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TEACHING ATLAS of

HEAD Gild NECK


IMAGING
Robert Lufkin
Alexandra Borges
Pablo Villablanca

Thieme
Teaching Atlas of
Head and Neck Imaging
Teaching Atlas of
Head and Neck Imaging

edited by

Robert Lufkin, MD
Professor
Department of Radiological Sciences
UCLA School of Medicine
Los Angeles, California

Alexandra Borges, MD
Hospital Assistant in Radiology
Instituto Portugues de
Oncologia-Centro de Lisboa
Lisboa, Portugal

and

Pablo Villablanca, MD
Assistant Professor
Director, Clinical Imaging Processing Service
Department of Radiological Sciences
UCLA School of Medicine
Los Angeles, California

2000
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Printed in the United States of America


5 4 3 2 I

TNY ISBN 0-86577-691-1


GTV ISBN 3-13-107881-2
To all who have a passion in life

AB
RL

To my loving and supportive family

PV
RL
CONTENTS
Preface ......................................... . . ................ . . . . . . . . . .. . . . . . . . . . . . . . . . . . . ..... . . . . . . . .................... XI

Acknowledgme n ts .... . .... ........................................................................................... Xill

I. CENTRAL SKULL BASE

Case 1 . Petroclival Meningioma 3

Case 2. Trigeminal Nerve Schwannoma . . . . . . . . . . .......... . . . . . ................................... 8

Case 3. Fibrous Dysplasia .... . .


.. .................. . ..... . ...................... . .
.. ..... .............. . .... 13

Case 4. Chondrosarcoma 18

Case 5. Clival Chordoma 22

Case 6. M e tastasis ..................................................... . . .. . . .. . ................................ . 27

U. LARYNX AND HYPOPHARYNX

Case 7. Epiglo ttitas ........................................................ ....................... ............. . 35

Case 8. Laryngocele . ............ . . . . ..... . . . . . .. . ........ . . . . . . . . . . . . . . .. . . ............. ........................ 38

Case 9. Cord Paralysis ... . ..


.. .... . . . .
.. .. ... ........... . ........................... . ..
.. . ...... . ... . ..
. ..... . . 42

Case 1 0. Posttraumatic Deformi ty . . . . . . . . . . . . . . . . . . . . . . ...................................... . .......... 46

Case 1 1 . Vallecular Cyst . . .......... . ..... . . . . .. . . . . . . ..


.. . . . . ....... . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . ..... .. . 50

Case 1 2 . Chondroid Tumor of the Laryn x .......................................................... 54

Case 1 3. Glottic Carcinoma ................... . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . ........ . . . . . . . ......... 58

Case 14. Transglo tlic/Supraglo ttic Carci noma . . . . . . . . . . . . . . . . ..... . . . . . . ...... . . . . . .. . . ......... 62

Case 1 5 . Pyriform Sinus Carcinoma ..... . . ... ........ . ................................. . . . . . . . ......... 66

Case 16. Metas tasis .................... ................................ ............... .............. ............... 69

m. NASOPHARYNX AND PARAPHARYNGEAL SPACE

Case 17. Tornwal d t Cyst ..... . ........ . . . . . . . . . . . . . . . . . . . .. . . . . . . . . .. . . ....................... ...............


... 75

Case 1 8. Juvenile Nasopharyngeal A ngiofibroma ... .. . . . . ......... .. . . . ..... . . . . . . . . . ..... . .. 78

Case 19. Glomus Vagale . . . . . . . . . . . . . .. . . . . . .. . . . . . . . .. . . . . .. . . . . . . . . . . . . . . . . . .. . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . 82

Case 20. Post-Styloid XII Nerve Schwannoma ... . . . . . . . . . . . .. . . . . . . . . . ...... . . . . . . . . .. . . ..... . . 86


Case 2 1 . Lymphoma ... . .................. . . . . . . .. . ........ . . . .. . . . . . . . . . . .. . ..... . . .. . . . . . . . . . . . . . . . . . ...... . . . . . 89

Case 22. Liposarcoma . . . . .... . . . . . . .... . . . . . . . .......................... ........................ ...............


.. 92
Case 23 . Rhabdosarcoma .... .. . . . . . . . .. . . . . . . . . . .. . . . . . . . .. . . . . . . . . . . . . . . . . . . ......... .. . . . . . . . . . . . .... ..
. .... 96

Case 24. Squamous Cell Carcinoma ...... . .


. . . . . . ... . . . . . . . .. . . ...... . . . .. . . . . .. .. .
... .. .. .. .. .. ... .. .. 101

IV. NECK, THYROID, PARATHYROID

Case 25. Thyroglossal Duct Cyst ............................................ . ............................. 1 07


I CONTENTS

Case 26. Branchial Cleft Cyst .... .. ... ................. ........... .. ........ .. .......... .. .. .. . . . . . . . . . . . . . . III
Case 27. Lymphangioma . . . . . . .. ... . . .. .. . . . . . . . . . . . . . . .. .. ... . . . . . . . . . . ..... . . ...
. . . . . . . . . . . . . .. .. . . . .. . .. . .. . 1 15

Case 28. Tortuous Carotid Artery ........................................................................ 1 18

Case 29. Jug Vein Thrombosis .. . . . . ... . . .....


.. .. .. . . . . . . ....... .. .... .. ........ .. .. ....... .... ..
. .. . . . . . . . 1 22

Case 30. Lipoma . ... . . . . .. . . .............................. ..... .............. . . . . . . . . . . . . . . . . .... ..


