ref-
erence to their response to roentgen therapy. Ann
Otol Rhinol Laryngol 64(2):546–566
123. Zhang H, Cai C, Wang S, Liu H, Ye Y, Chen X (2007)
Extracranial head and neck schwannomas: a clinical
analysis of 33 patients. Laryngoscope 117(2):278–
281. doi:10.1097/01.mlg.0000249929.60975.a7.
00005537-200702000-00017 [pii]
124. Zhang N, Pan L, Dai JZ, Wang BJ, Wang EM, Cai
PW (2002) Gamma knife radiosurgery for jugular
foramen schwannomas. J Neurosurg 97(5 Suppl):456–
458. doi:10.3171/jns.2002.97.supplement5.0456
125. Zimmer LA, Hirsch BE, Kassam A, Horowitz M,
Snyderman CH (2006) Resection of a recurrent para-
ganglioma via an endoscopic transnasal approach to
the jugular fossa. Otol Neurotol 27(3):398–402.
doi:00129492-200604000-00017 [pii]
 Other Cerebellopontine
Angle Tumors
Intra-Axial Tumors Extending
Secondarily to the Cerebellopontine
Angle
Intra-axial tumors originating in the brain stem,
cerebellum, or fourth ventricle can have an exo-
phytic part or become large enough to involve
the CP angle [4]. Clinically they can manifest as
a CP angle mass. Most common are astrocy-
tomas, ependymomas, and medulloblastomas,
followed by choroid plexus papillomas, lympho-
mas, hemangioblastomas, gangliogliomas, and
dysembryoplastic neuroepithelial tumors [8,
16, 30]. Their clinical presentation is nonspecific,
and the presenting symptoms are not related to
the tumor type: they depend rather upon the
affected cranial nerves or neural structures. The
preoperative diagnosis of a CP angle tumor is
based mainly on neuroimaging [4, 42].
Characteristic findings are narrow or undetect-
able CP angle cistern, absent brain–tumor
interface, and peritumoral edema.
It has been estimated that 0.3–2 % of all CP
angle lesions originate from primary brain tissue
[42]. Surgery rarely leads to cure of patients with
such tumors and should be regarded as a compo-
nent of the whole management concept. Its goal
is to provide histopathological diagnosis and to
remove the lesion as completely as safely possi-
ble, thereby achieving volume reduction, brain
stem decompression, and restoration of the CSF
circulation pathways. The operative approach is
selected considering tumor size and extension,
neurological and general condition of the patient,
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_11, © Springer-Verlag Berlin Heidelberg 2013
11
and goal of surgery. Besides the standard ret-
rosigmoid approach, its modification—the lat-
eral suboccipital approach to the craniocervical
junction, as described in the chapter on foramen
magnum meningiomas, can be utilized. Tumors
with both CP angle and fourth ventricular part
can be removed via a midline suboccipital cran-
iotomy with opening of the foramen magnum.
The fourth ventricle is accessed via the telovelar
route and—once the intraventricular tumor part
is removed—the CP angle is reached via the
dilated foramen of Luschka (see Case Illustration
11.3). Alternatively, tumors with larger CP angle
part may be approached via the retrosigmoid
route: once the cisternal part is removed, the
fourth ventricle may be reached via the enlarged
foramen of Luschka (see Case Illustration
11.1).
Brain Stem Gliomas
Brain stem gliomas are highly heterogeneous
tumors that occur at any age. Still, they affect
primarily children, with the mean age of diag-
nosis at 7–9 years. These tumors account for
10–20 % of all pediatric central nervous sys-
tem tumors [18]. Relatively rarely they mani-
fest as asymmetric expansion of the brain stem
into the CP angle or have an exophytic part in
the area—Fig. 11.1 [43]. Extension into and
even enlargement of the IAC has also been
reported [31, 45]. Primary CP angle gliomas
are exceedingly rare; they arise from the
703
704 11 Other Cerebellopontine Angle Tumors

vestibulocochlear nerve [3, 11, 20] or from the
trigeminal nerve [2]. Most cases reported in the
literature are either fibrillary or gemistocytic
astrocytomas [2, 5].
Three cases of primary extra-axial pilocytic
astrocytoma of the CP angle have been reported
[2, 3, 31]. These tumors were completely sepa-
rated from the brain stem, and a clear tissue plane
between the tumor capsule and the brain stem
could be identified.
On MR imaging, brain stem gliomas are
hypointense in T1-weighted images and hyper-
intense on T2-weighted images and may be sur-
rounded by hyperintense edematous area [4]. The
CISS sequence is very useful for delineating CP
angle anatomy and tumor borders. The signal
characteristics and the extent of contrast enhance-
ment are related to tumors’ grade. Mirone et al.
[31] pointed that a high index of suspicion is
warranted when evaluating a CP angle mass in a

a b

c d

Fig. 11.1 Pilocytic astrocytoma of the lower brain stem via a median suboccipital craniotomy and telovelar
in a 20-year-old patient. The tumor has a large pontomed- approach to the fourth ventricle (e–h: postoperative MR
ullary cystic part and an exophytic contrast-enhancing images)
part in the left CP angle (a–d). It was removed completely
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 705

e f

g h

Fig. 11.1 (continued)

child. Blurring of the margin between the tumor
and the brain stem or cerebellum, extensive peri-
tumoral edema, and widening of the lateral recess
of the fourth ventricle should raise the suspicion
for an intrinsic tumor with secondary CP angle
extension [31].
Surgery is part of the management of brain
stem gliomas. Its goal is to verify the histo-
logical tumor type, to perform tumor debulk-
ing, or to remove the safely accessible tumor
part (see Figs. 11.2, 11.3, and 11.4 and Case
Illustration 11.1).
706 11 Other Cerebellopontine Angle Tumors

a b

c d

Fig. 11.2 Heterogeneous lesion with MR characteristics stern sequence. (b, d) T2-weighted image. (c) T1-weighted
of a low-grade glioma. It originated from the pedunculus image after contrast administration. See next figures for
cerebelli above the level of the seventh to eighth cranial details on tumor removal
nerves and extended into the left CP angle. (a) T2-weighted
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 707

a b

Tu

7-8

c d

Fig. 11.3 Intraoperative images illustrating the steps of
tumor removal (patient from previous figure). (a) Initial
view of the tumor from a retrosigmoid perspective, present-
ing as expansion of the brain stem above the root exit zone
of the facial nerve (Tu tumor, 7–8 vestibulocochlear nerve
complex, R retractor, arrowhead petrous vein). (b–d) In
order to avoid major neurological deficit, the tumor was
removed only partially. Histological examination showed
that it was a low-grade glioma
708 11 Other Cerebellopontine Angle Tumors

a b

Fig. 11.4 Low-grade exophytic astrocytoma of the cervi- view of the tumor. (e, f) Gradual removal and exposure of
cal medulla with intracranial extension into the lower left the vertebral artery. (g) Complete tumor removal and pres-
CP angle. (a–c) Preoperative images. The patient has been ervation of all structures
operated in the early 1990s by the senior author. (d) Initial
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 709

d e

f g

Fig. 11.4 (continued)

710
Case Illustration 11.1
Brain Stem Low-Grade Glioma Removed
via the Lateral Suboccipital Approach with
Opening of the Foramen Magnum
Twenty-six-year-old male patient presented
with complaints of general weakness, vertigo,
deteriorating hearing on the right side, and occa-
sional swallowing difficulty since more than 12
months. Neurological examination revealed gait
instability, hearing loss, rotatory nystagmus, and
lack of pharyngeal reflex on the right side. MRI
demonstrated a large brain stem tumor with
extension in the right CP angle and fourth ven-
tricle through the foramen of Luschka (a–e).
The patient was operated via a lateral suboc-
cipital approach with opening of the foramen mag-
num (f–p). Following the dura incision along the
sigmoid sinus, the tonsil was elevated, and the
tumor part protruding into the CP angle was seen.
This part was gradually resected, which allowed
exposing and dissecting the seventh to eighth
nerve complex and later of the abducens nerve.
a b
(a–e) Preoperative MR examination of the patient,
demonstrating the tumor of the lower brainstem and its
extension into the CP angle and 4th ventricle through
the foramen of Luschka. (a, b) T1-weighted sequence,
following contrast administration. (c, d) T2-weighted
and (e) T2-weighted CISS sequences. The tumor
causes dilatation of the CP angle lateral to the tumor.
(f–p) Intraoperative images. (f) Lateral suboccipi-
tal approach with opening of the foramen magnum
(SS – sigmoid sinus). (g) The dura is opened along
the sigmoid sinus (C – cerebellum). (h) The cerebel-
11 Other Cerebellopontine Angle Tumors
Electrical stimulation was repeatedly applied to
identify the essential areas of the brain stem.
Tumor removal was then continued in a medial
direction through the enlarged foramen of
Luschka. The intra-axial tumor part, which
infiltrated the medulla oblongata, was not removed
in order to prevent major deficits; the nerve root
entry/exit zone of the lower cranial nerves was
located exactly in this area, which explained the
preoperative dysphagia. The tumor portion in the
fourth ventricle and in the CP angle was removed,
and decompression of the seventh to eighth cranial
nerves was achieved. Histopathological examina-
tion revealed a diffuse WHO grade II astrocytoma.
Immediately after surgery, the patient had
facial nerve weakness (HB grade III); the dys-
phagia was more pronounced and a tracheos-
tomy had to be performed. PEG tube was also
inserted. Within the hospital stay, his gait
improved significantly. In 2 months the tra-
cheostoma could be closed and the facial
nerve function improved to HB grade II.
lum has been slightly elevated, exposing the expanded
infiltrated by the tumor medulla (asteriks) and the lower
cranial nerves (LCN). (i) The exophytic tumor part (Tu)
was seen more rostrally to the LCN; the LCN entry/exit
zone was in the tumor-involved area of the medulla (j).
(k) Electrical stimulation was frequently applied.
(l–m) Gradual resection of the exophytic tumor, which
allowed to visualize the 7–8th nerve complex (n) and
later – of the abducent nerve (o) (6). (p) Tumor removal
was then continued in a medial direction through the
enlarged foramen of Luschka (V – 4th ventricle)
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 711

c d

h
712 11 Other Cerebellopontine Angle Tumors

i j

k l
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 713

m n

o p
714
Choroid Plexus Papillomas
Choroid plexus papillomas represent 0.4–1 % of
all intracranial neoplasms [44]. They originate
from the epithelial cells of the choroid plexus and
are similar histologically to the normal choroid
plexus [21]. Malignant forms are very rare.
Choroid plexus papillomas occur mainly in
children and are located in the lateral ventricles.
In adults their most common location is the fourth
ventricle. Papillomas originating from the chor-
oid plexus of the fourth ventricle can extend via
the foramina of Luschka into the CP angle. Very
rarely they develop primarily in the CP angle [10,
13, 25]. In 1 series of 245 patients of choroid
plexus papillomas, only 17 were located in the
CP angle [39].
These tumors present clinically with signs and
symptoms of a CP angle lesion that are related to
its size and precise location. On CT imaging, they
present as well-defined homogeneously enhancing
11 Other Cerebellopontine Angle Tumors
mass with lobulations and frond-like irregular
pattern—a cauliflower-like appearance [4, 24].
Calcifications are seen in 4–20 % of the tumors
[39]. Their radiological differential diagnosis is
difficult if a frond-like pattern, calcifications,
and/or enlargement of foramen of Luschka are
not present. Plexus papillomas are isointense on
T1- and T2-weighted MR images, have irregular
structure, and enhance intensely after contrast
injection. They are often associated with hydro-
cephalus, caused by obstruction of the CSF flow
or by CSF hypersecretion by the tumor [33]. In
the series of Talacchi et al. [46], 8 of 12 CP angle
choroid plexus papillomas had hydrocephalus.
The optimal treatment of plexus papillomas is
surgical removal: complete tumor removal is
related to an excellent prognosis [13, 21] (see
Case Illustration 11.2). The tumor may be very
adherent to the brain stem, however, and total
removal with preservation of neurological func-
tions may be challenging [25].
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle
Case Illustration 11.2
CP Angle Plexus Papilloma
This 65-year-old male patient presented with
complaints from headache, right-sided weak-
ness, and gait instability, as well as occasional
swallowing difficulty. MR examination (a–e)
demonstrated a tumor in the lower CP angle,
reaching the level of the foramen magnum. It
showed intense and homogeneous contrast
enhancement and appeared to be dura based.
Meningioma was hence the most plausible
diagnosis.
The patient was operated via the lateral
suboccipital approach with opening of the
foramen magnum (f–j). Initially the tumor
could not be seen, as it was located medially
and was hidden by the flocculus. The flocculus
was partially resected and retracted, and the
lower cranial nerves were followed in the
a b
Preoperative MR images, clearly demonstrating the
tumor and its location. (a) T1-weighted native sequence.
(b and c) T1-weighted sequences following gadolinium
administration. (d) T2-weighted sequence. (e) T2- CISS
sequence. Intraoperative images demonstrating the steps
of tumor removal. (f) Initial view of the CP angle (ret-
rosigmoid approach). The tumor is still hidden by the
flocculus (arrow seventh to eighth cranial nerves, flocc
715
direction of the brain stem, which allowed
exposing the tumor. The tumor was adherent
to the brain stem and to the lower cranial
nerves and engulfed the vertebral artery with
its small branches. Still, it could be gradually
dissected and removed completely. Frozen
section analysis showed that it was an adeno-
carcinoma metastasis.
The postoperative period was uneventful.
No new neurological deficits appeared. During
the hospital stay, the strength recovered com-
pletely and the patient was able to walk inde-
pendently. The definitive histopathological
study showed that the tumor was an atypical
(WHO grade II) plexus papilloma. Regular
follow-up with MRI every 6 months was rec-
ommended. At the first follow-up examina-
tion, the patient had no neurological deficits
and MRI signs of recurrence.
flocculus, LCN lower cranial nerves, asteriks PICA). (g, h)
The PICA has been dissected and the flocculus slightly
elevated, exposing the underlying tumor medial to the
lower cranial nerves and jugular foramen (Tu).
(i) Following removal of the main tumor bulk, its caudal
portion was removed between the fibers of the ninth to
tenth and the 11th cranial nerves. The vertebral artery
(VA) was also dissected free from the tumor (j)
716 11 Other Cerebellopontine Angle Tumors

c d

f
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 717

g h

i j
718
Ependymomas
Approximately 70 % of intracranial ependymo-
mas are infratentorial, arising from differentiated
ependymal cells lining the fourth ventricle and its
lateral recesses. Up to 15 % of these tumors may
extend secondarily into the CP angle through the
foramina of Luschka—Fig. 11.5 [50]. Primary
CP angle ependymomas originate presumably
from the ependymal cell rests within the foramen
of Luschka and extend both laterally into the CP
angle and medially into the fourth ventricle [7,
9, 17]. They rarely invade the floor of the fourth
ventricle but have a propensity to encase the
lower cranial nerves, the posteroinferior cerebel-
lar artery, and the basilar artery [12].
Radiologically ependymomas appear as irreg-
ular lobulated extra-axial tumors in the posterior
fossa (Fig. 11.4). They are hypointense on
T1-weighted MR images and hyperintense on
T2-weighted MR images and have an intermedi-
ate-to-high FLAIR signal intensity [4, 50].
Ependymomas have heterogeneous internal
structure due to the presence of calcifications,
hemorrhages, necrosis, and/or intratumoral cysts.
The tumor demonstrates irregular enhancement:
the strong enhancement of the soft tissue compo-
nents of the tumor is intermixed with poorly
a b
Fig. 11.5 Ependymoma of the fourth ventricle, extending into the left CP angle via the foramen of Luschka
11 Other Cerebellopontine Angle Tumors
enhancing or nonenhancing areas [50]. The
T2-weighted and the T2-CISS sequences may
demonstrate the encasement of the major arteries
and the relation of the tumor to cranial nerves
(Fig. 11.4). Spinal MRI is useful to rule out meta-
static disease. The main differential diagnosis of
ependymomas is medulloblastoma and astrocy-
toma. Medulloblastomas, which rarely extend
through the fourth ventricular exit foramina, have
a more bulbous extension compared to the thin
“tongues” of tissue seen in ependymomas.
Ependymomas have a propensity for CSF spread.
MR study of the whole neuraxis and CSF sam-
pling should be performed to evaluate the extent
of CNS involvement and for tumor staging.
CP angle ependymomas, especially in child-
hood, are often quite large before they become
clinically apparent. Their typical presentation is
rather intracranial hypertension than cranial nerve
deficits.
The current standard of care for patients with
ependymoma is maximal surgical resection and
evaluation of the presence of other foci [26, 34].
CP angle ependymomas pose particular surgical
challenges due to the frequent encasement of cra-
nial nerves and vessels [41]. Still the major deter-
minant of outcome and long-term survival is the
extent of tumor resection, and every effort should
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle
be made to achieve a gross total resection—if
safely possible [28, 41] (see Case Illustrations
11.3 and 11.4). Early postoperative MRI control
should be performed to assess the degree of resec-
tion. A rim of enhancement along the resection
cavity margins appears soon following surgery.
Hence, the imaging should be performed less than
24 h after surgery. In case a residual tumor is
detected that is deemed safely removable, reop-
eration should be considered. If the residual can-
not be removed completely, radiotherapy is
usually administered. The necessity of adjuvant
postoperative therapy is related to multiple fac-
tors, such as extent of resection, preoperative
staging, histologic tumor grade, tumor staging,
and patients’ age. Radiotherapy should be consid-
ered in all aggressive tumors and in case of subto-
tally resected nonanaplastic ependymomas. The
719
role of craniospinal irradiation in patients with
local disease and no evidence of metastasis is con-
troversial. In cases of metastatic disease, however,
it is certainly indicated. The efficacy of chemo-
therapy is uncertain; it may be recommended only
in case of failed surgery and radiotherapy.
Regular follow-up of the patients is important
to detect early possible recurrences. Most recur-
rences are local at the site of the primary tumor.
Recurrent ependymomas are managed best by
reoperation with involved-field radiotherapy.
Main prognostic factors of survival are com-
pleteness of resection, patients’ age, histological
tumor grade, and tumor location. Complete
removal (or “gross total resection”) is correlated
with improved prognosis. Children older than 3
or 4 years of age and infratentorial ependymomas
have a better general prognosis.
720
Case Illustration 11.3
Ependymoma with Extension in the CP Angle
Removed Through the Dilated Foramen
of Luschka removal with an Endoscope-
Assisted Microsurgical Technique
A 22-year-old female patient presented with
complaints of headache since 1 year and
increasing neck stiffness, nausea, and vomit-
ing since 1 month. Her neurological evalua-
tion revealed truncal ataxia and hyperreflexia
in all four extremities. MRI examination dem-
onstrated a large tumor, occupying the fourth
ventricle, extending caudally to the C2 level,
and expanding into the CP angle through the
enlarged left foramen of Luschka (a–f). The
tumor was hypointense on T1-sequences and
hyperintense on T2-sequences and had highly
inhomogeneous internal structure.
The patient was operated via the midline
suboccipital approach with C1 laminectomy
(g–i). The tumor part, protruding through the
foramen of Magendie, was debulked initially
with the ultrasonic aspirator. Then, the tumor
a b
(a–f) Extensive ependymoma of the 4th ventricle with
extension into the left CP angle through the enlarged
foramen of Luschka – preoperative MR images. (a–c)
T1-weighted sequences following Gadolinium admin-
istration. (d, e) T2-weighted sequences. (f) CISS
sequence. (g–j) Intraoperative images of the tumor:
the dura is incised in the midline (g) and fixed with 2
11 Other Cerebellopontine Angle Tumors
capsule was dissected stepwise from the PICA
on both sides, from the vertebral arteries, the
C1 nerve roots, and the accessory nerve. The
cerebellar tonsils were elevated, and further
debulking of the intraventricular tumor part
followed. The tumor extension in the CP angle
could be also removed through the dilated
foramen of Luschka, applying a combined
microsurgical and endoscopic technique. Due
to infiltration of the floor of the fourth ventri-
cle in the left lateral recess—the probable site
of tumor origin—complete tumor resection
was not safely possible. The postoperative
period was uneventful. No new neurological
deficits appeared, besides a slight temporary
swallowing difficulty. Figures k–p show the
early postoperative MRI control. In 4 days,
however, oral feeding was successfully initi-
ated. Histopathological diagnosis was ana-
plastic ependymoma (WHO grade III).
Adjuvant chemo- and radiotherapy was, there-
fore, recommended according to the HIT 2000
study protocol.
stay sutures, exposing the tumor (h, i). Elevation of the
lower pole of the tumor allowed to visualize the C1
roots and the lower cranial nerves (j). (k–p)
Postoperative MR images, demonstrating the extent of
tumor removal. (k–n) T1-weighted sequences follow-
ing Gadolinium administration. (o) T2-weighted
sequences. (p) T2- CISS sequence
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 721

c d

e f

g h
722 11 Other Cerebellopontine Angle Tumors

i j

k l

m n
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 723

o p
724
Case Illustration 11.4
Complete Removal of an Anaplastic
Ependymoma
This 39-year-old male patient had been operated
twice for a posterior cranial fossa ependymoma
at another neurosurgical facility—10 and 7 years
earlier. The last histopathological diagnosis was
anaplastic ependymoma; combined chemo- and
radiotherapy followed. Five to 6 weeks prior
to presentation, he experienced rapid hearing
deterioration on the right side, accompanied
by vertigo. Neurological examination showed
hypesthesia in V1, V2, and V3 branches and
severe hearing loss on the right side, as well
as dysmetria (right side) and gait ataxia. MR
examination demonstrated a large tumor in the
right CP angle (a–d) with imaging characteris-
tics, compatible with a recurrent ependymoma.
Large postoperative parenchymal defect involv-
ing the vermis and part of the right cerebellar
hemisphere were further seen. A certain growth
tendency was obvious when the tumor size was
compared to the previous images.
Considering the size and location of the
tumor and the progressive neurological
a b
Preoperative MRI of the patient. (a, b) T1-weighted
sequences following contrast administration. (c, d)
T2-weighted sequences. (e–h) Postoperative T1-weighted
11 Other Cerebellopontine Angle Tumors
deterioration, a decision for surgery was
taken. In this particular case, the RS approach
seemed to be less appropriate: (1) the large
postoperative parenchymal defect provided
sufficient working space and (2) retraction of
the cerebellum would certainly endanger its
remaining neurological functions and had to
be avoided. The tumor was approached, there-
fore, via a median suboccipital craniotomy. It
was very firm and vascularized. The vertebral
and basilar arteries were engulfed by the
tumor, and the lower cranial nerves were
compressed and dislocated caudally. Still,
following initial internal decompression, it
could be dissected from surrounding struc-
tures. The seventh to eighth cranial nerves
were also severely compressed but not
infiltrated, which allowed for their safe dis-
section and preservation. The tumor could be
removed completely, as shown on the postop-
erative MR imaging (e–h).
After the surgery, the patient had swallow-
ing difficulties, which rapidly improved.
Interestingly, the hearing also improved to a
functional level.
images following gadolinium administration (e, f)
and T2-weighted images (g, h), performed 6 months
postoperatively
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 725

c d

e f

g h
726
Subependymomas
Subependymomas are low-grade (WHO grade 1)
glial neoplasms typically located in the sub-
ependymal regions of the ventricles [6, 15].
They may become symptomatic at any age
but have a peak incidence in middle-aged and
elderly patients [37]. The tumor is characterized
by low proliferative activity and—according to
some authors—should be classified as a hama-
rtoma rather than as a true neoplasm [35].
Subependymomas cause slowly progressive clin-
ical symptoms or remain asymptomatic and are
common incidental finding at autopsy.
These tumors generally arise from the floor
of the fourth ventricle near the foramen of
Monro or—very rarely—are in relation to the
central canal of the spinal cord [6]. In contrast
to ependymomas, which tend to be paraven-
tricular, subependymomas tend to be intraven-
tricular. The CP angle might be secondarily
11 Other Cerebellopontine Angle Tumors
involved by tumors originating in the fourth
ventricle [22].
Neuroimaging features of subependymomas
are nonspecific: they are generally isointense or
hypointense on T1-weighted MR images and
hyperintense on T2-weighted MR images [6, 15,
40]. Heterogeneity is typical and is seen in up to
67 % of the patients [40]. Calcifications are seen
on CT in up to 50 % of the patients [6]. Following
gadolinium administration, most subependymo-
mas show at least some enhancement [40].
Notably, 21 % of subependymomas do not
enhance. Hoeffel et al. [15] even point that when
an adult patient presents with an intraventricular
tumor that is isodense or hypodense and shows
minimal or no enhancement, a subependymoma
should suspected. Symptomatic or enlarging sub-
ependymomas should be treated actively—com-
plete surgical removal is the optimal management
form and leads to excellent long-term outcome
(see Case Illustration 11.5).
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 727

Case Illustration 11.5
Fourth Ventricle Subependymoma with
Extension in the CP Angle
This 47-year-old male patient presented with
complaints of headache and nausea since sev-
eral months. His neurological examination
revealed no major abnormalities. The MRI,
presented on figures a and b showed a tumor in
the lower fourth ventricle with extension into
the right CP angle via the foramen of Luschka.
The patient was operated via the median sub-
occipital approach, and the tumor, which
proved to be a subependymoma, could be
removed completely (see figures c–j for details
on the operative technique). After the surgery,
the patient did not have any neurological
deficits and recovered rapidly. The postopera-
tive MRI is presented on figures k and l.

a b

c d

(a, b) Preoperative MR images (a) T1-weighted images
following gadolinium administration and CISS images
(b). The arrow points at the small tumor extension into
the right CP angle. (c–j) Consecutive steps of the
microsurgical tumor removal. (c) Median suboccipital
craniotomy with resection of foramen magnum was
performed. (d) The dura was incised parallel to the
midline to evade the occipital sinus. Two stay sutures
have been placed on both sides to provide the required
exposure. We avoid the classical “Y”-shaped incision
of the dura because of the difficulty to achieve water-
tight primary closure. (e) The tumor, protruding trough
the foramen of Magendie, was visualized. It was deb-
ulked carefully, preserving the PICA and its branches.
Then, the tumor capsule was dissected from the sur-
roundings (f–h). (i) The tumor part, extending into the
left foramen of Luschka and CP angle, was then dis-
sected from the cranial nerves and from the vertebral
artery and completely resected. The origin of the tumor
was the left recess of the fourth ventricle (j). (k, l)
Postoperative MRI: complete tumor removal
728 11 Other Cerebellopontine Angle Tumors

e f

g h
Intra-Axial Tumors Extending Secondarily to the Cerebellopontine Angle 729

i j

k l
730
Medulloblastomas
Medulloblastomas are neuroepithelial tumors
arising from remnants of the primitive neuroec-
toderm in the roof of fourth ventricle. They grow
usually in the cerebellar vermis, fill, and often
invade the floor of the ventricle [38]. Less com-
monly the tumor arises in the cerebellar hemi-
sphere. The CP angle may be involved due to
lateral tumor extension via the foramina of
Luschka or due to exophytic growth of the
tumor, originating from the cerebellum or from
the pons [1, 49].
On MRI medulloblastomas are hypointense
on T1- and iso-weighted sequences or hyperin-
tense on T2- weighted sequences with possible
cystic or necrotic components and surrounding
area of edema [29]. After administration of gado-
linium, the tumor enhances homogeneously. In
adults, however, a more heterogeneous pattern of
enhancement may be seen. MRI of the spine is
obligatory in order to rule out drop metastases.
Cytological examination of CSF, obtained via
lumbar puncture, from the ventricular drain or
from the cisterna magna at the time of surgery, is
mandatory for tumor staging. Symptoms dura-
tion prior to presentation is usually very short.
Patients present frequently with signs of intracra-
nial hypertension, as well as with symptoms or
signs of cerebellar, brain stem, and/or cranial
nerve dysfunction.
Less than 20 cases of CP angle medulloblasto-
mas have been reported in the world literature
[23, 27, 47]. Some authors suggest that these
medulloblastomas arise from remnants of the
external granular layer in the cerebellar hemi-
sphere, mainly the flocculus, which faces the CP
angle or from proliferating residue of the lateral
medullary velum. Medulloblastomas cannot be
reliably differentiated from other CP angle lesions
before surgery due to their similar clinical and
neuroradiological characteristics.
Clinical outcome of patients with medullo-
blastoma varies according to age, postoperative
tumor residuum, and metastatic stage [14]. They
are best managed with surgery, followed by cran-
iospinal radiotherapy [14, 36]. The role of che-
motherapy is controversial. Goal of surgery is
11 Other Cerebellopontine Angle Tumors
maximal but safe cytoreduction and restoration
of the CSF circulation. Unlike ependymomas that
may be more infiltrative to cranial nerves,
medulloblastomas extend laterally and tend to
displace (rather than encase) them. Normal arach-
noid plane is usually present, enabling tumor dis-
section. The combined radio- and chemotherapy
has been shown to improve the 5-year survival
rate to 55–76 % for high-risk patients and to
70–80 % for standard-risk patients [14].
Lymphomas
Less than 20 cases of CP angle lymphomas
have been reported in the literature. They
develop in the CP angle as primary extra-axial
lymphoma, as a leptomeningeal lymphoma, or
as an extension from an intra-axial lymphoma.
Lymphomas are hypointense on T1-weighted
MR images and hyperintense on T2-weighted
MR images and enhance after contrast agent
injection [19, 48]. Perifocal edema is typically
present and may suggest the intra-axial origin
of the tumor. Aggressive removal is not recom-
mended and adjuvant radiotherapy should be
always considered [32].
Rare Extra-axial Cerebellopontine
Angle Lesions
Extra-axial tumors and other mass lesions devel-
oping within the CP angle are separated from the
brain parenchyma (brain stem and cerebellum)—
in contrast to secondary CP angle tumors—by a
cleft of cerebrospinal fluid. In larger tumors/
lesions, such a cleft is absent, but a characteristic
radiological feature is the presence of vessels
between the lesion and the parenchyma [64].
The most frequent extra-axial tumors are
schwannomas, meningiomas, and arachnoid cysts.
The remaining lesions comprise less than 1 %:
metastases, melanomas and melanocytic tumors,
craniopharyngioma, pituitary adenoma, lym-
phoma, chloroma, hemangioblastoma, respiratory
cyst, solitary fibrous tumor, aneurysm, cavern-
oma, sarcoidosis, tuberculosis, Erdheim–Chester
Rare Extra-axial Cerebellopontine Angle Lesions
disease, neurocysticercosis, and hydatid cysts [64,
83, 96, 119, 141, 166].
Arachnoid Cysts
Arachnoid cysts are congenital collections of
CSF contained within the splitted or duplicated
arachnoidal membrane. According to the most
widely accepted theories, they originate from
arachnoid splitting due to alteration of the CSF
pressure and flow or from duplication of the
arachnoid membrane [137]. Duplicated arach-
noid membrane is found histologically in the
majority of the cases.
The natural history of arachnoid cysts is not
well known: some of these cysts are quiescent
throughout life, some remain asymptomatic for
many years before showing clinical manifesta-
tions, while other enlarge progressively and cause
compression of adjacent structures or CSF flow
disturbance. Spontaneous reduction is observed
very rarely [142]. Cyst enlargement may be due
to active secretion from the cyst wall and passive
fluid diffusion into the cyst due to the existing
osmotic gradient or intracystic hemorrhage.
Communicating cysts may grow due to the pres-
ence of a functional one-way valve between the
subarachnoid space and the cyst.
Ten to 11 % of all of intracranial arachnoid
cysts are located in the CP angle, which is the
second most common location of such cysts after
the Sylvian fissure area [57, 60, 142, 144, 147].
Arachnoid cysts may be located only in the CP
angle (58 % of the cases according to our previ-
ous study [144]) or may extend dorsally to the
brain stem (25 %) or even into the IAC (17 %).
Rarely, arachnoid cysts in adults may remain
confined within the IAC [68, 147]. The senior
author has operated three patients with pure intra-
canalicular arachnoid cysts.
The typical CT and MR appearance of arach-
noid cysts is that of a noncalcified homogeneous
extra-axial lesion, with imaging characteris-
tics similar to those of CSF. It is hypointense
on T1-weighted sequences and hyperintense
on T2-weighted images and does not enhance
after administration of gadolinium [65, 116].
731
Arachnoid cysts have smooth and well-defined
borders [107]. These cysts may be difficult
to differentiate from epidermoids of the CP
angle on conventional T1-weighted images
and T2-weighted images and from other cys-
tic lesions, such as cystic schwannomas, cystic
meningiomas, and neurocysticercosis (see the
appropriate chapters). Arachnoid cysts are gen-
erally more homogenous, have less mass effect,
and may cause adjacent bony reaction, usually
bone scalloping [65]. Furthermore—in contrast
to epidermoids—they displace adjacent arter-
ies and cranial nerves rather than encase them
[63]. Definitive clues for their correct diagnosis
are provided by fluid-attenuated inversion recov-
ery imaging, diffusion-weighted imaging, and
CISS spin-echo MR images [88]. Occasionally,
elevated signal intensity on T1-weighted images
can be seen, which is due to a higher protein con-
tent of the cyst [144]. Dynamic MRI technique,
as well as CT cisternogram, could allow differen-
tiating between communicating and noncommu-
nicating with the CSF arachnoid cysts.
The large majority of arachnoid cysts are
asymptomatic and found incidentally. They may
become symptomatic by causing compression or
stretching of surrounding structures or—more
rarely—by causing hydrocephalus [100, 144].
Spontaneous or traumatic intracystic hemorrhage
has rarely been described in the posterior fossa
[103]. The most frequent presenting symptom in
the series of Jallo et al. [107] was headache, while
in the series of the senior author it was vertigo/
dizziness [144], followed by hearing loss, gait dis-
turbance, tinnitus, and trigeminal dysfunction.
The management of patients with arachnoid
cysts is determined by their clinical and biologi-
cal behavior. Asymptomatic small cysts may be
followed with regular MRI, clinical, and audio-
logical control. In case enlargement of the cyst is
noted, surgery may be indicated. Symptomatic
cysts, especially those causing clinical worsen-
ing, should be treated actively, since their removal
leads to clinical improvement and the related
morbidity rate is very low [107, 144].
Surgical options include cyst aspiration, endo-
scopic or microsurgical cyst fenestration, cysto-
peritoneal shunt, cyst marsupialization into the
732
subarachnoid space, and complete or partial cyst
resection [51, 107, 121, 144].
We favor open surgery via the RS approach,
which the safest technique. It allows to confirm
the diagnosis, to coagulate any arachnoidal blood
vessels, and to fenestrate multiloculated cysts
[107]. Some authors recommend resecting only
the medial, lateral, and posterior membranes of
the cyst and fenestrating the anterior membrane
into the cisterns of the CP angle. Based on the
experience with 28 surgically treated cases (MS),
we believe that radical resection of the cyst and
careful removal of the cyst parts that are adherent
to vessels and cranial nerves reduce the risk of
recurrences (see Case Illustrative 11.7). Of
course, if extreme dense adherence between the
11 Other Cerebellopontine Angle Tumors
cyst wall and the surrounding neurovascular
structures is found, small parts of the capsule
may be left unremoved (see Case Illustrative
11.6). In cases of posterior fossa communicating
arachnoid cysts with associated hydrocephalus, a
VP shunting may be sufficient. However, if pre-
operative symptoms do not recover and the cyst
does not shrink, open surgery should be consid-
ered [144].
Generally, most symptoms regress after sur-
gery. In our experience, the headache, vertigo,
dizziness, and ataxia improved in the majority of
patients. Hearing dysfunction, however, improved
only rarely: hypacusis improved in two patients,
remained unchanged in three patients, and
became worse in two patients.
Rare Extra-axial Cerebellopontine Angle Lesions
Case Illustration 11.6
Arachnoid Cyst (Incomplete Resection
of the Medial Cyst Wall)
This is a 22-year-old female patient, com-
plaining of hearing loss on the right side,
headache, and vertigo for 15 months. The neu-
rological examination revealed no pathologi-
cal signs besides a profound right-sided
hearing loss. MR examination demonstrated a
cystic lesion in the right CP angle, compatible
with an arachnoid cyst (a–e).
The CP angle was exposed via the RS
approach, and the arachnoid cyst was seen in
its upper part between the seventh and eighth
a b
(a–e) MR images of the patient, demonstrating the cyst
in the CP angle. The contents of the cyst have signal
characteristics, identical with those of the CSF. Note
the mass effect on the adjacent neural structures: cranial
733
nerve complex (7–8) and the fifth nerve (f; T
tentorium). The outer membrane of the cyst
was relatively firm and non-translucent. It was
opened and resected with microscissors (g).
The inner or medial wall was densely attached
to the seventh to eighth cranial nerves, as well
as to the AICA branch, passing superior to the
nerves (h). An attempt to resect the medial
cyst wall was therefore not made. Therefore, a
wide fenestration to the medial subarachnoid
cisterns was made (figures i, j). The patient
recovered very rapidly from surgery and had
no new neurological deficits. Postoperative
MR images: k–n.
nerves and brainstem. (f–j) Intraoperative images, pre-
senting the removal of the cyst. See the text for expla-
nations. (k–n) Postoperative MRI: T1-weighted (k, l),
T2-weighted (m), and T2- CISS sequences (n)
734 11 Other Cerebellopontine Angle Tumors

c d

h
Rare Extra-axial Cerebellopontine Angle Lesions 735

i j

k l

n
736
Case Illustrative 11.7
Arachnoid Cyst (Complete Removal)
Nine-year-old girl complaining of headache
since 4–5 months. Her neurological status was
normal. MRI showed a cystic lesion in the right
CP angle with diameter of 2.6 × 1.8 × 1.7 cm.
Notably, a small contrast-enhancing part was
seen at the upper pole of the cyst, and the pre-
sumptive histological diagnosis was pilocytic
astrocytoma. Therefore, the decision for surgi-
cal removal of the lesion via the RS approach
was taken.
The cyst was located in the lower CP angle,
between the seventh and eighth and the lower
a b
Preoperative MRI: (a, b) T1-weghted sequence after
gadolinium administration. (c) T2-weighted sequences.
(d, e) CISS sequences. (f) Dark fluid sequence. (g–l)
Consecutive steps of surgery (arrow - arachnoid cyst).
11 Other Cerebellopontine Angle Tumors
cranial nerves (g). Initially, a small fenestra-
tion of its outer membrane away from the cra-
nial nerves at a nonvascular site was made
with microscissors (h). The remaining mem-
brane was resected under direct control using
both hands: the left hand was holding the cyst
and the right hand was holding the microscis-
sors. No mural nodule was found, and the
results from the histological examination
revealed an arachnoid cyst (i, j). Then the
remaining part of the membrane was carefully
dissected from the lower cranial nerves and
from the brain stem and removed in order to
prevent future recurrences (k, l).
See the text for explanations. (m–o) Postoperative
T1-weighted images after gadolinium administration
(m, n) and T2-weighted MR images (o)
Rare Extra-axial Cerebellopontine Angle Lesions 737