. . . . . . . . . . . . . . .. . . 1 27

Case 3 ] . Madelung Disease ...... . . .. . . ............... ..... . . . . .. . . . . . . . ... . . . . . . ...... .....


. . . . . . . . .. . . . . .. . 132

Case 32. Retropharyngeal Abscess . ..... .. . . ..... . . . . . . . . . . . .. . . . . .. .. . .. . . . . ..... . . . . . . . . . . . . ...


. . . . . .. 1 37

Case 33. Carotid Body Tumor .. . ............................. .. ........... .......... . ............. . .. . . . . . . 143

Case 34. Schwanoma .. .. .. . ... .. ........... . ..... . ....... .. ..... ..... . ....... ...............
. . . . . . . .. . .. .. .. . . . . . 147

Case 35. Adenopathy .. . . .. . . . . . . . . . . . . . . . .. . . . . . . . . . . . .. . . . . .. .. .. . . . .. . . ..... ......


. . . . . .. . . . . . . .. . . . . . . . . . .. .. 152

Case 36. Reidel's Thyroiditis . .... . .. . . . .. ... . .. . .. . ........... .. ........ .. ...... .


. .. . ... .. . . . ... . . .. . . . . . .. . 157

Case 37. Goiter . . ........ . . . . .. . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . .................... . . . . . ....... 1 63

Case 38. Anaplastic Thyroid Carcinoma . . . .. . .. .. .. . . .. .... . . .... ... . .. . . . . . . .. . . . . . . . . . . . .. . . . . . . . . 1 69

Case 39. Parathyroid Adenoma . .. . . . . . . . . . . . . . . . .. .. . . . . . . . . .. . .. ... ...... .. ... . . .. . .. . . . . . . .. . . . .. ... . .. 1 74

Case 40. Tracheal Carcinoma . . . .. ................................... . . . . . . . . . .. . . . .. . . . . . . .


. . . . . . . . . .. .. .. . 1 79

V. ORBIT AND VISUAL PATHWAYS

Case 4 1 . Cellulitis . . . ..... . . . . . . . . . . . . . . . . . . .. . . . . . . . . . .. . . . . . . . . .. . . .. ... ... .. ..


. . .. . . . . . . .. . .. . . . . . .. .. . . . .. . .. . . .. 1 85

Case 42. Dacroadenitis .. ...... . . ... . .. . .... .. . ...... .. .............. ......... ... ....... ...
. . . .. . . .. . .. .. . . . . . . . . 1 89

Case 43. Pseudotumor . ............ ........ ............ . . . ... . . . . .. . . . .. .... . . . . . . . . ....... ..


. . . . . . . . . . .. . .. .. . . . 1 92

Case 44. Graves' Ophthalmopath y ................................................ ...................... 1 97

Case 45. Fat Supression Artifact . . . . . . . . . . . . .... . . . . . ..... . . . . . . . . . . . . . . . . . ... ....... ... 203 . . . . .. . . . . . . . .. . . .

Case 46. Optic Neuritis ..... ...... .................... .. .. ........ .............. ............. ... ... ..
. . . . . . . . . . . 207

Case 47. Hemangioma .................. ............... ....... ........... ............... ....... . . . . . . . . . . . . . . . . . . 211

Case 48. Meningioma . . . ...... . . . . . ..... .............. . . . . ........ . . .. . . ..... . . . .. . . ....... ...
. . . . . . . . . . . . . . .. .. . 214

Case 49. Ocular Melanoma ................................ .. .. ... . . . . . . . . . . ... . . . . . .. .. . . . .. . . . . . . . . . . .. . .. . 219

Case 50. Retinoblastoma . . .. . ........ ... .... .. .......... ....... .. . ...... ... ... .. ,............ 223
.. . .. . . . . . . . . . . . .

V I. ORAL CAVITY AND OROPHARYNX

Case 5 1 . Lingual Thyroid .. ..... . . . . . . . . . . . . . . .. .. . . . . . . . ........... . . ......... . . . . . . ... ... . . . . .


. . . . . . . . . . . . . . . 233
Case 52. Dermoid ..... . .. .. . .. ........ . ........ . . ...... . .......... .......... .. ............... 237
. .. . . . . . .. . . . .. . . .. . . .

Case 53. Ranula ........... ......... ..... ........... . . . . . . ..


. . . . . . . . ..... . .. ..
. . .... .. .. . . . . . ..... .. .. . . . .. .. .
. . . ... . . 241

Case 54. X I I Nerve Denervation Atrophy ............................. ................... . ........ 244

Case 55. Tongue Carcinoma .... .. . . . . . . . . . . . . . . ...... . . . ... . . . ..... . ... ..


. . .. .. . .. . . . . . . . . . .. . . . .. ... . . . .... 247

VII. SALIVARY GLANDS

Case 56. Accessory Parotid Gland .. .... .. .... . . ....... .. .. ... .. .. . .... .. ....... . .. . .. . .. .. .. . . . . . . . . . . . 255
Case 57. Sialolith . . . . .. . . ......... . . . . . . .. .. . . . . .. . . ... . . . . . . . . . . . . . . . . . .. . . . . . ... .
. . . . . . . . .. . .. .. . . . .. . . . . .. . . .. . . .. 259

I
CONTENTSI

Case 5 8. Lymphoepithelial Cysts . . . . .. . . . . . . . . .... . .. . . ...... ................ .......... . .. ........... ... . 263

Case 59. Postoperative . . . . . . . . . . . . .... . . .. ..


. . . . . .. . . .... . .. . . ..... . . . ........ ............ ... ................
. . . 267

Case 60. Sjogrens Syndrome........................... . . ...................................... ............... 270

Case 6 1 . Pleomorphic Adenoma ....... ........ .. . .... . .. . . . . ...


. . . . .. . .......... ... ... . . .. . . .. .. .. ..... . . 275

Case 62. Warthin's Tumor . . . . . . . . . . . . .. . . . . . . ... ..... . . .. .


.. . ... . . . . .... ... . . . . ... .... ... .... . . . . ........ .
. . . . 279

Case 63. Oncocytoma . . . . . . . . . . . ........ . . . . . . . . . . . . . . ........ . ........... ... ............. . . . . . . . . . ....


. . ..... .. . 2 82

Case 64. Adenoid Cystic Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . ...... . . . . . . . . . . . . . . . . . . . . . . . 2 86

Case 65. Mucoepidermoid Carcinoma .. . . . . . . .... ...... . . . .. . .. .. . . . . . . . . . . .. . . . . . .. . . ..


... . . . . .. . . .. . 289

VIII. SINONASAL

Case 66. Dermoid Cyst .. .. ... . . . . . . . . . . . . . .... .. . . . . . .. . . . .


. . . . . .. . . . . . . ... . . . . . . . . .. .. . .
. . .. . . . ..... .. . . . . . . . . . 295

Case 67. Nasopalatine Duct Cyst .. ....... .. . . . . . ... .. .. ... . ...... . . .. ... . .. .. ...... .....
. . .. .. . . .. . . . . . . . 299

Case 68. Frontal Osteoma . . . . . . . . . . . . . . . .. . . . . . . .. . ...... .. . . . . . ....... . . . . . . . . . . . . . ..... . . . . . . . . . . . . ......... 303

Case 69. Mucocele . . . . . . . . .


. . . . . . . . .... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ..... . ......... . ................ . . . . . . . 30 8

Case 70. Fungal Disease ..................... ......... ............. ..................... .. .. ................. 3 1 4 . . .

Case TI. Inverted Papilloma . . . . . . . ..... .... ... . . . . . . . . .. . . . . . . . . . . ....


. ............ . . . . . . . . . . . .............. 319

Case 72. Nasal Lymphoma . . . . . . . ..... . . .


. . . . . . . . . . . . . . . .. . . . . . . . . . . ... . ..... .. .. . .
.. . ... . . . . . . .... ........... 324

Case 73. Carcinoma . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .......... .. . . . . . . . . . . . .. . .... . .... 328

Case 74. Esthesioneuroblastoma . . . . . . . . . . . . . . . . . . . . .. .


. . . ...... . . . . ........ .......... .. . .............. . . 333

IX. TEMPORAL BONE

Case 75. Aberrant I nternal Carotid Artery ...... ..... .. ... .................. ......... ... . . .. . . . .. . 339

Case 76. Atresia of the External Auditory Canal ............................................ . . . 344

Case 77. Congenital Anomalies of the I nner Ear .. . . . . . . . . . . . . . . ....... . . . . . . . . . . . . . . . . . . . . . . . 349

Case 78. Fracture ....... ... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . ....... . . . . . . . . . . . . . . . . . . . .................. 355

Case 79. Labyrinthitis Ossificans . . . . . . . . . . . . . . . . . . . . . .


. . . . . . . ... . . . . . . . . ....
. . . . . . . . . . .. . .
. . . . . . . . . . . . . . . 361

Case 80. Otosclerosis .... . . . . . . . .............. .. . . . . . . . . . . . . ....................................................


. . 365

Case 8 1 . Petrous Apicitis ...... . ........... . . . . . . . . . . . . .......... . ... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 370

Case 82. Malignant External Otitis .. .. .......... .. ... .................. .......... .................... . . 375
Case 83. Acquired Cholesteatoma ........ . . . . . . . . . . . . . . . .... .. . . . . . . ....... .. . . .. . ...... . .... . . . . . . . . . . 38 1

Case 84. Vestibular Schwannoma .... . . . . . .. . .. .... . . . . . . . ..


. . .. .. .. . . . . . ... .. .... . ... .... . . . . . .
. . . . . . . . . 3 88
Case 85. Neurofibromatosis Type n ....... . . .. ........
. . . ... . . . . .. . .. .... .. . ... . . . . . . .
. . . . . .. . . . . . . . . . . 395
Case 86. Glomus Tympanicum . . . . . . ................ ..... ...................... ......... .... ........
. . . .. . . 400

Case 87. Mastoidectomy . . .. ....


. . . . . . .. .. . . . . . . . . ....................................... . ... . . . . . . . ... . . . . . . . . 404

Case 88. Middle Ear Prostheses and Devices ... ... ... ... ... ..... .. .. .... ... .... ...... . . . . . . . . . . . 408

Case 89. Bell's Palsy ......................... .. .. .. .. . .. .. . .. . . .. .. .. . . .. . . . . . . . . .. . .. ..... . .... ..