c d

e f
738 11 Other Cerebellopontine Angle Tumors

g h

i j

k l
Rare Extra-axial Cerebellopontine Angle Lesions 739

m n

o
740
Dermoids
Dermoids are rare congenital nonneoplastic cys-
tic lesions with a capsule composed of epidermal
elements and dermal derivatives, such as hair fol-
licles, sebaceous, and even sweat glands. They
contain thick, buttery material that results from
epithelial desquamation and sebaceous gland
secretion, as well as variable amount of hair or
even teeth [69]. A dermal sinus tract may connect
the cyst to the skin surface. Dermoid cysts are
less common than epidermoid cysts. They tend to
be located in the midline, while epidermoid cysts
tend to occur in a more lateral position. The CP
a b
c d
Fig. 11.6 CT scan (a, b) and T1-weighted MR contrast-
enhanced images (c, d) of a patient with very large der-
moid cyst of the posterior and left middle cranial fossae.
11 Other Cerebellopontine Angle Tumors
angle is, therefore, not a favorable location of
dermoids.
Dermoids grow slowly and tend to produce
symptoms due to local compressive effect in the
CP angle or cause obstruction of CSF pathways
(Fig. 11.6). Hydrocephalus is frequently the
cause of initial signs and symptoms. Infectious
complications—bacterial meningitis or cerebel-
lar abscess—occurring due to bacterial contami-
nation via the dermal tract, may be the initial
presentation of these tumors [164]. Compression
of the trigeminal nerve can cause trigeminal neu-
ralgia [108]. Rare complication is rupture of the
dermoid and distribution of its content along the
Note the extension in both CP angles and the severe com-
pressive effect on the brain stem
Rare Extra-axial Cerebellopontine Angle Lesions
CSF. This can remain asymptomatic or cause a
wide range of symptoms including aseptic men-
ingitis, hydrocephalus, cranial nerve deficit, or
epilepsy [70, 133].
Dermoid cysts have various CT and MRI
appearance depending on the fat content of the
tumor (Fig. 11.6). They are well-circumscribed
lesions with a thick peripheral occasionally
enhancing capsule [63]. Most frequently they
have very high signal intensity on T1-weighted
and low signal intensity on T2-weighted MR
images. As pointed by Yasargil et al. [165],
dermoid tumors have a similar appearance to
epidermoids but with a wider range of attenua-
tion values. Calcifications are seen in approxi-
mately 20 % of the dermoids but are very rare
in epidermoid tumors [98]. Diffusion-weighted
MR imaging allows for their differentiation:
the typical for dermoids hyperintensity
is related to the decreased water proton diffu-
sion [133].
CP angle dermoids may be a greater operative
challenge than epidermoids due to the younger
age of most patients and the more rapid tumor
growth [133, 165]. Their capsule is thicker than
that of epidermoids and may be tightly attached
to adjacent structures. Goal of surgery is their
741
radical removal and preservation of all neurologi-
cal functions.
As in all CP angle tumors, the cyst is opened
and internal decompression is performed initially.
Then the cyst wall is then dissected. En bloc
removal, which is recommended for dermoids in
other locations, is not safely feasible in the CP
angle. Similarly to CP angle epidermoids, if a
small part of the capsule is very densely attached
to a cranial nerve or to the brain stem, it should be
left. If the dermoid is associated with dermal
sinus tracts, both should be removed during the
surgery. The sinus tract should be dissected very
carefully from the surrounding healthy tissue and
bone and excised.
Dermoids have a very low growth potential
and the risk for recurrence is low. Spillage of cyst
contents is to be limited to prevent recurrences or
aseptic meningitis. The risk is, however, rela-
tively low. In one case operated by the senior
author, extensively disseminated fat particles in
the subarachnoid space were found after surgery
(see Case Illustration 11.8). During the 8 years of
follow-up, neither neurological deterioration nor
growth of the multiple lesions was seen [70]. The
secondary trigeminal neuralgia has a good out-
come after the removal of the dermoid [108].
742
Case Illustration 11.8
CP Angle Dermoid Tumor
A 36-year-old woman presented with right-
sided trigeminal neuralgia (V3) for 4 years.
Besides a V3 hypesthesia on the right side, her
neurological examination was unremarkable.
CT and MRI were performed and demon-
strated a relatively large tumor in the medial
CP angle area on the right side that compressed
severely the brain stem (a). The lesion was
hyperintense on T1-weighted and heteroge-
neous—iso- and hypointense—on T2-weighted
MR sequences (b–d).
The tumor was exposed via the RS approach:
it was partially cystic and contained yellow
fluid. Its capsule could be dissected completely
from the involved third, fourth, fifth, seventh,
and eighth cranial nerves and from the brain
stem. The histopathological diagnosis was der-
a b
(a) CT scan showing the strongly hypodense tumor.
(b, c) The lesion was hyperintense on T1-weighted
MR images. (d) On T2-weighted sequences, the tumor
was more heterogeneous with isointense and hypoin-
tense zones. (e–h) T1-weighted and T2-weighted MR
sequences, demonstrating the widespread subarach-
11 Other Cerebellopontine Angle Tumors
moid tumor. After surgery the patient had
incomplete oculomotor paresis and slight hypa-
cusis on the right side. On the fifth postopera-
tive day, aseptic meningitis was diagnosed.
Treatment with dexamethasone (8 mg, 3 times/
day for 1 week) led to rapid resolution of his
symptoms. The accurate surgical manipula-
tions and the thorough irrigation of the opera-
tive field after tumor removal did not prevent
spillage of the cyst contents in this particular
case, which led to subarachnoid dissemination
of fatty material (a–d) and postoperative chem-
ical meningitis. Nevertheless, the patient recov-
ered quickly and was discharged in a good
condition on the 15th postoperative day. The
neurological deficits resolved completely
within several months. Annual MRI follow-up
studies showed no changes in the size and loca-
tion of the subarachnoid fat particles.
noid dissemination of fatty material, most probably
from the cyst contents. The disseminated lipid droplets
have hyperintense signal on T1-weighted images and
hypointense signal on T2-weighted images (Reprinted
from: Carvalho et al. [70]. Copyright © 2000, with
permission from Wolters Kluwer Health)
Rare Extra-axial Cerebellopontine Angle Lesions 743

c d

e f

g h
744
Hamartomas
Intracranial hamartomas are considered to be
nonneoplastic lesions formed as a result of abnor-
mal embryologic development. Glioneural hama-
rtomas involving the IAC are very infrequent:
only seven cases have been reported in the litera-
ture [97].
Radiologically hamartomas cannot be reli-
ably distinguished from VSs. Their clinical pre-
sentation is also nonspecific and is related to
facial or vestibulocochlear nerves dysfunction
[71]. The diagnosis of hamartomas is made at
11 Other Cerebellopontine Angle Tumors
histopathological examination, usually intraop-
eratively when a frozen section examination is
performed. These tumors are typically firmer
than VSs and are more tightly attached to the
seventh and eighth nerve complex. Attempt at
complete tumor resection may be related to high
risk of injury to neural structures. Therefore, in
case the tumor cannot be safely dissected or the
dissection causes lasting changes of the electro-
physiological monitoring parameters (EMG,
BAEP, or facial nerve MEP), this most adherent
part of the lesion should be left unremoved (see
Case Illustration 11.9).
Rare Extra-axial Cerebellopontine Angle Lesions
Case Illustration 11.9
IAC Hamartoma
A 40-year-old man presented with progressive
hearing loss and tinnitus on the right side for a
period of 16 months. The neurological exami-
nation revealed only hypacusis on the right
side: the hearing loss according to the audio-
gram was 30 dB in the main speech area.
Enhanced MR T1-weighted images demon-
strated a small lesion in the lateral one-third of
the IAC, which was supposed to be VS (a, b).
The patient was operated via the RS
approach, and the posterior wall of the IAC
was removed widely up to the fundus. The
tumor was found to have very hard consis-
tency. Arachnoid plane between the tumor
a b
(a, b) Native and contrast-enhanced T1-weighted MR
study of the patient. The lesion is seen only after gad-
olinium administration (b, arrow). (c) BAEP moni-
toring, demonstrating the reversible changes that
occur due to various surgical manipulations. Note the
repeated decrease of the amplitude and loss of com-
ponents, in particular of waves I, II, and III. (d) Initial
745
and adjacent structures was missing, and both
the facial and cochlear nerves were infiltrated.
Even the slightest manipulations of the lesion
caused major changes of the BAER (c) and of
the facial nerve EMG. Complete resection
with functional nerve preservation of these
nerves was hence deemed impossible and the
tumor was removed partially (d, e). The
patient recovered very rapidly from surgery.
He had normal facial nerve function and a
slightly deteriorated hearing function—hear-
ing loss of 40 dB in the main speech area.
Histopathological result was glioneural hama-
rtoma. The residual lesion remained
unchanged for the period of follow-up (>18
months).
view of the lesion, located in the lateral third of the
IAC (the posterior wall of the IAC has been removed
widely up to the fundus). (e) Partial removal of the
lesion and preservation of the facial and cochlear
nerves (Reprinted from: Carvalho et al. [71].
Copyright © 2003, with permission, Wolters Kluwer
Health)
746 11 Other Cerebellopontine Angle Tumors

II IV
C I III V
Healthy side

Tumor side, start

After drilling: hard tumor in IAC

Mobilization in IAC: wave I splitting

Dissection in IAC:
wave I amplitude decrease
Puting medial, away from function:
no waves

Release: V returns

Tumor biopsy: I, III and V present

Observation

Further resection: I and III lost

Release

Slow recovery, histology unclear

Decision: priority to function

partial resection

Complete wave recovery

150 w

0 10 m
Rare Extra-axial Cerebellopontine Angle Lesions 747

d e
748
Lipomas
Lipomas are rare extra-axial CP angle tumors:
approximately 100 cases have been reported in
the literature [105]. They are estimated to repre-
sent 0.14 % of all CP angle and IAC tumors
[158]. Patients with CP lipomas typically present
with cranial nerve symptoms/signs: hearing loss,
tinnitus, dizziness, trigeminal neuralgia, and/or
facial neuropathies [67].
Lipomas are reliably diagnosed with T1-weighted
and fat-suppression MRI (Fig. 11.7a, b). On native
a b
Fig. 11.7 This 50-year-old female presented with a his-
tory of hearing loss, vertigo, tinnitus, and gait instability
for 2 years. CT (a) and MRI (b) demonstrated characteris-
tic abnormal signal within the CP angle (arrow) that was
consistent with lipoma (hyperintense on T1, hypointense
11 Other Cerebellopontine Angle Tumors
T1 sequences, the tumor is hyperintense and does
not enhance after gadolinium application [67, 82].
No signal on fat-suppression sequences is charac-
teristically seen.
Patients with CP angle lipomas are followed
with regular neuroimaging and clinical control.
Surgical treatment is rarely indicated—in case of
intractable cranial neuropathies or brain stem com-
pression [67, 105, 122, 158]. Another indication
for surgery may be to achieve definitive histologi-
cal diagnosis, for example, in case liposarcoma
is suspected [82]. Lipomas are typically well
on T2, no mass effect or contrast enhancement). At sur-
gery, the diagnosis was confirmed and a partial removal
(internal debulking) was performed (c) (Reprinted from:
Schuhmann et al. [146])
Rare Extra-axial Cerebellopontine Angle Lesions
vascularized, do not respect natural borders, and are
densely adherent or infiltrate surrounding tissues,
in particular the brain stem and cranial nerves. The
attempt to remove them completely may be very
hazardous. The goal of surgery should be rather
decompression of neural structures than complete
tumor removal. Tankere et al. [158] reviewed the
treatment outcome in 98 patients with CP lipomas
(literature and own material). In most cases (67 %),
partial tumor removal was performed. After sur-
gery, the existing neurological symptoms remained
unchanged in 9.2 % and improved in 50 % of the
patients. New postoperative deficits developed in
two-thirds of the patients. Preservation of hearing
was possible in only 26 % of the patients.
Neurenteric Cysts
Neurenteric cysts, also known as enterogeneous,
endodermal, respiratory, or bronchogenic cysts,
are rare benign congenital lesions of the central
nervous system [59, 89, 143]. They occur at any
level of the neuraxis but most commonly are
found in the lower cervical and upper thoracic
regions [53]. They are generally located intradur-
ally ventral to the spinal cord [85]. Intracranial
neurenteric cysts are even more rare with the
majority—more than 90 %—located in the poste-
rior fossa [52, 59, 89]. The typical location of
such cysts is along the midline: anterior to the
brain stem (50 %), within the fourth ventricle
(21 %), or extending into the cervical canal
(18 %) [59]. The CP angle is involved in 17 %.
Spinal neurenteric cysts are associated fre-
quently (in 40–69 % of the cases) with other con-
genital malformations: vertebral body dysgenesis,
meningomyeloceles, segmentation abnormalities,
spina bifida, and respiratory or enteric tract malfor-
mations [131]. They may be connected by a fibrous
tract, fistula, or cleft to structures derived from the
primitive gut in the thoracic or abdominal cavities
[62]. Contrary to that, intracranial neurenteric cysts
are typically isolated congenital malformations.
Neurenteric cysts are thought to arise during
the embryologic development of the notochord
due to a failed dissolution of the transient neuren-
teric canal between the notochord and the foregut
749
or the respiratory buds. Their frequent associa-
tion with other congenital malformations is
regarded as an indirect confirmation of this the-
ory [91]. The walls of the cyst is lined with sim-
ple or pseudostratified cuboidal-to-columnar
epithelium with a basement membrane resem-
bling those of respiratory and intestinal tracts
mucosa, while the cyst is filled with mucin-like
secretions [101]. This fluid secretion leads to pro-
gressive cyst enlargement. The intracystic fluid is
usually milky, xanthochromic, or transparent
[91]. The endodermal origin of neurenteric cysts
determines their immunohistochemical charac-
teristics. They usually stain positively for keratin,
carcinoembryonic antigen, and epithelial mem-
brane antigen and negatively for glial fibrillary
acidic protein, neuron-specific enolase, S100,
and vimentin.
Neuroimaging
The imaging characteristics of neurenteric cysts
can be quite variable due to the presence of pro-
teinaceous or other hydrophilic contents [75]. CT
usually reveals a well-defined, noncalcified
hypodense lesion. On T1-weighted MR images,
they may appear hypointense, isointense, or
slightly hyperintense, depending on the content
of the cyst. They are typically hyperintense on
T2-weighted and FLAIR images. Peripheral rim
enhancement of the cyst wall is very rarely seen
[63, 91, 143].
The signal intensity of the cyst depends on its
protein content. It can rarely mimic CSF when
this content is low [74], but it is often isointense to
hyperintense relative to brain parenchyma on
T1-weighted images and hyperintense on
T2-weighted images when the protein concentra-
tion is high. Neurenteric cysts have low signal
intensity on DWI sequences (Fig. 11.8g). These
imaging features may enable their differentiation
from epidermoid cysts, which may exhibit similar
intensities on conventional sequences but have
characteristic high signal intensity on DWI [149].
Clinical Presentation
Neuroepithelial cysts are frequently detected
incidentally. Occasionally they may produce pro-
gressive neurological symptoms and/or chronic
750 11 Other Cerebellopontine Angle Tumors

a b

c d

Fig. 11.8 Typical MR characteristics of neurenteric cysts. (a) T1-weighted native sequence. (b–d) T1-weighted
sequences after gadolinium administration. (e) T2-CISS sequence. (f) FLAIR sequence. (g) DWI sequence

increased intracranial pressure [59]. Clinical Management

symptoms are due to the enlargement of the cyst,
caused by active secretion by the goblets cells
and compression of neighboring neurovascular
structures. Rarely, small microcysts can rupture
into the subarachnoid space, leading to recurrent
aseptic meningitis [143].
CP angle cysts lead to dysfunction of the fifth,
seventh, eighth, or of the lower cranial nerves and
corresponding symptoms. Further common com-
plaints are headaches, gait disturbance, and motor
and sensory disturbances.
The treatment of choice of neurenteric cysts is
complete excision of both the cyst contents and its
capsule (see Case Illustrations 11.10 and 11.11).
Importantly, the dissection should not cause injury
to the neighboring neurovascular structures
[59, 91]. In case of strong adhesion to surrounding
structures, small parts of the cyst wall may be left
in place to avoid postoperative morbidity (see Case
Illustration 11.10). Close clinical and neuroradio-
logical follow-up of these patients should be per-
formed to detect early possible cyst recurrence.
Rare Extra-axial Cerebellopontine Angle Lesions 751

e f

g
752
Case Illustration 11.10
Neurenteric Cyst 1
The CP angle tumor in this patient was discov-
ered incidentally after a road traffic accident.
At presentation he did not have any symptoms
and the neurological examination was normal.
MRI showed a lesion with a predominant loca-
tion in the left CP angle with an extension to
the contralateral side and encasement of the
basilar artery (a–d). The lesion was molded
around the brain stem with a relatively less
compressive effect, indicating its possible soft
consistence. The presumptive diagnosis was
epidermoid tumor. After thorough discussion
with the patient and his family, it was decided
to proceed with surgery in order to avoid neu-
rological deterioration in the future. The man-
agement concept was to remove the major
tumor bulk via a left-sided retrosigmoid
approach and—if necessary—to remove the
remaining part via a second right-sided
retrosigmoid craniotomy later on.
The RS approach was performed, the lat-
eral cerebellomedullary cistern was opened,
and sufficient CSF was drained, thus exposing
the cystic tumor without any retraction of the
a b
Preoperative CT (a) and MR images (b–d) of the
lesion, which was hyperintense on both T1- and
T2-weighted sequences. Consecutive steps of the cyst
removal. (e) Initial view of the cyst (arrows) located
medial to the seventh–eighth (7–8) and the lower
cranial nerves (asterik). (f) Opening of the cyst and
11 Other Cerebellopontine Angle Tumors
cerebellum (e). The cysts’ capsule was opened,
and the fluid contents, which were yellowish
and milky, were aspirated (f, g). The capsule
was then dissected stepwise; the more solid
tumor parts were exposed and also removed
(h, i). Although the capsule was found to be
tightly adherent to the brain stem and basilar
artery at certain points, it could be dissected
from them and excised. The very narrow CP
angle provided insufficient access to the con-
tralateral tumor part without endangering the
function of the cochlear and facial nerves,
even if the angled endoscope was used (j).
Therefore, no attempt was made to remove the
contralateral extension of the cyst.
Histopathologically, the lesion was found to
be a neurenteric (respiratory) cyst with focal
squamous epithelium metaplasia without ana-
plastic features and hemosiderin in the cyst
wall, indicating old hemorrhages.
The postoperative period was uneventful;
the patient did not have any neurological
deficit and recovered rapidly. The CT per-
formed 1 day after surgery is shown on figure
k. During a follow-up of more than 60 months,
the cyst remnant remained unchanged.
aspiration of its yellowish contents via the level
between the seventh and eighth and the lower cranial
nerves (f, g) and caudal to the lower cranial nerves
(h). Resection of the safely accessible cyst capsule (i).
(j) View of the CP angle at the end of surgery. (k)
Postoperative CT scan (first postoperative day)
Rare Extra-axial Cerebellopontine Angle Lesions 753

c
d

e f

g
h
754 11 Other Cerebellopontine Angle Tumors

i j

k
Rare Extra-axial Cerebellopontine Angle Lesions 755

Case Illustration 11.11 Following the opening of the dura, a multicys-

Neurenteric Cyst 2
This 46-year-old female patient presented
with a history of fluctuating weakness of the
right leg for 6 months. Her neurological exam-
ination revealed no abnormalities. MR imag-
ing demonstrated an extra-axial lesion in the
foramen magnum area that extended caudally
to the C2 level. The lesion was located ante-
rior and lateral to the medulla and spinal cord
and had a slight compressive effect. It was
isointense on T1-weighted images and slightly
hypointense on T2-weighted images (a–d).
The patient was operated via a lateral suboc-
cipital approach with a limited opening of the
foramen magnum, C1 hemilaminectomy, and
partial C2 hemilaminectomy on the right side.
tic tumor was seen, located around the verte-
bral artery and C1 rootlets. The cysts’ fluid
was thick and milky; its wall was adherent to
the brain stem and vertebral artery. Still, using
the bimanual preparation technique, the lesion
could be removed completely. An angled
endoscope was used at the end to examine the
whole surgical field, including the premedul-
lary space and to verify the completeness of
resection.
The patient recovered quickly from the sur-
gery and did not have any neurological deficits.
Her early postoperative MRI is presented on
figures e–g. Histopathological examination
proved that the lesion was a neurenteric (respi-
ratory) cyst without signs of malignancy.

a b

c d

(a–d) Preoperative T1- and T2- weighted MR images, demonstrating the extension of the lesion and its relation
to the surrounding structures. (e–g) Postoperative MRI control: no remnants can be visualized
756 11 Other Cerebellopontine Angle Tumors

e f

g
Rare Extra-axial Cerebellopontine Angle Lesions
Hemangioblastomas
Cushing and Bailey introduced the term heman-
gioblastoma in 1928: it refers to a benign vas-
cular neoplasm that occurs most frequently in
the posterior cranial fossa. Hemangioblastomas
account for 8–12 % of all tumors in this area
[78, 79, 139]. They are slow-growing tumors
of uncertain histological origin and occur spo-
radically or as a part of Von Hippel–Lindau
disease (VHL). Sporadic tumors present at
40–50 years of age and involve predominantly
the cerebellum. Hemangioblastomas in patients
with VHL present earlier—at 20–30 years of
age—and involve the cerebellum, brain stem,
or spinal cord [128]. Such hemangioblastomas
may be multiple. According to some studies,
up to 20 % of patients with VHL have lower
brain stem or craniocervical hemangioblasto-
mas [78, 92, 135]. Some of them arise in the
area of the obex or in the posterolateral aspect
of medulla oblongata and may infiltrate the
floor of fourth ventricle. Distinct margin to
neural tissue is usually absent, and attempt at
a b
Fig. 11.9 MR images of a patient with multiple heman-
gioblastomas, including a large right-sided cystic CP
angle hemangioblastoma. An opened biopsy has been
performed at another institution and an Ommaya reser-
voir was inserted. The patient presented because of con-
tinuous tumor enlargement. Considering the radiological
tumor characteristics, in particular the absence of clear
plain in relation to the brain stem, the histological tumor
757
radical surgery can cause severe morbidity.
Other tumors arise from the choroid plexus,
develop in the ventricle, and do not invade the
brain stem. The rare cervicomedullary heman-
gioblastomas may extend secondary into the
CP angle.
With respect to their internal structure, heman-
gioblastomas can be cystic, solid, or mixed [152].
Most frequently they are composed of a cherry
red solid nodule, associated with a cyst that con-
tains a clear yellowish fluid.
Hemangioblastomas are most reliably diag-
nosed on MRI (Fig. 11.9). On native T1-weighted
sequences, the solid tumor part is hypointense or
isointense; on T2-weighted images, it is hyperin-
tense [63, 117]. Strong enhancement after admin-
istration of contrast media with clear delineation
from surrounding neural tissue is typical for
hemangioblastomas. The cyst—if present—is
sharply demarcated and has homogeneous CSF-
like signal intensity: hyperintense on T1-weighted
images and hyperintense on T2-weighted images
[63]. Cerebral angiography, which is currently
largely replaced by MR angiography, demonstrates
type, and the rather mild symptoms, a decision was made
to perform tumor embolization. In case of further growth
or neurological deterioration, surgical excision would be
recommended. The follow-up of the patient is 12 months
and the tumor is stable. (a–c) T1-weighted sequences
after gadolinium administration. (d, e) T2-weighted
sequences. (f) Multiple scattered supra- and intracranial
hemangioblastomas
758 11 Other Cerebellopontine Angle Tumors

c d

e f

Fig. 11.9 (continued)

the highly vascular tumor blush. High-flow vessels
may be observed on MRI as flow voids at the
periphery of the mass [63]. A thorough medical
evaluation for other stigmata of VLH, including
MR study of the neuraxis, abdominal CT scan or
ultrasound, and ophthalmological examination, as
well as detailed family history, to exclude multiple
hemangioblastomas should be performed prior to
surgery.
Surgical removal is considered the standard
treatment of hemangioblastomas [78, 154]. They
are benign tumors and may be cured by complete
surgical excision. Exceptions are those cases, in
which the risk of operation outweighs its potential
benefits, such as in patients with poor general
condition. In case of critical location of the tumor
with infiltration of important neurovascular struc-
tures, the attempt at complete removal may have
devastating consequences (see Case Illustration
11.12). Staged multimodal management includ-
ing embolization, surgery, and radiosurgery can
be beneficial in such patients [73, 154].
Asymptomatic lesions in patients with multiple
hemangioblastomas may be initially observed
with regular MRI examinations to rule out tumor
enlargement.
Preoperative endovascular embolization of the
solid tumor component before its resection is very
Rare Extra-axial Cerebellopontine Angle Lesions
useful in large hypervascular hemangioblastomas
of the posterior fossa—see Case Illustration 11.12
[155]. It reduces the tumor vascular supply and pre-
vents major intraoperative bleeding, minimizing the
risk of neurological morbidity [156, 160]. However,
as noted by some authors [115], even superselective
embolization of large hemangioblastomas, espe-
cially those located in the medulla oblongata, is not
without infarction hazards and is not always safely
feasible. Occasionally the embolization inhibits
tumor growth control and even causes tumor shrink-
age. Surgery may be postponed and is required only
if the tumor continues to enlarge.
The operative strategy should be selected indi-
vidually, according to the location and size of the
tumor and of its feeding vessels. Important points
to consider are:
• Early identification and coagulation of feed-
ing vessels.
• Arterial feeders should be coagulated prior to
draining veins.
• In case a large cyst exists, its decompression
allows for improved access and easier delinea-
tion of the interface between tumor and neural
parenchyma.
759
• The tumor is usually well demarcated from
the surrounding brain or spinal cord and has a
clear gliotic margin.
• Its removal is performed by circumferential
dissection along the gliotic margin and divid-
ing its vascular supply.
• The surface of the tumor may be coagulated,
thereby shrinking the tumor. Penetration of
the tumor itself should be avoided because of
its extreme vascularity.
• Attempt to remove a hemangioma piecemeal
can lead to uncontrollable hemorrhage.
• The cyst if present is not composed of tumor
cells; hence, its wall should not be removed.
• In case a distinct dissection plane is absent or
the tumor infiltrates neural or major vascular
structures, no attempt should be made to
remove it completely.
Radiotherapy (fractionated radiotherapy or
radiosurgery) is an adjunct following incomplete
tumor removal or alternative treatment in patients
in poor general condition that obviates major sur-
gery [109, 111]. Of note, hemangioblastomas
with large cystic components are not good candi-
dates for radiosurgery.
760
Case Illustration 11.12
CP Angle Hemangioblastoma
This 25-year-old male patient had been oper-
ated in another neurosurgical facility for a
large tumor in the lower CP angle 5 months
prior to presentation. A biopsy was taken
and—due to extremely strong bleeding from
the tumor—several clips were placed; the sur-
gery was interrupted. The patient remained
ventilated for several weeks and then slowly
recovered. Histopathological diagnosis was
hemangioblastoma. At presentation he com-
plained of deteriorating gait and frequent
vomiting. He had horizontal nystagmus and
incomplete hypoglossal palsy on the right
side. Gag reflexes were absent. Babinski sign
was positive on the left side. The patient had
severe gait and truncal ataxia and was unable
to walk independently. His previous history
was remarkable for “treatment of angiomas”
(lack of precise records) on both eyes.
MRI study showed a large strongly enhanc-
ing tumor in the lower brain stem area and CP
angle (a–d). The tumor extended via the lat-
eral recess into the fourth ventricle and caused
significant brain stem compression. DSA
demonstrated a highly vascular lesion with
supply from the vertebral and the external
carotid arteries.
Considering the tumor size, the severe
brain stem compression, and the progressive
clinical deterioration of the patient, a decision
was made in favor of new attempt to remove
the lesion surgically. Initially superselective
embolization of the external vascular feeders
was performed. At a second stage immediately
before surgery, an attempt to embolize the
11 Other Cerebellopontine Angle Tumors
feeders form the vertebral artery was made.
However, selective occlusion of the tumor
feeders only turned out to be impossible.
The patient was operated via the RS
approach. Exposure of the hemangioblastoma
required meticulous dissection of the severe
adhesions caused by the previous surgery. The
tumor was extremely vascularized and even
slightest manipulation caused strong bleeding
(e). Due to its large size, critical location, and
infiltration of adjacent structures, circumferen-
tial dissection was not a reasonable option.
Alternating bipolar coagulation and removal of
the tumor with the ultrasonic aspirator were
therefore initiated (f). Gradually the caudal and
dorsal parts were dissected and resected from
the brain stem. The lower cranial nerves were
engulfed by the tumor. Nevertheless, we pro-
ceeded with tumor removal until the seventh to
eighth nerves were seen. In the root exit zone
of the facial nerve, the adhesions were so
strong that further resection would inevitably
damage the nerve. The caudal limit of the
resection was the tumor part, engulfing the ver-
tebral artery and its small branches. In general,
significant volume reduction of the tumor and
brain stem decompression was achieved (g).
Following surgery, the patient had incom-
plete abducens nerve palsy on the right side
and was unable to swallow, so that a tracheos-
tomy was placed. After a prolonged intensive
care management (4 weeks), he could be
transferred to a normal ward. His condition
gradually improved. The swallowing recov-
ered and the tracheostoma could be closed. At
discharge the patient was able to take care of
himself and walk without support.
Rare Extra-axial Cerebellopontine Angle Lesions 761

a b

c d

Preoperative T2-weighted (a) and T1-weighted MRI of initial view of the tumor (e), partial removal (f), and
the tumor following gadolinium administration (b–d). view at the end of surgery (g). Postoperative T2- (h)
Note the flow voids, corresponding to large vessels in and T1-weighted MR sequences after contrast applica-
the periphery of the tumor (a). Intraoperative images: tion (i–k) demonstrating the extent of tumor resection
762 11 Other Cerebellopontine Angle Tumors

e f

g
Rare Extra-axial Cerebellopontine Angle Lesions 763

h i

j k
764
CP Angle Metastases
Most secondary CP angle tumors are benign and are
usually of neuroectodermal origin. As recalled by
Tu et al. [159], Cornil reported the first case of CP
angle metastasis in the 1930s. With the widespread
of modern neuroimaging, secondary metastatic
tumors to the CP angle and IAC are increasingly
frequently being diagnosed [56, 77, 99].
Metastases comprise 0.2–0.7 % of all CP
angle tumors [66, 125]. They spread to the area
either by hematogenous or by leptomeningeal
dissemination [168]. The three most common
primary malignancies that metastasize to the CP
angle are breast and lung carcinoma and mela-
noma [66, 150, 167]. Other tumors reported to
metastasize to the area are colon carcinoma, renal
cell carcinoma, adenocarcinoma, nasopharyngeal
carcinoma, thyroid carcinoma, ovarian carci-
noma, gallbladder carcinoma and lymphoma
[54, 94, 102, 112, 136, 167].
The radiologic appearance of metastatic
tumors can mimic that of common benign lesions
of the CP angle such as schwannoma and menin-
gioma [168]. They are usually smaller lesions,
isointense or hypointense on T1-weighted MR
sequences and hypointense on T2-weighted MR
sequences, and show strong enhancing after gad-
olinium administration [63, 162]. Perifocal edema
in the adjacent cerebellum/brain stem, which is
seen as high signal on FLAIR and T2-weighted
images, can be observed. Warren et al. [162]
pointed that metastatic tumors are eccentric from
the IAC because they do not arise from the sev-
enth to eighth nerves but from the flocculus and
choroid plexus. Further clues are the evidence of
bilateral CP angle lesions and the presence of
multifocal cerebral or neck lesions
[162]—Fig. 11.10.
Clinically metastatic tumors in the CP angle
cannot be distinguished from benign tumors.
Still, some clinical factors should raise the suspi-
cion of malignancy: rapid clinical deterioration,
advanced age at presentation, progressive sen-
sorineural hearing loss, facial nerve weakness,
bilateral cranial nerve symptoms, and history of
11 Other Cerebellopontine Angle Tumors
Fig. 11.10 Contrast-enhancing lesion in the left CP
angle and IAC. The presence of a second round cerebellar
lesion with similar imaging characteristics raised the sus-
picion of metastases. Clinical work-up revealed multiple
lung metastatic lesions and the primary focus—a breast
carcinoma
cancer [90, 150, 159, 162]. Metastases cause
neurological dysfunction by compression or
direct infiltration of neural structures. The mass
effect of the tumor may be further enhanced by
the surrounding edema. Hence, even small metas-
tases—in contrast to benign CP tumors—may
cause major neurological dysfunction. Facial
nerve weakness at presentation is relatively fre-
quently seen in metastatic tumors but is excep-
tional in VSs and rare in CP angle meningiomas.
On the other hand, if patients with known pri-
mary cancer have symptoms such as vertigo, tin-
nitus, hearing loss, or facial palsy, metastasis in
the CP angle—despite its rarity—should be sus-
pected [66, 125]. Huang and Young [102] exam-
ined radiologically 174 cancer patients with such
symptoms and found CP angle tumors in just 6 of
them. Interestingly only 3/6 tumors turned out to
be metastatic: a history of previous malignancy
and a secondarily discovered CP angle lesion
does not necessarily imply a metastasis.
Generally, metastases to the CP angle are
believed to have rapid clinical course and carry
Rare Extra-axial Cerebellopontine Angle Lesions
poor prognosis. In patients with CP angle tumors
that turn out to be metastatic, the primary focus
should be identified and managed appropriately.
The management of CP angle tumors in patients
with known cancer should be part of their general
treatment concept. Factors to consider are general
765
condition of the patient, primary tumor control,
presence of multiple metastases, as well as size,
location, and symptoms caused by the CP angle
tumor. Surgical removal of isolated CP angle
metastases along with appropriate chemotherapy
and radiation can provide local tumor control.
766 11 Other Cerebellopontine Angle Tumors

Case Illustration 11.13 followed with MRI studies. Several weeks

Metastatic Tumor to the CP Angle
This 54-year-old female patient suffered from
an invasive ductal carcinoma of the right breast.
Partial breast resection had been performed
1 year ago, followed by therapy with Herceptin
(the HER-2-neu was positive). She has been
diagnosed to have 2 lesions in the posterior
cranial fossa, which were highly suspicious for
metastases. The large tumor in the right cere-
bellar hemisphere—metastasis from the ductal
carcinoma—has been removed surgically 2
months prior to presentation at our department
and whole brain radiation was planned. The
second one, located in the left CP angle, was
prior to presentation, increasing weakness of
the extremities and headache appeared. MRI
demonstrated a certain growth tendency of the
tumor and a compressive effect on the brain
stem and cerebellum (a, b).
Considering the size of the tumor, its pro-
gressive enlargement with resultant clinical
deterioration and the good general condition
of the patient, as well as her personal wish, a
decision was taken to remove the tumor surgi-
cally. The RS route was selected, and the
tumor could be removed completely, preserv-
ing the function of the seventh to eighth cra-
nial nerves.