.. .. ... . . ... . . .. 412

Case 90. Facial Neuroma ......... .... ......... . .. ... ............... .......... ............... .... ......
. .. . . . . . . 417

ixl
I CONTENTS
Case 9 1 . Stapes Replacement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 423

x. TMJ AND MANDIBLE

Case 92. TMJ D islocation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . .. . . . . . . . . . . . . . . . . . . . . 43 1

Case 93. Radicular Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437

Case 94. Giant Cell Granulomaffumor ................................. ................ . . ........... 443

Case 95. Ameloblastoma . . . . . . . . . . . . . . . . . . . . . . . . . ... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 448

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . 451
Preface
Vocatus atque non vocatus deus aderit.
Carl lung

Head and neck imaging is more important than ever before. In the past, patients re
ferred for imaging studies of the head and neck region were closely examined by
primary care doctors and specialists who would obtain a focused clinical history and
perform a detailed physical examination. This filtering process resulted in patients
referred for head and neck imaging with finely crafted clinical questions of impor
tant prognostic and management significance that the image study was well suited
to answer. With changes in modern medical care, work-up of patients' complaints
has been "streamlined" through a number of mechanisms that has ultimately re
sulted in less access to medical resources and expertise. This results in increasing
numbers of patients referred for head and neck imaging studies that have only a
limited clinical work-up. I n order to adequately handle this situation, it is more im
portant than ever that the radiologist have a thorough knowledge of head and neck
imaging. Patients with conditions that in the past would have been diagnosed and
managed in the clinic are now being sent to the radiology department for examina
tion and ultimate triage. I n an increasing number of patients, the radiologist may be
the only head and neck specialist that the patient sees.

This book is intended for health care professionals in training and those in clinical
practice interested in the head and neck. It is a case review of important examples
of head and neck pathology but is not designed to be encyclopedic in scope. We
hope that the readers of this casebook have as much fun using it as we had writing
and preparing the material.

Robert Lufkin, MD
Alexandra Borges, MD
Pablo Villablanca, MD
Los Angeles, California 1 999
Acknowledgments

We wish to express our sincere appreciation to our colleagues for sbaring their ex
periences. We thank Dr. Jane Pennington and the staff at Thieme for their invalu
able help. We also thank Mr. Kyu Na for his assistance with manuscript preparation
and editing. Most important, this work could not have been completed without the
endless support and encouragement from our families.

RL
AB
py

I would like to thank Jeffrey Wensel and Eric Spak for all the good tips, editing help,
and support, and Fernando Torrinha [or all the great images be sent me from
Lisbon.

AB

xiii
I.

Central Skull Base


CENTRAL SKULL BASEl

Case 1

Clinical Presentation
A 59-year-old female presented with a 2-year history of pain and paresthesia in
volving the left side of her face.

A B
I CENTRAL SKULL BASE

Radiologic Findings
MRI of the skull base: Axial TIW (Fig. I-A) and axial enhanced T I W ( Fig. I-B) im
ages at the level of the pons and axial enhanced T l W image (Fig. I -C) at the level
of the middle cerebellar peduncles. These images show a well circumscribed, oval
shaped mass centered upon the left prepontine cistern. This mass is isointense to
gray matter on the TIW image and shows intense homogeneous enhancement on
the post-contrast images. A dural tail is seen along the posterior aspect of the
petrous ridge and superiorly along the left tentorium. This mass is compressing
but not invading the left cerebral peduncle. The internal auditory canal is free of
tumor.

Differential Diagnosis: Prepontine


Cistern Mass
Trigeminal schwan noma
Clivallpetroclival ligament meningioma
Parasagittal chordoma
Metastasis
Basilar artery aneurysm/ectasia

Diagnosis
Petroclival meningioma (tentorial type)

Discussion
The differential diagnosis of petroclival meningiomas includes cerebellopontine an
gie (CPA) lesions and lesions arising within or in the immediate vicinity of the
petrous apex. Systemic diseases such as multiple myeloma, eosinophilic granuloma,
lymphoma, and metastasis should also be included in the differential diagnosis. Le
sions in these locations may have components in both the posterior and middle cra
nial fossa by extension over the petrous tip.

Epidemiology
Meningiomas constitute approximately 1 5 to 20% of intracranial neoplasms. How
ever, tumors arising from the skull base are much less common. The most frequent
sites of origin are the sphenoid ridge, cribiform plate, paraseUar region, clivus, CPA,
tentorium, and cavernous sinus. These tumors are more frequent in females and the
peak age at presentation is in the 6th decade.

Pathophysiology
Meningiomas are benign tumors that arise from meningothelial cells. Petroclival
meningiomas originate from the meningeal folds surrounding the junction of the
occipital bone, petrous apex, and sphenoid bone. Several classifications have been
proposed because the exact location of the tumor has both clinical and thera
peutic implications. The classification proposed by Kawase et al (1996) divides
petroclival meningiomas into four groups: upper clival (origin in the clivus medially
to the trigeminal nerve), cavernous sinus (clival origin and growth into the cav
ernous sinus), tentorial (tentorial origin above the trigeminal nerve), and petrous
apex (petrous origin lateral to the trigeminal nerve). This system, based on the
site of dural attachment of the tumor, is useful in selecting the optimal surgical
approach. Also, the location of the tumor can be inferred from the presenting
CENTRAL SKULL BASEl

symptoms, which are a consequence of impingement upon the surrounding cranial


nerves.

Clinical Fin dings


Patients with petroclival meningiomas usually present with cranial nerve palsies or
uncommonly, with signs of increased intracranial pressure, when the tumors are
large enough to cause obstructive hydrocephalus. Cerebellar signs may also be
present. The cranial nerves involved depend on the exact tumor location. Deafness,
vertigo, and tinnitus are common presenting symptoms when the tumor impinges
upon the cisternal segment of the VIIIth and VIIth nerves complex or grows along
the LAC. lnvolvement of the trigeminal nerve in its cisternal segment or in the
Meckel's cave results in trigeminal neuralgia or facial hypesthesia. Diplopia is also
a frequent presenting symptom due to compression of the VIth nerve. This may oc
cur in its cavernous segment or in its epidural course passing through Dorello's
canal. The other cranial nerves coursing in the cavernous sinus ( I I I and IVth) may
also be involved. Headaches, imbalance, and papilledema can be the presenting
symptoms in patients with large tumors. Other focal neurologic findings may result
from brainstem or cerebellar compression. W hen small and separate from cranial
nerves, petroclival meningiomas can be completely asymptomatic, discovered inci
dentally on imaging performed for other reasons.

Imaging Findings
CT and MRI are the techniques of choice for imaging skull base meningiomas.
On CT meningiomas appear as discrete, iso- or hypodense masses with a broad
dural base. Calcification may be present and hyperostosis of the adjacent bone may
also be seen, but these are more common with convexity meningiomas. After con
trast administration there is strong enhancement. A dural tail may be recognized
but is not a specific finding. Petroclival meningiomas usually show a flat base on the
posterior surface of the petrous bone and a dome shaped medial surface contacting
the brainstem. Unlike acoustic tumors, meningiomas show an obtuse angle with the
petrous bone and are usually medial to the lAC. However, they can extend along
the intracanalicular portion of the nerve sheath.
CT is optimal for detection of associated bony erosion or destruction of the
petrous apex, which would suggest an alternative, more aggressive process. Lesions
arising within the petrous apex usually cause bony expansion. Pneumatization of the
petrous apex and petrous apicitis are readily recognized on CT but can be mislead
ing on MRI. Confusing petrous apicitis with tumor is common on MRI.
M Rl is particularly helpful in defining the exact location of the tumor and pattern
of spread, factors that are critical in selecting the best surgical approach. On M RI,
meningiomas are seen as well-defined globular or plaque-like masses, isointense to
gray matter on both Tl W and TIW images. I ntense homogeneous enhancement is
usually seen, unless calcification is present. Due to the multiplanar capability and
high soft tissue contrast of MR, it is possible to determine the site of tumor attach
ment and relation to the adjacent cranial nerves. In an MRI of the skull base axial
(Fig. I-D) and coronal (Fig. I-E) enhanced T1W images show an enhancing extra
axial mass located posterolateral to the left cavernous sinus. This mass is well cir
cumscribed and impinges upon the uncus and hippocampal formation of the left
temporal lobe. The mass appears to originate from the lateral margin of the cav
ernous sinus and does not involve Meckel's cave. The cavernous carotid artery is un
remarkable and there is no evidence of edema in the surrounding brain
parenchyma. The diagnosis is cavernous sinus meningioma.
Petroclival meningiomas may extend inferiorly into the jugular foramen and
along the tentorium, laterally into the lAC or anteriorly into the cavernous sinus,
Meckel's cave, and medial cranial fossa. The signal characteristics of meningiomas
allow ready diferentiation from petroclival cystic lesions. In an MRI of the skull
I CENTRAL SKULL BASE

D E

base, an axial T I W image (Fig. I-F) and an enhanced coronal TI W image (Fig. J -G )
show a right petroclival lobulated mass, isointense to gray matter on Tl W images
and strongly enhancing after contrast administration. The lesion is attached to the
posterosuperior aspect of the petro us apex and extends superiorly through the am
biens cistern into the choroidal fissure. The mass impinges upon the right ccrebral
peduncle and pons. There is also significant mass efrect upon the right hippocampal
rormation. An enhancing dural tail is seen along the cisternal segment of the V I I I
and V I [ nerves complex. The diagnosis i s petroclival meningioma (petrous apex
type).

F G
CENTRAL SKULL BASEl

Management
PEARLSIPITFALLS
MRI s trongly enhances masses The treatment for skull base meningiomas is surgical resection. Radiation therapy
tha t are isoi n tense to gray matter is usually considered for incomple te resections and recurrences. More recen tly,
and show a "dural tail." s tereotatic radiosurgery has been used in the primary treatment of skull base
meningiomas when surgical resection is expected to cause unacceptable neurologi
An MRI defines the exact loca cal defici ts or to tre a t postsurgical macroscopic disease. This modality appears to al
tion and e x tension of the tumor low con trol of further grow th without severe morbidi ty, and is a promising tech
i n to the cavernous sinus, nique in the management of these benign slow growing tumors.
Meckel's cave, ten torium, or Surgical approaches vary according to the location of the tumor, with the focus
lA e. upon functional preservation of the cranial nerves. Nevertheless, complete resection
cannot be achieved in approximately 35% of cases due to tumor adherence to the
A cr scan is optimal for d e tect brainstem, cranial nerves, or cavernous carotid artery. Compl e te removal should not
ing for calcification and focal hy be pursued a t the cost of neurologic impairmen t. Expectant managemen t is also ac
perostosis. ceptable for small, asymptomatic tumors in difficult locations.