a b

(a, b) MRI study of a patient with breast carcinoma metastases

Rare Extra-axial Cerebellopontine Angle Lesions
Collision Tumors
Collision tumors are defined as neoplasms that
grow in one area, collide, and become intermin-
gled [118, 157]. In the CP angle area, such
findings have been occasionally reported.
Muzumdar et al. [127] described a case of a col-
lision tumor comprising a vestibular schwannoma
and a petroclival meningioma in a 30-year-old
male patient. Both tumors were resected via a ret-
rosigmoid route: the caudal portion of the mass
proved to be VS, while the rostral portion in the
petroclival was a meningioma. The authors’ lit-
erature review showed that occurrence of VS and
a petroclival meningioma in “collision” has never
been reported. Another mechanism is the devel-
opment of carcinoma metastases in a primary CP
angle tumor, usually in VS. Leeman et al. [118]
reported on a case of breast carcinoma metasta-
sizing to a CP angle meningioma and forming a
collision tumor.
Izci et al. [106] described a schwannoma and
meningioma in the CP angle in a 57-year-old
patient that were not in continuity. The tumors
were removed successfully via the retrosigmoid
approach. Wilms et al. [163] presented a vestibu-
lar schwannoma extending into the IAC and a
meningioma with broad implantation on the
Fig. 11.11 MR images of a combined schwannoma and meningioma in a single tumor (Reproduced from: Lüdemann
et al. [120]. Copyright © 2000, with permission from Wolters Kluwer Health)
767
petrous apex that were also clearly separate.
Similar to these cases, one of our patients had a
coincidental occurrence of two different tumors
in the CP angle that were not in contact and can-
not be strictly defined as “collision tumors” (see
Case Illustration 11.14). According to some
authors, “combined” or “mixed” tumors have
been found in up to 25 % of all schwannomas in
patients with NF-2 [93]. In sporadic cases, how-
ever, the association is rare. In the case described
here, however, no history of previous irradiation
or association with NF-2 was present. The possi-
bility of such concomitantly growing tumors in
the CP angle should be considered when plan-
ning the surgical strategy.
The condition should be differentiated from
the development of combined schwannoma and
meningioma in a single tumor, which we have
reported earlier [120] in a 59-year-old female
patient with VS that was found to infiltrate the
facial nerve—Fig. 11.11. In order to achieve
complete tumor removal, the facial nerve was
cut and then reconstructed with an interposi-
tion sural nerve graft. Interestingly, the histo-
logical and immunohistochemical examination
showed that the tumor was a typical schwan-
noma with intermingled islets of meningothelial
meningioma.
768
Case Illustration 11.14
Sporadic Unilateral VS and Petroclival
Meningioma
This patient (55 years old) presented with a
3-year history of occipital pain and vertigo.
Since 1 year she noticed that hearing on the
left side deteriorated progressively. Later on
bilateral papilledema was diagnosed and MRI
study was performed, which demonstrated a
large CP angle tumor and obstructive hydro-
cephalus. Ventriculoperitoneal shunt was
therefore placed. At presentation the patient
had trigeminal hypesthesia (V1–3) and com-
plete hearing loss on the left side, as well as
severe gait instability. MRI examination
showed 2 separate CP angle tumors: a large
VS (extension grade T4b) and a petroclival
tumor, most probably a meningioma that
invaded the posterior cavernous sinus (neu-
roimaging: a–f, before surgery, and e–i, after
surgery). Considering the size of the tumors,
the extent of brain stem compression, and the
clinical condition of the patient, the primary
goal of surgery was to remove the VS und
(a–i) MR images of the patient with two different spo-
radic tumors in the left CP angle: VS and petroclival
meningioma that extends into the posterior cavernous
sinus. Although both tumors show intense contrast
enhancement, the signal characteristics are obviously
different. (a–c) T1-weighted MR sequences following
contrast administration. (d) T2- CISS sequence.
Postoperative images: (e) CT scan 1 day after surgery.
(f, g) T1-weighted MR sequences following contrast
administration. (h, i) T2-CISS sequences. The VS is
11 Other Cerebellopontine Angle Tumors
possibly to remove the posterior cranial fossa
part of the meningioma.
The patient was operated via the RS
approach. Complete removal of the VS and
preservation of the facial nerve was achieved
(j–l). The petroclival tumor, which proved to
be a meningioma, was engulfing the abducens
nerve. It was removed carefully along this
nerve until the Dorello’s canal was reached.
Complete removal of the tumor was not
attempted due to the infiltration of the cavern-
ous sinus. The patient recovered well and at
discharge did not have any new neurological
deficits besides a slight (HB grade II) facial
weakness on the left side. The postoperative
MR study is presented on figures f–i.
As discussed in detail in the chapter on
Neurofibromatosis, the occurrence of unilat-
eral VS and additional ipsilateral meningioma
may be observed in the mosaic type of NF-2,
caused by a mutation at a later stage of embryo-
genesis. In this case, however, no clinical clues
for NF-2 existed. Genetic testing was recom-
mended but not performed by the patient.
removed completely and the IAC is plugged with fat,
generating the characteristic signal on T1 and T2
sequences. (j–l) Intraoperative images. (j) The VS has
been removed completely and the facial nerve (arrows)
has been preserved. Note the widely opened IAC. (k)
The IAC has been sealed with fibrin glue and fat
pieces. (l) In the depth of the operative field—in the
petrous apex area—a meningioma was seen (C) and
was also removed completely. (5 trigeminal nerve, BS
brain stem, arrowheads lower cranial nerves)
Rare Extra-axial Cerebellopontine Angle Lesions 769

c
770 11 Other Cerebellopontine Angle Tumors

d e

f g
Rare Extra-axial Cerebellopontine Angle Lesions 771

h i
772 11 Other Cerebellopontine Angle Tumors

j k

l
5

BS
Rare Extra-axial Cerebellopontine Angle Lesions
Vascular Lesions
Aneurysms, arteriovenous malformations,
venectasias, or tortuous segments of the vertebral
and basilar arteries or some of their branches can
present as CP angle mass and have to be differen-
tiated from neoplastic lesions [64, 80, 104, 124].
They become clinically apparent either with acute
symptoms due to SAH or with more insidious
symptoms of a CP angle mass lesion [80].
CP Angle Aneurysms
Giant or fusiform aneurysms extending to the CP
angle usually arise from the vertebral or basilar
arteries [76, 110]. Peripheral aneurysms of the
anteroinferior cerebellar artery are rare, with a
reported incidence between 0.0003 and 0.5 %
[55, 123]. Typically they develop at the meatal loop
or from the dorsolateral arterial branch. Peripheral
aneurysms of the superior cerebellar artery in the
CP angle also have been reported [114]. Extension
into the IAC is exceedingly rare: according to Sun
et al. [153], only 13 cases of aneurysms located in
the IAC have been so far reported in the literature.
In case of high-flow aneurysms, MRI demon-
strates oval or round masses that have flow void
773
(no signal) on all spin-echo sequences. Extreme
hypointensity on T2-weighted MR images is
highly suggestive for such lesions. Thrombosed
aneurysms with methemoglobin content have
hyperintense signal on T1-weighted images
(Fig. 11.12). Importantly, enhancement of the
mass can be observed due to organization of the
thrombus and has to be differentiated from neo-
plasms [126].
Patients with distal anteroinferior cerebellar
or superior cerebellar artery aneurysms are usu-
ally managed surgically because the endovascu-
lar treatment seems to be less suitable.
Developmental Venous Anomalies
of CP Angle
Developmental venous anomalies (DVAs) are
regarded as extreme variations of normal venous
drainage, despite the fact that structural abnor-
malities of their walls have been reported [140].
They are thought to represent anatomically disor-
dered but physiologically essential venous drain-
age of surrounding normal cerebral tissue
and—due to the high risk of venous stroke—
should not be directly treated (see Case
Illustration 11.15).
774 11 Other Cerebellopontine Angle Tumors

a b

Fig. 11.12 T1- and T2-weighted MR images of a giant partially thrombosed aneurysm of the basilar artery, presenting
as a CP angle mass
Rare Extra-axial Cerebellopontine Angle Lesions
Case Illustration 11.15
Developmental Venous Anomaly
A 51-year-old male patient presented to the
senior author in the early 1990s, complaining
from vertigo, aggravated by vertical eye move-
ments. His neurological examination revealed
no deficits. Native and contrast-enhanced CT,
MRI, and DSA were performed (a–d) and
demonstrated a lesion in the right cerebellum
with characteristics of a developmental venous
anomaly. In the right CP angle, a contrast-
enhancing mass that could not be unequivo-
cally related to the DVA was seen.
The patient was operated via the RS approach,
and a complex venous malformation tightly
adherent to and compressing the seventh to eighth
nerve complex was seen (e, f). The adhesions
were carefully dissected; the lesion was mobi-
lized away from the nerves and coagulated (g).
a b
The CT (a, b), MRI (c), and venous phase angiogra-
phy (d) images show the typical appearance of a
developmental venous anomaly in the right cerebel-
lum. The tributary and the dilated collector veins are
well seen. An enhancing mass with unknown rela-
tion to the DVI is visible in the right CP angle (b, c).
(e–g) Intraoperative images demonstrating the
775
The patient could be extubated soon after
the surgery and did not have any neurological
deficits. Few hours later, however, he lost con-
sciousness. CT was performed immediately
and revealed massive edema of the brain stem
and the right cerebellar hemisphere (h). The
patient was intubated and appropriate antie-
dematous therapy was started. He regained
consciousness quickly but suffered from cere-
bellar ataxia and glossopharyngeus palsy with
dysphagia and oculomotor palsy on the right
side.
On the fifth day after surgery, he was suc-
cessfully extubated and begun slowly to
improve (i). CT control 30 days later (j)
showed significant reduction of the edema. All
neurological deficits resolved completely
within the following months, and at 6 months
follow-up, he had no neurological symptoms.
dilated veins and their relation to the seventh to
eighth cranial nerve complex (e, f). (g) The veins
have been dissected form the nerves and coagulated.
(h–j) CT scans of the patient demonstrating the
extensive venous infarction and its subsequent reso-
lution (h: second day, i: fifth day, j: 30 days)
776 11 Other Cerebellopontine Angle Tumors

c d

e f

g
Rare Extra-axial Cerebellopontine Angle Lesions 777

h i

j
778
CP Angle Arteriovenous Malformations
Arteriovenous malformations (AVM) develop
primarily within the CP angle or in the IAC very
rarely. Nozaki et al. [130] reported on eight
patients with such AVMs: all with hemorrhage as
initial presentation. On admission all patients had
some cranial nerve palsy and three patients had
cerebellar ataxia. The goal of treatment of CP
angle/IAC AVMs is complete excision and avoid-
ance of new neurological deficits [86, 151]. The
senior author’s experience encompasses four
intracanalicular AVMs treated surgically
(Fig. 11.13). In all cases, the malformation could
be removed completely and the functional out-
come was excellent.
Cavernomas
Cavernous malformations or cavernomas are vas-
cular malformations, composed of sinusoidal
vascular spaces separated by connective tissue
a b
c d
Fig. 11.13 (a, b) Intraoperative images of the subsequent
steps of resection of an AVM, extending deep into the
IAC. Its complete and safe removal necessitated wide
11 Other Cerebellopontine Angle Tumors
without intervening neural elements and sur-
rounded by a gliotic peripheral capsule. They
occur with an incidence of 0.3–0.5 % and consti-
tute 10–20 % of all vascular malformations [61].
Extra-axial cavernomas of the cranial nerves are
uncommon.
Cavernomas develop in the IAC or CP angle
exceedingly rarely [66, 95, 113, 145]. They may
present with mass effect and have to be differen-
tiated from tumors developing in the area.
Cavernomas of the IAC are thought to arise from
the capillary plexus of the epineurium of Scarpa’s
ganglion in the IAC—see Case Illustration 11.16
[58]. Some lesions may extend from the IAC into
the CP angle. On the other hand, brain stem cav-
ernomas may have an exophytic part, extending
into the CPA (Fig. 11.14).
The clinical presentation of such cavernomas
is related to compression and/or dislocation of
the adjacent cranial nerves and is similar to that
opening of the IAC (c, d). The patient presented with
increasing hearing deficit and vertigo, which after the sur-
gery showed a gradual recovery
Rare Extra-axial Cerebellopontine Angle Lesions 779

a b

Fig. 11.14 Pontine cavernoma with a large exophytic
parapontine part in a 33-year-old male patient. (a–e)
Preoperative images. Note the large DVA, extending into
the CP angle. The lesion was removed via a midline pos-
terior fossa craniotomy. (f–j) Intraoperative images,
demonstrating the gradual removal of the cavernoma (BS
brain stem, C cerebellum, R retractor). Following partial
removal, the PICA and the DVA (arrowhead) became
visible. Then, the ventral exophytic part, adherent to the
seventh to eighth cranial nerve complex (*), was
removed. Complete removal was possible with preserva-
tion of the DVA (arrowhead) and of the cranial nerves
(*). (k) Postoperative MRI, demonstrating the
intact DVA
780 11 Other Cerebellopontine Angle Tumors

e f
C

BS

Fig. 11.14 (continued)

Rare Extra-axial Cerebellopontine Angle Lesions
i j
Fig. 11.14 (continued)
of VSs. In case of bleeding, abrupt onset of symp-
toms or worsening of existing symptoms occurs.
Clinical signs/symptoms include sensorineural
hearing loss, tinnitus, dizziness, vertigo, and pro-
gressive facial nerve palsy [58, 95, 148]. Important
clues to the diagnosis are the presence of facial
weakness and rapidly progressive or abrupt hear-
ing deficit [84, 148]. A possible vascular genesis
could be suspected in case of episodic exacerba-
tion or disproportionate severity of the symptoms
regarding the small size of the lesion.
Radiologically it may be difficult to distinguish
a cavernoma from a VS or other IAC/CP angle
lesions—Fig. 11.15. They are well-circumscribed
lesions of mixed signal intensity on T1-weighted
781
sequences and hyperintense signal on
T2-weighted sequences with variable contrast
enhancement [58, 132, 145]. Typically, caverno-
mas are surrounded by a peripheral rim of hemo-
siderin that shows hypointensity on all sequences
[61, 64, 84]. Tumors with hyperintensity on T2
sequence are rare in the IAC/CP angle. In gen-
eral, the differential diagnosis is between lesions
containing fat, melanin, or intratumoral hemor-
rhage: hemorrhagic tumor (particularly VS),
cavernoma, dermoid, lipoma, melanoma, and
cholesterol granuloma [161]. CT scans may
demonstrate calcium deposits and/or IAC
enlargement in lesion with intracanalicular
extension.
782 11 Other Cerebellopontine Angle Tumors

a b

Fig. 11.15 Pure intracanalicular cavernoma. (a) CT scan administration. (c) View of the cavernoma after opening
with bone-window setting: the IAC is significantly wid- of the IAC. (d) Complete removal of the cavernoma and
ened. (b) T1-weighted MR image following contrast preservation of all cranial nerves
Rare Extra-axial Cerebellopontine Angle Lesions 783

Case Illustration 11.16
Cavernoma in the IAC
This 43-year-old patient presented to the
senior author in 1992 with a history of gait
imbalance. CT and MRI were performed,
which demonstrated widened IAC and an
intracanalicular tumor on the left side. Taking
into consideration the possible management
options, the patient decided for follow-up
with regular MRI and hearing level examina-
tions. However, the next MRI was performed
more than 3 years later when he noticed pro-
gressive hearing loss (a). Despite the fact that
the tumor showed a growth tendency, the
patient decided to postpone surgery. One year
later, he presented with severe vestibular
problems, further hearing loss, and tinnitus.
The patient was operated via the RS approach
(b). The lesion, which turned out to be a cav-
ernoma, was removed completely, and both
the facial and the cochlear nerves could be
preserved.

a b

(a) Preoperative MRI, demonstrating the intracanalicular cavernoma. (b) Intraoperative photomicrograph of the
lesion, located in its lateral part of the IAC
784
The treatment of choice of IAC/CP angle cav-
ernomas is total microsurgical resection in order
to prevent recurrences. At surgery intense adhe-
sions between the cavernoma and the seventh or
eighth cranial nerves are often found. Pappas
et al. [134] found dense attachment of the caver-
noma to the facial nerve in five of their six cases.
Considering the high risk of recurrence and the
inevitably related to it loss of function, complete
cavernoma removal even with the option of
reconstruction of the injured/interrupted facial
nerve should be aimed even in such cases.
Although the issue of the most appropriate tim-
ing of cavernoma surgery is controversially
addressed in the literature, we believe that early
surgery after the diagnosis is related to best func-
tional outcome: multiple bleedings may irrevers-
ibly injure the cranial nerves. Moreover, the
chances for preservation of the facial nerve are
lower in larger lesions.
The optimal approach is the RS one—it offers
an excellent overview of the cavernoma and of
the whole CP angle and IAC (see Case Illustration
11.17). The lesion is identified on the basis of its
typical reddish-blue color. Its relation to the cra-
nial nerves and the degree of IAC extension
11 Other Cerebellopontine Angle Tumors
should be carefully assessed. Once the IAC is
opened, an attempt is made to identify the facial
and cochlear nerves medial and lateral to the cav-
ernoma. In large lesions, internal decompression
is performed initially, followed by dissection of
the capsule from the neural structures.
In all 7 patients, operated by the senior author,
the lesion was located in the IAC and extended
into the CP angle in only 2 cases. Complete cav-
ernoma removal was achieved in a single-stage
surgical procedure in all cases. The facial nerve
could be preserved in 5 cases and the cochlear
nerve in 4 cases. The intimate adherence between
the facial nerve and the lesion rendered complete
removal without its sacrifice impossible in 2
cases; one of these patients had pronounced facial
nerve paresis before surgery. Facial nerve recon-
struction with a sural nerve graft was performed
in both cases.
Early after surgery, some degree of facial palsy
was noted in all patients. At follow-up, however,
it improved to House–Brackmann grade I in 1
patient, grade II in 1 patient, and grade III in the
remaining 5 patients. Hearing preservation was
not an issue because all patients had severe hear-
ing loss at presentation.
Rare Extra-axial Cerebellopontine Angle Lesions
Case Illustration 11.17
IAC Cavernoma Extending in the CP Angle
Twenty-three-year-old female patient presented
with a history of progressive hearing loss. Two
weeks prior to admission, she experienced sud-
den facial weakness on the left side (HB grade
III) that in 5 days worsened to HB grade IV.
Audiogram revealed left-sided hearing loss of
60–70 dB. CT showed widening of the left IAC,
while MRI demonstrated an intra- and extra-
canalicular lesion with signal characteristics,
suspicious for recent hemorrhage (a–e). The
patient was operated via the RS approach (f–i).
a b
Preoperative images of the cavernoma. (a) Bone-
window CT demonstrates an enlargement of the IAC.
(b) CISS sequence. (c) TOF sequence. (d) T2-weighted
sequence. (e) T1-weighted sequence. Intraoperative
images presenting the stages of cavernoma removal.
(f) Initial view of the cavernoma (arrow), protruding
into the CP angle. Arrowhead—seventh and eighth
785
A reddish-blue lesion was seen, which was inti-
mately adherent to the seventh to eighth nerves
and highly suspicious for a cavernoma. The
lesion was found to originate within the IAC
and to protrude along the nerves in the CP angle.
It could be completely removed, and—despite
the dense adhesions to the nerves—both the
facial and the cochlear nerves were preserved
anatomically. Histopathological examination
proved that the lesion was a cavernoma. The
postoperative MR study is presented on figures
j and k. The facial palsy improved steadily and
at 12 months follow-up was HB grade II.
nerve complex, P pyramid. The posterior wall of the
IAC has been removed, and the intracanalicular part is
seen and gradually dissected from the nerves (g).
(h) Complete removal of the cavernoma and preserva-
tion of both cochlear and facial nerves (small arrows).
Postoperative T1-weighted MR images, following
contrast application (i, j)
786 11 Other Cerebellopontine Angle Tumors

c d

f
e

g
Rare Extra-axial Cerebellopontine Angle Lesions 787

h i

j
788
Neurocysticercosis
Neurocysticercosis is caused by infestation of the
central nervous system by the larval form of
Taenia solium—the cysticerci [81, 138]. Their
radiological and clinical manifestations are
highly variable and are related to the location of
the lesion and the host response. Neurocysticercosis
in the CP angle have been reported and usually
occur in the racemose form [72, 129]. Their
pathognomonic radiological sign, which however
is not frequently seen, is a 1–3-mm mural nodule
in the cyst (the invaginated scolex). They may
appear as lobulated cysts with no mural nodule or
enhancement, as focal cisternal widening or as a
high signal rim due to surrounding adjacent tis-
sue reaction and have signal intensity similar to
that of CSF with all sequences [87]. The differen-
tial diagnosis includes other cystic lesions: arach-
noid cyst, epidermoid cyst, cystic schwannoma,
and cystic meningioma.
Temporal Bone and Petrous
Apex Lesions
Neoplastic or inflammatory lesions that originate
from the posterior surface of the petrous bone or
the petrous apex may present as CP angle masses.
They have to be differentiated from incidental
nonoperative findings, such as asymmetric fatty
marrow, trapped fluid, or variations of the struc-
ture of normal bone [239]. The petrous apex is
marrow filled in 84 %, pneumatized in 9 %, and
sclerotic in 7 % of temporal bones [188].
Asymmetric pneumatization of the petrous apex
may be confused with a neoplasm. The differen-
tial diagnosis is based on the absence of bone
destruction or expansion on CT, the absence of
contrast enhancement, and the hypointensity of
the signal on T2-weighted MR sequences [175].
Cholesterol granulomas are the most common
lesions intrinsic to the petrous apex [270]. They
are estimated to be ten times more common than
cholesteatomas and 40 times more common than
petrous apex mucoceles [175]. Mucoceles are
nonenhancing lesions, developing due to occlu-
sion of the petrous air cells. They are hypointense
11 Other Cerebellopontine Angle Tumors
on T1-weighted MR images and hyperintense on
T2-weighted MR images. Symptomatic mucoce-
les may require drainage. Apex petrositis is an
inflammatory lesion of the petrous apex second-
ary to otitis media [189]. Patients present with ear
and/or retroorbital pain and trigeminal and
abducens nerve symptoms, which constitute the
Gradenigo’s syndrome. Other symptoms are pho-
tophobia, excessive lacrimation, and fever. CT
scans demonstrate a destructive expansive lesion
of the petrous apex with irregular margins. MR
imaging can show an enhancing mass at the
petrous tip [195]. Osteomyelitis of the skull base,
usually observed in diabetics or immunocompro-
mised patients, may involve the petrous bone. It
appears as an area of demineralization or as an
irregular lytic lesion on bone-window CT and has
soft tissue density. MR imaging demonstrates an
enhancing area with hyperintense signal on
T2-weighted sequences. Aneurysms of the
petrous portion of the carotid artery cause
smoothly marginated bone erosion and present as
complex structures with flow voids and even con-
trast enhancement on MRI.
Cholesterol Granulomas
of the Petrous Apex
Manasse first reported cholesterol granuloma of
the temporal bone in the mastoid and the middle
ear in 1894 [231]. Two distinct entities have been
later recognized: the more rare petrous apex and
the unaggressive tympanomastoid cholesterol
granuloma, which is typically observed in asso-
ciation with chronic otitis media [270].
Cholesterol granulomas are relatively rare benign
lesions of the petrous apex. They may enlarge
enough to expand in the CP angle and compro-
mise cranial nerves function.
The incidence of cholesterol granuloma of
the petrous apex is less than 0.6 cases per mil-
lion population per year. Their prevalence has
been assessed to be 30–40 times less than that
of VSs [175, 215, 228]. Cholesterol granu-
loma is a histologic term used to describe a
chronic granulomatous foreign body giant-
cell reaction to the by-products of hemoglobin
Temporal Bone and Petrous Apex Lesions
degradation with associated fibrosis and vascular
proliferation [180]. According to the classic
obstruction-vacuum theory of the physiopathol-
ogy of these lesions, the cascade of events is trig-
gered by obstruction of the air cell outflow tracts
of the petrous apex [205]. This causes transuda-
tion of blood or rupture of vessels into the unven-
tilated mucosal space and anaerobic breakdown
of red blood cells, which liberates cholesterol.
The cycle repeats and leads to accumulation of
cholesterol crystals, which are strong irritant
to surrounding tissue. Ultimately a cholesterol
granuloma is formed [180]. An alternative theory
asserts that the causative factor is hemorrhage
from exposed bone marrow [217].
Cholesterol granulomas are round or ovoid
cysts filled with brown-yellow fluid that contains
globular material, lipids, and cholesterol crystals,
surrounded by a thick fibrous capsule [270].
Actually, they are not true cysts because they are
not lined with epithelium.
Neuroimaging
High-resolution thin-slice CT and MRI exami-
nations are mandatory for the preoperative
planning [241, 249, 268]. The essential infor-
mation is the location and size of the cyst, as
well as its relation to surrounding structures:
brain stem, carotid artery, sigmoid sinus, jugular
Fig. 11.16 Bone-window CT demonstrating an area of
bone erosion with smooth margins in the left petrous apex.
The corresponding MRI slice (T1-weighted image) shows
789
bulb, bony labyrinth, posterior wall of the sphe-
noid, IAC, and facial nerve. On CT cholesterol
granulomas appear as nonenhancing isodense
with the brain parenchyma lesions. Bone-
window CT demonstrates well-defined bony
expansile lytic lesion, with remodeling and ero-
sion of the bone (Fig. 11.16a). They have sharp
and smooth margins—Fig. 11.16a. The MR
image of cholesterol granulomas is more
specific—Fig. 11.16b. The lesion is nonenhanc-
ing and has high signal intensity on both T1-
and T2-weighted sequences [241, 242]. Rarely
foci of low signal intensity, related to hemosid-
erin, may be seen. Areas of contrast enhance-
ment correspond to granulation tissue [249].
When desiccated, cholesterol granulomas can
have areas of low signal intensity that give this
homogeneous T1-hyperintense lesion an het-
erogeneous T2 pattern, which in this location is
very suggestive of the diagnosis [279].
The differential diagnosis of cholesterol gran-
uloma includes epidermoid, cholesteatoma, chor-
doma, chondrosaroma, metastasis, mucocele, and
petrous apicitis [214]. Cholesteatomas and epi-
dermoids, in contrast to cholesterol granulomas,
have a low T1 MR signal intensity, which is
slightly greater than CSF signal intensity. On
T2-weighted sequences, however, all three lesions
are hyperintense [183].
petrous apex cholesterol granuloma, which has a small
extension to the middle cranial fossa. (The patient was
operated in the late 1980s by the senior author)
790
Clinical Presentation
Cholesterol granulomas are slow-growing lesions,
causing initially vague or indistinct symptoms,
such as temporal or retroorbital headaches, atypi-
cal facial pain, Eustachian tube dysfunction, and
middle ear effusion. Recurrent serous otitis
media, caused by the obstruction of the Eustachian
tube, may be the primary clinical manifestation.
The involvement of the adjacent structures
leads to more specific symptoms: diplopia due to
abducens nerve compression, hearing loss and
vestibular complaints, caused by compression of
the eighth cranial nerve in the IAC or CP angle or
destruction of the bony labyrinth, and facial nerve
dysfunction, which can appear in more advanced
lesions [180, 241]. In a review of 92 patients,
Terao et al. [267] found that the most common
clinical presentations were hearing loss (37 %),
vertigo (28.3 %), and headache (27.2 %).
Management
Cholesterol granuloma can have variable bio-
logical behavior. Accordingly two clinical sub-
types have been described: aggressive and
nonaggressive lesions [241]. The nonaggressive
lesions are uncommon. They are usually inci-
dentally discovered and do not progress for long
periods. Such lesions do not require interven-
tion: long-term follow-up is recommended with
regular MRI and CT control. It has been reported,
however, that rapid growth can occur even after
years of stability [269].
Symptomatic or growing lesions should be
treated surgically. The management options
range from complete resection to simple drain-
age and fenestration. The necessity to reestab-
lish an adequate aeration of the evacuated cavity
is frequently stressed, while the use of stents is
debated [172, 256, 258]. Some authors recom-
mend extensive skull-base approaches aiming
at more radical direct cyst excision: subtempo-
ral transzygomatic, preauricular subtemporal–
infratemporal, or a variant of the transpetrous
approaches, such as the translabyrinthine, tran-
sotic, transcochlear, infralabyrinthine, or petrosal
ones [172, 173, 182, 187, 202, 268].
In our opinion, cholesterol granulomas should
be resected as extensively as possible without
11 Other Cerebellopontine Angle Tumors
causing new neurological deficits. Considering
the lack of a true epithelial lining, complete surgi-
cal excision is not essential, especially if this
would endanger some important structure
[180, 182]. The choice of operative approach
depends on the exact location and extension of
the lesion, its relation to adjacent structures, level
of preoperative hearing, and goal of surgery [228].
Older patients with progressing lesions may be
considered for more conservative surgery—cyst
drainage and partial resection—via a less invasive
approach, such as the endonasal one.
Lesions located purely in the petrous apex,
especially those abutting the posterior wall of
the sphenoid, can be accessed via the endonasal
(transsphenoidal) endoscope-assisted approach.
Alternatively, the pure endoscopic technique may
be applied. The cyst can be safely drained and even
resected [209, 236, 252]. With this less invasive
approach, a more extensive surgery is avoided, the
labyrinth and, respectively, the hearing are pre-
served and the hospitalization costs are reduced.
Some granulomas, however, due to their exten-
sion and/or location of the carotid artery, are not
adequately accessible via this route. The subtem-
poral approach with extradural resection of the
petrous apex is one of the alternatives that we
favor. Large lesions with extensive bone destruc-
tion may be accessed via the transmastoid infral-
abyrinthine approach. Cholesterol granulomas,
extending more caudally, are best approached via
a retrosigmoid craniotomy with drilling of the
overlying bone with diamond drill. The exact
amount of bone removal is determined by the
extension of the cyst and the degree of bone ero-
sion. To access cholesterol granulomas, located
below the level of the IAC, the inframeatal bone
should be drilled off intradurally (retrosigmoid
inframeatal approach, which was elaborated by
the senior author and used initially in 2004 in a
patient with a petrous bone chordoma)—see Case
Illustration 11.18. Lesions with more cranial
extension, medial to the IAC, might require some
degree of suprameatal bone removal (retrosigmoid
suprameatal approach).
Following the evacuation of the cyst contents
and the resection of its wall, the cyst may be
inspected endoscopically. The possibility to look
Temporal Bone and Petrous Apex Lesions
around the corner with the angled optic and the
superior illumination allows to detect and to
remove possible remnants invisible with the micro-
scope. We recommend obliteration of the cyst cav-
ity with multiple fat pieces, sealed with fibrin glue
in order to prevent CSF leaks and reoccurrence of
the cyst. As noted by some authors, the most com-
mon cause for recurrence is obstruction of the
drainage site by fibrous tissue [268]. Fat oblitera-
tion of the cavity obviates the need for cyst drain-
age. Although some authors recommend using
muscle [202], we have found that it tends to shrink
and—in a long run—is less reliable material.
New neurologic deficit is exceptional after
surgery, and symptomatic relief is achieved in
791
more than 85 % of the patients. Cranial nerve
dysfunction generally tends to recover after the
evacuation of the cyst.
Cholesterol granulomas may recur even late
after surgery. The recurrence rate can reach 15 %
according to some publications [182]. Strict fol-
low-up of the patients is therefore recommended,
including clinical, audiometric and vestibular
examinations, and neuroimaging. Decrease of the
signal intensity on T1-weighed images and reduc-
tion of the size of the lesion are the accepted cri-
teria for successful evacuation [218]. At
follow-up, the fat-suppression techniques allow
to differentiate recurrence from cavities filled
with fat.
792
Case Illustration 11.18
Cholesterol Granuloma Removal via the
Retrosigmoid Intradural Inframeatal
Approach
This 45-year-old patient has been operated
15 years prior to presentation to our depart-
ment for a cystic tumor of the right pyramid
via a subtemporal–intradural approach. The
tumor was removed partially; both epidermoid
and dermoid were suspected, but the histo-
pathological diagnosis remained unclear.
During the follow-up, a steady growth ten-
dency was obvious, but the patient had no
major new symptoms. At presentation the
patient had just a hypesthesia in the area of the
first and second branches of the trigeminal
nerve—probably resulting from the initial
surgery—and complained of annoying tinni-
tus. The HR bone-window CT showed an
extensive zone of erosion of the pyramid with
smooth margins (a, b). MRI showed a cystic
lesion, located medial and inferior to the IAC,
with characteristics compatible with a choles-
terol granuloma (c–h).
Considering the extent of caudal exten-
sion of the lesion, it would not be adequately
accessed via the subtemporal approach.
A decision was therefore taken to approach it
via the retrosigmoid intradural inframeatal
approach (i–w). Following a standard RS
craniotomy, dural opening, and release of
CSF, the CP angle was well exposed. A yel-
lowish lesion was seen to protrude intradur-
ally from the pyramid in the area immediately
dorsal to the jugular foramen. The lesion did
not have any relation to the cranial nerves,
which could explain the lack of corresponding
11 Other Cerebellopontine Angle Tumors
neurological symptoms. Using high-speed
diamond drill, the petrous bone dorsal to the
jugular foramen and inferior to the IAC was
removed until the underlying lesion was ade-
quately exposed—the so-called intradural
inframeatal approach was performed. The
lesion had a bluish appearance, moderately
thick capsule and mixed content, consisting
of a dark brown fluid part and yellowish cho-
lesterol crystals. These finding corresponded
mostly to that of a cholesterol granuloma,
which was proven later by the histopatholog-
ical examination. The entire content of the
cyst could be removed with suction, tumor
forceps, platelet knifes of variable size, and
angled curettes under alternating microscopic
and endoscopic control. The capsule was
then dissected from the surrounding bony
structures and removed. Those portions that
were invisible with the microscope could be
visualized with the rigid endoscope control
and subsequently removed. Once the com-
pleteness of removal was verified (micro-
scopic and endoscopic), the large bone cavity
was filled with multiple fat pieces sealed with
fibrin glue. Interestingly, the BAEP monitor-
ing showed twice temporary decreased
amplitude and increased latency of the wave
V during endoscopic observation. These
changes recovered almost immediately fol-
lowing the removal of the endoscope.
Following surgery the patient did not have
any new neurological symptoms and was dis-
charged 12 days after surgery. The audiogram
showed that the preoperative hearing level
was preserved. The CT examination early
after surgery is presented on figures x and y.
Temporal Bone and Petrous Apex Lesions
a b
c
(a, b) Preoperative CT, demonstrating a lytic lesion
of the medial petrous bone with sharp and smooth
margins. (c–h) MRI of the patient, demonstrat-
ing precisely the extension of the lesion and its rela-
tion to surrounding structures (c) FLAIR sequence.
(d) T1-weighted sequence. (e, f) CISS sequences.
(g, h) T1-weighted sequences after contrast adminis-
tration. (i–w) Intraoperative images, demonstrating the
consecutive steps of surgery. (i) Standard RS approach
and dural incision. Initial CSF drainage from the lateral
cerebellomedullary cistern (R – retractor; arrowhead –
transverse/sigmoid sinus knee; asteriks – sigmoid sinus).
(j, k) Initial view of the CP angle and the tumor (Tu).
(SA – subarcuate artery; 7–8 – 7–8th cranial nerves;
793
d
LCN – lower cranial nerves; C – cerebellum). (l) Removal
of the dura (arrows) and drilling (m) of the bone, dorsal
to the jugular foramen and inferior to the IAC, which
allowed for exposure of the whole lesion (n), Single
arrowheads and double arrowheads. (o, p) Microscopic
view, indicating complete removal of the lesion; the
inspection with angled endoscope, however, demon-
strated some remaining parts, which were removed
under direct endoscopic guidance (q–v). The bone
opening was filled at the end with multiple fat pieces
and sealed with fibrin glue (w). (x, y) Postoperative CT,
demonstrating the extent of bone removal. The fat tis-
sue appears as a strongly hypodense signal
794 11 Other Cerebellopontine Angle Tumors

e f

i
Temporal Bone and Petrous Apex Lesions 795

j k

l m

n
0
796 11 Other Cerebellopontine Angle Tumors

p q

r
s

t
u
Temporal Bone and Petrous Apex Lesions 797

v w

x y
798
Chordomas
Virchow first described chordomas in 1857.
These tumors are derived from rests of the embry-
onal notochord—a primitive cell line, around
which the skull base and the vertebral column
form [203]. Chordomas account for 0.1–0.2 % of
all primary intracranial neoplasms [170, 263].
They are locally aggressive tumors, causing
expansive destruction of the bone at the site of
origin, followed by subsequent infiltration and
penetration of the dura and eventually intracra-
nial extension [225]. Chordomas originate usu-
ally in the vicinity of the clivus (sphenooccipital
bones) and exhibit various extension patterns in
all directions. Chordomas arising from the body
and the dorsal aspect of the clivus manifest usu-
ally as sphenooccipital or petrosal lesions. In a
recently published analysis [260], we found that
the most frequent direction of tumor growth was
into the posterior cranial fossa (81 %). In 35 % of
these patients, the brain stem was compressed.
Extension in the CP angle (defined as at least
2-cm posterolateral growth) was seen in 14 %.
Clinical Presentation
Symptoms are typically related to compression
of adjacent structures and vary with lesion loca-
tion and proximity to critical structures [203].
Diplopia caused by abducens nerve palsy is the
most frequent initial symptom—in 46–90 % of
all cases [211, 260, 274]. Other symptoms/signs
include headache caused by dural stretching
and cranial nerve irritation, oculomotor or tro-
chlear palsy, visual changes, facial numbness or
weakness, hearing loss, dysphagia, hoarseness,
and hypoglossal nerve palsy. Further tumor
growth causes symptoms and signs related to
brain stem compression and intracranial hyper-
tension [235, 281].
Neuroimaging
High-resolution bone-window CT scanning
reveals lytic bone destruction along with a soft
tissue mass that is isodense or hypodense com-
pared to brain parenchyma and enhances moder-
ately after contrast application. In our series CT
showed evidence of clivus destruction in all cases
11 Other Cerebellopontine Angle Tumors
[260]. The petrous apex area was eroded in 54 %,
while in 21 % the erosion extended more later-
ally. Intratumoral calcifications appear irregular
at CT and are thought to represent sequestrae
from bone destruction.
Chordomas are isointense or hypointense
lesions on T1-weighted MR images and are typi-
cally hyperintense on T2 sequences [203,
225, 263]. Contrast enhancement is typically
moderate to high [281]. Multiplanar reconstruc-
tion of the images is essential to evaluate pre-
cisely the tumor extension and to plan surgery.
MR angiography demonstrates the main vessels
and their relationship to tumor. Arterial narrow-
ing is rarely observed, due to the fact that these
tumors are generally soft and easily to dissect
from adjacent vessels [203].
The differential diagnosis of skull-base chor-
domas includes chondrosarcomas, meningiomas,
nasopharyngeal malignancies, trigeminal neuro-
mas, hemangioblastomas, craniopharyngiomas,
pituitary adenomas, lymphoma, fibrous dyspla-
sia, and metastases [199, 203]. In contrast to
chordomas, which are midline tumors, the major-
ity of chondrosarcomas arise along the petro-
occipital fissure. The rare midline chondrosarcomas
can be hardly differentiated from chordomas pre-
operatively. These two pathological entities,
however, should not be mixed: they have specific
biological features, clinical presentation, natural
evolution, and outcome. Histologically they may
share some features, but immunohistochemistry
allows for their precise differentiation.
Management
Skull-base chordomas have relatively poor prog-
nosis; if untreated, the life expectancy from diag-
nosis is 6 months to 2 years [220]. Despite surgery
and radiotherapy, recurrences occur early—
usually within 2–3 years.
The most effective management of skull-base
chordomas is maximal surgical excision fol-
lowed by heavy particle radiotherapy [192, 194,
225, 246]. Controversial is the issue of the extent
of surgery. Some surgeons favor an aggressive
surgical approach with maximal tumor resection
based on the assumption that longer survival
rates have been associated with more extensive
Temporal Bone and Petrous Apex Lesions 799

tumor removal [192, 206, 272]. Growing experi-
ence has shown, however, that true oncological
resection of chordomas can hardly, if ever, be
achieved [194, 225, 250]. Complete removal of
petroclival chordomas with a clear margin of tis-
sue is virtually impossible. Furthermore, long-
term survival rates and recurrence-free intervals
have not been substantially prolonged after
extensive surgeries. The alternative strategy is to
perform safe cytoreductive resection, thereby
avoiding the significant procedure-related mor-
bidity risk related to aggressive surgery. The goal
of surgery is to remove as much of the tumor as
possible and to decompress the brain stem, so
that safe and more efficient radiotherapy can be
performed [194, 265].
Previously for midline clival chordomas, we
utilized the transethmoid transsphenoidal
approach (Fig. 11.17). Currently, however, we
favor the endonasal-combined microsurgical and
endoscope-controlled technique (Fig. 11.18).
With this technique, tumors extending from the
level of dorsum sellae to C2 level can be accessed.
The paramedian tumor extension can be

a b

Fig. 11.17 Giant skull-base chordoma (a–c), removed via the transethmoid transsphenoidal approach (d, e)
800
d e
Fig. 11.17 (continued)
visualized and removed under endoscopic guid-
ance with appropriate angled instruments.
Chordomas with a more lateral extension,
however, cannot be safely removed via an endo-
nasal route. The approach to such tumors is
selected individually, considering the tumor
extension pattern, the degree of bone destruction,
the presence of intradural extension, the degree
of brain stem compression, and the neurological
status of the patient. Extradural chordomas that
already caused significant petrous bone destruc-
tion, even if they have a limited intradural exten-
sion, are operated via a presigmoid transpetrous
approach. In case of relatively intact pyramid or
large intradural tumor part causing brain stem
compression, the standard RS approach is pre-
ferred. This approach allows for removal of both
the intra- and extradural tumor parts. Furthermore,
the tumor can be safely dissected from the brain
stem and from the cranial nerves under direct
visual control, and the risks related to the extended
petrous bone drilling are avoided (see Case
Illustration 11.19).
Extensive skull-base chordomas may require a
2-stage removal via different operating corridors.
11 Other Cerebellopontine Angle Tumors
The senior author followed this strategy in 4
patients: in 3 of them, bilateral retrosigmoid sur-
geries were necessary and in 1 subtemporal and
RS approaches were made [260].
Initial surgery affords the best opportunity for
maximal tumor excision, and radicality should be
aimed, unless it endangers some essential struc-
ture. The involved bone should be extensively
removed, whenever feasible. Importantly, tumor
invasion or surgery itself may impact the stability
of the craniovertebral junction. The operative
morbidity is typically related to cranial nerve pal-
sies, CSF leak, hemorrhages, and hydrocephalus.
The cranial nerves in the CP angle are usually
compressed and dislocated by the tumor; they are
infiltrated very rarely, which allows for safe dis-
section. In large chordomas, however, cranial
nerves may be embedded within the tumor tissue.
The main reason for iatrogenic injury remains
their difficult or late visualization. Early
identification of the essential neural and vascular
structure is, therefore, the prerequisite for their
preservation. In chordomas with intradural exten-
sion, due to the petrous bone destruction or
approach-related bone drilling, the risk of CSF
Temporal Bone and Petrous Apex Lesions 801

a b

Fig. 11.18 Endonasal-combined microsurgical and feedback control on the extent of resection and location of
endoscope-controlled removal of a clival chordoma (a–d) possible remnants. The images (f–i) are acquired intraop-
under navigational guidance (e). The surgery was per- eratively after the tumor removal
formed in the INI BrainSuite, which allows for immediate
802 11 Other Cerebellopontine Angle Tumors

Fig. 11.18 (continued)