Whe n diagnosing this condition,


the clinician may confuse a Suggested Readings
meningioma w i th a vestibular
Couldwell, W. T., Fukushima, T., Gianno tta, S.L., Weiss, M.H. P e trocLival menin
schwannoma.
giomas: surgical experience in 1 09 cases. Journal of Neurosurgery 84( 1 ):20-8, 1996
Whe n diagnosing this condition, Jan.
the clinician may mistake unilat Fazi, S., Barthelemy, M. P e troclival meningioma mimicking the prese n ta tion of a
eral re te n tion of secre tions for a transie n t ischemic a ttack. Acta Neurologica Scandinavica 89( 1 ):75--{j, 1 994 Jan .
tumor. Kawase, T., Shiobara, R., Ohira, T., Toya, S. Developme n tal pa tterns and character
istic symptoms of p e troclival meningiomas. Neurologia Medico-Chirurgica
Whe n diagnosing this condition, 36( 1 ): 1 --{j, 1 996 Jan.
the clinician may fail to recog
Nicolato, A., Ferraresi, P , Foroni, R., Pasqualin, A., Piovan, E . , Severi, F., Masotto,
nize involveme n t of s tructures
B., Gerosa, M. Gamma K nife radiosurgery i n skull base meningiomas. Preliminary
which would preclude surgery.
experience wi th 50 cases. S tereotactic and Functional Neurosurgery 66 Suppl
1: 1 1 2-20,1996.
Pomeranz, S., UmanskY,I, Elidan, 1., Ashkenazi, E., Valarezo, A., Shalit, M. Giant
cranial base tumours. Acta Neurochirurgica 1 29(3--4) : 1 2 1 -6, 1 994.
Sekhar, L.N., Wrigh t, D. C, Richardson, R., Monacci, W. P e troclival and foramen
magnum meningiomas: surgical approaches and p i tfalls. lournal of Neuro
Oncology 29(3) :249-59, 1 996 Sep.
Ta tagiba, M., Samii, M., Ma tthies, C, Vorkapic, P. Manageme n t of petroclival menin
giomas: a cri tical analysis of surgical tre a tmen t. Acta Neurochirurgica Supplernen
tum 65:92--4, 1 996.
Thomas, N.W., King, T.T. Meningiomas of the cerebellopontine angle: a report of 4 1
cases. British lournal of Neurosurgery 10(1) :59-68, 1996 Feb.
Zen tner, 1., Meyer, B., Vieweg, u., Herberhold, C, Schramm, 1. P e troclival menin
giomas: is radical resection always the best option? lournal of Neurology, Neuro
surgery and Psychiatry Apr, 62(4):341-5, 1 997.
icE TRAL SKULL BASE

Case 2

Clinical Presentation
A 42-year-old female pre ented with progre sive left-sided facial pain and pares
the ia.

A B

I
CENTRAL SKULL BASEl

Radiologic Findings
On an M R I of the skull base the axial T1 W ( Fig. 2-A), and axial (Fig. 2-B) and coro
nal (Fig. 2-C) post-contrast T l WI show a dumbbell-shaped lesion involving the
Meckel's cave and left prepontine ciste rn, following the course of the left trigeminal
nerve. The mass is of intermediatellow signal intensity on Tl WI and enhances het
erogeneously, showing a nonenhancing anterior component. The coronal image
shows denervation atrophy of the left pterygoid muscles. There is no evidence of tu
mor extension through the foramen ovaIe.

Differential Diagnosis: Mass or Abnormal


Enhancement of the Trigeminal Nerve
Trigeminal nerve schwannoma/neurofibroma
Lipoma
Hemangioma
Perineural spread of a tumor
Metastasis
Inflammatory disease (sarcoid)
Infection ( herpes zoster, Lyme disease, actinomycosis)
Lesions in the Meckel's cave: meningioma, dermoid, epidermoid, metastasis

Diagnosis
Trigeminal nerve schwan noma

Discussion
Epidemiology
Schwannomas are benign nerve sheath tumors arising from cranial, spinal or sym
pathetic nerves. TIley occur exclusively in sensory or mixed nerves. I ntracranial
schwannomas are common, comprising 8% of all intracranial tumors. Among these,
95% originate in cranial nerve V I I I . Trigeminal schwan noma, is in a distant second
place, and occurs with equal frequency in men and women with a peak incidence in
the 4th decade. The trigeminal nerve is the one most commonly involved in the cen
tral skull base. Trigeminal schwannomas may occur in isolation or in association
with neurofibromatosis.

Pathophysiology
Trigeminal schwannomas are benign encapsulated tumors which originate from the
nerve sheath. They are composed of two different types of tissue designated as An
toni A and Antoni B (see vestibular schwannoma). These tumors grow slowly, do
not tend to recur and rarely show malignant transformation. Malignant trigeminal
schwannomas "de novo" are also rare with only a few cases described in the litera
ture and are more common in the setting of neurofibromatosis (plexiform neurofi
broma ) . TIlese malignant tumors are characterized by increased cellularity and
nuclear pleomorphism and may be confused with sarcomas, speci fically with fi
brosarcoma. Most malignant trigeminal schwannomas in the head and neck are
localized in the retromaxillary or pterygomandibular region.
Trigeminal schwannomas are classified according to their location along the
course of the nerve. Location determines both the clinical symptoms and the surgi
cal approach to the lesion. Type I tumors, the most common, occur in the middle cra
nial fossa in the region of the gasserion ganglion. Type I I I tumors are the second
most common, typically seen as dumbbell-shaped tumors with a posterior fossa
I CENTRAL SKULL BASE

component in the prepontine cistern and a middle cranial fossa component in


Mecke l 's cave, separated by a waist at the level of the porus trigeminus. Type I I tu
mors are limited to the posterior fossa, and involve only the cisternal segment of the
nerve. Finally, type IV comprises the extracranial tumors with or without minimal
intracranial extent.

Clinical Findings
Most patients present with pain, paresthesias. and numbness. usually along the
course of the first or second divisions of the trigeminal nerve. The association of
trigeminal schwannoma with trigeminal neuralgia is controversial. Some experts as
sociate this neuralgic pain with involvement of the trigeminal root, whereas others
relate it to involvement of the gasserian ganglion. Headaches and symptoms of com
pression of adjacent cranial nerves are common. Diplopia, facial weakness and sen
sorineural hearing loss are among the most frequent, and result from compression
of the cavernous segment of cranial nerve V I and cisternal segments of cranial
nerves V l l and V l l l by large trigeminal tumors.
Extension of a tumor along the third division of the Vth nerve may result in tris
mus or weakness of the masticator muscles. Large tumors in the posterior fossa may
cause cerebellar and pyramidal symptoms. Extension along the V I division may pre
sent with exophthalmos and papilledema. Physical exam may reveal loss of corneal
reflex.

Imaging Findings
Cross-sectional imaging allows precise depiction of tumor location and extent and
has largely improved the outcome of surgical treatment. Imaging is also helpful in
establishing the diagnosis and in the follow-up of these patients. CT and MRI are
used complimentarily to assess bony changes and soft tissue extent, respectively.
Plain films of the skull base may suggest the diagnosis by showing enlargement,
remodeling or erosion of the skull base neural foramina through which the three di
visions of the trigeminal nerve exit the skull (superior orbital fissure-V l , foramen
rotundum-V2, and foramen ovale-V3). Large tumors may also remodel or erode the
petrous apex, lateral aspect of the sphenoid in the region of the cavernous sinus, an
terior c1inoids or dorsum sellae.
CT, due to its higher spatial resolution, shows to advantage these bony changes
and at the same time depicts the soft tissue component of the tumor. Trigeminal
schwannomas are usually iso - to slightly hyperdense to the brain and enhance on
the postcontrast images. Large tumors may be heterogeneous due to cystic degen
eration, necrosi or intra tumoral hemorrhage. Schwannomas of the Vth nerve tend
to be oblong in shape following the long axis of the nerve. They may be seen any
where from the nerve root, in the lateral aspect of the prepontine cistern to the pe
ripheral branches of the nerve. A bilobed, dumbbell-shaped tumor is typical of
trigeminal schwannomas extending from the cisternal segment in the posterior fossa
into the cavernous segment in the middle cranial fossa with constriction of the tu
mor mass at the level of the porus trigeminus.
M RI is the best imaging modality to assess the extent of these tumors. Gadolin
ium enhanced M R T may depict small tumors that would otherwise be missed.
Trigeminal schwannomas tend to be isointense to t he brai n on T l WI, hyperintense
on T2WI and enhance after contrast administration. Fluid-fluid levels have been re
ported in these lesions resulting from hemorrhage, or layering of debris, or fluids
with different protein composition.
MRI better delineates the relationship of the tumor to adjacent structures, par
ticularly with the brainstem, petrous apex, VU and V I l l th nerve complex, cavernous
sinus and intracranial segments of the internal carotid artery. Replacement of the fat
signal adjacent to the skull base foramina and pterygomaxillary fissure is a useful
sign of tumor extension into these foramina and is better depicted with noncontrast
T l WI .
CENTRAL SKULL BASEl

D E

Cross sectional studies for patie n ts wi th trigeminal nerve symp toms should in
clude all segmen ts of the nerve, including the posterior fossa, skull base, orbi ts, and
infratemporal fossa. A trophy and fa tty infiltration of the muscles of mas tication may
be seen, indicating denervation atrophy. This imaging finding should prompt a
search for trigeminal nerve pathology.
Differe n tial diagnosis of this lesion includes other cranial nerve schwannomas, es
pecially scbwannomas of the cis ternal segme n ts of the V II and V l l l th nerves, petro
c1ival or cavernous meningiomas, perineural spread of tumor, and rare infectious/in
flammatory processes.
Differe n ti a tion from schwannomas of o ther cranial nerves may be difficult when
tumors are very l arge, but is usually possible once the radiologist is familiar wi th the
anatomy of the cranial nerves. V I I and V I T I nerve schwannomas are more l a teral in
position in the prepontine cistern and course in the direction of the internal audi
tory canal, which may be enlarged.
Schwan nom as grow along the nerve sheath by direct extension, as a single bulky
lesion. The presence of "skip enhancing lesions" along the trigeminal nerve is typi
cal of perineural spread of a tumor and should prompt a search for the primary neo
plasm. Neoplasms mos t likely to show this pattern of spread include adenoid cystic
carcinoma, mucoepidermoid carcinoma, lymphoma, and squamous cell carcinoma.
The second division of the trigeminal nerve is particularly prone to perineural
spread of head and neck tumors due to i ts e x tensive branching n e twork. Usually this
spread is re trograde from the peripheral branches to the main trunk. Hematoge
neous me tastasis to the trigeminal nerve may also occur and are more commonly
seen in patients with melanoma, breast, and lung carcinoma. On an MRI of the skull
base axial (Fig. 2-D) and coronal (Fig. 2 - E ) post-contrast Tl W images show abnor
mal enlargeme n t and enhanceme n t of the right Meckel's cave in a patien t wi th
known breast carcinoma. The diagnosis is me tasta tic breast disease (hematogeneous
dissemination). Inflamma tory "neuritis" manifes ts as abnormal segmental, smooth
enhancement along the course of the nerve.
I CENTRAL SKULL BASE