Temporal Bone and Petrous Apex Lesions
f g
h
Fig. 11.18 (continued)
leak is very high. We recommend the utilization
of multiple small fat pieces sealed with fibrin
glue to plug the areas of bone destruction and to
augment the watertight dural closure. The fat can
be harvested from the lower part of the incision
or from the abdomen, in case a larger amount is
necessary.
Irradiation with heavy particles (protons, car-
bon ions, helium, and combined proton–photon
803
i
or carbon–photon therapy) has been shown to
improve local control and survival rates and is
recommended in case of incomplete tumor
removal, in case of recurrence, or is delivered to
the surrounding bone following each surgery,
regardless of the extent of resection [174, 197,
262, 266].
Chondroid chordoma is a pathological variant
of chordomas. At surgery these 2 variants cannot
804
be differentiated; they both present similar opera-
tive challenges. Immunohistochemical staining
allows to distinguish them. Although it has been
speculated that they may have better prognosis
[211], recent studies found that the biological
behavior and outcome of chondroid chordoma is
similar to that of conventional chordoma [171].
Local recurrence of intracranial chordomas is
still common regardless of the therapy mode,
and strict follow-up of the patients is therefore
recommended. A recurrence is best visualized
on contrast-enhanced MRI images (area of con-
trast enhancement) or on T2-weighted sequences
11 Other Cerebellopontine Angle Tumors
(hyperintensive area). In case of growth ten-
dency or symptomatic recurrences, repeated sur-
gery is indicated with the goal of decompression
of neural structures and obtaining maximal safe
tumor removal. Of note, the operation-related
morbidity rate is higher in repeated surgeries,
especially after radiotherapy. The orientation
and identification of normal landmarks and
structures are more difficult, more intense adhe-
sions of the tumor to cranial nerves and/or brain
stem are frequently found, and the large vessels
may be very fragile due to radiation-induced
vasculopathy.
Temporal Bone and Petrous Apex Lesions
Case Illustration 11.19
Chordoma Removal via the Retrosigmoid
Approach
Forty-two-year-old female patient operated
twice elsewhere before presentation. The first
surgery was in 2001: partial removal of a
chordoma via a frontotemporal craniotomy.
One year later, the patient was reoperated via
a left-sided retrosigmoid craniotomy, and
postoperative proton beam treatment has
been performed. Her recent MRI showed an
extensive tumor of the clivus with major
intradural growth, engulfment of the verte-
bral and basilar arteries, and compression
with suspected brain stem infiltration (a–c).
a b
Preoperative MRI, demonstrating the location of the
tumor, its relation to the basilar artery, and the deep
penetration in the brain stem. (a) Native T1-weighted
sequence. (b) T1-weighted sequence after gadolinium
administration. (c) T2-weighted sequence. (d) Initial
view of the CP angle and of the tumor (Tu), located
medially to the lower cranial nerves (LCN), the
seventh–eighth nerve complex (7–8), and the trigeminal
805
Considering the relations to the brain stem,
the two previous surgeries, and the proton
beam therapy, radical tumor removal was not
deemed possible. A new surgery via a left-
sided RS approach was planned with the goal
of decompressing the brain stem and volume
reduction of the tumor, as far as safely pos-
sible. The steps of tumor removal are illus-
trated on figures d–i.
Six days after the surgery, a CSF leak
appeared. The patient was reoperated and a
small opening between the dural stitches was
identified as source of the leak. Fat, harvested
from the abdomen, and fibrin glue were used
to watertight seal this area.
nerve (5). (e) Tumor removal was performed via sev-
eral CP angle levels: initially between the LCN and the
seventh–eighth nerves, followed by the level below the
LCN (f) and the level between the seventh–eighth and
the fifth nerves (g). The ipsilateral vertebral artery
(VA), initially hidden by the tumor, is visible on (h). (i)
All neural and vascular structures of the CP angle,
preserved after the tumor removal (PV petrosal vein)
806 11 Other Cerebellopontine Angle Tumors

c d

i
Temporal Bone and Petrous Apex Lesions
Chondrosarcomas
Chondrosarcomas (CSA) originate from chon-
drocytes in the areas of residual enchondral carti-
lage or from multipotent mesenchimal cells
[206, 211]. Cranial CSA originate at or near the
petroclival area and comprise approximately 6 %
of all skull-base tumors [171, 193, 222]. In the
senior author’s material, CSA were typically
located paramedian and had most frequently pos-
terior or posterolateral extension [259]. Ninety-
two percent of them expanded into the posterior
cranial fossa and 64 % involved the CP angle.
CSA are subdivided into 3 grades based on
their cellularity and nuclear atypia [255]: from
well differentiated (grade 1) to least differentiated
(grade 3). These categories correlate to the risk of
recurrence and the survival rate, although in our
experience, significant difference between grades
1 and 2 was not noted [259]. Four histological
CSA types have been reported: conventional, ded-
ifferentiated, clear cell, and mesenchymal [255,
278]. Mesenchymal CSA are associated with
worse prognosis and have an average life expec-
tancy of less than 28 months [193].
Neuroimaging
CSA have a tendency to invade and destruct sur-
rounding bones. The typical CT appearance of
CSA is that of a destructive lesion with patchy
infiltration, occasionally with a rim of calcification.
Erosion of the clivus is always found, while the
petrous apex is destructed in 83 % of the cases
[259]. These tumors are hypo- to isointense on
T1-weighted sequences and hyperintense on
T2-weighted sequences with frequent signal het-
erogeneity. They usually demonstrate an intense
but heterogeneous contrast uptake [244, 278].
CSA should be differentiated from chordoma,
chondroid chordoma, chondroma, meningioma,
osteogenic sarcoma, glomus jugulare, and metas-
tases. Neuroimaging data may be insufficient to
differentiate reliably chordoma, chondroid chor-
doma, and CSA because their characteristics are
similar [171, 244]. Tumor location, however, may
provide a clue: CSA tend to be lateral to chordo-
mas. In the series of Al-Mefty et al. [171], the
petrous bone with the clivus was involved in
807
12 % of chordoma patients, in 32 % of chondroid
chordoma patients, and in 80 % of chondrosar-
coma patients.
CSA and chordomas are completely different
pathological entities with specific biological
behavior and prognosis. Histologically these
tumors are, however, difficult to differentiate.
The diagnosis is based on immunohistochemis-
try: both CSA and chordoma contain S-100 and
vimentin but that only chordoma contains keratin
and epithelial membrane antigen.
Clinical Presentation
The initial clinical presentation is related to tumor
location and size (Figs. 11.19, 11.20, and 11.21).
Most frequent initial sign is abducens nerve
palsy: it was found in more than half of the
patients in our series. Less frequent symptoms/
signs are headache, ataxia, oculomotor nerve
palsy, hypacusis, tinnitus, vertigo, facial palsy,
swallowing difficulties, and pyramidal signs.
Management
CSA, extending to the CP angle, similar to all
other CSA, can be managed with surgery, radio-
therapy, or combination thereof. Extensive and
aggressive surgical resection, even total en bloc
removal with carotid artery and cranial nerve
sacrifice, has been proposed by some authors as
the “ideal” surgical goal [206, 244, 273].
Considering the high risk of functional deficits
related to aggressive surgery and the long-term
outcome, the dominating alternative treatment
concept is to achieve maximal safe cytoreduction
and brain decompression while preserving func-
tion [193, 248, 278]. The rationale for this approach
is that small tumor remnants do not influence
significantly the risk of tumor recurrence and
patients’ survival. Skull-base CSA are benign
slow-growing tumors that can be controlled effec-
tively with surgical resection. The 5- and 10-year
survival rates in largest series are 85–95 and
71–95 %, respectively [171, 193, 248, 261, 278].
We believe that the goal of surgery should be
to achieve radical tumor removal and neurovas-
cular decompression, but not at the expense of
compromising neurological functions and quality
of life of the patient. The management of each
808
a b
Fig. 11.19 Foramen jugular chondrosarcoma. Preoperative
MRI and postoperative bone-window CT of a 69-year-old
patient with a WHO grade I chondrosarcoma of the right
jugular foramen (arrow). The patient presented with pro-
gressive dysphagia, dysphonia, and accessory nerve palsy
patient should be individualized according to his
personal expectation, neurological status, and
tumor characteristics. In CSA of the CP angle,
the major goal is to achieve brain stem decom-
pression and—on the second place—to remove
the tumor as radically and as safely as possible.
The retrosigmoid or/and the retrosigmoid
suprameatal approaches have been found to be
most suitable for achieving these goals (see Case
Illustrations 11.21 and 11.22). In case of exten-
sive involvement and destruction of the pyramid,
alternative approaches, such as the presigmoid or
11 Other Cerebellopontine Angle Tumors
with atrophy of trapezius muscle. MRI (a, b) demonstrated
a cystic lesion in the right jugular foramen without intrac-
ranial extension. The tumor was removed completely
via the transmastoid infralabyrinthine transcervical
approach (c)
the anterior transpetrosal ones, may be preferred
(see Case Illustration 11.20). Following this con-
cept, the 5- and 10-year survival rates in the
senior author’s series were 95 % [259].
Proton beam radiation is an adjunctive man-
agement method [176, 247]. The survival rates
and the risks of recurrence and metastases, how-
ever, are similar in patients undergoing only sur-
gery and in those managed with adjunctive proton
beam radiotherapy. Therefore, such management
is recommended in grade III CSA or after surgery
of early recurrences.
Temporal Bone and Petrous Apex Lesions 809

a b

c d

Fig. 11.20 Preoperative T1-weighted axial and coronal petrous apex and has a smaller part in the middle cranial
MR images (a–c) and T2-weighted images (d) of a patient fossa. The tumor was removed via the standard RS
with a CSA with a very large CP angle part, which causes approach (Reproduced from: Samii et al. [259]. Copyright
severe brain stem compression. The tumor involves the © 2008, with permission of Springer-Verlag)

a b

Fig. 11.21 Postoperative T1-weighted MR images (patient from Fig. 11.20) demonstrating the complete removal of
the CP angle tumor part and the brain stem decompression. The remnant in the middle fossa is followed radiologically
810
Case Illustration 11.20
Chondrosarcoma Removal via the Anterior
Petrosal Route
This 52-year-old female patient presented
with a 6-month history of double vision to the
left side. The neurological examination
revealed incomplete left-sided abducens nerve
palsy. MRI demonstrated a tumor in the
petrous apex area on the left side with a lim-
ited extension into the CP angle (a–d). The
bone erosion was well visible on the HR bone-
window CT scans. Considering the predomi-
nant tumor location within the petrous bone,
the small CP angle tumor component and the
neurological condition of the patient, she was
operated via an anterior petrosal route. A low
a
c
(a–d) Preoperative CT (a) and MRI (b– T2-weighted;
c– CISS; d– T1-weighted contrast enhanced
sequences), demonstrating a petrous apex lesion on
11 Other Cerebellopontine Angle Tumors
temporal craniotomy was performed after
resection of the zygoma and the petrous apex
was approached extradurally. The eroded bone
covering the area was drilled off with diamond
drill, and the petrous segment of the carotid
artery was exposed to provide sufficient access
to the lesion. Following tumor removal, the
patient had an uneventful recovery. The histo-
pathological diagnosis was grade 1 chondro-
sarcoma. Radiosurgery was performed in
order to lower the risk of recurrence, as
assumed at that time. Gradually, the abducens
nerve function recovered almost completely.
The patient is followed for more than 72
months and has a very good quality of life
without any evidence of tumor activity.
b
d
the left side. (e–f) Postoperative T1-weighted
sequence after gadolinium administration (e) and
T2-weighted sequence (f)
Temporal Bone and Petrous Apex Lesions 811

e f
812
Case Illustration 11.21
Chondrosarcoma Removal (Endoscope-
Assisted Retrosigmoid Suprameatal
Approach)
This 32-year-old male patient complained
from intermittent double vision for 2 months
prior to presentation. Neurological examina-
tion revealed abducens nerve palsy on the
right side. High-resolution bone-window CT
showed an area of bone erosion in the medial
part of the right pyramid (a), while on MRI a
homogeneously enhancing lesion involving
the petrous apex was seen (b–f). The lesion
was isointense to brain parenchyma on
T1-weighted sequences and hyperintense on
a b
(a–f) Preoperative CT (a), T1-weighted MR image
following contrast administration (b–d), CISS (e) and
T2-weighted MR sequence (f), demonstrating an
enhancing lesion in the area of the petrous apex with
extension into the medial CP angle. (g–l) Tumor
removal: (g) initial view of the tumor (Tu) and the sur-
rounding structures in the CP angle (5 – trigeminal
nerve; 7–8 – 7–8th nerve complex; S – suprametala
tubercle). (h) The suprametal tubercle was drilled off,
thus exposing the whole length of the trigeminal nerve
11 Other Cerebellopontine Angle Tumors
T2-weighted sequences and had an intracra-
nial extension that reached and slightly com-
pressed the lateral midpons. The patient was
operated via the RS suprameatal approach.
This approach was selected, considering the
limited bone erosion of the petrous apex and
the presence of intradural tumor portion com-
pressing the brain stem. The lesion, which
proved to be a grade I chondrosarcoma, was
removed completely using combined micro-
scopic and endoscopic control (g–l).
Postoperative CT scans are presented on
figures m and n. Early after surgery, the
abducens nerve function started to improve
and at follow-up resolved almost completely.
up to the Meckels’ cave and enhancing the approach
to the tumor (i). Following initial internal debulking
(j, arrowhead), the tumor was removed completely
(k). Note the preserved abducent nerve (arrow), medial
to the tumor. (l) Endoscopic inspection to verify the
completeness of resection. (m, n) Postoperative CT
images: 1 day after surgery (m) and 10 days later (n).
Note the moderate pneumocephalus after surgery
(m) and its complete resorption (n)
Temporal Bone and Petrous Apex Lesions 813

c d

e f

g h
814 11 Other Cerebellopontine Angle Tumors

i j

k l

m n
Temporal Bone and Petrous Apex Lesions
Case Illustration 11.22
Chondrosarcoma in the Jugular Foramen
Area (Retrosigmoid Endoscope-Assisted
Approach)
This 64-year-old male patient has been oper-
ated at another hospital 12 years prior to pre-
sentation: a tumor in the jugular foramen area
on the right side, presumably a schwannoma,
has been removed partially. The residual has
been followed neuroradiologically at irregular
intervals. The last imaging showed substantial
increase of its size. At presentation the patient
had severe gait ataxia and complete hearing
loss on the right side. MR examination showed
an extensive tumor of the right petrous bone
with large extracranial and intracranial parts
(a–h). The tumor was approached via the ret-
rosigmoid route; it had a thick capsule and
was filled with gelatinous content. Following
dissection of the surrounding scar tissue, the
actual extension of the tumor, which reached
a b
(a–h) Preoperative MR examination: native T1-weighted
(a, b), T2-weighted (c–e) and T1-weighted sequences
following contrast application (f–h). (i–l) Postoperative
815
anteriorly the external auditory canal, antero-
medially the petrous carotid artery, and supe-
riorly the seventh to eighth nerve complex,
became evident. Its capsule could be dissected
from all surrounding structures, and the tumor
could be removed completely with endoscopic
assistance. The space was filled with multiple
gelatin sponges (later on, we begun using fat
tissue for such purposes). After surgery the
patient recovered quickly and had no new
deficits. Considering the invasion of the exter-
nal ear, a new surgery was performed 10 days
later—the external ear canal was closed and
an incidentally found cholesteatoma was
removed. The postoperative MRI is shown on
figures i–l.
The tumor turned out to be a grade II chon-
drosarcoma and regular MR follow-up was
recommended. For the period of follow-
up—48 months—no recurrences have been
diagnosed.
CT (i) and MRI (j–l) images demonstrating the com-
plete tumor removal
816 11 Other Cerebellopontine Angle Tumors

c d

f
e

h
g
Temporal Bone and Petrous Apex Lesions 817

i j

k l
818
Chondroblastomas
Chondroblastoma was first reported by Codman
in 1931 and occurs typically in the epiphyses of
the long bones. Chondroblastomas involve the
skull base extremely rare. According to Hatano
et al. [210], approximately 70 cases of such
tumors involving the temporal bone have been
reported. Chondroblastoma arises from imma-
ture cartilage and is typical for the young age
group. Histologically they are benign tumors but
clinically show malignant behavior with inva-
sion and/or metastasis to adjacent structures
[238]. Surgery is the mainstay of treatment of
such tumors, and the goal is to achieve total
tumor removal, if safely possible [224]. Partial
removal or curettage is related to high risk of
recurrence [223]. Radiation may be effective in
suppressing tumor growth and is regarded as an
adjunctive therapy mode in case of tumor rem-
nants. Primary radiotherapy is not a plausible
option: some authors have shown that it may
induce malignant transformation to chondrosar-
comas [280].
In our series, we had one patient with a petrous
bone chodroblastoma, presented on Fig. 11.22.
This 16-year-old patient presented recently with
trigeminal hypesthesia (V2), facial palsy (HB
grade III), and anacusis on the left side. He had
been operated 5 months earlier in another depart-
ment: tumor removal had been performed via a
retrosigmoid approach; the histopathological
diagnosis was aneurysmal bone cyst. The CT and
MR images at admission are presented on
Fig. 11.22a–i. Considering the extension pattern
of the tumor and the wide destruction of the
petrous bone, a decision in favor of subtemporal
epidural transpetrous tumor removal was taken.
The tumor was widely enucleated and dissected
from the surrounding structures, including the
internal carotid artery. The most medial and pos-
terior parts, however, were strongly adherent; any
attempt to mobilize or dissect them would endan-
ger the integrity of the facial nerve, of the lower
cranial nerves, or of the carotid artery. Therefore,
this portion was not removed. The resection
cavity was filled with fat tissue, well visible on
the postoperative CT scans (Fig. 11.22j, k).
11 Other Cerebellopontine Angle Tumors
The histopathological result was chodroblastoma.
The patient did not have new neurological deficits
after surgery and recovered rapidly. A follow-up
MRI is planned in 3 months: the decision if to
undertake radiotherapy will depend on the
findings of this study.
Endolymphatic Sac Tumors
Endolymphatic sac tumors (ELST) originate
from the endolymphatic sac or duct [212]. Their
exact anatomical origin is supposed to be the
osseous portion (vestibular aqueduct) of the
endolymphatic duct/sac system [229]. These
tumors were first established as a distinct patho-
logical entity by Heffner in 1989 [212] and were
recognized as part of the Von Hippel–Lindau
syndrome by Manski et al. in 1997 [232]. They
are rare tumors of the posterior temporal bone,
whose incidence is actually underestimated
because they have been misdiagnosed as a variety
of other tumors [213].
Previously known as aggressive papillary
middle ear tumors, ELST are histologically
benign slow-growing low-grade adenocarcino-
mas. They are locally invasive and frequently
expand intracranially in the CP angle area [212,
226]. ELST tend to recur locally but do not
metastasize.
Though most cases are sporadic, ELST have
been described in association with Von Hippel–
Lindau disease, an autosomal dominant cancer
syndrome [275]. The incidence of ELST in
patients with this syndrome is 15 % [191]. In
30 % of the patients with VHL syndrome, ELST
occur bilaterally and cause significant neurologi-
cal disability: deafness and severe disequilibrium
[234]. VHL syndrome is linked to a germline
mutation of the VHL gene [275, 276], mapped to
chromosome 3p25. The VHL protein is involved
in the regulation of the activity of a hypoxia-
inducible factor, which enhances cellular metab-
olism and increases the expression of angiogenic
and mitogenic factors. With absent or abnormal
VHL protein function, angiogenesis is stimu-
lated, which explains the typical hypervascular
pattern of the VHL-related tumors [219].
Temporal Bone and Petrous Apex Lesions 819

a b

c d

Fig. 11.22 Petrous bone chondroblastoma with a large enhanced images. (h, i) T2-CISS images. The early post-
cp angle and supratentorial extension. Preoperative CT operative CT images are presented on (j, k) (See the text
(a–c) and MR (d–i) images. (d–g) T1-weighted contrast- for details)
820 11 Other Cerebellopontine Angle Tumors

e f

g h

Fig. 11.22 (continued)

Temporal Bone and Petrous Apex Lesions
i j
Fig. 11.22 (continued)
Neuroimaging
ELST are centered at or near the posteromedial
surface of the petrous bone. Bone-window CT
reveals bone erosion with “moth-eaten” bone
margins [191, 227]. Frequently, bone spicules or
fragments are seen within the tumor. The radio-
graphic finding in case of small ELST is typically
limited bony erosion adjacent to the vestibular
aqueduct. The cystic tumor areas are hyperin-
tense on T2- and T1- weighted MR sequences,
while the solid portions have heterogeneous
intensity on both sequences. ELST are generally
highly vascular tumors.
821
ELST should be differentiated from other
destructive tumors involving the temporal bone:
glomus tumor, chordoma, chondrosarcoma, men-
ingiomas, CP angle choroid plexus papilloma,
ceruminous gland tumors, metastatic thyroid or
renal cell carcinoma, benign adenomatous
tumors, and primary bone tumors [243].
Clinical Presentation
ELST present most frequently (in up to 95 % of
the patients) with unilateral hearing loss: of grad-
ual or of sudden onset [191, 232, 234]. The puta-
tive mechanism of hearing loss in case of large
822
ELST is tumor invasion of the otic capsule (the
bony covering of the inner ear). Sudden hearing
loss is supposed to be caused by intralabyrinthine
hemorrhage, while the slowly progressing hear-
ing loss mimicking that of Menière’s disease is
due to endolymphatic hydrops [185]. Other com-
mon symptoms are tinnitus (90 % of patients),
vertigo or disequilibrium (66 %), and aural full-
ness (30 %) [229, 232, 253]. The vestibular
symptoms related to ELST follow usually a pat-
tern consistent with unilateral vestibulopathy:
severe symptomatology with subsequent milder
or asymptomatic periods. Possible pathophysio-
logical mechanisms are either the “burn out” phe-
nomenon or the development of central
compensation [191].
Further symptoms in patients with ELSC are
due to involvement of adjacent cranial nerves: the
facial nerve is involved most frequently, followed
by ninth, tenth, and fifth cranial nerves. Tumor
expansion causes cerebellar and long-tract signs,
intracranial hypertension, and/or hydrocephalus.
In some cases, a vascular mass in the external
auditory canal or a bluish-red discoloration—in
case the tympanic membrane is intact—can be
visualized [212]. The natural evolution of ELST
tumors is unpredictable, and remarkable
variability, ranging from more indolent to very
aggressive progress, has been observed (see Case
Illustrations 11.23 and 11.24).
Management
The ideal treatment of ELST is their complete
resection, which is curative. In small tumors, this
can even alleviate vestibular symptoms and allow
for preservation of residual hearing [191].
11 Other Cerebellopontine Angle Tumors
Complete tumor excision has been shown to lead
to better results than subtotal or partial excision
followed by radiotherapy: the cure rate after total
tumor removal is up to 90 %, while in case of
subtotal resection with adjuvant radiation of the
residual tumor, it is just 50 % [212, 229, 234].
Early surgery has been recommended to
reduce the morbidity related to ELST because of
the unpredictable onset of audiovestibular mor-
bidity, which is not related to tumor size
[229, 234]. In advanced tumors, however, com-
plete excision is rarely possible due to involve-
ment of lower cranial nerves, sigmoid sinus, and/
or petrous portion of the carotid artery [254].
Therefore, high-resolution MR and CT imaging
of the vestibular aqueduct and endolymphatic sac
area are recommended in all patients with VHL
syndrome.
The RS approach is best suited for the removal
of both small and large ELST (see Case Illustration
11.25). It allows for radical surgery and—in case
of small tumors—offers the possibility for hear-
ing preservation. The petrous bone can be drilled
off additionally, as described earlier in the book,
providing adequate access to the whole tumor. In
case of large ELST, the dissection of the CP angle
tumor part from the cranial nerves or the brain
stem is safer when performed under the better
visual control, provided by the RS approach. In
case the tumor is confined to the petrous bone
and hearing is completely lost due to destruction
of the inner ear, the transmastoid transperosal
approach may be an alternative (see Case
Illustrations 11.23 and 11.24). Preoperative
embolization of major feeders can be performed
to reduce intraoperative bleeding [243].
Temporal Bone and Petrous Apex Lesions
Case Illustration 11.23
Sporadic ELST with Relatively Slow
Development, Removed in Two Steps via
Transmastoid and Retrosigmoid Approaches
Twenty-nine-year-old female patient pre-
sented with a history of progressive hearing
loss on the right side since her 14th year.
One year ago, dysesthesia in the area of the
right ear and slight facial palsy appeared. She
has been diagnosed of having a large tumor
involving the petrous bone and the CP angle
that was removed partially elsewhere via the
transmastoid approach. Following surgery
she had anacusis and complete facial palsy.
On the last bone-window CT, an area of bone
erosion in the pyramid with irregular con-
tours and small bony fragments in the center
of the mass were seen (a). MRI revealed a
heterogeneous partially cystic tumor, which
destroyed the lateral petrous bone (b–h). Its
solid portions enhanced intensely after gad-
olinium application. The tumor had major
intracranial and extracranial extensions in the
infrapetrous area, and the comparison with
previous images showed a certain growth
tendency.
823
Considering the hypervascularity of the
tumor, its extension pattern, and the presence
of compete facial palsy, a decision was taken
to perform first embolization of its feeding
arteries and then remove it in two separate
operative steps with reconstruction of the
facial nerve, if necessary.
The tumor feeders were successfully embo-
lized, and 4 days later its petrous and extracra-
nial portions were removed via a right-sided
transmastoidal approach. The proximal seg-
ment of the facial nerve was infiltrated by the
tumor. Therefore, it was reconstructed by per-
forming an end-to-end anastomosis between
its distal healthy part and the hypoglossal nerve
(i, j: postoperative CT scans). After surgery,
the patient suffered from minor dysphagia,
which improved rapidly. Two weeks later, the
intracranial tumor part was removed com-
pletely via the retrosigmoid route (k–m: post-
operative CT scans).
Summarizing, this is a case of a sporadic
ELST with relatively slow development for
more than 14 years, good outcome of surgery,
and no recurrence up to present (30 months of
follow-up).
824 11 Other Cerebellopontine Angle Tumors

a b

d
c

Preoperative CT (a) and MR images of the patient,
demonstrating the location of the tumor and the extent
of bone erosion. (b, c) Native T1-weighted sequence.
(d–g) T1-weighted sequence following contrast
administration. (h) T2-weighted sequence. Native (i)
and bone-window (j) CT scans after the first surgery.
Native (k) and bone-window CT scans (l, m) follow-
ing the last surgery. The hyperdense signal corresponds
to fat tissue, used to seal the petrous bone
Temporal Bone and Petrous Apex Lesions 825

f g

h i
826 11 Other Cerebellopontine Angle Tumors

j k

l
Temporal Bone and Petrous Apex Lesions
Case Illustration 11.24
VHL Syndrome-Associated ELST with
Aggressive Biological Behavior
This 13-year-old boy presented with nonfunc-
tional hearing on the right side that occurred
suddenly 4 months earlier. The MRI demon-
strated a large destructive tumor involving the
right petrous bone with extension from the
external auditory canal to the jugular foramen,
as well as into the CP angle (a). The presump-
tive radiological diagnosis was glomus jugu-
lare tumor. Digital subtraction angiography
was done and showed a highly vascularized
tumor, supplied predominantly by a strongly
dilated occipital meningeal artery—a branch
of the external occipital artery. Further feeders
were originating from the ascending pharyn-
geal artery, the posterior temporal artery, the
internal carotid artery, and the temporo-occip-
ital branch of the middle meningeal artery,
while a small medial tumor part was supplied
by the slightly dilated anteroinferior cerebel-
lar artery. The carotid artery was not infiltrated
by the tumor. The venous outflow system was
not significantly affected, although the right
jugular bulb was narrowed due to tumor com-
pression. Superselective catheterization with
embolization of the major feeding arteries was
then performed.
The retrosigmoid approach was selected for
tumor removal, taking into account the absence
of tumor extension into the lateral petrous bone
and the mastoid, as well as the normal facial
nerve function. This route would allow further
to perform the dissection of the tumor capsule
from the cranial nerves and brain stem under
direct visual control. In case the petrous tumor
part would remain inaccessible, the approach
could be extended presigmoidally.
At surgery the tumor was found to be
purely extradural and could be completely
removed with preservation of all cranial
nerves. The histopathological diagnosis was
ELST. MRI of the whole neuraxis was there-
fore performed but showed no further lesions.
Ultrasound of the abdomen ruled out further
827
parenchymal tumors or cysts. Ophthalmological
examination, however, revealed a retinal
angioma on the left side so that a suspicion of
VHL syndrome aroused; genetic testing was
recommended. The patient made an unevent-
ful recovery from the surgery without new
neurological deficits and follow-up MR imag-
ing showed no signs of recurrence.
Two years later, however, a slight facial
palsy (HB grade II) appeared and the MRI
showed a large tumor recurrence (b–e).
Considering the relatively early recurrence, a
decision for radical surgery—removal of the
tumor and of the affected bone structures via
the transpetrous approach—was taken. The
external auditory canal was closed plastically,
the facial nerve was identified distal to the sty-
lomastoid foramen, and a lateral subtotal neuro-
navigation-guided petrosectomy was performed.
The facial nerve was exposed along its whole
petrous segment—it was partially infiltrated by
the tumor. The affected segment of the nerve
(approximately 1.5 cm) was resected, and its
continuity was reconstructed with a sural nerve
interposition graft. The tumor was gradually
removed, reaching the petrous segment of the
carotid artery and the middle fossa dura. Then,
the presigmoid dura was opened, and the CP
angle tumor part was removed, dissecting its
capsule from the severely compressed lower
cranial nerves. The surgical cavity was filled
with fat tissue, harvested from the abdomen
(postoperative MR images: f–i).
Postoperatively the patient, besides the
complete facial palsy, had lower cranial
nerves dysfunction, and a PEG tube had to be
placed before discharge. Four months later he
presented to our outpatient department with
complete recovery of the swallowing and
the speech. One year later, his facial nerve
function improved to HB grade III. On MRI,
however, a small round contrast-enhancing
nodule, highly suspicious for a recurrence,
was seen in the petrous apex area (j, k).
Due to the good clinical improvement and
the absence of any new neurological symp-
828
toms, further close follow-up control was
recommended. Six months later, the finding
remained unchanged.
Summarizing, this ELST was most proba-
bly associated to VHL syndrome and had
(a) Initial MR images of the tumor. (b–e) MRI show-
ing the large tumor recurrence (T1-weighted images
after gadolinium administration). (f–i) Postoperative
MR images, demonstrating the completeness of
tumor removal (T1-weighted images after gadolinium
11 Other Cerebellopontine Angle Tumors
more aggressive biological behavior. It
occurred early in life, the clinical history was
short, and the tumor tended to infiltrate the
neural structures and to recur relatively early,
even after complete removal.
administration). Note the fat tissue used to fill the sur-
gical cavity. (j, k) Follow-up MRI, showing a small
round contrast-enhancing nodule in the petrous apex
area (arrow), which is highly suspicious for a
recurrence
Temporal Bone and Petrous Apex Lesions 829

b c

d e

f g
830 11 Other Cerebellopontine Angle Tumors

h i

j k
Temporal Bone and Petrous Apex Lesions
Case Illustration 11.25
Sporadic Cystic ELST with a Predominant
Part in the CP Angle
This 43-year-old patient complained since 5
years of progressive hearing loss, tinnitus, and
vertigo on the left side. Later on progressive
gait instability appeared. He has been diag-
nosed to have a large left-sided CP angle tumor,
involving the petrous bone, and was operated
in another neurosurgical facility. The tumor
was removed partially, and the histopathologi-
cal examination revealed a cholesterol granu-
loma. He presented to our institute 2 years later
and reported to have a progressive facial weak-
ness since 12 months and swallowing difficulty
since 6 months. Neurological examination
revealed hypesthesia in the V2 and V3 areas,
HB grade VI facial nerve palsy, complete hear-
ing loss, and lack of gag reflex. He had further
severe gait ataxia and slight right-sided hemi-
paresis (grade 4/5).
CT and MRI examination demonstrated a
very large tumor in the left CP angle with severe
compression of the brain stem (a–g). Although
the fourth ventricle was occluded, the ventricu-
lar system was only slightly dilated. Extensive
destruction of the pyramid was evident.
Tumor removal via the retrosigmoid
approach was planned with subsequent, at a
later stage, hypoglossal–facial anastomosis
for reconstruction of facial nerve function.
The tumor was found to be predominantly
cystic and very tightly adherent to the lower
831
cranial nerves and brain stem. Initially, the
fluid cyst contents were aspirated; then the
capsule was dissected from the lower cranial
nerves in the area of the jugular foramen. The
dissection proceeded to the brain stem, which
allowed identifying the severely atrophic
facial nerve. The trigeminal and the abducens
nerves could be freed from the tumor and pre-
served. Gradually the whole tumor capsule
was separated and resected. The petrous
tumor portion was removed thereafter, and
the bone defect was filled with multiple small
fat pieces, sealed with fibrin glue (h, i).
The patient did not have new neurological
deficits after the surgery. He recovered quickly,
and 2 weeks later the planned facial nerve
reconstruction was performed. The histo-
pathological and immunohistochemical study
of the specimen showed that it was an endo-
lymphatic sac tumor. Considering this finding,
a thorough screening was performed but no
further stigmata of Von Hippel–Lindau dis-
ease were discovered. Active speech therapy
and medialization of the vocal cord in case no
improvement appeared in 6 months were
recommended.
Summarizing, this patient had a very rare
cystic sporadic ELST with a predominant part
in the CP angle. Despite the previous surgery
and the tight adhesions of the cyst capsule to
the brain stem and cranial nerves, the tumor
could be removed completely via the retrosig-
moid approach.
832 11 Other Cerebellopontine Angle Tumors

a b

c
d

(a–g) Preoperative CT of the patient (a, b) and MRI
((c) Axial T1-weighted sequence. (d) Coronal
T1-weighted sequence. (e) Axial T2-weighted
sequence. (f) Axial T1-weighted sequence with
Gadolinium. (g) Coronal T1-weighted sequence with
Gadolinium), demonstrating the extension of the
tumor, the destruction of the pyramid and the degree
of brainstem compression. (h, i) Postoperative CT.
The fat plug is seen as a more hypodense signal
Temporal Bone and Petrous Apex Lesions 833

f g

h
i
834
Fibrous Dysplasia
Fibrous dysplasia (FD) is an idiopathic benign
slowly progressive disorder, in which mature
bone is replaced by fibro-osseous tissue [204].
This developmental anomaly can affect any bone
in the body: it represents 2.5 % of all bone tumors
and 7 % of the benign bone tumors [201]. FD
typically presents during the first three decades
of life.
Histologically, FD consists of varying amounts
of spindle cell bundles and irregular bony trabe-
culae of immature woven bone. Osteoblasts and
osteoclasts are scarce or absent. Common finding
are hemorrhagic areas with associated
inflammatory and giant-cell reactions.
Three patterns of development of FD have
been described: (1) monostotic or involvement
of a single bone (70 % of patients), (2) polyos-
totic or involvement of multiple bones (30 %),
and (3) McCune–Albright syndrome, which is a
rare variant of polyostotic disease, in which FD
is associated with multiple endocrine dysfunc-
tion and café-au-lait spots [216]. The skull and
facial bones are affected in 10–30 % of patients
with the monostotic pattern and in 50 % of
patients with polyostotic fibrous dysplasia
[179, 190]. Most frequently affected bones are
the maxilla, the mandible, the frontal, and the
sphenoid [179, 230].
Three CT variants of FD have been described.
Most frequently seen is the “ground glass”
appearance (56 %) or bone expansion with alter-
nating areas of radiolucency and radiopacity, fol-
lowed by the sclerotic or homogeneously dense
pattern (23 %) and the pagetoid or cystic pattern
(21 %) [184, 190]. The fibrous tissue in FD is
metabolically active and well vascularized.
Therefore, it enhances strongly and on MRI may
resemble soft tissue tumors [184]. On
T1-weighted images, it has low or intermediate
signal intensity depending on the ratio of fibrous
tissue to mineralized matrix [190]. On
T2-weighted images, the signal intensity varies
from low to high.
11 Other Cerebellopontine Angle Tumors
FD should be differentiated form inflammatory
or neoplastic lesions, especially if T2-weighted
images show high signal intensities. In case the
bone expansion conforms to the general shape of
the bone of origin, the diagnosis may be more
straightforward [190]. The more rare localized
bone involvement, however, might represent a
diagnostic challenge. The radiological differential
diagnosis of FD includes chordoma, chondrosar-
coma, chondroblastoma, carcinomas nasopharyn-
geal, metastasis, plasmacytoma, giant-cell tumors,
hemangiomas, and Paget’s disease [169].
Fibrous dysplasia affects the temporal bone
very rarely. Its usual presentation is progressive
hearing loss. Expanding mass over the postau-
ricular region may be seen. Hearing loss is usu-
ally conductive and is caused by external auditory
canal obliteration, although sensorineural deaf-
ness may develop if the lesion involves the inner
ear structures [186]. The intracranial extension of
the lesion causes symptoms, due to neural
compression.
The clinical evolution of FD is unpredictable:
most of the lesions tend to stabilize with the onset
of puberty, while other may show aggressive
growth and may even undergo malignant trans-
formation. The management options vary respec-
tively: from close observation to resection of the
lesions. Surgery is generally indicated in case of
cosmetic problems or compression of neural
structures and is aimed at correcting or prevent-
ing functional problems and achieving normal
facial esthetics [169, 198]. Depending on the par-
ticular site of origin, extension pattern, and clini-
cal presentation, the goal is to decompress the
cranial nerves or brain stem, to remove the bulk
of the lesion, and to resolve the esthetic problem
(see Case Presentation: Fibrous Dysplasia of the
Petrous Bone).
Radiotherapy is not recommended even in
case of incomplete removal because of the risk
of malignant transformation [240]. Some
reports, however, suggest that even without
radiotherapy spontaneous malignant transfor-
mation can occur [271].
Temporal Bone and Petrous Apex Lesions
Case Illustration 11.26
Fibrous Dysplasia of the Petrous Bone
This 17-year-old boy presented with a history
of dizziness, vertigo, hearing loss, and tinnitus
on the right side for more than 2 years. Several
months later, progressive facial palsy appeared.
MRI was performed that demonstrated a con-
trast-enhancing lesion in the petrous apex area
with small extension toward the CP angle
(a, b). Follow-up of the lesion with MRI was
recommended by the consulting neurosurgeon.
One month prior to presentation, double vision
to the right side appeared, and the new MRI
demonstrated further growth of the lesion.
At examination the patient had peripheral
facial paresis (HB grade V), nonfunctional
hearing, and absent gag reflex on the right
side. CT showed an area of bone destruction
involving the medial part of the petrous bone,
clivus, and sphenoid bone (c, d). The lesion
had the typical “ground glass” appearance:
bone expansion with alternating areas of
increased and decreased density. On MRI the
lesion was heterogeneous, intensely enhanc-
ing, and had smooth boundary to adjacent
structures (e–h). Compared to the initial
images (a, b), a certain growth tendency was
evident with predominant expansion in the CP
angle, in the cavernous sinus, and in sphenoid
sinus areas. A presumptive diagnosis of sphe-
nopetroclival fibrous dysplasia was made.
The huge size and the extension of the
lesion precluded its safe resection in one
stage. Considering the presence of brain stem
compression, the retrosigmoid approach was
favored. Besides the facial nerve, EMG,
BAEP, SEP, as well as the abducens and the
835
lower cranial nerves were also monitored and
the standard RS approach was performed.
Once the lesion in the CP angle was exposed,
its capsule was coagulated and internal deb-
ulking was done. The lesion was very well
vascularized and relatively firm with areas of
calcification. Using CUSA and high-speed
diamond drill, the whole CP angle tumor part,
as well as part of the petrous part, was
removed. The abducens nerve was identified
in the area of its exit at the brain stem and fol-
lowed to the Dorello’s canal. The lower cra-
nial nerves were severely compressed and
dislocated caudally but could be freed from
the tumor and preserved. The continuity of
the facial and the cochlear nerves was also
preserved. Complete removal of the lesion
without undue risk was considered impossi-
ble because of the encasement of the carotid
artery’s petrous segment and the presence of
cavernous sinus extension. Thorough hemo-
stasis was achieved, and the tumor cavity was
filled with fat pieces. The patient had an
uneventful recovery with recovery of the
swallowing and improvement of the facial
nerve palsy to HB grade III in 6 months. (i, j:
early postoperative CT control).
The histopathological diagnosis was fibrous
dysplasia. Follow-up CT and MRI scans at 6
months were recommended with the option of
second surgery via an endonasal approach in
case of growth of the remnant. Twelve months
later, he sent his CT, which showed a large
tumor recurrence with obstructive hydroceph-
alus (k–n). A VP shunt was inserted elsewhere.
The patient is scheduled for a new surgery at
our institute.
836 11 Other Cerebellopontine Angle Tumors

a b

c d

e f

(a, b) Initial MRI: T1-weighted MR images after
gadolinium application. CT scans (c, d) and MR
images scans performed at presentation to our insti-
tute. (e, f) T1-weighted sequences after gadolinium
administration. (g) T2-weighted sequence. (h)
T2-CISS sequence. (i, j) Postoperative CT, demon-
strating the extent of removal of the lesion and the
decompression of the brain stem. (k–n) Last CT
scans, showing a large tumor recurrence and obstruc-
tive hydrocephalus
Temporal Bone and Petrous Apex Lesions 837

g h

i j
838 11 Other Cerebellopontine Angle Tumors

k l

m n
Temporal Bone and Petrous Apex Lesions 839

Osteomas ders, and genetic defects may contribute to

Osteomas are rare benign slow-growing cranial
tumors originating typically from the inner or
outer table of the calvarium, the bones of the
face or mandible [178]. They are round to oval
sessile lesions, composed of compact bone.
Skull-base osteomas in the fronto-ethmoid area
have been described relatively frequently. The
pyramid is rarely affected and the CP angle and
IAC are extremely rare [237]. Less than 25
such cases have been reported in the literature
[181, 257, 264]. It has been proposed that
trauma, inflammation, developmental disor-
their etiology. Recently we presented the first
case of bilateral osteomas arising from the
IAC and extending to the CP angle:
Fig. 11.23a–d [207].
Osteomas are usually asymptomatic for many
years and are discovered incidentally. They may
cause symptoms due to local compressive effect,
depending on their specific origin and extension:
trigeminal neuralgia, sensorineural hearing loss,
vertigo, and tinnitus or even symptoms/signs of
brain stem compression [257, 264, 277].
Clinically they may be undistinguishable from
VS or CP meningiomas.

a b

IAC

c d

Fig. 11.23 CT (a) axial view, (b) coronal view, (c) sagittal view and MRI (d) of a patient with an osteoma in the area
of the porus of the IAC
840
Osteomas should be differentiated from osteo-
chondroma, osteosarcoma, ossifying fibroma,
osteoblastic metastasis, isolated eosinophilic
granuloma, Paget’s disease, giant-cell tumor,
osteoid osteoma, calcified meningioma and
monostotic fibrous dysplasia, as well as temporal
bone exostoses. Exostoses are usually multiple
broad-based bone elevations, characterized histo-
logically by parallel, concentric layers of subpe-
riosteal bone [221]. Osteomas are usually solitary,
pedunculated lesions, with typical internal struc-
ture: they are composed of discrete fibrovascular
channels surrounded by irregularly oriented
lamellated bone [181, 277].
Asymptomatic tumors should be observed and
followed with regular CT examinations. The
treatment of choice of symptomatic or enlarging
osteomas is surgical removal [181, 237].
Following surgery, symptoms usually completely
resolve or at least improve [196, 207]. Davis et al.
[196] analyzed 12 patients, 2 from the authors
own experience and 10 from the literature. Three
of the 8 operated patients showed improvement
of hearing loss, resolution of their dizziness
occurred in 5 patients, and resolution of tinnitus
occurred in 4 patients. In case of chronic
11 Other Cerebellopontine Angle Tumors
compression of the cochlear and vestibular
nerves, however, surgery may be less beneficial.
Pleomorphic Adenomas
Pleomorphic adenoma is the most common tumor
of the parotid, submandibular, and minor salivary
glands. It is composed of both glandular epithelial
and mesenchymal elements, hence the name mixed
tumor. Intracranial invasion of the tumor is very
rare [282]. Pleomorphic adenomas of the external
auditory canal are benign tumors of the ceruminal
glands, arising from the lateral cartilaginous por-
tion of the canal [177]. Ceruminal glands are
modified sweat glands of the skin of the external
auditory meatus. These tumors are usually benign:
wide local excision is the mainstay of treatment
[233]. They may, however, extend to the petrous
bone and cause wide bone destruction with respec-
tive symptoms. Peters et al. [251] described a case
of a pleomorphic adenoma of the middle ear and
mastoid, causing extensive bony destruction and
extending into the posterior cranial fossa. Primary
posterior fossa or CP angle pleomorphic adenomas
are exceedingly rare [283].
Temporal Bone and Petrous Apex Lesions
Case Illustration 11.27
Pleomorphic Adenoma of the Petrous Bone
and CP Angle
A 41-year-old male patient presented with a
recurrent tumor of the left petrous bone and
the left CP angle. He has been operated at
another hospital for a pleomorphic adenoma 7
years earlier via a cervical approach on the left
side, followed by fractionated radiotherapy
(50 Gy total dosage).
Seven years later, a MRI follow-up study
showed a lesion in the left petrous bone with
large CP angle portion. An open transmastoid
biopsy was done, confirming the diagnosis of
pleomorphic adenoma. The tumor was then
removed via a transmastoid transpetrous
approach. MRI study, performed 6 months
later, demonstrated tumor involvement of the
left cavernous sinus and of the adjacent skull-
base area, which was treated with gamma
knife radiosurgery.
At presentation to our department 3 years
later, the patient had gait ataxia, trigeminal
hypesthesia (V2, 3), abducens nerve palsy,
complete hearing loss, and tinnitus on the left
side. MRI demonstrated a large CP angle
841
tumor, extending caudally to the level of the
jugular foramen (a–d). The tumor, which
proved to be a pleomorphic adenoma, was
removed via the retrosigmoid route. After sur-
gery the patient recovered rapidly and had a
slight (HB grade II) facial palsy at discharge.
His gait improved considerably and in 6
months was normal.
The patient presented 3 years later with
CSF leak on the left side via a small perfora-
tion of the tympanic membrane. His gait, as
well as the facial nerve function, was normal.
The trigeminal hypesthesia (V2,3), abducens
nerve palsy, complete hearing loss, and tinni-
tus on the left side, however, persisted. MRI
and CT revealed liquid collection in the mas-
toid and a suspicious bone defect in the lower
medial part of the mastoid process. Considering
the clinical and radiological findings, a deci-
sion for surgical treatment was taken: dural
repair with fat pieces was performed trans-
mastoidally and the Eustachian tube, as well
as the external ear canal, was closed. At 12
months follow-up examination, the patient
had no new complaints. The follow-up MRI is
shown on figures e–g.
842 11 Other Cerebellopontine Angle Tumors

a b

c d

MR examination, demonstrating the tumor in the left Postoperative MR images: (e, f) T1-weighted sequences
CP angle and the involvement of the petrous bone. (a, b) after gadolinium administration. (g) T2-weighted
T1-weighted sequences after gadolinium administra- sequence
tion. (c) T2-weighted sequence. (d) CISS sequence.
Temporal Bone and Petrous Apex Lesions 843

e f

g
844
Intralabyrinthine Schwannomas
Mayer has described for the first time in 1917.
Since then less than 60 cases have been presented
in the literature. These tumors originate from the
distant branches of the cochlear, superior vestibu-
lar, or inferior vestibular nerves. Previously, most
cases have been found at autopsy or incidentally
during labyrinthectomy for Meniere’s syndrome
or intractable vertigo [208, 245]. Nowadays, gad-
olinium-enhanced MRI allows for preoperative
diagnosis of such tumors [200].
Patients with intralabyrinthine schwannomas
present typically with hearing loss, vertigo, or
tinnitus [245]. Hearing loss might be of mixed
type: a conductive component secondary to inter-
ference with stapes movement might be present.
Green and McKenzie [208] stressed that symp-
toms associated with intralabyrinthine schwan-
noma may be confused with Meniere’s disease
(hearing loss, tinnitus, aural fullness, and epi-
sodic vertigo). Therefore, they recommend pre-
operative high-resolution MRI in all patients with
serviceable hearing and intractable vertigo,
undergoing surgery for Meniere’s disease.
Patients with profound hearing loss are best
managed surgically. Surgical removal of such
tumors leads to complete hearing loss. Therefore,
in case of preserved functional hearing, initial
observation with serial audiograms and MRI
might be an alternative [200, 208].
References
Intra-Axial Tumors Extending
Secondarily to the Cerebellopontine
Angle
1. Ahn MS, Jackler RK (1997) Exophytic brain tumors
mimicking primary lesions of the cerebellopontine
angle. Laryngoscope 107(4):466–471
2. Arnautovic KI, Husain MM, Linskey ME (2000) Cranial
nerve root entry zone primary cerebellopontine angle
gliomas: a rare and poorly recognized subset of extra-
parenchymal tumors. J Neurooncol 49(3):205–212
3. Beutler AS, Hsiang JK, Moorhouse DF, Hansen LA,
Alksne JF (1995) Pilocytic astrocytoma presenting as
an extra-axial tumor in the cerebellopontine angle:
case report. Neurosurgery 37(1):125–128
11 Other Cerebellopontine Angle Tumors
4. Bonneville F, Savatovsky J, Chiras J (2007) Imaging
of cerebellopontine angle lesions: an update. Part 2:
intra-axial lesions, skull base lesions that may invade
the CPA region, and non-enhancing extra-axial
lesions. Eur Radiol 17(11):2908–2920. doi:10.1007/
s00330-007-0680-4
5. Brackmann DE, Bartels LJ (1980) Rare tumors of the
cerebellopontine angle. Otolaryngol Head Neck Surg
88(5):555–559
6. Chiechi MV, Smirniotopoulos JG, Jones RV (1995)
Intracranial subependymomas: CT and MR imaging
features in 24 cases. AJR Am J Roentgenol 165(5):
1245–1250
7. Cosgrove GR, Villemure JG, Robitaille Y, Melanson
D (1985) Extraaxial ependymoma of the posterior
fossa. Surg Neurol 24(4):433–436
8. Cuthikonda B, Buckleair LJ, Goodman JC, Powell
SZ, Rose JE (2009) Ganglioglioma in the cerebello-
pontine angle of an adult without seizures. J La State
Med Soc 161(3):143–146
9. Donich D, Lee JH, Prayson R (1999) Giant extra-axial
cerebellopontine angle/cavernous sinus ependymoma:
case report. Neurosurgery 44(1):195–198
10. Enomoto H, Mizuno M, Katsumata T, Doi T (1991)
Intracranial metastasis of a choroid plexus papilloma
originating in the cerebellopontine angle region: a
case report. Surg Neurol 36(1):54–58
11. Forton G, Verlooy J, Cras P, Parizel P, Van de Heyning
P (1992) Problems with flute playing: an otological
problem? Case report of a peculiar cerebellar
astrocytoma. Acta Otorhinolaryngol Belg 46(4):
405–410
12. Fukui MB, Hogg JP, Martinez AJ (1997) Extraaxial
ependymoma of the posterior fossa. AJNR Am J
Neuroradiol 18(6):1179–1181
13. Furuya K, Sasaki T, Saito N, Atsuchi M, Kirino T
(1995) Primary large choroid plexus papillomas in the
cerebellopontine angle: radiological manifestations
and surgical management. Acta Neurochir (Wien)
135(3–4):144–149
14. Gilbertson RJ (2004) Medulloblastoma: signalling a
change in treatment. Lancet Oncol 5(4):209–218.
doi:10.1016/S1470-2045(04)01424-X
15. Hoeffel C, Boukobza M, Polivka M, Lot G,
Guichard JP, Lafitte F, Reizine D, Merland JJ (1995)
MR manifestations of subependymomas. AJNR Am J
Neuroradiol 16(10):2121–2129
16. House JL, Burt MR (1985) Primary CNS tumors
presenting as cerebellopontine angle tumors. Am J
Otol (Suppl):147–153
17. Ikezaki K, Matsushima T, Inoue T, Yokoyama N,
Kaneko Y, Fukui M (1993) Correlation of microana-
tomical localization with postoperative survival in
posterior fossa ependymomas. Neurosurgery 32(1):
38–44
18. Jallo G (2006) Brainstem gliomas. Childs Nerv Syst
22(1):1–2. doi:10.1007/s00381-005-1267-5
19. Kariya S, Nishizaki K, Aoji K, Akagi H (1998)
Primary malignant lymphoma in the cerebellopontine
angle. J Laryngol Otol 112(5):476–479
References
20. Kasantikul V, Palmer JO, Netsky MG, Glasscock ME
3rd, Hays JW (1980) Glioma of the acoustic nerve.
Arch Otolaryngol 106(8):456–459
21. Kieserman S, Linstrom C, McCormick S, Petschenik AJ
(1996) Choroid plexus papilloma of the cerebellopon-
tine angle. Am J Otol 17(1):119–122
22. Koral K, Kedzierski RM, Gimi B, Gomez A,
Rollins NK (2008) Subependymoma of the cerebel-
lopontine angle and prepontine cistern in a 15-year-old
adolescent boy. AJNR Am J Neuroradiol 29(1):190–
191, ajnr.A0821 [pii]
23. Kumar R, Achari G, Banerjee D, Chhabra DK (2001)
Uncommon presentation of medulloblastoma. Childs
Nerv Syst 17(9):538–542; discussion 543
24. Kumar R, Achari G, Benerji D, Jain VK, Chhabra DK
(2002) Choroid plexus papillomas of the cerebello-
pontine angle. Neurol India 50(3):352–358
25. Martin N, Pierot L, Sterkers O, Mompoint D,
Nahum H (1990) Primary choroid plexus papilloma
of the cerebellopontine angle: MR imaging.
Neuroradiology 31(6):541–543
26. Massimino M, Giangaspero F, Garre ML, Genitori L,
Perilongo G, Collini P, Riva D, Valentini L, Scarzello
G, Poggi G, Spreafico F, Peretta P, Mascarin M,
Modena P, Sozzi G, Bedini N, Biassoni V, Urgesi A,
Balestrini MR, Finocchiaro G, Sandri A, Gandola L
(2006) Salvage treatment for childhood ependymoma
after surgery only: pitfalls of omitting “at once” adju-
vant treatment. Int J Radiat Oncol Biol Phys
65(5):1440–1445, S0360-3016(06)00675-4 [pii]
27. Mehta JS, Sharr MM (1998) An unusual cause of
acute labyrinthine failure. J Laryngol Otol 112(2):
193–195
28. Merchant TE, Boop FA, Kun LE, Sanford RA (2008)
A retrospective study of surgery and reirradiation for
recurrent ependymoma. Int J Radiat Oncol Biol Phys
71(1):87–97, S0360-3016(07)04225-3 [pii]
29. Meyers SP, Kemp SS, Tarr RW (1992) MR imaging
features of medulloblastomas. AJR Am J Roentgenol
158(4):859–865
30. Milligan BD, Giannini C, Link MJ (2007)
Ganglioglioma in the cerebellopontine angle in a
child. Case report and review of the literature. J
Neurosurg 107(4 Suppl):292–296. doi:10.3171/PED-
07/10/292
31. Mirone G, Schiabello L, Chibbaro S, Bouazza S,
George B (2009) Pediatric primary pilocytic astrocy-
toma of the cerebellopontine angle: a case report.
Childs Nerv Syst 25(2):247–251. doi:10.1007/
s00381-008-0690-9
32. Nishimura T, Uchida Y, Fukuoka M, Ono Y,
Kurisaka M, Mori K (1998) Cerebellopontine angle
lymphoma: a case report and review of the literature.
Surg Neurol 50(5):480–485, S0090-3019(97)00362-5
[pii]; discussion 485–486
33. Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, Kalifa C,
Brunelle F, Sgouros S, Meyer P, Cinalli G, Zerah M,
Pierre-Kahn A, Renier D (1998) Papillomas and carcino-
mas of the choroid plexus in children. J Neurosurg
88(3):521–528. doi:10.3171/jns.1998.88.3.0521
845
34. Pollack IF, Gerszten PC, Martinez AJ, Lo KH,
Shultz B, Albright AL, Janosky J, Deutsch M (1995)
Intracranial ependymomas of childhood: long-term
outcome and prognostic factors. Neurosurgery
37(4):655–666; discussion 666–657
35. Prayson RA, Suh JH (1999) Subependymomas:
clinicopathologic study of 14 tumors, including
comparative MIB-1 immunohistochemical analysis
with other ependymal neoplasms. Arch Pathol Lab
Med 123(4):306–309, 10.1043/0003-9985(1999)
123<0306:S>2.0.CO;2
36. Raffel C (2004) Medulloblastoma: molecular genet-
ics and animal models. Neoplasia 6(4):310–322.
doi:10.1593/neo.03454
37. Rea GL, Akerson RD, Rockswold GL, Smith SA
(1983) Subependymoma in a 2 1/2-year-old boy. Case
report. J Neurosurg 59(6):1088–1091. doi:10.3171/
jns.1983.59.6.1088
38. Rossi A, Caracciolo V, Russo G, Reiss K, Giordano A
(2008) Medulloblastoma: from molecular pathology
to therapy. Clin Cancer Res 14(4):971–976.
doi:10.1158/1078-0432.CCR-07-2072, 14/4/971 [pii]
39. Rovit RL, Schechter MM, Chodroff P (1970) Choroid
plexus papillomas. Observations on radiographic
diagnosis. Am J Roentgenol Radium Ther Nucl Med
110(3):608–617
40. Rushing EJ, Cooper PB, Quezado M, Begnami M,
Crespo A, Smirniotopoulos JG, Ecklund J, Olsen C,
Santi M (2007) Subependymoma revisited: clinico-
pathological evaluation of 83 cases. J Neurooncol
85(3):297–305. doi:10.1007/s11060-007-9411-6
41. Sanford RA, Merchant TE, Zwienenberg-Lee M,
Kun LE, Boop FA (2009) Advances in surgical tech-
niques for resection of childhood cerebellopontine
angle ependymomas are key to survival. Childs Nerv
Syst 25(10):1229–1240. doi:10.1007/s00381-009-
0886-7
42. Springborg JB, Poulsgaard L, Thomsen J (2008)
Nonvestibular schwannoma tumors in the cerebello-
pontine angle: a structured approach and management
guidelines. Skull Base 18(4):217–227. doi:10.1055/s-
2007-1016959
43. Swaroop GR, Whittle IR (1997) Exophytic pon-
tine glioblastoma mimicking acoustic neuroma.
J Neurosurg Sci 41(4):409–411
44. Tacconi L, Delfini R, Cantore G (1996) Choroid
plexus papillomas: consideration of a surgical series
of 33 cases. Acta Neurochir (Wien) 138(7):802–810
45. Takada Y, Ohno K, Tamaki M, Hirakawa K (1999)
Cerebellopontine angle pilocytic astrocytoma mim-
icking acoustic schwannoma. Neuroradiology 41(12):
949–950
46. Talacchi A, De Micheli E, Lombardo C, Turazzi S,
Bricolo A (1999) Choroid plexus papilloma of the
cerebellopontine angle: a twelve patient series.
Surg Neurol 51(6):621–629, S0090-3019(99)00024-5
[pii]
47. Yamada S, Aiba T, Hara M (1993) Cerebellopontine
angle medulloblastoma: case report and literature
review. Br J Neurosurg 7(1):91–94
846
48. Yang PJ, Seeger JF, Carmody RF, Mehta BA (1987)
Cerebellopontine angle lymphoma. AJNR Am J
Neuroradiol 8(2):368–369
49. Yoshimura J, Nishiyama K, Fukuda M, Watanabe M,
Igarashi H, Fujii Y (2009) Adult cerebellopontine
angle medulloblastoma originating in the pons mim-
icking focal brainstem tumor. J Neuroimaging
19(4):385–387, JON298 [pii]
50. Yuh EL, Barkovich AJ, Gupta N (2009) Imaging of
ependymomas: MRI and CT. Childs Nerv Syst
25(10):1203–1213. doi:10.1007/s00381-009-0878-7
Rare Extra-axial Cerebellopontine
Angle Lesions
51. Abbott R (2004) The endoscopic management of
arachnoidal cysts. Neurosurg Clin N Am 15(1):9–17.
doi:10.1016/S1042-3680(03)00071-8
52. Abe K, Oyama K, Mori K, Ishimaru S, Eguchi M,
Maeda M (1999) Neurenteric cyst of the craniocervi-
cal junction – case report. Neurol Med Chir (Tokyo)
39(12):875–880, JST.Journalarchive/nmc1959/39.875
[pii]
53. Agnoli AL, Laun A, Schonmayr R (1984) Entero-
genous intraspinal cysts. J Neurosurg 61(5):834–840.
doi:10.3171/jns.1984.61.5.0834
54. Aguiar PH, Agner C, Tavares FR, Yamaguchi N (2001)
Unusual brain metastases from papillary thyroid carci-
noma: case report. Neurosurgery 49(4):1008–1013
55. Andaluz N, Pensak ML, Zuccarello M (2005)
Multiple, peripheral aneurysms of the anterior inferior
cerebellar artery. Acta Neurochir (Wien) 147(4):419–
422. doi:10.1007/s00701-004-0453-7; discussion 422
56. Arriaga MA, Lo WW, Brackmann DE (1995)
Metastatic melanoma to the cerebellopontine angle.
Clinical and imaging characteristics. Arch Otolaryngol
Head Neck Surg 121(9):1052–1056
57. Babu R, Murali R (1991) Arachnoid cyst of the
cerebellopontine angle manifesting as contralateral
trigeminal neuralgia: case report. Neurosurgery 28(6):
886–887
58. Barrera JE, Jenkins H, Said S (2004) Cavernous
hemangioma of the internal auditory canal: a case
report and review of the literature. Am J Otolaryngol
25(3):199–203, S0196070903001571 [pii]
59. Bejjani GK, Wright DC, Schessel D, Sekhar LN
(1998) Endodermal cysts of the posterior fossa. Report
of three cases and review of the literature. J Neurosurg
89(2):326–335. doi:10.3171/jns.1998.89.2.0326
60. Bengochea FG, Blanco FL (1955) Arachnoidal cysts
of the cerebellopontine angle. J Neurosurg 12(1):
66–71. doi:10.3171/jns.1955.12.1.0066
61. Bertalanffy H, Benes L, Miyazawa T, Alberti O,
Siegel AM, Sure U (2002) Cerebral cavernomas in the
adult. Review of the literature and analysis of 72 sur-
gically treated patients. Neurosurg Rev 25(1–2):1–53;
discussion 54–55
11 Other Cerebellopontine Angle Tumors
62. Birch BD, McCormick PC (1996) High cervical split
cord malformation and neurenteric cyst associated
with congenital mirror movements: case report.
Neurosurgery 38(4):813–815; discussion 815–816
63. Bonneville F, Sarrazin JL, Marsot-Dupuch K,
Iffenecker C, Cordoliani YS, Doyon D, Bonneville JF
(2001) Unusual lesions of the cerebellopontine angle:
a segmental approach. Radiographics 21(2):419–438
64. Bonneville F, Savatovsky J, Chiras J (2007) Imaging
of cerebellopontine angle lesions: an update. Part 1:
enhancing extra-axial lesions. Eur Radiol 17(10):
2472–2482. doi:10.1007/s00330-007-0679-x
65. Bonneville F, Savatovsky J, Chiras J (2007) Imaging
of cerebellopontine angle lesions: an update. Part 2:
intra-axial lesions, skull base lesions that may invade
the CPA region, and non-enhancing extra-axial
lesions. Eur Radiol 17(11):2908–2920. doi:10.1007/
s00330-007-0680-4
66. Brackmann DE, Bartels LJ (1980) Rare tumors of the
cerebellopontine angle. Otolaryngol Head Neck Surg
88(5):555–559
67. Brodsky JR, Smith TW, Litofsky S, Lee DJ (2006)
Lipoma of the cerebellopontine angle. Am J Otolaryngol
27(4):271–274, S0196-0709(05)00218-8 [pii]
68. Brooks ML, Mayer DP, Sataloff RT, Myers DL,
Spiegel JR, Ruiz S (1992) Intracanalicular arachnoid
cyst mimicking acoustic neuroma: CT and MRI.
Comput Med Imaging Graph 16(4):283–285
69. Caldarelli M, Massimi L, Kondageski C, Di Rocco C
(2004) Intracranial midline dermoid and epidermoid
cysts in children. J Neurosurg 100(5 Suppl
Pediatrics):473–480. doi:10.3171/ped.2004.100.5.0473
70. Carvalho GA, Cervio A, Matthies C, Samii M (2000)
Subarachnoid fat dissemination after resection of a
cerebellopontine angle dysontogenic cyst: case report
and review of the literature. Neurosurgery 47(3):
760–763; discussion 763–764
71. Carvalho GA, Matthies C, Osorio E, Samii M (2003)
Hamartomas of the internal auditory canal: report of
two cases. Neurosurgery 52(4):944–948; discussion
948–949
72. Celis MA, Mourier KL, Polivka M, Boissonnet H,
Kato T, Lot G, George B, Cophignon J (1992)
Cisternal cysticercosis of the cerebellopontine angle.
A surgically-treated case and review of the literature.
Neurochirurgie 38(2):108–112
73. Chang SD, Meisel JA, Hancock SL, Martin DP,
McManus M, Adler JR Jr (1998) Treatment of heman-
gioblastomas in von Hippel-Lindau disease with lin-
ear accelerator-based radiosurgery. Neurosurgery
43(1):28–34; discussion 34–35
74. Chaynes P, Thorn-Kany M, Sol JC, Arrue P,
Lagarrigue J, Manelfe C (1998) Imaging in neuren-
teric cysts of the posterior cranial fossa. Neuroradiology
40(6):374–376
75. Cheng JS, Cusick JF, Ho KC, Ulmer JL (2002)
Lateral supratentorial endodermal cyst: case report
and review of literature. Neurosurgery 51(2):493–499;
discussion 499
References
76. Chihara Y, Iwasaki S, Ushio M, Sugasawa K,
Murofushi T (2009) Fusiform aneurysm of the basilar
artery presenting as a cerebellopontine angle mass.
Eur Arch Otorhinolaryngol 266(1):151–152.
doi:10.1007/s00405-008-0635-7
77. Chiong Y, Mulroy L, Fleetwood IG, Younis T (2009)
Isolated metastasis to the cerebellopontine angle sec-
ondary to breast cancer. Can J Surg 52(5):E213–E214
78. Constans JP, Meder F, Maiuri F, Donzelli R,
Spaziante R, de Divitiis E (1986) Posterior fossa
hemangioblastomas. Surg Neurol 25(3):269–275
79. Conway JE, Chou D, Clatterbuck RE, Brem H,
Long DM, Rigamonti D (2001) Hemangioblastomas
of the central nervous system in von Hippel-Lindau
syndrome and sporadic disease. Neurosurgery 48(1):
55–62; discussion 62–63
80. Dalley RW, Robertson WD, Nugent RA, Durity FA
(1986) Computed tomography of anterior inferior
cerebellar artery aneurysm mimicking an acoustic
neuroma. J Comput Assist Tomogr 10(5):881–884
81. Davis LE, Kornfeld M (1991) Neurocysticercosis:
neurologic, pathogenic, diagnostic and therapeutic
aspects. Eur Neurol 31(4):229–240
82. Debelius A, Rudolph J, Schrom T (2006) Lipoma of the
cerebellopontineangle–acasereport.Laryngorhinootologie
85(2):128–131. doi:10.1055/s-2005-870116
83. Depper MH, Carlow TJ, Crooks LA, Orrison WW Jr
(1996) Intracranial dissemination of a pituitary adenoma:
presentation as an unusual mass in the cerebellopontine
angle. AJR Am J Roentgenol 166(6):1500–1501
84. Deshmukh VR, Albuquerque FC, Zabramski JM,
Spetzler RF (2003) Surgical management of cavern-
ous malformations involving the cranial nerves.
Neurosurgery 53(2):352–357; discussion 357
85. Devkota UP, Lam JM, Ng H, Poon WS (1994) An
anterior intradural neurenteric cyst of the cervical
spine: complete excision through central corpectomy
approach – case report. Neurosurgery 35(6):
1150–1153; discussion 1153–1154
86. Drake CG, Friedman AH, Peerless SJ (1986)
Posterior fossa arteriovenous malformations. J Neuro-
surg 64(1):1–10. doi:10.3171/jns.1986.64.1.0001
87. Duchene M, Benoudiba F, Iffenecker C, Hadj-Rabia
M, Caldas J, Doyon D (1999) Neurocysticercosis.
J Radiol 80(12):1623–1627, MDOI-JR-12-1999-80-
12-0221-0363-101019-ART2 [pii]
88. Dutt SN, Mirza S, Chavda SV, Irving RM (2002)
Radiologic differentiation of intracranial epidermoids
from arachnoid cysts. Otol Neurotol 23(1):84–92
89. Eynon-Lewis NJ, Kitchen N, Scaravilli F, Brookes
GB (1998) Neurenteric cyst of the cerebellopontine
angle: case report. Neurosurgery 42(3):655–658
90. Falcioni M, Piccirillo E, Di Trapani G, Romano G,
Russo A (2004) Internal auditory canal metastasis.
Acta Otorhinolaryngol Ital 24(2):78–82
91. Filho FL, Tatagiba M, Carvalho GA, Weichhold W,
Klekamp J, Samii M (2001) Neurenteric cyst of the
craniocervical junction. Report of three cases.
J Neurosurg 94(1 Suppl):129–132
847
92. Filling-Katz MR, Choyke PL, Patronas NJ,
Gorin MB, Barba D, Chang R, Doppman JL,
Seizinger B, Oldfield EH (1989) Radiologic screen-
ing for von Hippel-Lindau disease: the role of
Gd-DTPA enhanced MR imaging of the CNS. J
Comput Assist Tomogr 13(5):743–755
93. Geddes JF, Sutcliffe JC, King TT (1995) Mixed cra-
nial nerve tumors in neurofibromatosis type 2. Clin
Neuropathol 14(6):310–313
94. Geetha N, James FV, Ramachandran K,
Ajithkumar TV, Nair MK (1999) Ovarian carcinoma
relapsing as a cerebellopontine angle tumour.
Australas Radiol 43(3):388–390
95. Gjuric M, Koester M, Paulus W (2000) Cavernous
hemangioma of the internal auditory canal arising
from the inferior vestibular nerve: case report and
review of the literature. Am J Otol 21(1):110–114
96. Gokcan MK, Batikhan H, Calguner M, Tataragasi
AI (2006) Unilateral hearing loss as a presenting
manifestation of granulocytic sarcoma (chloroma).
Otol Neurotol 27(1):106–109, 00129492-
200601000-00019 [pii]
97. Gonul E, Yetiser S, Tasar M, Ongoru O (2006)
Glioneural hamartoma of the VIIIth nerve. J Laryngol
Otol 120(1):70–73, S0022215105009515 [pii]
98. Guidetti B, Gagliardi FM (1977) Epidermoid and
dermoid cysts. Clinical evaluation and late surgical
results. J Neurosurg 47(1):12–18. doi:10.3171/
jns.1977.47.1.0012
99. Guilemany JM, Alobid I, Gaston F, Morrello A,
Bernal-Sprekelsen M (2005) Cerebellopontine angle
and internal auditory canal metastasis from ductal
carcinoma of the breast. Acta Otolaryngol 125(9):
1004–1007
100. Hadley MN, Grahm TW, Daspit CP, Spetzler RF
(1985) Otolaryngologic manifestations of posterior
fossa arachnoid cysts. Laryngoscope 95(6):
678–681
101. Harris CP, Dias MS, Brockmeyer DL, Townsend JJ,
Willis BK, Apfelbaum RI (1991) Neurenteric cysts
of the posterior fossa: recognition, management, and
embryogenesis. Neurosurgery 29(6):893–897; dis-
cussion 897–898
102. Huang TW, Young YH (2002) Differentiation
between cerebellopontine angle tumors in cancer
patients. Otol Neurotol 23(6):975–979
103. Ikeda H, Deinsberger W, Boker DK (2000)
Retroclival arachnoid cyst presenting with spontane-
ous intracystic haemorrhage – case presentation.
Acta Neurochir (Wien) 142(11):1317–1318
104. Ildan F, Gocer AI, Bagdatoglu H, Uzuneyupoglu
Z, Tuna M, Cetinalp E (1996) Isolated trigeminal
neuralgia secondary to distal anterior inferior cer-
ebellar artery aneurysm. Neurosurg Rev 19(1):
43–46
105. Iplikcioglu C, Bikmaz K, Gokduman CA, Bek S
(2006) Cerebellopontine angle lipoma with extracra-
nial extension. J Clin Neurosci 13(10):1045–1047,
S0967-5868(06)00471-1 [pii]
848
106. Izci Y, Secer HI, Gonul E, Onguru O (2007)
Simultaneously occurring vestibular schwannoma
and meningioma in the cerebellopontine angle: case
report and literature review. Clin Neuropathol 26(5):
219–223
107. Jallo GI, Woo HH, Meshki C, Epstein FJ, Wisoff JH
(1997) Arachnoid cysts of the cerebellopontine
angle: diagnosis and surgery. Neurosurgery 40(1):
31–37; discussion 37–38
108. Jamjoom AB, Jamjoom ZA, al-Fehaily M,
el-Watidy S, al-Moallem M, Nain Ur R (1996)
Trigeminal neuralgia related to cerebellopontine
angle tumors. Neurosurg Rev 19(4):237–241
109. Jawahar A, Kondziolka D, Garces YI, Flickinger JC,
Pollock BE, Lunsford LD (2000) Stereotactic radio-
surgery for hemangioblastomas of the brain. Acta
Neurochir (Wien) 142(6):641–644; discussion
644–645
110. Kalavagunta S, Karkanevatos A, Swift AC (2006)
Giant vertebro-basilar aneurysm: an unusual cere-
bello-pontine angle lesion. J Laryngol Otol
120(2):e8
111. Kamitani H, Hirano N, Takigawa H, Yokota M,
Miyata H, Ohama E, Watanabe T (2004) Attenuation
of vascularity by preoperative radiosurgery facili-
tates total removal of a hypervascular hemangioblas-
toma at the cerebello-pontine angle: case report.
Surg Neurol 62(3):238–243. doi:10.1016/j.
surneu.2003.09.030; discussion 243–234
112. Kawamata T, Kawamura H, Kubo O, Sasahara A,
Yamazato M, Hori T (1999) Central nervous system
metastasis from gallbladder carcinoma mimicking a
meningioma. Case illustration. J Neurosurg 91(6):
1059. doi:10.3171/jns.1999.91.6.1059
113. Kohan D, Downey LL, Lim J, Cohen NL, Elowitz E
(1997) Uncommon lesions presenting as tumors of
the internal auditory canal and cerebellopontine
angle. Am J Otol 18(3):386–392
114. Komotar RJ, Connolly ES Jr, Khandji A,
Teitelbaum GP, Lavine SD (2005) Clinicoradiological
review: symptomatic superior cerebellar artery
aneurysm imitating cerebellopontine angle tumor.
Neurosurgery 56(4):836–840; discussion 836–840
115. Krishnan KG, Schackert G (2006) Outcomes of
surgical resection of large solitary hemangioblasto-
mas of the craniocervical junction with limitations in
preoperative angiographic intervention: report of
three cases. Zentralbl Neurochir 67(3):137–143.
doi:10.1055/s-2006-933360
116. Lakshmi M, Glastonbury CM (2009) Imaging of the
cerebellopontine angle. Neuroimaging Clin N Am
19(3):393–406, S1052-5149(09)00040-9 [pii]
117. Lee SR, Sanches J, Mark AS, Dillon WP, Norman D,
Newton TH (1989) Posterior fossa hemangioblasto-
mas: MR imaging. Radiology 171(2):463–468
118. Leeman DJ, Chandrasekhar SS, Brackmann DE,
Poletti BJ (1997) Collision tumors at the cerebello-
pontine angle: case report with literature review.
Otolaryngol Head Neck Surg 117(6):S76–S80,
S0194-5998(97)70063-9 [pii]
11 Other Cerebellopontine Angle Tumors
119. Link MJ, Driscoll CL, Giannini C (2002) Isolated,
giant cerebellopontine angle craniopharyngioma in a
patient with Gardner syndrome: case report.
Neurosurgery 51(1):221–225; discussion 225–226
120. Lüdemann W, Stan AC, Tatagiba M, Samii M (2000)
Sporadic unilateral vestibular schwannoma with
islets of meningioma: case report. Neurosurgery
47(2):451–452; discussion 452–454
121. Marin-Sanabria EA, Yamamoto H, Nagashima T,
Kohmura E (2007) Evaluation of the management of
arachnoid cyst of the posterior fossa in pediatric pop-
ulation: experience over 27 years. Childs Nerv Syst
23(5):535–542. doi:10.1007/s00381-006-0284-3
122. Marton E, Basaldella L, Longatti PL (2009) Minimal
surgery for a cerebellopontine angle lipoma. J Clin
Neurosci 16(1):129–132, S0967-5868(08)00185-9
[pii]
123. Mizushima H, Kobayashi N, Yoshiharu S, Kazuo H,
Dohi K, Sasaki K, Matsumoto K (1999) Aneurysm
of the distal anterior inferior cerebellar artery at the
medial branch: a case report and review of the litera-
ture. Surg Neurol 52(2):137–142, S0090-
3019(99)00042-7 [pii]
124. Moffat DA, O’Connor AF (1980) Bilateral internal
carotid aneurysms in the petrous temporal bones.
Arch Otolaryngol 106(3):172–175
125. Moffat DA, Saunders JE, McElveen JT Jr, McFerran
DJ, Hardy DG (1993) Unusual cerebello-pontine
angle tumours. J Laryngol Otol 107(12):1087–1098
126. Morris DP, Ballagh RH, Hong A, Moffat DA,
Hardy DG (1995) Thrombosed posterior-inferior
cerebellar artery aneurysm: a rare cerebellopontine
angle tumour. J Laryngol Otol 109(5):429–430
127. Muzumdar DP, Goel A, Desai KI (2001) Pontine
glioma and cerebellopontine angle epidermoid
tumour occurring as collision tumours. Br J Neuro-
surg 15(1):68–71
128. Neumann HP, Eggert HR, Weigel K, Friedburg H,
Wiestler OD, Schollmeyer P (1989) Hemangio-
blastomas of the central nervous system. A 10-year
study with special reference to von Hippel-Lindau
syndrome. J Neurosurg 70(1):24–30. doi:10.3171/
jns.1989.70.1.0024
129. Noujaim SE, Rossi MD, Rao SK, Cacciarelli AA,
Mendonca RA, Wang AM, Coelho FH (1999) CT
and MR imaging of neurocysticercosis. AJR Am J
Roentgenol 173(6):1485–1490
130. Nozaki K, Hashimoto N, Kikuta K, Takagi Y,
Kikuchi H (2006) Surgical applications to arterio-
venous malformations involving the brainstem.
Neurosurgery 58(4 Suppl 2):ONS-270–ONS-278.
doi:10.1227/01.NEU.0000210001.75597.81; discus-
sion ONS-278–ONS-279
131. Odake G, Yamaki T, Naruse S (1976) Neurenteric cyst
with meningomyelocele. Case report J Neurosurg
45(3):352–356. doi:10.3171/jns.1976.45.3.0352
132. Omojola MF, al Hawashim NS, Zuwayed MA, al
Ferayan A (1997) CT and MRI features of cavernous
haemangioma of internal auditory canal. Br J Radiol
70(839):1184–1187
References
133. Orakcioglu B, Halatsch ME, Fortunati M,
Unterberg A, Yonekawa Y (2008) Intracranial der-
moid cysts: variations of radiological and clinical
features. Acta Neurochir (Wien) 150(12):
1227–1234; discussion 1234
134. Pappas DG, Schneiderman TS, Brackmann DE,
Simpson LC, Chandra-Sekar B, Sofferman RA
(1989) Cavernous hemangiomas of the internal audi-
tory canal. Otolaryngol Head Neck Surg 101(1):
27–32
135. Pavesi G, Berlucchi S, Munari M, Manara R,
Scienza R, Opocher G (2010) Clinical and surgical
features of lower brain stem hemangioblastomas in
von Hippel-Lindau disease. Acta Neurochir (Wien)
152(2):287–292. doi:10.1007/s00701-009-0512-1
136. Redleaf MI, Bauer CA, Laurenzo JF, Gantz BJ
(1994) Squamous cell carcinoma metastatic to the
cerebellopontine angle. Ann Otol Rhinol Laryngol
103(11):908–910
137. Rengachary SS, Watanabe I (1981) Ultrastructure
and pathogenesis of intracranial arachnoid cysts.
J Neuropathol Exp Neurol 40(1):61–83
138. Revuelta R, Soto-Hernandez JL, Vales LO,
Gonzalez RH (2003) Cerebellopontine angle cystic-
ercus and concurrent vascular compression in a case
of trigeminal neuralgia. Clin Neurol Neurosurg
106(1):19–22, S0303846703000623 [pii]
139. Richard S, David P, Marsot-Dupuch K, Giraud S,
Beroud C, Resche F (2000) Central nervous system
hemangioblastomas, endolymphatic sac tumors, and
von Hippel-Lindau disease. Neurosurg Rev 23(1):
1–22; discussion 23–24
140. Rigamonti D, Johnson PC, Spetzler RF, Hadley MN,
Drayer BP (1991) Cavernous malformations and
capillary telangiectasia: a spectrum within a single
pathological entity. Neurosurgery 28(1):60–64
141. Rodriguez F, Scheithauer BW, Ockner DM,
Giannini C (2004) Solitary fibrous tumor of the
cerebellopontine angle with salivary gland heteroto-
pia: a unique presentation. Am J Surg Pathol 28(1):
139–142
142. Russo N, Domenicucci M, Beccaglia MR, Santoro A
(2008) Spontaneous reduction of intracranial arach-
noid cysts: a complete review. Br J Neurosurg
22(5):626–629, 905633552 [pii]
143. Sahara Y, Nagasaka T, Takayasu M, Takagi T, Hata
N, Yoshida J (2001) Recurrence of a neurenteric cyst
with malignant transformation in the foramen mag-
num after total resection. Case report J Neurosurg
95(2):341–345. doi:10.3171/jns.2001.95.2.0341
144. Samii M, Carvalho GA, Schuhmann MU, Matthies C
(1999) Arachnoid cysts of the posterior fossa. Surg
Neurol 51(4):376–382, S0090-3019(98)00095-0 [pii]
145. Samii M, Nakamura M, Mirzai S, Vorkapic P, Cervio
A (2006) Cavernous angiomas within the internal
auditory canal. J Neurosurg 105(4):581–587.
doi:10.3171/jns.2006.105.4.581
146. Schuhmann MU, Ludeman WO, Schreiber H, Samii
M (1997) Cerebellopontine angle lipoma. Skull Base
7(4):199–205
849
147. Schuknecht HF, Gao YZ (1983) Arachnoid cyst in
the internal auditory canal. Ann Otol Rhinol
Laryngol 92(6 Pt 1):535–541
148. Sepehrnia A, Rebolledo Godoy AP, Reusche E
(2000) A cavernous hemangioma simulating an
intracanalicular acoustic neurinoma – a case report.
Zentralbl Neurochir 61(4):194–197
149. Shakudo M, Inoue Y, Ohata K, Tanaka S (2001)
Neurenteric cyst with alteration of signal intensity
on follow-up MR images. AJNR Am J Neuroradiol
22(3):496–498
150. Shinogami M, Yamasoba T, Sasaki T (1998) Bilateral
isolated metastases of malignant melanoma to the
cerebellopontine angle. Otolaryngol Head Neck
Surg 118(2):276–279, S0194599898000539 [pii]
151. Solomon RA, Stein BM (1986) Management of arte-
riovenous malformations of the brain stem. J Neurosurg
64(6):857–864. doi:10.3171/jns.1986.64.6.0857
152. Spetzger U, Bertalanffy H, Huffmann B, Mayfrank
L, Reul J, Gilsbach JM (1996) Hemangioblastomas
of the spinal cord and the brainstem: diagnostic and
therapeutic features. Neurosurg Rev 19(3):147–151
153. Sun Y, Wrede KH, Chen Z, Bao Y, Ling F (2009)
Ruptured intrameatal AICA aneurysms – a report of
two cases and review of the literature. Acta Neurochir
(Wien) 151(11):1525–1530. doi:10.1007/s00701-
009-0269-6
154. Symon L, Murota T, Pell M, Bordi L (1993) Surgical
management of haemangioblastoma of the posterior
fossa. Acta Neurochir (Wien) 120(3–4):103–110
155. Takeuchi S, Tanaka R, Fujii Y, Abe H, Ito Y (2001)
Surgical treatment of hemangioblastomas with pre-
surgical endovascular embolization. Neurol Med
Chir (Tokyo) 41(5):246–251, JST.JSTAGE/
nmc/41.246 [pii]; discussion 251–242
156. Tampieri D, Leblanc R, TerBrugge K (1993)
Preoperative embolization of brain and spinal
hemangioblastomas. Neurosurgery 33(3):502–505;
discussion 505
157. Tang GC, Piao YS, Zhao L, Lu DH (2007) Lung
adenocarcinoma metastasizing to cerebellopontine
angle schwannoma (collision tumor). Acta Neurochir
(Wien) 149(1):87–90. doi:10.1007/s00701-006-
1049-1; discussion 90
158. Tankere F, Vitte E, Martin-Duverneuil N, Soudant J
(2002) Cerebellopontine angle lipomas: report of
four cases and review of the literature. Neurosurgery
50(3):626–631; discussion 631–622
159. Tu AS, Wang H, Harris-McCorkle L, Saunders JR Jr
(1994) Bilateral involvement of the cerebellopontine
angles by malignant melanoma metastasis: a case
report. Md Med J 43(11):967–970
160. Vazquez-Anon V, Botella C, Beltran A, Solera M,
Piquer J (1997) Preoperative embolization of solid
cervicomedullary junction hemangioblastomas:
report of two cases. Neuroradiology 39(2):86–89
161. Warakaulle DR, Anslow P (2003) Differential diag-
nosis of intracranial lesions with high signal on T1
or low signal on T2-weighted MRI. Clin Radiol
58(12):922–933, S000992600300268X [pii]
850
162. Warren FM, Shelton C, Wiggins RH 3rd, Herrod
HC, Harnsberger HR (2008) Imaging characteristics
of metastatic lesions to the cerebellopontine angle.
Otol Neurotol 29(6):835–838. doi:10.1097/
MAO.0b013e31818133ae
163. Wilms GPC, Goossens L et al (1992) Differentiation
of acoustic neurinoma and meningioma occurring
together in the cerebellopontine angle. Neurosurgery
30:443–445
164. Yang B, Jin HM, Sun LP, Cai W, Shi CR (2008)
Posterior fossa abscesses secondary to dermal
sinus associated with dermoid cyst in children.
Neuropediatrics 39(1):39–42. doi:10.1055/s-
2008-1077086
165. Yasargil MG, Abernathey CD, Sarioglu AC (1989)
Microneurosurgical treatment of intracranial der-
moid and epidermoid tumors. Neurosurgery 24(4):
561–567
166. Yilmaz C, Altinors N, Sonmez E, Gulsen S, Caner H
(2010) Rare lesions of the cerebellopontine angle.
Turk Neurosurg 20(3):390–397. doi:10.5137/1019-
5149.JTN.2961-10.0
167. Young YH, Lin CY, Lou PJ, Hsu MM (2001)
Intracranial relapse of nasopharyngeal carcinoma
manifested as sudden deafness. Otol Neurotol
22(3):392–396
168. Yuh WT, Mayr-Yuh NA, Koci TM, Simon JH,
Nelson KL, Zyroff J, Jinkins JR (1993) Metastatic
lesions involving the cerebellopontine angle. AJNR
Am J Neuroradiol 14(1):99–106
Temporal Bone and Petrous Apex
Lesions
169. Adada B, Al-Mefty O (2003) Fibrous dysplasia of the
clivus. Neurosurgery 52(2):318–322; discussion 323
170. Al-Mefty O, Borba LA (1997) Skull base chordo-
mas: a management challenge. J Neurosurg
86(2):182–189. doi:10.3171/jns.1997.86.2.0182
171. Al-Mefty K, Pravdenkova S, Colli BO, Al-Mefty O,
Gokden M (2007) Chordoma and chondrosarcoma:
similar, but quite different, skull base tumors. Cancer
110(11):2457–2467. doi:10.1002/cncr.23073
172. Altschuler EM, Jungreis CA, Sekhar LN, Jannetta
PJ, Sheptak PE (1990) Operative treatment of intrac-
ranial epidermoid cysts and cholesterol granulomas:
report of 21 cases. Neurosurgery 26(4):606–613;
discussion 614
173. Amedee RG, Marks HW, Lyons GD (1987)
Cholesterol granuloma of the petrous apex. Am J
Otol 8(1):48–55
174. Amichetti M, Cianchetti M, Amelio D, Enrici RM,
Minniti G (2009) Proton therapy in chordoma of the
base of the skull: a systematic review. Neurosurg Rev
32(4):403–416. doi:10.1007/s10143-009-0194-4
175. Arriaga MA, Brackmann DE (1991) Differential
diagnosis of primary petrous apex lesions. Am J Otol
12(6):470–474
11 Other Cerebellopontine Angle Tumors
176. Austin-Seymour M, Munzenrider J, Goitein M,
Verhey L, Urie M, Gentry R, Birnbaum S,
Ruotolo D, McManus P, Skates S et al (1989)
Fractionated proton radiation therapy of chordoma
and low-grade chondrosarcoma of the base of the
skull. J Neurosurg 70(1):13–17. doi:10.3171/
jns.1989.70.1.0013
177. Ayers LS, Depasquale K, Marlowe FI, Ghaderi M
(2010) Pleomorphic adenoma of the external audi-
tory canal: a case report and review of the literature.
Ear Nose Throat J 89(3):E1–E3
178. Bartlett JR (1971) Intracranial neurological
complications of frontal and ethmoidal osteomas. Br
J Surg 58(8):607–613
179. Bibby K, McFadzean R (1994) Fibrous dysplasia of
the orbit. Br J Ophthalmol 78(4):266–270
180. Bockmuhl U, Khalil HS, Draf W (2005)
Clinicoradiological and surgical considerations in
the treatment of cholesterol granuloma of the
petrous pyramid. Skull Base 15(4):263–267.
doi:10.1055/s-2005-918618; discussion 267–268
181. Boedts M, Hermans R, Feenstra L (1997) Osteoma
of the internal auditory canal. Acta Otorhinolaryngol
Belg 51(3):191–193
182. Brackmann DE, Toh EH (2002) Surgical manage-
ment of petrous apex cholesterol granulomas. Otol
Neurotol 23(4):529–533
183. Brodkey JA, Robertson JH, Shea JJ 3rd, Gardner G
(1996) Cholesterol granulomas of the petrous
apex: combined neurosurgical and otological man-
agement. J Neurosurg 85(4):625–633. doi:10.3171/
jns.1996.85.4.0625
184. Brown EW, Megerian CA, McKenna MJ, Weber A
(1995) Fibrous dysplasia of the temporal bone:
imaging findings. AJR Am J Roentgenol 164(3):
679–682
185. Butman JA, Kim HJ, Baggenstos M, Ammerman JM,
Dambrosia J, Patsalides A, Patronas NJ, Oldfield
EH, Lonser RR (2007) Mechanisms of morbid
hearing loss associated with tumors of the endolym-
phatic sac in von Hippel-Lindau disease. JAMA
298(1):41–48, 298/1/41 [pii]
186. Chee GH, Chen JM (2002) Fibrous dysplasia of the
temporal bone. Otol Neurotol 23(3):405–406
187. Chen JM, Fisch U (1993) The transotic approach
in acoustic neuroma surgery. J Otolaryngol 22(5):
331–336
188. Chole R (1985) Petrous apicitis: surgical anatomy.
Ann Otol Rhinol Laryngol 94:251–257
189. Chole RA, Donald PJ (1983) Petrous apicitis.
Clinical considerations. Ann Otol Rhinol Laryngol
92(6 Pt 1):544–551
190. Chong VF, Khoo JB, Fan YF (2002) Fibrous
dysplasia involving the base of the skull. AJR Am J
Roentgenol 178(3):717–720
191. Choo D, Shotland L, Mastroianni M, Glenn G,
van Waes C, Linehan WM, Oldfield EH (2004)
Endolymphatic sac tumors in von Hippel-Lindau
disease. J Neurosurg 100(3):480–487. doi:10.3171/
jns.2004.100.3.0480
References
192. Colli BO, Al-Mefty O (2001) Chordomas of the
skull base: follow-up review and prognostic factors.
Neurosurg Focus 10(3):E1, 100301 [pii]
193. Crockard HA, Cheeseman A, Steel T, Revesz T,
Holton JL, Plowman N, Singh A, Crossman J (2001)
A multidisciplinary team approach to skull base
chondrosarcomas. J Neurosurg 95(2):184–189.
doi:10.3171/jns.2001.95.2.0184
194. Crockard HA, Steel T, Plowman N, Singh A,
Crossman J, Revesz T, Holton JL, Cheeseman A
(2001) A multidisciplinary team approach to skull
base chordomas. J Neurosurg 95(2):175–183.
doi:10.3171/jns.2001.95.2.0175
195. Dave AV, Diaz-Marchan PJ, Lee AG (1997) Clinical
and magnetic resonance imaging features of
Gradenigo syndrome. Am J Ophthalmol 124(4):
568–570
196. Davis TC, Thedinger BA, Greene GM (2000)
Osteomas of the internal auditory canal: a report of
two cases. Am J Otol 21(6):852–856
197. Di Maio S, Temkin N, Ramanathan D, Sekhar LN
(2011) Current comprehensive management of
cranial base chordomas: 10-year meta-analysis of
observational studies. J Neurosurg 115(6):
1094–1105. doi:10.3171/2011.7.JNS11355
198. DiCaprio MR, Enneking WF (2005) Fibrous dyspla-
sia. Pathophysiology, evaluation, and treatment.
J Bone Joint Surg Am 87(8):1848–1864, 87/8/1848
[pii]
199. Doucet V, Peretti-Viton P, Figarella-Branger D,
Manera L, Salamon G (1997) MRI of intracranial
chordomas. Extent of tumour and contrast enhance-
ment: criteria for differential diagnosis.
Neuroradiology 39(8):571–576
200. Doyle KJ, Brackmann DE (1994) Intralabyrinthine
schwannomas. Otolaryngol Head Neck Surg 110(6):
517–523, S0194-5998(94)70620-4 [pii]
201. Edgerton MT, Persing JA, Jane JA (1985) The
surgical treatment of fibrous dysplasia. With empha-
sis on recent contributions from cranio-maxillo-
facial surgery. Ann Surg 202(4):459–479
202. Eisenberg MB, Haddad G, Al-Mefty O (1997)
Petrous apex cholesterol granulomas: evolution
and management. J Neurosurg 86(5):822–829.
doi:10.3171/jns.1997.86.5.0822
203. Erdem E, Angtuaco EC, Van Hemert R, Park JS,
Al-Mefty O (2003) Comprehensive review of
intracranial chordoma. Radiographics 23(4):
995–1009
204. Feldman MD, Rao VM, Lowry LD, Kelly M (1986)
Fibrous dysplasia of the paranasal sinuses.
Otolaryngol Head Neck Surg 95(2):222–225
205. Friedmann I (1959) Epidermoid cholesteatoma and
cholesterol granuloma; experimental and human.
Ann Otol Rhinol Laryngol 68(1):57–79
206. Gay E, Sekhar LN, Rubinstein E, Wright DC, Sen C,
Janecka IP, Snyderman CH (1995) Chordomas and
chondrosarcomas of the cranial base: results and
follow-up of 60 patients. Neurosurgery 36(5):887–
896; discussion 896–897
851
207. Gerganov VM, Samii A, Paterno V, Stan AC,
Samii M (2008) Bilateral osteomas arising from the
internal auditory canal: case report. Neurosurgery
62(2):E528–E529. doi:10.1227/01.neu.0000316023.
81786.b6; discussion E529
208. Green JD Jr, McKenzie JD (1999) Diagnosis and
management of intralabyrinthine schwannomas.
Laryngoscope 109(10):1626–1631. doi:10.1097/
00005537-199910000-00015
209. Griffith AJ, Terrell JE (1996) Transsphenoid endo-
scopic management of petrous apex cholesterol
granuloma. Otolaryngol Head Neck Surg 114(1):-
91–94, S0194599896000174 [pii]
210. Hatano M, De Donato G, Falcioni M, Sanna M
(2011) Chondroblastoma of the temporal bone. Acta
Otolaryngol 131(8):890–895. doi:10.3109/0001648
9.2011.566579
211. Heffelfinger MJ, Dahlin DC, MacCarty CS,
Beabout JW (1973) Chordomas and cartilaginous
tumors at the skull base. Cancer 32(2):410–420
212. Heffner DK (1989) Low-grade adenocarcinoma of
probable endolymphatic sac origin A clinicopatho-
logic study of 20 cases. Cancer 64(11):2292–2302
213. Heffner DK (1996) Are papillary adenomas endo-
lymphatic sac tumors? Ann Otol Rhinol Laryngol
105(3):251–252
214. Hentschel S, Durity F (2002) Petrous apex granulo-
mas: CT and MR imaging. Can J Neurol Sci
29(2):169–170
215. Howitz MF, Johansen C, Tos M, Charabi S, Olsen JH
(2000) Incidence of vestibular schwannoma in
Denmark, 1977–1995. Am J Otol 21(5):690–694
216. Itshayek E, Spector S, Gomori M, Segal R (2002)
Fibrous dysplasia in combination with aneurysmal
bone cyst of the occipital bone and the clivus: case
report and review of the literature. Neurosurgery
51(3):815–817; discussion 817–818
217. Jackler RK, Cho M (2003) A new theory to explain
the genesis of petrous apex cholesterol granuloma.
Otol Neurotol 24(1):96–106; discussion 106
218. Jackler RK, Parker DA (1992) Radiographic differ-
ential diagnosis of petrous apex lesions. Am J Otol
13(6):561–574
219. Kaelin WG Jr (2002) Molecular basis of the VHL
hereditary cancer syndrome. Nat Rev Cancer
2(9):673–682. doi:10.1038/nrc885
220. Kamrin RP, Potanos JN, Pool JL (1964) An evalua-
tion of the diagnosis and treatment of chordoma.
J Neurol Neurosurg Psychiatry 27:157–165
221. Kemink JL, Graham MD (1982) Osteomas and exo-
stoses of the external auditory canal – medical and
surgical management. J Otolaryngol 11(2):101–106
222. Korten AG, ter Berg HJ, Spincemaille GH, van der
Laan RT, Van de Wel AM (1998) Intracranial chond-
rosarcoma: review of the literature and report of 15
cases. J Neurol Neurosurg Psychiatry 65(1):88–92
223. Kurokawa R, Uchida K, Kawase T (2005) Surgical
treatment of temporal bone chondroblastoma. Surg
Neurol 63(3):265–268, S0090-3019(04)00514-2
[pii]; discussion 268
852
224. Kutz JW Jr, Verma S, Tan HT, Lo WW, Slattery WH
3rd, Friedman RA (2007) Surgical management of
skull base chondroblastoma. Laryngoscope 117(5):-
848–853. doi:10.1097/MLG.0b013e3180337da6
225. Lanzino G, Dumont AS, Lopes MB, Laws ER Jr
(2001) Skull base chordomas: overview of disease,
management options, and outcome. Neurosurg Focus
10(3):E12, 100312 [pii]
226. Li JC, Brackmann DE, Lo WW, Carberry JN, House
JW (1993) Reclassification of aggressive adenoma-
tous mastoid neoplasms as endolymphatic sac
tumors. Laryngoscope 103(12):1342–1348.
doi:10.1288/00005537-199312000-00004
227. Lo WW, Applegate LJ, Carberry JN, Solti-Bohman
LG, House JW, Brackmann DE, Waluch V, Li JC
(1993) Endolymphatic sac tumors: radiologic
appearance. Radiology 189(1):199–204
228. Lo WW, Solti-Bohman LG, Brackmann DE, Gruskin P
(1984) Cholesterol granuloma of the petrous apex: CT
diagnosis. Radiology 153(3):705–711
229. Lonser RR, Kim HJ, Butman JA, Vortmeyer AO,
Choo DI, Oldfield EH (2004) Tumors of the endo-
lymphatic sac in von Hippel-Lindau disease. N
Engl J Med 350(24):2481–2486. doi:10.1056/
NEJMoa040666
230. Mallina S, Philip R, Chong AW, Gurdeep S (2007)
Fibrous dysplasia of the temporal bone. Med J
Malaysia 62(2):160–161
231. Manasse P (1894) Uber granulations geschwulste
mit fremdkorperriesen- zellen. Virchows Arch
136:245
232. Manski TJ, Heffner DK, Glenn GM, Patronas NJ,
Pikus AT, Katz D, Lebovics R, Sledjeski K, Choyke
PL, Zbar B, Linehan WM, Oldfield EH (1997)
Endolymphatic sac tumors. A source of morbid hear-
ing loss in von Hippel-Lindau disease. JAMA
277(18):1461–1466
233. Markou K, Karasmanis I, Vlachtsis K, Petridis D,
Nikolaou A, Vital V (2008) Primary pleomorphic
adenoma of the external ear canal. Report of a case
and literature review. Am J Otolaryngol 29(2):142–
146, S0196-0709(07)00047-6 [pii]
234. Megerian CA, Haynes DS, Poe DS, Choo DI,
Keriakas TJ, Glasscock ME 3rd (2002) Hearing
preservation surgery for small endolymphatic sac
tumors in patients with von Hippel-Lindau syn-
drome. Otol Neurotol 23(3):378–387
235. Menezes AH, Gantz BJ, Traynelis VC, McCulloch
TM (1997) Cranial base chordomas. Clin Neurosurg
44:491–509
236. Michaelson PG, Cable BB, Mair EA (2001) Image-
guided transphenoidal drainage of a cholesterol
granuloma of the petrous apex in a child. Int J Pediatr
Otorhinolaryngol 57(2):165–169, S0165-5876(00)
00456-0 [pii]
237. Moffat DA, Saunders JE, McElveen JT Jr,
McFerran DJ, Hardy DG (1993) Unusual cerebello-
pontine angle tumours. J Laryngol Otol 107(12):
1087–1098
11 Other Cerebellopontine Angle Tumors
238. Moon IS, Kim J, Lee HK, Lee WS (2008) Surgical
treatment and outcomes of temporal bone chondro-
blastoma. Eur Arch Otorhinolaryngol 265(12):
1447–1454. doi:10.1007/s00405-008-0660-6
239. Moore KR, Harnsberger HR, Shelton C, Davidson HC
(1998) ‘Leave me alone’ lesions of the petrous apex.
AJNR Am J Neuroradiol 19(4):733–738
240. Mortensen A, Bojsen-Moller M, Rasmussen P
(1989) Fibrous dysplasia of the skull with acromeg-
aly and sarcomatous transformation. Two cases with
a review of the literature. J Neurooncol 7(1):25–29
241. Mosnier I, Cyna-Gorse F, Grayeli AB, Fraysse B,
Martin C, Robier A, Gardini B, Chelikh L, Sterkers
O (2002) Management of cholesterol granulomas of
the petrous apex based on clinical and radiologic
evaluation. Otol Neurotol 23(4):522–528
242. Muckle RP, De la Cruz A, Lo WM (1998) Petrous
apex lesions. Am J Otol 19(2):219–225
243. Mukherji SK, Albernaz VS, Lo WW, Gaffey MJ,
Megerian CA, Feghali JG, Brook A, Lewin JS,
Lanzieri CF, Talbot JM, Meyer JR, Carmody RF,
Weissman JL, Smirniotopoulos JG, Rao VM, Jinkins
JR, Castillo M (1997) Papillary endolymphatic sac
tumors: CT, MR imaging, and angiographic findings
in 20 patients. Radiology 202(3):801–808
244. Neff B, Sataloff RT, Storey L, Hawkshaw M,
Spiegel JR (2002) Chondrosarcoma of the skull
base. Laryngoscope 112(1):134–139. doi:10.1097/
00005537-200201000-00023
245. Neff BA, Willcox TO Jr, Sataloff RT (2003)
Intralabyrinthine schwannomas. Otol Neurotol
24(2):299–307
246. Noel G, Feuvret L, Calugaru V, Dhermain F,
Mammar H, Haie-Meder C, Ponvert D, Hasboun D,
Ferrand R, Nauraye C, Boisserie G, Beaudre A,
Gaboriaud G, Mazal A, Habrand JL, Mazeron JJ
(2005) Chordomas of the base of the skull and upper
cervical spine. One hundred patients irradiated by
a 3D conformal technique combining photon and
proton beams. Acta Oncol 44(7):700–708,
N47554P2G1676823 [pii]
247. Noel G, Habrand JL, Jauffret E, de Crevoisier R,
Dederke S, Mammar H, Haie-Meder C, Pontvert D,
Hasboun D, Ferrand R, Boisserie G, Beaudre A,
Gaboriaud G, Guedea F, Petriz L, Mazeron JJ (2003)
Radiation therapy for chordoma and chondrosar-
coma of the skull base and the cervical spine.
Prognostic factors and patterns of failure. Strahlenther
Onkol 179(4):241–248. doi:10.1007/s00066-003-
1065-5
248. Oghalai JS, Buxbaum JL, Jackler RK, McDermott
MW (2005) Skull base chondrosarcoma originating
from the petroclival junction. Otol Neurotol
26(5):1052–1060, 00129492-200509000-00037
[pii]
249. Palacios E, Valvassori G (1999) Petrous apex lesions:
cholesterol granuloma. Ear Nose Throat J 78(4):234
250. Pallini R, Maira G, Pierconti F, Falchetti ML, Alvino
E, Cimino-Reale G, Fernandez E, D’Ambrosio E,
References
Larocca LM (2003) Chordoma of the skull base:
predictors of tumor recurrence. J Neurosurg
98(4):812–822. doi:10.3171/jns.2003.98.4.0812
251. Peters BR, Maddox HE 3rd, Batsakia JG (1988)
Pleomorphic adenoma of the middle ear and mastoid
with posterior fossa extension. Arch Otolaryngol
Head Neck Surg 114(6):676–678
252. Presutti L, Villari D, Marchioni D (2006) Petrous
apex cholesterol granuloma: transsphenoid endo-
scopic approach. J Laryngol Otol 120(6):e20,
S0022215106009121 [pii]
253. Richard S, David P, Marsot-Dupuch K, Giraud S,
Beroud C, Resche F (2000) Central nervous system
hemangioblastomas, endolymphatic sac tumors, and
von Hippel-Lindau disease. Neurosurg Rev 23(1):1–
22; discussion 23–24
254. Roche PH, Dufour H, Figarella-Branger D, Pellet W
(1998) Endolymphatic sac tumors: report of three
cases. Neurosurgery 42(4):927–932
255. Rosenberg AE, Nielsen GP, Keel SB, Renard LG,
Fitzek MM, Munzenrider JE, Liebsch NJ (1999)
Chondrosarcoma of the base of the skull: a clinico-
pathologic study of 200 cases with emphasis on its
distinction from chordoma. Am J Surg Pathol
23(11):1370–1378
256. Royer MC, Pensak ML (2007) Cholesterol
granulomas. Curr Opin Otolaryngol Head
Neck Surg 15(5):319–322. doi:10.1097/
MOO.0b013e3282a08f74
257. Ruelle A, Datti R, Andrioli G (1994) Cerebellopontine
angle osteoma causing trigeminal neuralgia: case
report. Neurosurgery 35(6):1135–1137
258. Sabin HI, Bordi LT, Symon L (1987) Epidermoid
cysts and cholesterol granulomas centered on the
posterior fossa: twenty years of diagnosis and man-
agement. Neurosurgery 21(6):798–805
259. Samii A, Gerganov V, Herold C, Gharabaghi A,
Hayashi N, Samii M (2009) Surgical treatment of skull
base chondrosarcomas. Neurosurg Rev 32(1):67–75.
doi:10.1007/s10143-008-0170-4; discussion 75
260. Samii A, Gerganov VM, Herold C, Hayashi N,
Naka T, Mirzayan MJ, Ostertag H, Samii M (2007)
Chordomas of the skull base: surgical management
and outcome. J Neurosurg 107(2):319–324.
doi:10.3171/JNS-07/08/0319
261. Samii M, Faupel G (1977) Chondrosarcoma with
intraspinal, intra- and extrathoracal extension (author’s
transl). Acta Neurochir (Wien) 39(1–2):71–80
262. Schulz-Ertner D, Karger CP, Feuerhake A,
Nikoghosyan A, Combs SE, Jakel O, Edler L,
Scholz M, Debus J (2007) Effectiveness of carbon
ion radiotherapy in the treatment of skull-base
chordomas. Int J Radiat Oncol Biol Phys 68(2):-
449–457, S0360-3016(06)03669-8 [pii]
263. Sen C, Triana AI, Berglind N, Godbold J,
Shrivastava RK (2010) Clival chordomas: clinical
management, results, and complications in 71
patients. J Neurosurg 113(5):1059–1071. doi:10.3171
/2009.9.JNS08596
853
264. Singh V, Annis JA, Todd GB (1992) Osteoma of the
internal auditory canal presenting with sudden
unilateral hearing loss. J Laryngol Otol 106(10):
905–907
265. Stuer C, Schramm J, Schaller C (2006) Skull
base chordomas: management and results. Neurol
Med Chir (Tokyo) 46(3):118–124, JST.JSTAGE/
nmc/46.118 [pii]; discussion 124–125
266. Takahashi S, Kawase T, Yoshida K, Hasegawa A,
Mizoe JE (2009) Skull base chordomas: efficacy of
surgery followed by carbon ion radiotherapy. Acta
Neurochir (Wien) 151(7):759–769. doi:10.1007/
s00701-009-0383-5
267. Terao T, Onoue H, Hashimoto T, Ishibashi T,
Kogure T, Abe T (2001) Cholesterol granuloma in
the petrous apex: case report and review. Acta
Neurochir (Wien) 143(9):947–952
268. Thedinger BA, Nadol JB Jr, Montgomery WW,
Thedinger BS, Greenberg JJ (1989) Radiographic
diagnosis, surgical treatment, and long-term
follow-up of cholesterol granulomas of the petrous
apex. Laryngoscope 99(9):896–907. doi:10.1288/
00005537-198909000-00003
269. Thorne MC, Gebarski SS, Telian SA (2006) Rapid
expansion in a previously indolent cholesterol cyst: a
need for lifelong follow-up. Otol Neurotol 27(1):
124–126, 00129492-200601000-00022 [pii]
270. Tringali S, Linthicum FH Jr (2010) Cholesterol gran-
uloma of the petrous apex. Otol Neurotol 31(9):-
1518–1519. doi:10.1097/MAO.0b013e3181d279c3
271. Tsai EC, Santoreneos S, Rutka JT (2002) Tumors of
the skull base in children: review of tumor types and
management strategies. Neurosurg Focus 12(5):e1,
120501 [pii]
272. Tzortzidis F, Elahi F, Wright D, Natarajan SK,
Sekhar LN (2006) Patient outcome at long-term fol-
low-up after aggressive microsurgical resection of
cranial base chordomas. Neurosurgery 59(2):230–
237. doi:10.1227/01.NEU.0000223441.51012.9D;
discussion 230–237
273. Tzortzidis F, Elahi F, Wright DC, Temkin N,
Natarajan SK, Sekhar LN (2006) Patient outcome at
long-term follow-up after aggressive microsurgical
resection of cranial base chondrosarcomas.
Neurosurgery 58(6):1090–1098. doi:10.1227/01.
NEU.0000215892.65663.54; discussion 1090–1098
274. Volpe NJ, Liebsch NJ, Munzenrider JE, Lessell S
(1993) Neuro-ophthalmologic findings in chordoma
and chondrosarcoma of the skull base. Am J
Ophthalmol 115(1):97–104
275. Vortmeyer AO, Choo D, Pack S, Oldfield E,
Zhuang Z (2000) VHL gene inactivation in an
endolymphatic sac tumor associated with von
Hippel-Lindau disease. Neurology 55(3):460
276. Vortmeyer AO, Huang SC, Koch CA, Governale L,
Dickerman RD, McKeever PE, Oldfield EH,
Zhuang Z (2000) Somatic von Hippel-Lindau gene
mutations detected in sporadic endolymphatic sac
tumors. Cancer Res 60(21):5963–5965
854
277. Vrabec JT, Lambert PR, Chaljub G (2000) Osteoma
of the internal auditory canal. Arch Otolaryngol
Head Neck Surg 126(7):895–898, ocn90069 [pii]
278. Wanebo JE, Bristol RE, Porter RR, Coons SW,
Spetzler RF (2006) Management of cranial base
chondrosarcomas. Neurosurgery 58(2):249–255.
doi:10.1227/01.NEU.0000194834.74873.FB; dis-
cussion 249–255
279. Warakaulle DR, Anslow P (2003) Differential diag-
nosis of intracranial lesions with high signal on T1
or low signal on T2-weighted MRI. Clin Radiol
58(12):922–933, S000992600300268X [pii]
280. Weatherby RP, Dahlin DC, Ivins JC (1981)
Postradiation sarcoma of bone: review of 78 Mayo
Clinic cases. Mayo Clin Proc 56(5):294–306
11 Other Cerebellopontine Angle Tumors
281. Weber AL, Liebsch NJ, Sanchez R, Sweriduk ST Jr
(1994) Chordomas of the skull base. Radiologic and
clinical evaluation. Neuroimaging Clin N Am
4(3):515–527
282. Yamasaki T, Kikuchi H, Yamabe H, Yamashita J
(1990) Multiple intracranial metastases following
malignant evolution in recurrent pleomorphic ade-
noma of the lacrimal gland – case report. Neurol
Med Chir (Tokyo) 30(13):1038–1042, JST.
Journalarchive/nmc1959/30.1038 [pii]
283. Yano H, Funakoshi T, Shinoda J, Sakai N,
Kokuzawa G, Shimokawa K (1997) Primary pleo-
morphic adenoma in posterior cranial fossa. Brain
Tumor Pathol 14(1):75–78
 Management of Facial Palsy
Despite the major advancement in CP angle surgery,
in certain cases it is impossible to preserve normal
facial nerve function. Even if the nerve is pre-
served anatomically, complete and lasting paraly-
sis may ensue. Comprehensive management of
patients with facial nerve paralysis aims at resto-
ration of function and appearance, as well as pre-
vention of negative sequelae.
Restoration of Facial Nerve Function
Restoration of facial nerve function can be
performed by (1) nerve reconstruction, either
directly or via an interposition graft, or (2) nerve
reanimation with donor nerves. Direct end-to-end
neurorrhaphy yields the best results, followed by
reconstruction with interposition graft, and then
reanimation with a donor nerve [19, 31].
The goal of any restorative procedure is to
achieve normal facial appearance at rest, symme-
try with movement, and restoration of muscle
control [5]. Timing is a key factor with regard to
functional outcome of facial nerve reconstruc-
tion: best results are achieved if the reconstruction
is performed within the first year after onset of
the palsy [26, 27].
Principles of Nerve Anastomosis
• Careful microsurgical technique of prepara-
tion of the stump and precise approximation
of the anastomotic ends.
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9_12, © Springer-Verlag Berlin Heidelberg 2013
12
• Preparation of the stump; 1–2 mm of the
epineurium of the stump and of the graft has to
be resected with microscissors to create
smooth interface.
• Tensionless anastomosis to prevent scar for-
mation and subsequent hindering of axonal
regeneration.
• Suture, glue, or other alternative. The evalua-
tion of various neurorrhaphy techniques—
epineural vs. perineural sutures, laser
neurorrhaphy, tissue adhesives, tubulization,
and neurotrophic factors—revealed no major
differences in regard to outcome [33]. In a pre-
vious study of the senior author, the degree of
reinnervation following nerve anastomosis
with fibrin glue and with suture has been com-
pared, and no significant differences were
found [31]. The use of fibrin glue is technically
easier and seems to lead to less scar formation.
The facial nerve in its intracranial segment has
no epineurium, and the anastomosis can be
made with fibrin glue, instead of placing a
suture through the substance of the nerve. In
case of hypoglossal–facial anastomosis, we
apply two microsutures that are additionally
secured with fibrin glue.
Facial Nerve Reconstruction
Indications
Direct reconstruction of the facial nerve leads to
optimal regeneration. It can be performed in case
the proximal nerve stump at the brain stem and
855
856
the distal stump are available. The minimum
length of the available proximal stump should be
3–5 mm. Importantly, the anastomosis should be
absolutely tension-free. An experimental study
performed by the senior author has shown that
optimal outcome is achieved in case of tension-
less end-to-end anastomosis, followed by recon-
struction using an interpositional neural graft.
The worse regeneration occurs in case the anasto-
mosis is performed under tension. If significant
gap between the proximal and distal ends exists,
interposition graft should be used. All these
techniques for primary facial reconstruction,
however, lead to better functional results than the
reanimation techniques, for example, the hypo-
glossal–facial anastomosis.
End-to-End Anastomosis
If both the proximal and the distal nerve stumps
are available within the CP angle, direct end-
to- end anastomosis can be performed at the time
of tumor surgery. It is feasible only in case of
large VSs, in which the facial nerve has been
elongated during tumor growth. In such cases, a
defect of up to 10–15 mm can be bridged without
creating an undue tension. The ends of the stumps
are laid on gelatin sponges, freshened, and fixed
with fibrin glue. The benefits of the method, if
compared with the alternatives, are that sponta-
neous facial movement can recover, the need of
second surgery is obviated, and the time to recov-
ery is shortened.
Reconstruction with an Interposition
Graft
The functional restoration with an interposition
graft is inferior to that of the direct tensionless
end-to-end anastomosis but is still superior when
compared with the facial nerve reanimation
techniques.
Nerve Grafts
• Length of the graft. The length of the graft and
the number of grafts do not affect the extent of
recovery [15, 27, 31].
• Diameter of the donor nerve. Optimally, the
diameter of the donor nerve should match that
of the facial nerve (after the epineurium has
12 Management of Facial Palsy
Fig. 12.1 The sural nerve graft is harvested using two
short transverse skin incisions (arrows)
been removed). Both the sural nerve and the
greater auricular nerve grafts are appropriate
in this regard [28].
• The harvesting of the graft should not have
undesirable cosmetic effect. Avoid donor site
morbidity!
• Graft nutrition!
Sural Nerve Graft
The obtainable graft length is up to 40 cm. The
sural nerve is a sensory nerve formed from the
medial sural cutaneous nerve of the tibial nerve
and the peroneal communicating nerve of com-
mon peroneal nerve. It supplies the skin of the
posterolateral aspect of the leg, lateral malleolus,
and lateral side of the foot up to little toe. The
nerve runs between the two heads of the gastroc-
nemius muscle, then pierces the deep fascia, and
runs distally lateral to the small saphenous vein
between the lateral malleolus and the calcaneus.
Harvesting of sural nerve leads to mild sensory
deficit.
Technique: The nerve is exposed first distally
by performing a skin incision (appr. 1 cm long)
at the junction of the middle and anterior thirds
of the line between the tip of calcaneus and lat-
eral malleolus (Fig. 12.1). With blunt dissection,
the nerve is identified subcutaneously—it is
usually slightly anterior to the small saphenous
vein. A small hemostatic forceps is inserted
beneath it, and slight traction is performed.
Thus, with palpation or visually, the proximal
Restoration of Facial Nerve Function 857

course of the nerve can be identified. A second and can be followed to the junction of the upper
incision is placed accordingly to the expected and middle thirds of the posterior sternocleido-
location of the nerve 10–15 cm proximally. The mastoid border (Erb’s point).
nerve is dissected and—using the same tech-
nique of slight traction with a hemostatic Reconstruction Within the CP Angle
forceps—followed more proximally as long as with an Interposition Graft
necessary, and appropriate skin incisions are If both the proximal and the distal nerve stumps
made. The course of the nerve from distal to are available but cannot be coapted without undue
proximal is lateral to medial, and in the popliteal tension, an interposition graft with an appropriate
fossa, it lies approximately in the midline. length (usually 5–20 mm) may be used (Figs. 12.2,
Another important consideration is that the sural 12.3, 12.4, and 12.5).
nerve pierces the deep fascia in the junction area
between upper 2/3 and lower 1/3 of the posterior
leg. Large branches, usually from the middle
part of the nerve, should be cut via additional
skin incisions. The nerve is incised at the most
proximal level and stripped out by traction with
the forceps.

Great Auricular Nerve Graft

The maximal obtainable graft length is 7–10 cm.
Greater auricular nerve grafting, however, may
cause more disabling hypesthesias than sural
nerve resection.
Technique: The retrosigmoid skin incision has
to be extended inferior along the posterior border
of the sternocleidomastoid muscle as long as Fig. 12.2 Schematic illustration of a direct end-to-end
facial nerve reconstruction with an interposition graft
required. The nerve is identified under the skin

Fig. 12.3 MR images demonstrating large VS on the segment of the facial nerve was resected (priority was
right side. At surgery, the facial nerve was found to be given to complete tumor removal due to the relatively
intimately adherent to the tumor capsule over a length of young age of the patient). The technique used for recon-
2–3 cm. In order to remove the tumor completely, this struction of the nerves’ integrity is presented on Fig. 12.4
858
a b
d e
Fig. 12.4 Reconstruction of the facial nerve in the CP
angle with a sural nerve interposition graft—intraopera-
tive images. The tumor (see previous image) has been
removed completely. (a, b) Healthy stumps are prepared
proximally and distally. A piece of Gelfoam is used as a
platform to allow better adaptation. (c) Sural nerve graft
The early experience of the senior author with
facial nerve reconstruction in the CP angle was
presented in 1994 [31]. The series included 16
patients with VSs and 1 with meningioma.
Reinnervation was first noted in 4–12 months
following the procedure. At follow-up, 24 % of
the patients had HB grade III + facial nerve func-
tion, 47 % had HB grade III, and 29 % had HB
grade IV.
Intracranial–Intratemporal Technique
If only the proximal nerve stump is available, the
nerve can be reconstructed by creating an anasto-
mosis to its distal segment within the temporal
bone using the intracranial–intratemporal tech-
nique developed by Samii in 1975 (the first case
12 Management of Facial Palsy
c
f
of appropriate length is selected and approximated end-to-end
to the stumps. The diameter of the graft usually corre-
sponds well to the diameter of the facial nerve. Fibrin glue
is used to seal the ends—in the CP angle (c) and in the
IAC (d, e). The opened IAC is then filled with fat pieces to
prevent CSF leak (f)
was performed with Dr. Weigand; afterwards, all
cases were performed with Dr. Draf) [11].
The mastoid segment of the facial nerve is
identified and exposed by performing a mas-
toidectomy (Figs. 12.6 and 12.7). It is transected
below the lateral semicircular canal and trans-
posed posterior towards the sinodural angle. The
sigmoid sinus is skeletonized, and the posterior
surface of the pyramid at sinodural angle is
removed. The nerve graft—usually 30–50 mm
long—is coapted to the proximal stump and
brought out of the CP angle into the mastoid
through a presigmoid dural incision. Then it is
united to the distal facial nerve stump.
As presented in the earlier cited study, 24 % of
the patients after such reconstruction had HB
Restoration of Facial Nerve Function 859

a b

Fig. 12.5 (a) Intraoperative image of a direct facial nerve reconstruction in the CP angle with a nerve graft. (b–d)
Photos of the patient immediately after surgery (b), 9 months, (c) 15 months and (d) later

grade III+, 53 % had HB grade III, and 18 % had
HB grade IV [29].
Intracranial–Extracranial Technique
Occasionally, if the petrous segment of the facial
nerve is destroyed by infection or neoplastic
growth, an intracranial–extracranial reconstruction,
distal to the exit site of the nerve at stylomastoid
foramen, might be necessary (Fig. 12.8). An 80- to
150-mm graft is required to reach this facial nerve
segment [25, 28]. The technique was presented
by Dott in 1958 [10], who performed the grafting
860
in two stages: initially, the graft was united with
the proximal stump, brought out of the CP angle,
and tunneled subcutaneously between the ster-
nocleidomastoid and the splenius capitis muscles.
The distal end was marked with a silver clip. In 3
months, the distal end of the graft was anastomo-
sed to the peripheral stump of the facial nerve.
The senior author has performed such recon-
structions in ten patients. In contrast to the origi-
nal description of Dott, the surgery was performed
in one step. One patient (10 %) recovered to HB
Fig. 12.6 Schematic illustration of an intracranial–
intratemporal facial nerve reconstruction. A proximal
facial nerve stump, B nerve graft, C distal facial nerve
stamp in mastoid, D trigeminal nerve
a b
Fig. 12.7 Intracranial–intratemporal anastomosis. (a)
CP angle part: the proximal facial nerve stamp and the
graft are well visible. (b) Anastomosis in the mastoid,
12 Management of Facial Palsy
grade III+, 60 % recovered to HB grade III, and
20 % to HB grade IV.
Reinforcement Technique
A
A novel technique, introduced recently by the
senior author, is the end-to-end reinforcement of
the facial nerve in the CP angle. It was applied in
one case of large cystic VS. Tumor dissection led
to partial loss of facial nerve fibers and, respec-
tively, to a marked reduction of response inten-
sity during proximal electrical stimulation.
Stimulation of the facial nerve in the IAC, how-
ever, elicited a normal response. A sural nerve
graft was used to reinforce the remaining func-
tional facial nerve fibers: it was coapted with
fibrin to the normally appearing proximal and
distal nerve ends (Figs. 12.9 and 12.10). The con-
tinuity of the facial nerve was thus not inter-
rupted. Eighteen months after surgery, good
recovery of facial function was recorded
(House–Brackmann grade III).
B
Second variant of the facial nerve reinforce-
ment technique is the end-to-side insertion of
an interposed sural nerve graft. It was used ini-
tially in a case of large VS (extension grade
T4b) and published in 2006 [30]. During the
dissection of the tumor capsule from the facial
which is presented schematically on figure (c). Photo of
the patient immediately after the surgery (d) and 12
months later (e, f)
Restoration of Facial Nerve Function 861

c d

Fig. 12.7 (continued)

nerve, approximately 70 % of the nerve fibers continuity (Fig. 12.11). At 18 months follow-
were lost. The graft was interposed end-to-side up, the patient’s facial function had improved
between the normal proximal and distal parts of markedly to an HB grade III. The patient was
the facial nerve without interrupting its unfortunately lost to follow-up.
862
Fig. 12.8 Schematic illustration of an intracranial–
extracranial facial nerve reconstruction
a b
Fig. 12.9 Preoperative MRI examination of a patient with large cystic VS grade T4B on the right side. See next figures
for operative details
12 Management of Facial Palsy
Facial Nerve Reanimation with a Donor
Nerve (or Indirect Facial Nerve
Reconstruction)
Indications
Reanimation with donor nerve is indicated in
case of complete facial nerve loss or loss of its
proximal stump at the brain stem during tumor
surgery, as well as in case the anatomically intact
nerve shows no signs of recovery at follow-up. In
case of nerve loss, the procedure is performed
within 1 or 2 weeks after tumor removal. If a
complete facial palsy persists for 10–12 months
without signs of spontaneous functional or elec-
tromyographic recovery, even though the integ-
rity of the nerve has been preserved, nerve
reanimation procedure is also indicated. The
Restoration of Facial Nerve Function
a b
d e
g h
j k
Fig. 12.10 Intraoperative images illustrating the technique
of facial nerve reinforcement in the CP angle. The patient
had a 4.2-cm large cystic VS (T4b) (See previous figure). (a)
Initial view of the tumor (TS transverse sinus, SS sigmoid
sinus, R retractor). (b) The IAC has been opened widely,
exposing the intracanalicular tumor portion. (c) Partial
removal of the intracanalicular tumor and exposure of the
distal facial nerve (7). (d) Identification of the medial segment
of the facial nerve, close to the brain stem. (e, g) Progressive
863
c
f
i
dissection of the nerve from the tumor capsule. (h) Removal
of the last piece of tumor (Tu) from the nerve. (i) Part of the
facial nerve fibers were damaged. The stumps are marked
with arrows. Although the nerve responded to electrical
stimulation, the response was rather weak. Therefore, a sural
nerve graft was prepared (j) and fixed with fibrin glue to the
ends of the interrupted facial nerve fibers (k) (arrowheads:
anastomotic sites)
864
Fig. 12.11 Schematic drawing of the end-to-side facial
nerve reinforcement technique (Reprinted from Samii
et al. [30])
ipsilateral muscles can be partially reinnervated
by collaterals from the contralateral facial nerve.
Therefore, the patient should be examined both
clinically and with EMG for signs of nerve recov-
ery. The functional outcome is optimal if the sur-
gery is performed up to 12 months after the onset
of the paralysis.
Reanimation can be performed with various
donor nerves: hypoglossal, accessory, phrenic
nerves, or contralateral facial nerve. Best results
are achieved with the hypoglossal nerve, which
could be explained by the close relations of the
facial and hypoglossal cortical representations
and the existing physiologically close association
of facial and tongue movements.
Hypoglossal–Facial Anastomosis
Classical hypoglossal–facial nerve anastomosis
is performed end-to-end between the proximal
part of the hypoglossal nerve and the distal seg-
ment of the facial nerve at the stylomastoid
process. This method is the most reliable one to
promote reinnervation because it incorporates the
maximum number of axons.
Technique
The head is turned to the contralateral side.
A slightly curved linear retromastoid retroauricular
12 Management of Facial Palsy
skin incision is performed that is extended down
along the anterior border of the sternocleidomas-
toid muscle (Fig. 12.12). The facial nerve is
identified between the mastoid tip and the angle
of mandible as it courses distal to the stylomas-
toid foramen. Useful landmark for its location is
the “pointer” of the cartilaginous auditory canal.
Another helpful landmark is the digastric branch,
which can be dissected back to the main facial
trunk. Dissection is continued distally to the point
of nerves’ bifurcation.
The hypoglossal nerve is identified using the
posterior belly of the digastric muscle and the
anterior border of the sternocleidomastoid mus-
cle as landmarks. The nerve is traced distally as
far as possible, transected, and brought up in
front of the stylomastoid foramen medial to the
posterior belly of the digastric muscle. Its
descending ramus can be divided to acquire addi-
tional length. The facial nerve is then transected
proximally—at the stylomastoid foramen—and
brought in proximity to the hypoglossal stump.
Anastomosis is performed with two 9-0 perineu-
ral sutures or with fibrin glue. In order to avoid
complete hemitongue denervation, the proximal
part of ansa cervicalis can be connected to the
distal part of the hypoglossal nerve.
Complete transection of the hypoglossal
nerve, however, causes ipsilateral paralysis and
hemitongue atrophy, which interferes with
mastication, speech, and swallowing [33].
Hammerschlag [16] observed both speech and
swallowing problems in 45 % of patients using
this technique. Conley and Baker [6] reported
severe tongue atrophy in 25 % of their series of
137 cases. Although these problems may be tran-
sient, some patients show persistent difficulties
that interfere with their daily lives [8, 18].
Variations of the classic techniques that allow
for preservation of hypoglossal nerve function
have been introduced, such as end-to-side anasto-
mosis, jump interposition graft hypoglossal–
facial anastomosis, hypoglossal nerve longitudinal
split, and use of a descending hypoglossal branch
or of ansa cervicalis [1, 6, 7, 9, 17, 19].
May et al. [22] described the technique of
indirect hypoglossal–facial anastomosis. The
hypoglossal and facial nerves are anastomosed
with the interposition of a free nerve “jump” graft,
Restoration of Facial Nerve Function
a b
d e
Fig. 12.12 (a) A slightly curved linear retromastoid ret-
roauricular skin incision is performed. The digastric mus-
cle (DM) is identified. (M, mastoid). (b) The facial nerve
(arrow) was identified anterior to the stylomastoid fora-
men. (c) Identification of the hypoglossal nerve (double
arrow) and transected distally (d). (e) Partial mastoidectomy
end-to-end to the peripheral facial nerve stump,
and end-to-side to the hypoglossal nerve. With
this method, postoperative tongue dysfunction
and swallowing or speech problems can be com-
pletely avoided, as shown by Manni et al. [20] in
a series of 39 consecutive patients. However, as
with other crossover techniques, synkinesis can
still occur. Twenty-one percent of the patients in
this series recovered to HB grade II, 45 % recov-
ered to grade III, 24 % recovered to grade IV, and
7 % to grade V, and in 3 % no recovery was noted.
The disadvantages of this technique are the exis-
tence of two suture lines, which may act as obsta-
cles to the regenerating axon sprouts, and the
donor site morbidity [13, 16, 18].
865
c
f
was performed, and the facial nerve was identified in the
fallopian canal (black arrow), approximately 5 mm proxi-
mally to the stylomastoid foramen. (f, g) The distal part of
the facial nerve is mobile, coapted to the hypoglossal
nerve (end-to-end) and fixed with fibrin glue
The use of split hypoglossal nerve for facial
reanimation is another alternative [1, 6, 9].
Longitudinal splitting of the hypoglossal nerve is
performed, and one-half is routed cranially and
connected in an end-to-end fashion with the
proximal stump of the facial nerve. The hypo-
glossal nerve, however, is not a polyfascicular
nerve, and the longitudinal incision may transect
many interweaving axons. Therefore, although
the technique leads to good facial reanimation, in
all patients, mild to moderate tongue atrophy is
described. The degree of atrophy, however, is
much less severe as that observed in some patients
with classic hypoglossal–facial nerve anastomo-
sis [1]. Another complication related to the
866
technique is fibrosis involving the exposed nerve
after being split [18]. To avoid such outcome,
Cusimano and Sekhar [7] described a technique
of partial hypoglossal to facial anastomosis after
partial division and limited splitting of the hypo-
glossal nerve.
Following the experimental works of Viterbo
[34, 35] on the end-to-side neurorrhaphy, a new
hypoglossal nerve-preserving technique was pro-
moted: 30–50 % of its cross-sectional area of the
hypoglossal nerve are sectioned transversally,
and the facial nerve distal stump is coapted
directly to the sidewall of the hypoglossal nerve.
The fibers remaining intact enable good hemi-
tongue function. Alternatively, an epineural win-
dow is made.
Our favorite technique currently is the
side-to- end hypoglossal–facial anastomosis
(Fig. 12.13). A retromastoid retroauricular skin
incision is performed. The hypoglossal nerve
is identified and dissected proximally. Then the
facial nerve is found anterior to foramen stylo-
mastoideum. In order to obtain sufficient length
of the facial nerve, its mastoid segment up to
the geniculate ganglion is exposed following a
mastoidectomy. It is transected as proximally
as possible, dissected from the facial canal, and
reflected inferiorly. Two perineural sutures (9-0
or 8-0 monofilament nylon sutures) are sutured to
unite the facial to a partially sectioned (approxi-
mately 50 %) hypoglossal nerve [3, 4]. Fibrin
glue is applied as an additional support for the
anastomosis. Although theoretically fewer nerve
fibers are transferred to the facial muscles when
compared with the end-to-end technique, collat-
eral axonal sprouting provides sufficient func-
tional motor reinnervation.
Accessory Nerve
Reanimation with the accessory nerve is recom-
mended for patients with dysphagia or in case the
potential alteration of the voice is a major con-
cern. With this donor nerve, however, the func-
tional outcome is poorer, and disturbing
concomitant movements of the shoulder can
occur. The experience of the senior author with
this technique is that facial symmetry at rest is
very rarely achieved. Active movements of the
12 Management of Facial Palsy
face recover but are possible only with concomi-
tant arm abduction. Furthermore, all patients
have weakness of the muscles innervated by the
accessory nerve.
Facial to Facial Anastomosis
(Cross-Face Graft)
Facial–facial nerve anastomosis is an option in
patients in whom no other cranial nerve can be
sacrificed, such as those with lower cranial nerve
palsy, isolated hypoglossal nerve palsy, or those
with profession for which speech is essential
(speech is dependent on intact hypoglossal
function!). It is used further to achieve some
functional recovery after other reanimation tech-
niques had failed or to augment partial recovery
after hypoglossal–facial anastomosis in patients
with incomplete facial paralysis showing only
limited recovery over time.. The anatomical
premise for the procedure is the fact that cutting
of approximately 1/2 to 1/3 of the plexiform
facial nerve branches does not cause obvious
functional deficit. Adjacent branches take over
the function completely. The facial nerve of the
healthy side is connected with nerve grafts to the
corresponding branches of the plegic side. In
such cases, the muscle tone on the affected side is
partially preserved, whereas the graft enhances
the voluntary movements [14, 33].
Technique: The zygomatic branches are
exposed by performing vertical skin incisions
slightly anterior to the masseter muscles below the
zygomatic arch on both sides. These are the stron-
gest facial branches, innervating the orbicularis
oculi and the orbicularis oris muscles. Intraoperative
electrostimulation is essential to estimate the role
of each branch because of their anatomical vari-
ability. Sural nerve graft is used as an end-to-end
anastomosis between the proximal part of the
facial branch on the contralateral healthy side to
the distal nerve part on the ipsilateral side. For the
subcutaneous tunneling of the graft, auxiliary skin
incisions in the nasolabial folds are helpful.
Additional plastic surgeries are generally
required to achieve good cosmetic effect because
the reinnervation appears slow and is generally
weaker compared to the one achieved when the
hypoglossal nerve is used as a donor.
Restoration of Facial Nerve Function 867

a b c

d e f

g h i

j k l

Fig. 12.13 Intraoperative images illustrating the end-to-
side hypoglossal–facial anastomosis. (a) Skin incision and
exposure of the mastoid (M) and digastric muscle (DM);
asterisk—ear lobule. (b) Identification of the facial nerve
distal to the stylomastoid foramen (FN). (c–f) Mastoidectomy
and identification of the facial nerve as close to the genicu-
late ganglion, as possible. (g, h) Mobilization of the facial
nerve (arrows). (i, j) Identification of the hypoglossal nerve
(two small arrows). (k, m) Sectioning of appr. 50 % of its
diameter (m, arrowheads). The mobilized facial nerve
(arrow) is attached to the sectioned part of the hypoglossal
nerve (double arrows) and fixed with two 9-0 monofilament
nylon sutures and fibrin glue
868
Table 12.1 Facial nerve functional outcome following various reconstructive procedures
Facial nerve function (House–Brackmann scale) (in %)
Type of reconstruction Grade II–III
Reconstruction in the CP angle 21
Intracranial–intratemporal reconstruction 25
Intracranial–extratemporal reconstruction 10
Hypoglossal–facial anastomosis 21
Facial–facial cross face reanimation
Modified from [25, 32]
Outcome
The reinnervation in case of end-to-end facial
nerve reconstruction should become clinically
obvious within 12–18 months. Recovery after
this endpoint is unlikely [19]. In case of recon-
struction with an interposition graft, the endpoint
for recovery is approximately 24 months, while
clinical improvement following hypoglossal–
facial anastomosis can occur 2 years after nerve
repair and continue even after the third year. In
the series of Malik et al. [19], best results, defined
as HB grades £ III, were achieved with the pri-
mary end-to-side coaptation (85 %), followed by
reconstruction with a cable graft (56 %), and
hypoglossal–facial anastomosis (25 %).
Arriaga and Brackmann [2] reviewed the out-
come of facial nerve repair in cases of CP angle
tumors using end-to-end coaptation and cable nerve
graft interposition. They found that HB grade IV
was achieved in 7/13 cases for end-to- end coapta-
tion and 4/8 cases of cable nerve graft and con-
cluded that good results are obtained by immediately
reestablishing anatomic continuity of the nerve with
direct reanastomosis or with interposition graft.
The senior author’s experience with various
types of facial nerve reconstruction is presented
on Table 12.1. Reconstruction in the CP angle
led to satisfactory reinnervation (HB grade III or
better) in 69–74 % (reconstruction within the CP
angle vs. intracranial–intratemporal anastomosis).
The functional outcome after hypoglossal–facial
anastomosis was even better: House–Brackmann
grade III had 75 %, and function between grades
II and III had 21 %.
Timing of surgery is the factor most closely
related to outcome. Late surgery—more than
1 year after the onset of facial dysfunction—leads
to poorer reinnervation. According to some studies,
12 Management of Facial Palsy
Grade III Grade IV Grade V–VI
53 21 5
44 19 6
60 20 10
75 4
100
increasing age is associated with a poorer outcome
[19, 23, 24]. The degree of recovery of facial func-
tion was significantly better in patients without
facial nerve symptoms prior to tumor surgery [2].
Further determining factors are the degree of atro-
phy of the facial nerve and mimetic muscles [36].
Falcioni et al. [12] reported that tumor type also
correlated to the outcome: the recovery rate
decreased progressively in iatrogenic lesions, VSs,
petrous bone cholesteatomas, and facial nerve
tumors. However, this relationship was also
influenced by the duration of the preoperative
facial nerve dysfunction in every group. In our
experience, as well as according to other authors
[19], tumor type does not influence outcome.
Improvement of facial function more than HB
grades II to III is unattainable, whatever tech-
nique is applied. The recovery of facial nerve
function is a gradual process that has to be sup-
plemented by consequential physiotherapy.
Throughout the period, the patient should receive
psychological support.
Following prolonged facial paralysis, the regen-
erative process is often associated with misdirected
axonal regrowth. As a result, synkinesis, epiphora,
and varying degrees of hemifacial contracture can
occur, which are difficult to manage. A potential
effective therapy in case of involuntary blepharos-
pasm is botulinum A toxin injection. Generally,
synkinesis tends to decrease after several years.
Plastic Surgical Procedures
Additional plastic surgical correction should be con-
sidered if the recovery is insufficient and can be per-
formed without any time limit. Muscle transposition
Eye Care
techniques are used when the distal neuromuscular
unit is absent or nonviable due to fibrosis [21]. Most
effective are the temporalis and masseter muscles
transpositions. Facial slings with fascia lata or allo-
plasts, such as expanded polytetrafluoroethylene,
can improve facial symmetry [33].
Physiotherapy
Transcutaneous electrical stimulation may
adversely affect recovery by promoting the devel-
opment of synkinesis. Physiotherapy is of key
role to prevent muscle atrophy or synkinesis and
to promote functional recovery. The specific type
of physiotherapy is related to the degree of rein-
nervation and risk of synkinesis.
The authors’ concept for the supportive
physiotherapy has been described in a previous
publication [32]:
1) In case of complete paralysis, the patient needs
to participate in a physiotherapy program for at
least 3 years. The program consists of daily indi-
vidual exercises that are performed six times for 5
to 10 minutes. During the first 1 to 2 years, the
patient needs to be accompanied by a specialized
physiotherapist for two to three weekly sessions.
2) Each exercise consists of the trial of an active
movement and then a massage in the requested
direction, as follows: a) lifting eyebrows without
any help and striking eyebrows up with the
fingertips, b) frowning and pushing eyebrows
together, c) closing eyes and sliding down eyelids,
d) wrinkling the nose and pushing cheeks together,
e) blowing the cheeks and holding the mouth angles
tight, f) forming a small “o” with the lips and push-
ing the mouth angles together, g) smiling and pull-
ing mouth angles apart, and h) smiling strongly and
pushing the healthy side toward the midline to
facilitate some action on the sick side. 3) If eye clo-
sure is especially weak, it is combined with strong
biting until it becomes complete. Then the patient
tries very slowly to relax the masseter muscles
while imagining closed eyes; after complete relax-
ation, the eyes are opened. This whole sequence is
repeated three to five times. The same exercise is
helpful in reducing synkinesis between eye closure
and mouth angle. 4) If eye closure is weak in hypo-
glossal–facial combination, a similar exercise is
performed. The eye is closed and supported by
pressing the tongue against the teeth. The tongue is
then very slowly loosened while the eye is imag-
ined to be closed. Within weeks to months, inde-
pendent eye closure without any tongue movement
869
becomes possible. 5) In case of a weak mouth
angle, the healthy side is pushed toward the midline
and fixed with the fingertips. The sick mouth angle
can then be trained more easily. 6) Any electrical
stimulation of nerves or muscles must be avoided
to minimize the development of contractures.
Eye Care
In patients with facial nerve palsy, eye care is the
most important concern. Maintenance of mois-
ture and protection from mechanical injury are
critical to avoid complications, such as blurred
vision, exposure keratopathy, corneal abrasions,
and even blindness. Dry eye is caused by a com-
bination of factors: reduced lacrimation, dimin-
ished corneal reflex, or inability to close the eye
completely (lagophthalmos) and occasionally by
eyelid malposition (paralytic ectropion). Although
the intermediate nerve function correlates with
the degree of facial nerve dysfunction, diminished
lacrimation can occur even in case of normal or
nearly normal function of the facial nerve.
The management is directed at maintaining a nor-
mal corneal epithelium, which provides comfort and
preserves visual acuity. Patients with severe muscle
atrophy, as well as those with trigeminal nerve deficits,
need special protection. The coexistence of corneal
anesthesia and decreased tear production or lago-
phthalmos accelerates the corneal degeneration:
• Topical eye lubricants such as artificial tears.
They should be applied frequently enough to
keep the eye moist, for example, every 3–4 h.
• Gels are thicker than artificial tears and pro-
vide more lubrication than eyedrops. However,
they are thick and can blur vision.
• Moisture chamber or hourglass dressing. It cre-
ates a moist ocular environment and lessen tear
evaporation but allows better vision than a patch.
• Nighttime protection. Lubricants (gels and
ointments) combined with a patch provide the
optimal protection.
• Punctal plug insertion or partial blocking the
lacrimal duct. A relatively simple procedure
that might be necessary if dryness of the cor-
nea is a persistent problem.
• Tarsorrhaphy. In case a prolonged period of
recovery of facial nerve function is antici-
870
pated, the outer or inner corner of the lids can
be sutured (lateral or medial tarsorrhaphy).
Generally, a lateral tarsorrhaphy of <5 mm is
not noticeable and is cosmetically acceptable.
Medial tarsorrhaphies should be avoided
because of their disfiguring effect.
• If a 5-mm tarsorrhaphy is not sufficient to
manage the exposure keratitis, a reanimation
procedure should be considered. Skin-tone
external eyelid weights or gold eyelid weights
can be used. The weights are attached to the
upper lid and improve mechanical blink.
• Botulinum toxin. It is injected at the upper
border of the tarsus and produces complete
ptosis to protect the cornea.
• Ophthalmic follow-up with slit-lamp examina-
tion and fluorescein staining are advised to assess
corneal and visual status after any lid procedure.
References
1. Arai H, Sato K, Yanai A (1995) Hemihypoglossal-
facial nerve anastomosis in treating unilateral facial
palsy after acoustic neurinoma resection. J Neurosurg
82(1):51–54. doi:10.3171/jns.1995.82.1.0051
2. Arriaga MA, Brackmann DE (1992) Facial nerve
repair techniques in cerebellopontine angle tumor sur-
gery. Am J Otol 13(4):356–359
3. Asaoka K, Sawamura Y (1999) Hypoglossal-facial
nerve side-to-end anastomosis. J Neurosurg
91(1):163–164
4. Campero A, Socolovsky M (2007) Facial reanimation
by means of the hypoglossal nerve: anatomic com-
parison of different techniques. Neurosurgery 61(3
Suppl):41–49. doi:10.1227/01.
neu.0000289710.95426.19; discussion 49–50
5. Cheney ML, McKenna MJ, Nath R, Healy C, Bartlett
SP (1999) Facial nerve reconstruction and facial
reanimation following oncologic surgery. Head
Neck 21(3):276–284. doi:10.1002/(SICI)1097-
0347(199905)21:3<276::AID-HED15>3.0.CO;2-K
[pii]
6. Conley J, Baker DC (1979) Hypoglossal-facial nerve
anastomosis for reinnervation of the paralyzed face.
Plast Reconstr Surg 63(1):63–72
7. Cusimano MD, Sekhar L (1994) Partial hypoglossal
to facial nerve anastomosis for reinnervation of the
paralyzed face in patients with lower cranial nerve
palsies: technical note. Neurosurgery 35(3):532–533;
discussion 533–534
8. Darrouzet V, Guerin J, Bebear JP (1999) New tech-
nique of side-to-end hypoglossal-facial nerve attach-
ment with translocation of the infratemporal facial
12 Management of Facial Palsy
nerve. J Neurosurg 90(1):27–34. doi:10.3171/
jns.1999.90.1.0027
9. Dellon A (1992) Restoration of facial nerve function:
an approach for the twenty-first century. Neurosurg Q
2:199–222
10. Dott NM (1958) Facial paralysis; restitution by extra-
petrous nerve graft. Proc R Soc Med 51(11):900–902
11. Draf W, Samii M (1980) Intracranial-intratemporal
anastomosis of the facial nerve (author’s transl).
Laryngol Rhinol Otol (Stuttg) 59(5):282–287
12. Falcioni M, Taibah A, Russo A, Piccirillo E, Sanna M
(2003) Facial nerve grafting. Otol Neurotol
24(3):486–489
13. Flores LP (2007) Surgical results of the Hypoglossal-
Facial nerve Jump Graft technique. Acta Neurochir
(Wien) 149(12):1205–1210. doi:10.1007/s00701-
007-1412-x; discussion 1210
14. Frey M, Giovanoli P, Michaelidou M (2006)
Functional upgrading of partially recovered facial
palsy by cross-face nerve grafting with distal end-to-
side neurorrhaphy. Plast Reconstr Surg 117(2):597–
608. doi:10.1097/01.prs.0000197136.56749.c6
15. Gidley PW, Gantz BJ, Rubinstein JT (1999) Facial
nerve grafts: from cerebellopontine angle and beyond.
Am J Otol 20(6):781–788
16. Hammerschlag PE (1999) Facial reanimation with
jump interpositional graft hypoglossal facial anasto-
mosis and hypoglossal facial anastomosis: evolution
in management of facial paralysis. Laryngoscope
109(2 Pt 2 Suppl 90):1–23
17. Kessler LA, Moldaver J, Pool JL (1959) Hypoglossal-
facial anastomosis for treatment of facial paralysis.
Neurology 9(2):118–125
18. Magliulo G, D’Amico R, Forino M (2001) Results
and complications of facial reanimation following
cerebellopontine angle surgery. Eur Arch
Otorhinolaryngol 258(1):45–48
19. Malik TH, Kelly G, Ahmed A, Saeed SR, Ramsden
RT (2005) A comparison of surgical techniques used
in dynamic reanimation of the paralyzed face. Otol
Neurotol 26(2):284–291. doi:00129492-200503000-
00028 [pii]
20. Manni JJ, Beurskens CH, van de Velde C, Stokroos RJ
(2001) Reanimation of the paralyzed face by indirect
hypoglossal-facial nerve anastomosis. Am J Surg
182(3):268–273. doi:S0002-9610(01)00715-2 [pii]
21. May M (1984) Muscle transposition for facial reani-
mation. Indications and results. Arch Otolaryngol
110(3):184–189
22. May M, Sobol SM, Mester SJ (1991) Hypoglossal-
facial nerve interpositional-jump graft for facial reani-
mation without tongue atrophy. Otolaryngol Head
Neck Surg 104(6):818–825
23. Pitty LF, Tator CH (1992) Hypoglossal-facial nerve
anastomosis for facial nerve palsy following surgery
for cerebellopontine angle tumors. J Neurosurg
77(5):724–731. doi:10.3171/jns.1992.77.5.0724
24. Sabin HI, Bordi LT, Symon L, Compton JS (1990)
Facio-hypoglossal anastomosis for the treatment
References
of facial palsy after acoustic neuroma resection. Br J
Neurosurg 4(4):313–317
25. Samii M (1981) Preservation and reconstruction of
the facial nerve in the cerebellopontine angle. In:
Samii M, Janetta P (eds) The cranial nerves. Springer,
Berlin, pp 438–450
26. Samii M (1984) Facial nerve grafting in acoustic
neurinoma. Clin Plast Surg 11(1):221–225
27. Samii M (1986) Surgery of facial nerve paralysis. No
Shinkei Geka 14(8):943–955
28. Samii M, Draf W (1989) Surgery of the skull base.
Springer, Heidelberg
29. Samii M, Jannetta P (eds) (1981) The cranial nerves:
anatomy, pathology, pathophysiology, diagnosis,
treatment. Springer, New York
30. Samii M, Koerbel A, Safavi-Abbasi S, Di Rocco F,
Samii A, Gharabaghi A (2006) Using an end-to-side
interposed sural nerve graft for facial nerve reinforce-
ment after vestibular schwannoma resection. Technical
note. J Neurosurg 105(6):920–923. doi:10.3171/
jns.2006.105.6.920
31. Samii M, Matthies C (1994) Indication, technique and
results of facial nerve reconstruction. Acta Neurochir
(Wien) 130(1–4):125–139
871
32. Samii M, Matthies C (1997) Management of 1000
vestibular schwannomas (acoustic neuromas): the
facial nerve–preservation and restitution of function.
Neurosurgery 40(4):684–694; discussion 694–685
33. Tate JR, Tollefson TT (2006) Advances in facial rean-
imation. Curr Opin Otolaryngol Head Neck Surg
14(4):242–248. doi:10.1097/01.moo.0000233594.
84175.a0
34. Viterbo F, Trindade JC, Hoshino K, Mazzoni A (1994)
Two end-to-side neurorrhaphies and nerve graft with
removal of the epineural sheath: experimental study in
rats. Br J Plast Surg 47(2):75–80
35. Viterbo F, Trindade JC, Hoshino K, Mazzoni Neto A
(1994) End-to-side neurorrhaphy with removal of the
epineural sheath: an experimental study in rats. Plast
Reconstr Surg 94(7):1038–1047
36. Ylikoski J, Brackmann DE, Savolainen S (1982)
Facial nerve abnormalities after acoustic tumor
removal. Morphological and clinical study of seven
patients with postoperative facial paralysis. Arch
Otolaryngol 108(12):795–800
 Index
A Arteriovenous malformations (AVMs), 105–108, 778
AAO-HNS hearing classification, 255
Abducens nerve schwannoma, 581–583
Accessory nerve, 10, 41, 55, 60–61
Accessory nerve schwannoma, 637, 649–651
Acidophilic adenoma, 93–95
Acoustic neuromas
anatomy, 34
arterial relationships, 43, 45, 48
brain stem relationships, 39
history of, 1
middle neurovascular complex, 34
retrosigmoid approach, 35, 37–39
venous relationships, 44–47
Acute bacterial sive purulent meningitis, 84–86
Adamantinomatous craniopharyngioma, 97–98
Adenohypophysis, spindle cell oncocytoma, 96
Aggressive papillary middle ear tumors.
See Endolymphatic sac tumors (ELST)
AICA. See Anteroinferior cerebellar artery (AICA)
Anaplastic ependymoma, 100, 724–725
Anaplastic hemangiopericytoma, 81–82
Anaplastic meningiomas, 81
Anastomosis
facial-facial nerve, 866
hypoglossal-facial, 864–867
Ancient schwannomas, 74–75
Aneurysmal bone cyst, 90, 91
Angiomatous meningiomas, 78
Anteroinferior cerebellar artery (AICA)
anterior view, 15
cerebellopontine angle, 11, 15–16
CNs VII and VIII in, 15
compression, 26
Antineutrophil cytoplasmic antibody (ANCA)-mediated
inflammations, 112
Arachnoid cysts
complete removal, 736–739
CT and MR appearance, 731
description, 731
medial cyst wall, incomplete resection of, 733–735
patient management, 731
surgical options, 731–732
symptoms, 732
Arterial relationships
middle neurovascular complex, 43, 45, 48
upper neurovascular complex, 26–29
M. Samii, V. Gerganov, Surgery of Cerebellopontine Lesions,
DOI 10.1007/978-3-642-35422-9, © Springer-Verlag Berlin Heidelberg 2013
Aspergillosis, 86–88
Aspergillus ssp., 87
Atypical choroid plexus papillomas, 101
Atypical meningiomas, 79
Audiometric examinations, 165
Auditory brain stem implants (ABI)
cochlear implantation, 371
complications, 368, 370
factors, 370
indications, 364
intraoperative images, 364, 368–370
lip-reading enhancement, 370
MRI, large left-sided VS, 364, 368
penetrating electrodes, 370–371
prosthesis
magnetless ABI, 364, 367
removal of right-sided tumor, 364–366
rehabilitation, 370
Auditory midbrain implant (AMI), 371
Autonomous nervous system pathology, 77
Autosomal dominant polycystic kidney disease, 108
AVMs. See Arteriovenous malformations (AVMs)
B
Bacterial meningitis
acute bacterial sive purulent meningitis, 84–85
sarcoidosis, 86, 87
tuberculous meningitis, 85–87
Basophilic adenoma, 95
Besnier–Boeck–Schaumann disease, 86, 87
Bilateral cerebellopontine (CP) angle tumors
bilateral osteomas, 354–358
extensive skull base meningioma, 352, 354
foramina of Luschka, 352–353
melanoma metastases, 359–363
metastatic lesions in cerebellar hemispheres, 352
Bilateral vestibular schwannomas, 74
Bill’s bar, 37
Blood vessels and circulation pathology
arteriovenous malformation, 105–108
hypertensive angiopathy and hemorrhage,
109–111
ischemic brain infarct, 109–110
saccular aneurysm, 107–109
vasculitis, 110–112
873
874 Index

Brain stem Chemodectoma, 77

acoustic neuromas, 39 Cholesterol granuloma of petrous apex
middle neurovascular complex, 39 classic obstruction-vacuum theory, 789
Brain stem gliomas clinical presentation, 790
description, 703 CT and MRI, 789
low-grade exophytic astrocytoma, 705, 708–709 description, 788–789
low-grade gliomas, 706, 710–713 differential diagnosis, 789
MR imaging, 704 incidence, 788
pilocytic astrocytoma, 703–705 management options, 790–791
surgery, 705 recurrence rate, 791
tumor removal steps, 705, 707 retrosigmoid intradural inframeatal
Breast carcinoma metastases, 766 approach, 792–797
Chondroblastomas, 90, 91, 818–821
Chondrosarcomas (CSA)
C clinical presentation, 807, 809
Cavernomas grades, 807
clinical presentation, 778, 781 histological types, 807
in IAC, 783, 785–787 in jugular foramen area, 815–817
incidence, 778 neuroimaging, 807
intracanalicular, 781, 782 origin, 807
recurrence risk, 784 pathology, 90, 91
retrosigmoid approach, 784–787 removal, anterior petrosal route, 810–811
signs/symptoms, 781 retrosigmoid suprameatal approach, 812–814
total microsurgical resection, 784 Chordoid meningiomas, 79
Cell-mediated inflammations, 112 Chordomas
Cellular ependymoma, 100 chondroid, 803–804
Cellular schwannomas, 74–75 endonasal-combined microsurgical removal, 799,
Cerebellar arteries, 16–18 801–803
Cerebellar brain stem fissures, 14–17 iatrogenic injury, 800
Cerebellar hemispheres management, 798–800, 803–804
edema in, 777 neuroimaging, 798
metastatic lesions in, 352 origin, 798
Cerebellar surfaces, 11, 14–17 pathology, 91–92
Cerebellomedullary fissure, 16 retrosigmoid approach, 805–806
Cerebellomesencephalic fissure, 14, 16 symptoms/signs, 798
Cerebellopontine angle transethmoid transsphenoidal approach, 799–800
anteroinferior cerebellar artery, 11, 15–16 Choriocarcinoma, 104
cleavage plane, 12, 13 Choroid plexus
dura, 12 AVM, 107
epidermoids (see Epidermoids) carcinoma, 101
flocculus and choroid plexus, 12 flocculus and, 12, 58
meatal dura, 12, 13 and foramen of Luschka, 44
meningiomas (see Meningiomas) papillomas, 100–101, 714–717
middle neurovascular complex, 36 Chromophobic adenoma, 95, 96
posteroinferior cerebellar artery, 11, 17 Clear cell ependymoma, 100
superior cerebellar artery, 11, 14 Clear cell meningiomas, 79
superior petrosal vein, 12 Clival chordoma, 799, 801–803
surgical management Clival meningiomas, 380–382
hearing preservation, 6 Cochlear nerves, 37
microsurgical technique, 5–6 Cochlear schwannomas, 605
middle fossa approach, 5 Collision tumors, 767–772
radiosurgery, 6 Communicating hydrocephalus, 275, 277–278
surgical improvements, 3–4 Cranial nerves
surgical microscope, 5–6 VS, 297
translabyrinthine approach, 4–5 posterior cranial fossa, 9–11
transpetrosal approach, 5 Craniopharyngioma, 97–98
vestibular schwannomas, 2 Cryptococcosis, 88–89
vestibulocochlear nerve, 13 Cryptococcus ssp., 88
Cerebellopontine fissure, 16 CSA. See Chondrosarcomas (CSA)
Index 875

CSF leak, 300–302 Ependymomas

Cysticercosis, 88, 89 description, 718
Cystic jugular foramen schwannomas, 614–615, 637, differential diagnosis, 718
652–655 pathology, 99–100
Cystic tumors, 252 patient care, 718
Cystic VS prognostic factors, 719
capsule, 234–235 radiotherapy, 719
MR imaging, 233, 236–237, 239–240 recurrences, 719
nerve adhesion, 238 Epidermoid cyst, 105, 107
proteolytic enzymes, 232 Epidermoids
tumor debulking, 234 angled endoscope usage, 515
case study
endoscopic inspection, 524, 529, 535, 539, 540
D patients clinical history, 519, 524, 530, 535, 540
Deep noncortical AVM, 107 postoperative periods, 519, 523, 530, 531, 534,
Delayed facial nerve palsy, 254 535, 540, 541
Dermoid cyst, 105, 107 preoperative images, 524–526, 530–532,
Dermoids 536–537, 540, 541
case illustration, 742–743 RS approach, 526–528, 530, 532–534, 537–538,
complications, 740–741 541–544
CT and MRI, 740, 741 CISS MR sequence, 509, 511
description, 740 CT and MRI images, 509, 510, 512–514
vs. epidermoids, 741 diffusion-weighted imaging, 511, 516–517
signs and symptoms, 740 features, 509
Developmental venous anomalies (DVAs), 773, 775–777 FLAIR sequence, 509, 511
Diffuse melanocytosis, 83 frontotemporal approach, 519
Dumbbell tumors, 559–568, 575–576 location/extension, 509, 515
management, 511–512, 514–515
MR cisternography, 511
E petrous bone, 515, 518
Echinococcosis, 88, 89 retrosigmoid approach, 511, 523
Echinococcus granulosus, 89 signs/symptoms, 511
Ehlers–Danlos syndrome type IV, 108 supra and infratentorial extension, 519–521
ELST. See Endolymphatic sac tumors (ELST) tumor capsule dissection, 515
Embryonal carcinoma, 104 Erdheim–Chester disease, 103
Endolymphatic sac tumor, 92–93 Extended MF approach, 118–119
Endolymphatic sac tumors (ELST) Extra-axial tumors
case illustration aneurysms, 773, 774
sporadic cystic ELST, 831–833 arachnoid cysts
sporadic ELST removal, 823–826 CT and MR appearance, 731
VHL syndrome-associated ELST, 827–830 description, 731
incidence, 818 medial cyst wall, incomplete
management, 822 resection of, 733–735
neuroimaging, 821 patient management, 731
origin, 818 removal, 736–739
symptoms, 822 surgical options, 731–732
unilateral hearing loss, 821–822 symptoms, 732
Endonasal-combined microsurgical technique, 799, arteriovenous malformations, 778
801–803 cavernomas
Endoscopy case illustration, 783, 785–787
clival chordoma removal, 799, 801–803 clinical presentation, 778, 781
microsurgical technique, 720–723 incidence, 778
transcervical tumor removal, 618–624 intracanalicular, 781, 782
End-to-end facial nerve reinforcement technique, 860, recurrence risk, 784
862, 863 RS approach, 784–787
End-to-side facial nerve insertion technique, 860–861, signs/symptoms, 781
864 total microsurgical resection, 784
Eosinophilic adenoma, 95, 96 collision tumors, 767–772
Eosinophilic granuloma, 102 dermoids, 740–743
876
Extra-axial tumors (cont.)
developmental venous anomalies, 773, 775–777
hamartomas, 744–747
hemangioblastomas
case illustration, 760–763
cerebral angiography, 757–758
description, 757
MRI, 757–758
operative strategy considerations, 759
preoperative endovascular
embolization, 758–759
radiotherapy, 759
staged multimodal management, 758
surgical removal, 758
lipomas, 748–749
metastasis
case illustration, 766
clinical factors, 764
management, 765
primary malignancies, 764
radiologic appearance, 764
neurenteric cysts
case illustration, 752–756
clinical presentation, 749–750
endodermal origin, 749
management, 750
MR characteristics, 749–751
neuroimaging, 749
spinal, 749
vascular lesions, 773
Extrameatal tumor removal
tumor debulking/decompression, 187, 191–192
tumor variables, 190–191
ultrasonic aspirator, 188, 190
F description, 834
Facial-facial nerve anastomosis, 866
Facial motion classification, 251
Facial nerve
arterial compression, 58–59
infrafloccular exposure, 56–57
meatal relationships, 37, 40–42
operative techniques development
translabyrinthine approach, 4–5
transpetrosal approach, 5
vestibular schwannomas
cystic tumors, 252
delayed facial nerve palsy, 254
facial nerve palsy, 253–254
grading systems, 250
intermediate nerve dysfunction, 254–255
intraoperative monitoring, 252
NF-2, 252
operative approach, 253
patient age, 252
preoperative facial palsy, 252–253
preservation, 250
radiosurgery, 252
Index
reoperations, 252
taste disturbance, 255
tumor characteristics, 250–252
Facial nerve palsy, 253–254
Facial nerve reconstruction
end-to-end anastomosis, 856
end-to-end reinforcement, 860, 862, 863
end-to-side insertion technique, 860–861, 864
eye care, 869–870
functional outcome, 868
goal, 855
indications, 855–856
with interposition graft
in CP angle, 857–859
great auricular nerve graft, 857
requirements, 856
sural nerve graft, 856–857
intracranial-extracranial technique, 859–860, 862
intracranial-intratemporal technique, 858–861
nerve anastomosis, principles of, 855
physiotherapy, 869
plastic surgical procedures, 868–869
with reanimation techniques
accessory nerve, 866
facial-facial nerve anastomosis, 866
hypoglossal-facial anastomosis, 864–867
indications, 862, 864
timing, 855, 868
Facial schwannomas
case illustration, 590–593, 596, 599–308
complete tumor removal with resection, 596–598
ganglion geniculi, 584, 588–589
in mastoid segment, 584–587
middle cranial fossa, 584
radiological patterns, 594–595
Fibrous dysplasia (FD)
development patterns, 834
pathology, 90
of petrous bone, 835–838
radiological differential diagnosis, 834
Fibrous meningiomas, 78
Flocculus, 12
Fluid-attenuated inversion recovery imaging
(FLAIR), 509, 511
Foramen jugular chondrosarcoma, 807, 808
Foramen magnum meningiomas
clinical presentation, 482
complete tumor removal, 489–491
facial and vestibulocochlear nerve
complex, 489, 492–493
factors, 483
headache and vertigo, 496–498
iatrogenic injury avoidance, 487
lateral suboccipital approach, 487–489
limits, 482
lower clivus extendsion, 483, 486
occipital headache and gait instability, 499–500
outcome, 489, 493
Index 877

preoperative neuroradiological Hearing and VS

examinations, 482–483 classification, 255–256
rupture contralateral vertebral artery, 489, 494–495 preservation rate, 262, 266–267
size of craniotomy and dentate ligament, 483–485 risk factors, 256
Foramen of Luschka, 10, 39, 44 tumor removal and preservation, 257–271
Fourth ventricle Hearing loss, 201
anatomy, 18–19 Hearing preservation, history of, 6
choroid plexus pathology, 100–101 Hemangioblastomas
subependymoma, 727–729 case illustration, 760–763
telovelar approach to, 64–65, 70 cerebral angiography, 757–758
Functioning adenomas, 94 description, 757
Fungal meningitis MRI, 757–758
aspergillosis, 86–88 operative strategy considerations, 759
cryptococcosis, 88–89 preoperative endovascular embolization, 758–759
radiotherapy, 759
staged multimodal management, 758
G
surgical removal, 758
Gardner–Robertso scale, 255
Hemangiopericytoma, 81–82
Gardner-type NF-2, 315
Hematopoietic system pathology
Germ cells pathology
Langerhans cell histiocytosis, 102–103
dysontogenetic cysts, 105–107
non-Langerhans cell histiocytosis, 103
tumors, 104–106
primary CNS lymphomas, 102
Germinoma, 104
Hemorrhagic complications, 201
Giant partially thrombosed aneurysm, 773, 774
Hereditary hemorrhagic telangiectasia, 109
Giant (extension grade T5) VS
High jugular bulb
bone-window MRI, 207–210
classification, 173, 174
hearing loss, 201
grade III, 181, 182
hemorrhagic complications, 201
intraoperative images, 177–179
neurological examination, 207
postoperative imaging, 180
operated seven times, 245–246
preoperative imaging, 176
preoperative MRI, 202
House–Brackmann grading system, 250
RS approach, 204–205
Hydrocephalus associated VS
Glomus jugulare tumors
clinical management, 273, 282
acute lower cranial nerve dysfunction, 668
communicating, 275, 277–278
autonomous nervous system, 77
MRI, 274–275
clinical presentation, 666, 668
obstructive, 275
features, 664
retrosigmoid approach, 280
Fisch classification, 665
Hypertensive angiopathy and brain hemorrhage, 109–111
Glasscock–Jackson classification, 665
Hypoglossal-facial anastomosis, 864–867
neuroimaging, 665–667
Hypoglossal nerve, 59
pathogenesis, 664–665
Hypoglossal nerve schwannomas, 664
surgery of
carotid artery, jugular vein, lower cranial
nerves, 670–673
I
embolization, 670, 680–685
Immature teratoma, 105, 106
neuronavigation screenshots, 670, 676
Immune-complex-mediated inflammations, 112
radiotherapy/radiosurgery, 670, 673
Indirect facial nerve reconstruction. See Reanimation
transmastoid infralabyrinthine approach, 670,
techniques, facial nerve reconstruction
674–675, 677–679
Inframeatal meningiomas
watchful waiting, 668
case illustration, 407–408
Glomus tympanicum tumor, 77
foramen magnum level, 401
Glossopharyngeal nerve, 59, 61–63, 646, 664
pattern of dislocation, 376, 379, 380
Granular cell tumor, 97
Internal acoustic meatus, 38, 41
Great auricular nerve graft, n facial nerve
Intra-axial tumors
reconstruction, 857
brain stem gliomas
description, 703
H low-grade exophytic astrocytoma, 705, 708–709
Hamartomas, 744–747 low-grade gliomas, 706, 710–713
Hand–Schüller–Christian disease, 102, 103 MR imaging, 704
878
Intra-axial tumors (cont.)
pilocytic astrocytoma, 703–705
surgery, 705
tumor removal steps, 705, 707
choroid plexus papillomas, 714–717
clinical manifestation, 703
ependymomas
anaplastic, 724–725
description, 718
differential diagnosis, 718
patient care, 718
prognostic factors, 719
radiotherapy, 719
recurrences, 719
lymphomas, 730
medulloblastomas, 730
subependymomas, 726–729
Intracanalicular meningiomas, 416–418
Intracanalicular tumor grading, 152
Intracanalicular (extension grade 1) VS
adhesions to nerves, 219–221
capsule and adhesion, 217–218
CT and MRI, 195
hearing loss, 195
microsurgery, 193–197
retrosigmoid approach, 193–194
stereotactic radiosurgery, 193
Intracranial-extracranial facial nerve
reconstruction, 859–860, 862
Intracranial-intratemporal facial nerve
reconstruction, 858–861
Intralabyrinthine schwannomas, 844
Intraosseous meningiomas, 419–421
Ischemic brain infarct, 109–110
J CSF leak, 626
Jugular foramen (JF) meningiomas
clinical manifestation, 691
facial nerve, 691
features, 686
neuroimaging
en plaque appearance, 686–688
foramen magnum area, 686, 688
primary meningioma, 686, 689–690
operative treatment
with large CP angle part, 693–697
parameters, 691
outcome, 692
recurrence rate, 691–692
Jugular foramen (JF) schwannomas
accessory nerve schwannoma, 637, 649–651
classifications, 627
clinical presentation, 628–629
neuroimaging
cystic schwannomas, 637, 652–655
type A with large intracranial part and
smaller JF part, 629
type B, axial and coronal T1-weighted
MRI, 629–630
Index
type C, T1-weighted and T2-weighted
MRI, 629–630
type D, dumbbell-shaped tumor, 629, 631
origin, 627
retrosigmoid endoscope-assisted approach, 637,
656–659
staged removal in two steps, 637, 660–663
type A schwannomas
with intracranial growth, 632–636
pure intracranial location, 637, 646–648
RS approach and opening of JF, 637, 640–644
type D, 637–639
type II cervical and extradural transmastoid
infralabyrinthine approach, 637, 645
vagus nerve schwannoma, 627–628
Jugular foramen (JF) tumors
clinical presentation and diagnosis, 610–611
craniectomy, mastoidectomy, 616
electrophysiological monitoring, 612
endoscope-assisted tumor removal, 617–624
extended endoscopic endonasal approach, 625
infratemporal approach, 612
intradural exposure, 616
lateral suboccipital approach, 612–615
lower cranial nerves dysfunction, 625
management, 611
neck dissection and exposure of cervical region,
vessels, and nerves, 616
neuroimaging
digital subtraction angiography, 610
MR signal characteristics, 610
native and contrast-enhanced MRI, 609
typical radiological characteristics, 609–610
pars nervosa, 611
postoperative care
aspiration pneumonia, 625–626
speech-language therapy, 626
primary bone tumors, 609
radiosurgery and stereotactic radiotherapy, 626–627
reconstruction of dura and skull base, 616–617
structures, 609
tumor removal, 616–617
Jugulotympanic paraganglioma, 77
Juvenile xanthogranuloma, 103
L
Langerhans cell histiocytosis (LCH), 102–103
Lateral suboccipital approach, 710–713
Leptomeningeal melanocytic lesions, 83, 84
Lipomas, 748–749
Lower cranial nerve schwannomas. See Jugular foramen
(JF) tumors
Lower neurovascular complex
glossopharyngeal nerve, 61–63
neural relationships, 59–61
vascular compression, 63–64
vascular relationships, 62–63
Low-grade exophytic astrocytoma, 705, 708–709
Index 879

Lymphomas, 730 hormonal therapy and chemotherapy, 435

Lymphoplasmacyte-rich meningiomas, 78 IAC involvement
case illustration, 411, 413–415
high-speed drill, 411–412
M neuroimaging, 410–411
Malignant melanoma, 83 variable extension patterns, 409–410
Malignant peripheral nerve sheath tumor inframeatal, 376, 379, 380
(MPNST), 76–77 internal decompression, 394
Marfan’s syndrome, 108 intracanalicular, 416–418
Mature teratoma, 105, 106 intraosseous, 419–421
Meatal dura, 12, 13, 36–38 jugular foramen
Meckel’s cave, 21, 32, 40 clinical manifestation, 691
Medial VS facial nerve, 691
caudal tumor extension, 229, 231 features, 686
cranial nerve dissection, 229 neuroimaging, 686–690
preoperative imaging, 228–229 operative treatment, 691, 693–697
RS approach, 230 outcome, 692
Medulloblastomas, 730 recurrence rate, 691–692
Melanocytoma, 83 lateral retromeatal
Melanocytosis, 83 case illustration, 398–400
Melanomatosis, 83 CISS and T2-weighted sequences, 396–397
Melanotic schwannomas, 76 sigmoid sinus involvement, 396
Meningeal hemangiopericytoma, 81, 82 management
Meninges pathology adhesive characteristics, 393–394
acute bacterial sive purulent meningitis, 84–85 hearing preservation, 393
aspergillosis, 86–88 hydrocephalus, 394
cryptococcosis, 88–89 treatment options, 392
cysticercosis, 88, 89 multiple, 424–431
echinococcosis, 88, 89 neuroimaging
hemangioblastoma, 82–84 cavernous sinus and retrosellar growth, 382,
hemangiopericytoma, 81–82 384–385
leptomeningeal melanocytic lesions, 83 CSF signal characteristics, 390
meningioma, 78–81 dural tail sign, 382, 386
meningiosis and solid metastatic tumors, 83, 84 intratumoral calcification, 380, 382
sarcoidosis, 86, 87 mushrooming effect, 383, 387
tuberculous meningitis, 85–87 petroclival meningioma with perifocal edema,
Meningiomas 383, 388–389
atypical and anaplastic, 423–424 secondary intracanalicular extension,
centered at IAC, 376, 379 382, 385–386
clinical presentation, 390–392 tumor-feeding arteries, 390
CP angle definitions, 376 outcome, 422–423, 472
epidemiology, 375 pathogenesis and pathology, 375–376
examination, 392 pathology
foramen magnum age distribution, 78
clinical presentation, 482 anaplastic, 81
complete tumor removal, 489–491 angiomatous, 78, 79
facial and vestibulocochlear nerve complex, atypical, 79, 81
489, 492–493 chordoid, 79, 81
factors, 483 classification, 376
headache and vertigo, 496–498 clear cell, 79, 80
iatrogenic injury avoidance, 487 epidemiology, 78
lateral suboccipital approach, 487–489 fibrous, 78, 79
limits, 482 grading, 78
lower clivus extension, 483, 486 histology, 78
occipital headache and gait instability, 499–500 lymphoplasmacyte-rich, 78, 80
outcome, 489, 493 meningothelial, 78, 79
preoperative neuroradiological examinations, metaplastic, 78–80
482–483 microcystic, 78, 80
rupture contralateral vertebral artery, 489, 494–495 NF-2 mutations, 375–376
size of craniotomy and dentate ligament, 483–485 papillary, 81
880
Meningiomas (cont.)
psammomatous, 78, 79
rhabdoid, 81
secretory, 78, 80
sex distribution, 78
transitional, 78, 79
perioperative management, 392
petroclival
case illustration, 438, 440–442
cavernous sinus, 435–436
endoscopeassisted removal, 452, 477–481
features, 435–437
jugular foramen and foramen magnum, 472–476
with large supratentorial part, 451–453
located medial to trigeminal nerve, 435–436
management of, 437
massive venous infarction, 447, 449–450
Meckel’s cave, 435–436
pattern of dislocation, 376, 379
presigmoid approach, 444–447
RSMA (see Retrosigmoid suprameatal approach)
small petrous apex, 438–439
surgical management, 443
two-staged removal, 453–458
venous phase of DSA, 447–448
postoperative management, 422
premeatal, 376, 378, 380
pure clival, 380–382
recurrence, 423
removal, retrosigmoid approach, 65, 66
retromeatal, 376, 378, 380
retrosigmoid approach, 394–395
sphenopetroclival, 376–377
suprameatal, 376, 378, 380
tentorial, 432–434
Meningiosis and solid metastatic tumors, 83–84
Meningitis, 302
Meningothelial meningiomas, 78
Metaplastic meningiomas, 78
Metastasis
breast carcinoma metastases, 766
case illustration, 766
clinical factors, 764
management, 765
primary malignancies, 764
radiologic appearance, 764
Microcystic meningiomas, 78
Microsurgical anatomy, of CA
cerebellar arteries, 16–18
cerebellar brain stem fissures, 14–17
cerebellar surfaces, 11, 14–17
cerebellopontine angle
anteroinferior cerebellar artery, 11, 15–16
cleavage plane, 12, 13
dura, 12
flocculus and choroid plexus, 12
meatal dura, 12, 13
posteroinferior cerebellar artery, 11, 17
superior cerebellar artery, 11, 14
superior petrosal vein, 12
vestibulocochlear nerve, 13
Index
fourth ventricle, 18–19
lower neurovascular complex
glossopharyngeal nerve, 61–63
neural relationships, 59–61
vascular compression, 63–64
vascular relationships, 62–63
middle neurovascular complex
acoustic neuromas, 34
arterial relationships, 43, 45, 48
brain stem relationships, 39
cerebellopontine angle, 36
meatal relationships, 36–38
neural relationships, 39, 42–44
retrosigmoid approach, 35, 37–39
vascular compression, 49–57
venous relationships, 44–47
posterior cranial fossa, 9, 10
telovelar approach, to fourth ventricle,
64–65, 70
tumor removal
distorted nerves, 65, 69
dural attachment, 65, 67
meningioma attachment, 65, 66
nerve displacement, 65, 67
routes, 65
upper neurovascular complex
arterial relationships, 26–29
suprameatal extension, 32–33
trigeminal root anatomy, 20–24
vascular compression, 24–26
venous relationships, 29–32
Microsurgical technique, history of, 5–6
Middle fossa (MF) approach
advantages and drawbacks, 115
extended, 118–119
history of, 5
IAC, 115
standard, 115–118
Middle neurovascular complex
acoustic neuromas, 34
arterial relationships, 43, 45, 48
brain stem relationships, 39
cerebellopontine angle, 36
meatal relationships, 36–38
neural relationships, 39, 42–44
retrosigmoid approach, 35, 37–39
vascular compression, 49–57
acoustic neuromas removal, 53
cochlear and vestibular nerve
compression, 53–54, 56
geniculate neuralgia, 56, 57, 62
hemifacial spasm, 51
venous relationships, 44–47
Monostotic fibrous dysplasia (MFD), 90
Multiple endocrine neoplasia type 1
(MEN 1), 93
Multiple meningiomas
differential diagnosis, 344
MRI findings, 426–432
in NF-2 patients, 424
treatment, 425
Index 881

N hemangiopericytoma, 81–82
Neural relationships leptomeningeal melanocytic lesions, 83
lower neurovascular complex, 59–61 meningioma, 78–81
middle neurovascular complex, 39, 42–44 meningiosis and solid metastatic tumors, 83–84
Neurenteric cysts sarcoidosis, 86, 87
case illustration, 752–756 tuberculous meningitis, 85–87
clinical presentation, 749–750 osseous skull base
endodermal origin, 749 aneurysmal bone cyst, 90, 91
management, 750 chondroblastoma, 90, 91
MR characteristics, 749–751 chondrosarcoma, 90, 91
neuroimaging, 749 chordoma, 91–92
spinal, 749 endolymphatic sac tumor, 92–93
Neurocysticercosis, 788 fibrous dysplasia, 90
Neurofibromatosis type 2 (NF-2) osteochondroma, 90, 91
classification, 315 peripheral nervous system
clinical presentation MPNST, 76–77
bilateral VS, 316–317 schwannomas, 74–76
case illustration, 317–320 regional glial tumors
manifestation, 316 ependymoma, 99–100
multiple spinal schwannomas, 321–323 pilocytic astrocytoma, 99
differential diagnosis, 343 pilomyxoid astrocytoma, 99–100
facial nerve and VS, 252 sellar region
Gardner-type, 315 craniopharyngioma, 97–98
genetics, 315–316 granular cell tumor, 97
large VSs, 328–332 pituicytoma, 96
management, 321, 323–328 pituitary adenoma, 93–96
modified NIH criteria, 315–316 pituitary carcinoma, 95–97
multiple meningiomas, 424 spindle cell oncocytoma, 96
mutations, 375–376 xanthogranuloma, 98–99
personal series, 337–342, 345–351 New Hannover hearing classification, 256
revised clinical criteria, 315–316 NF-2. See Neurofibromatosis type 2 (NF-2)
smaller VS, 328, 332–335 Nonfunctioning adenomas, 94
surgery on hearing side, 332–333, 336 Non-langerhans cell histiocytosis, 103
Neurohypophysis, granular cell tumor, 97 Nonvestibular schwannomas
Neuropathology abducens nerve, 581–583
autonomous nervous system, 77 cochlear, 605
blood vessels and circulation facial
arteriovenous malformation, 105–108 case illustration, 590–593, 596, 599–308
hypertensive angiopathy and brain complete tumor removal with resection,
hemorrhage, 109–111 596–598
ischemic brain infarct, 109–110 ganglion geniculi, 584, 588–589
saccular aneurysm, 107–109 in mastoid segment, 584–587
vasculitis, 110–112 middle cranial fossa, 584
choroid plexus radiological patterns, 594–595
carcinoma, 101 lower cranial nerve (see Jugular foramen (JF) tumors)
papilloma, 100–101 oculomotor nerve, 578
germ cells trigeminal
tumorlike dysontogenetic cysts, 105, 107 classification, 547, 548
tumors, 104–106 clinical presentation, 549
hematopoietic system dumbbell tumors, 559–568, 575–576
langerhans cell histiocytosis, 102–103 infratemporal extracranial location, 569–574
non-langerhans cell histiocytosis, 103 management, 549–550
primary CNS lymphomas, 102 neuroimaging, 547–549
meninges operative technique, 569, 577
acute bacterial sive purulent meningitis, outcome, 577
84–86 posterior fossa location, 553–558
aspergillosis, 86–88 predominant location in middle cranial
cryptococcosis, 88–89 fossa, 550–553
cysticercosis, 88, 89 radiotherapy, 577–578
echinococcosis, 88, 89 surgical treatment, 550
hemangioblastoma, 82–83 trochlear, 578–580
882
O incidence, 788
Obstructive hydrocephalus, 275
Oculomotor nerve schwannoma, 578
Osseous skull base pathology
aneurysmal bone cyst, 90, 91
chondroblastoma, 90, 91
chondrosarcoma, 90, 91
chordoma, 91–92
endolymphatic sac tumor, 92–93
fibrous dysplasia, 90
osteochondroma, 90, 91
Osteoblastic metaplasia, 80
Osteochondroma, 90, 91
Osteomas, 839–840
P Pituitary carcinoma, 95–97
Papillary craniopharyngioma, 98
Papillary ependymoma, 100
Papillary meningiomas, 81
Parasitic meningitis, 88, 89
Pars nervosa, 611
Partial translabyrinthine approach, 121
Peripheral aneurysms, 773
Peripheral nervous system pathology
MPNST, 76–77
schwannoma
bilateral vestibular, 74, 76
cellular, 74–75
histology, 74
melanotic, 76
nonvestibular, 74
Petroclival meningiomas
case illustration, 438, 440–442
cavernous sinus, 435–436
endoscopeassisted removal, 452, 477–481
features, 435–437
jugular foramen and foramen magnum, 472–476
with large supratentorial part, 451–453
located medial to trigeminal nerve, 435–436
management of, 437
massive venous infarction, 447, 449–450
Meckel’s cave, 435–436
pattern of dislocation, 376, 379
presigmoid approach, 444–447
RSMA (see Retrosigmoid suprameatal approach)
small petrous apex, 438–439
sporadic unilateral VS and, 768–772
surgical management, 443
two-staged removal, 453, 456–458
venous phase of DSA, 447–448
Petrosal veins, 29, 48, 49
Petrous apex
cholesterol granuloma
classic obstruction-vacuum theory, 789
clinical presentation, 790
CT and MRI, 789
description, 788–789
differential diagnosis, 789
Index
management options, 790–791
recurrence rate, 791
removal of, 792–797
description, 788
mucoceles, 788
Petrous bone epidermoids, 515, 518
Pilocytic astrocytoma, 99, 703–705
Pilomyxoid astrocytoma, 99–100
Pituicytoma, 96
Pituitary adenoma
classification, 93–94
ectopic, 93
functioning, 94
microadenomas, 93
nonfunctioning, 94
Pleomorphic adenoma, 840–843
Plexiform schwannoma, 76
Pneumocephalus, 300
Polyostotic fibrous dysplasia (PFD), 90
Posterior cranial fossa
anatomy, 9
cranial nerves, 9–11
neurovascular complexes, 20
Posteroinferior cerebellar artery (PICA)
cerebellopontine angle, 11, 17–18
CNs X and XI, 17
Postoperative hydrocephalus, 302–304
Premeatal meningiomas
case illustration, 401, 404–406
complete removal of, 401, 403
limited growth into IAC, 401–402
pattern of dislocation, 376, 378, 380
Primary CNS lymphomas (PCNSL), 102
Proteolytic enzymes, 232
Psammomatous meningiomas, 78
R
Radiosurgery, history of, 6
Reanimation techniques, facial nerve reconstruction
accessory nerve, 866
facial-facial nerve anastomosis, 866
hypoglossal-facial anastomosis, 864–867
indications, 862, 864
Regional glial tumors pathology
ependymomas, 100
pilocytic astrocytoma, 99
pilomyxoid astrocytoma, 99, 100
Retrolabyrinthine approach, 121
Retromeatal meningiomas
case illustration, 398–400
CISS and T2-weighted sequences, 396–397
pattern of dislocation, 376, 378, 380f
sigmoid sinus involvement, 396
Retrosigmoid approach
anesthesia considerations, 123, 126
benefits of, 122–123
Index 883

chordomas, 805–806 Sinus thrombosis, 299–300

drawbacks Sphenopetroclival meningiomas, 376–377
limited exposure of the fundus, 134–135, 138 Spinal neurenteric cysts, 749
need of cerebellar retraction, 136 Spindle cell oncocytoma, 96
pneumocephalus and tension Sporadic unilateral vestibular schwannomas, 768–772
pneumocephalus, 137–140 Standard MF approach, 115–118
postoperative headache, 136–137 Subependymomas, 726–729
suboptimal operative technique, 134, 137 Suboccipital approach, 487–489, 612–615, 710–713
dura incision, 131–132 Suboccipital headache, 300
hemostasis, dural closure and mastoid Superficial cortical AVM, 107
occlusion, 132–134 Superior cerebellar artery (SCA)
microinstrument, 128, 129 cerebellopontine angle, 11, 14, 16, 18
patient positioning, 123–125 compression, 26
posterior skull base reconstruction, 132–134 Superior petrosal vein, 12, 29
semi-sitting position, 126–128 Suprameatal extension
sporadic ELST removal, 823–826 retrosigmoid approach
Retrosigmoid intradural inframeatal approach, 792–797 cerebellum, 33
Retrosigmoid suprameatal approach (RSMA) intraosseous structures, 32
approach/Samii approach neural structures, 32
CP angle levels, 453 petrosal vein, 33
extensive brain stem edema, 455, 459–460 tubercle removal, 32
hearing loss on right side, 454, 463–469 tumor removal, 34
intradural resection of petrous apex, 451–452 upper neurovascular complex, 32–33
Meckel’s cave and posterior cavernous Suprameatal meningiomas, 376, 378, 380
sinus, 455, 461–462 Sural nerve grafts, in facial nerve
slight hearing loss on right side, 454, 470–471 reconstruction, 856–857
Rhabdoid meningiomas, 81
Rosai–Dorfman disease, 103
T
Tanycytic ependymoma, 100
S Telovelar approach, to fourth ventricle, 64–65, 70
Saccular aneurysm, 107–109 Temporal bone, 40, 41
Samii approach/RSMA Teratomas, 105, 106
CP angle levels, 453 Thrombosed aneurysms, 773, 774
extensive brain stem edema, 455, 459–460 Tinnitus, 158, 298
hearing loss on right side, 454, 463–469 Torulosis, 88–89
intradural resection of petrous apex, 451–452 Transcochlear approach, 121
Meckel’s cave and posterior cavernous Transethmoid transsphenoidal approach, 799–800
sinus, 455, 461–462 Transitional meningiomas, 78
slight hearing loss on right side, 454, 470–471 Translabyrinthine (TL) approach
Sarcoidosis, 86, 87 advantages, 119
Schwannoma anatomical variations, 119
bilateral vestibular, 74, 76 history of, 4–5
cellular, 74–75 labyrinthectomy, 120
histology, 74 mastoidectomy, 119–120
jugular foramen (See Jugular foramen (JF) operative technique, 119
schwannomas) partial approach, 121
melanotic, 76 vs. retrolabyrinthine approach, 121
nonvestibular, 74 surgical outcome, 120–121
Schwannomatosis, 344 TL-retrosigmoid approach, 121–122
Secretory meningiomas, 78 TL-transtentorial approach, 122
Sellar region pathology transcochlear, 121
craniopharyngioma, 97–98 Transmastoid approach, 823–826
granular cell tumor, 97 Transpetrosal approach, history of, 5
pituicytoma, 96 Trigeminal nerve
pituitary adenoma, 93–95 arterial compression sites, 27–29
pituitary carcinoma, 95–97 retrosigmoid approach, 25
spindle cell oncocytoma, 96 and SCA, 26
xanthogranuloma, 98–99 venous compression sites, 30–31
884 Index

Trigeminal root anatomy meatal relationships, 37

anastomoses, 23 retrosigmoid exposure, 13
Meckel’s cave, 21 superior, 37, 41
motor rootlets, 21 vestibular schwannomas, 150
nerve hook, 23 Vestibular schwannomas (VS)
pons, 22, 23 audiometric and audiological
sensory root and motor rootlets, 22 examinations, 165
upper neurovascular complex, 20–24 bilateral, 74, 76
Trigeminal schwannomas brain stem, 214
classification, 547, 548 classification, 152–156
clinical presentation, 549 clinical presentation
dumbbell tumors, 559–568, 575–576 cisternal stage, 158–159
infratemporal extracranial location, 569–574 intracanalicular, 157–158
management, 549–550 MR imaging, 157, 158
neuroimaging, 547–549 tinnitus, 158
operative technique, 569, 577 conservative management, 162–163
outcome, 577 cystic VS
posterior fossa location, 553–558 capsule, 234–235
predominant location in middle cranial MR imaging, 233, 236–237, 239–240
fossa, 550–553 nerve adhesion, 238
radiotherapy, 577–578 proteolytic enzymes, 232
surgical treatment, 550 tumor debulking, 234
Trochlear schwannomas, 578–580 electronystagmography, 165
Tuberculous meningitis, 85–87 epidemiology, 147–148
Tumorlike dysontogenetic cysts, 105, 107 facial nerve
Tumor removal, multiple neurovascular complexes cystic tumors, 252
distorted nerves, 65, 69 delayed facial nerve palsy, 254
dural attachment, 65, 67 facial nerve palsy, 253–254
meningioma attachment, 65, 66 grading systems, 250
nerve displacement, 65, 67 intermediate nerve dysfunction, 254–255
routes, 65 intraoperative monitoring, 252
Type A trigeminal schwannomas, 550–553 NF-2, 252
Type B trigeminal schwannomas, 553–558 operative approach, 253
Type C trigeminal schwannomas, 559–568, 575–576 patient age, 252
Type D trigeminal schwannomas, 569–574 preoperative facial palsy, 252–253
preservation, 250
radiosurgery, 252
U reoperations, 252
Upper neurovascular complex taste disturbance, 255
arterial relationships, 26–29 tumor characteristics, 250–252
suprameatal extension, 32–33 giant (extension grade T5) VS
trigeminal root anatomy, 20–24 bone-window MRI, 207–210
vascular compression, 24–26 hearing loss, 201
venous relationships, 29–32 hemorrhagic complications, 201
neurological examination, 207
operated seven times, 245–246
V preoperative MRI, 202
Vascular compression RS approach, 204–205
lower neurovascular complex, 63–64 hearing
middle neurovascular complex, 49–57 classification, 255–256
upper neurovascular complex, 24–26 preservation rate, 262, 266–267
Vasculitis, 110–112 VS removal and preservation, 257–271
Venous relationships risk factors, 256
middle neurovascular complex, 44–47 history, of removal, 1–3
upper neurovascular complex, 29–32 hydrocephalus associated
Ventricular roof, 18 clinical management, 273, 282
Vestibular nerve communicating, 275, 277–278
cerebellopontine angle, 12 MRI, 274–275
compression syndromes, 53–54, 56 obstructive, 275
inferior, 37, 41 retrosigmoid approach, 280
Index 885

IAC opening retrosigmoid approach, 165

canal opening, 168 extrameatal tumor removal, 187–192
CT imaging, 168–169 high jugular bulb, 172–174
dura incision, 168 IAC opening, 168–173
MR imaging, 170 intracanalicular tumor removal, 187–189
posterior wall, 171 intracanalicular (extension grade 1)
intracanalicular (extension grade 1), 193–197 VS, 193–194
with large petrous bone involvement vascular considerations, 174–175, 183–187
brain stem, 214 surgical management
CT scan, 214 following previous radiosurgery, 287–291
debulking, 213 following previous surgery and
facial nerve, 213 radiosurgery, 292–296
massive destruction, 211–212 neurological symptoms, 284
large VS removal with primary debulking, 166–167 tumor removal, 163–165
of lateral IAC tumor adhesion/capsule
postoperative period, 198 incomplete tumor removal, 224–227
preoperative MRI, 198–200 intracanalicular VS, 217–221
medial VSs microsurgery, 215
caudal tumor extension, 229, 231 preoperative MRI, 215–216
cranial nerve dissection, 229 without obvious tumor capsule, 222–223
preoperative imaging, 228–229 Vestibulocochlear nerve
RS approach, 230 cerebellopontine angle, 13
molecular genetics, 148 foramen of Luschka, 44
neuroimaging, 156 infrafloccular exposure, 56
NF2 protein, 148–149 left cerebellopontine angle, 36
operative complications and management neural relationships, 42
cerebellar symptoms, 297–298 in petrosal surface, 10, 12
cranial nerves, 297 retrosigmoid exposure, 13
CSF leak, 300–302 von Hippel-Lindau syndrome (VHL)
meningitis, 302 with endolymphatic sac tumor, 93, 818, 827–830
mortality, 304 with hemangioblastoma, 83, 757
pneumocephalus, 300 VS. See Vestibular schwannomas (VS)
postoperative hydrocephalus, 302–304
sinus thrombosis, 299–300
suboccipital headache, postoperative, 300 W
tinnitus, 298 Waterhouse-Friderichsen syndrome, 85
vascular complications, 298–299 Willis’ circle, atherosclerosis of, 109
vestibular symptoms, 298 Wishart-type NF-2, 315
pathogenesis
growth pattern and rate, 150–152
nerve, 150 X
origin, 149–150 Xanthogranuloma, 98–99
site, 150 Xanthomatous metaplasia, 80
preoperative examinations, 165
radiosurgical management, 282–283
recurrent VS Y
after middle fossa removal, 248–249 Yolk sac tumor, 104
clinical management, 247
MRI, 241–242
preoperative MRI, 243–244 Z
prevalence, 240 Zellballen, 77