Man agement
PEARLSIPITFALLS
A trigeminal schwannoma can be llle treatme nt for trigemi nal schwa nnomas is surgical excision. The surgical ap
see n as a n e nhancing mass fol proach is dictated by the locatio n a nd exte nt of the tumor. As a ge neral guideline,
lowing the course of the trigemi type 1 tumors are approached through a fro ntotemporal cra niotomy via a tra nsyl
nal nerve. via n approach. Type 2 tumors are approached through retrosigmoid cra niotomy.
Type 3 tumors are approached through a combi ned subtemporal retromastoid ap
cr and M R I together give a pre proach, a nd type 4 tumors through a n extradural i nfratemporal approach. The best
cise picture of bony changes a nd m a nageme nt of maligna nt schwa nnomas appears to be surgery followed by radia
soft tissue extent. tion therapy.

Image all segments of the trigem


inal nerve.
Suggested Readings
Catal a no, P., Fang-Hui, E., Som, P.M. Fluid-fluid levels i n be nign neuroge nic tumors.
It is importa nt not to miss the American Journal of Neuroradiology 1 8(2):385-7, 1 997 Feb.
full exte nt of the tumor due to an
Charabi, S., M a ntoni, M., Tos, M .. lllOmse n. 1. Cystic vestibular schwa nnomas:
incomplete study.
neuroimagi ng a nd growth rate. Journal of Laryngology and Otology 108(5):375-9,
1 994 May.
Avoid mistaking perineural
spread of a tumor for a schwan Krish namurthy, S., Holmes, B., Powers, S. K. Schwa nnomas limited to the i nfratem
noma. poral fossa: report of two cases. Journal of Neuro-Oncology 36(3):269-77, 1998 Feb.
Maut ner, VF.. L i nde nau, M., Baser, M.E., Hazim, W., Tatagiba, M., Haase, W.,
It is important not to miss dener Samii, M., Wais, R., Pulst, S.M. The neuroimagi ng a nd cli nical spectrum of neuro
vation atrophy of the muscle of fibromatosis 2. Neurosurgery 38(5 ):880-5, discussio n 885-6, 1 996 May.
mastication.
Samii, M., Migliori, M.M., Tatagiba, M., Babu. R. Surgical treatme nt of trigemi nal
schwa nnomas. Journal of Neurosurgery 82( 5 ) :7 1 1-8, 1 995 May.
Tegos, S ., Georgouli, G.. Gogos. c., Polythothorakis, 1., Sa nidas. V. Mavrogiorgos. C.
Primary, maligna nt schwa nnoma i nvolvi ng simulta neously the right Gasserian ga n
glion a nd the distal part of the right ma ndibular nerve. Case report. Journal of Neu
rosurgical Sciences 41 (3) :293-7, 1 997 Sep.
Turgut, M., Palaoglu, S., Akpi nar, G., Saglam, S. Gia nt schwa nnoma of the trigemi
nal nerve misdiagnosed as maxillary si nusitis. A case report. South African Journal
of Surgery 35(3): 1 3 1 -3, 1 997 Aug.
Yamashiro, S., Nagahiro, S., Mimata, c., Kuratsu, 1., Ushio, Y. Maligna nt trigemi nal
schwa nnoma associated with xeroderma pigmentosum--case report. NeLII'ologia
Medico-Chimrgica 34( 1 2 ) : 8 1 7 -20, 1 9 94 Dec.
CENTRAL SKULL BASEl

Case 3
Clinical Presentation
A 35-year-old male presented w i th facial dismorphism, anosmia and pain on
chewing.

A B
/cENTRAL SKULL BASE

Radiologic Findings
Axial cr sections through the skull and facial bones (bone window) ( Figs. 3-A,
3 -B, and 3 -C) show extensive bony changes. These consist of expansion of the
medullary cavi ty wi th cystic and ground glass pallerns merging imperceptibly with
normal surrounding bone.

Differential Diagnosis: Diffuse Fibroosseus


Lesions in the Skull Base or Facial Bones
Common
Fibrous dysplasia

Paget's disease

M e tastasis

Ossifying fibroma

M u l tiple os teomas

Hyperparathyroidism

Rare
Meningioma tosis

Osteogenesis imperfecta

Juvenile Paget (hereditary hyperphosphatasia)

Progressive diaphyseal dysplasia (Camura tti -Englemann disease)

Pyknodysostosis

Diagnosis
Polyostotic fibrous dysplasia (leontiasis ossea)

Discussion
Epidemiology
Fibrous dysplasia is an idiopathic skeletal disorder characterized by a defect in os
teoblastic ma tura tion. I t is a common disorder, first described in 1938, by Lichen
stein and Jaffe.
There are three main forms of disease, depending on the number of bones in
volved and the presence or absence of other clinical fea tures. The monostotic form
is the most common, accounting for 75 to 80% of all cases, and tends to preferen
tially involve such long bones as the femur and ribs. Isolated involveme n t of a sin
gle craniofacial bone is seen in 10 to 25% of cases with the maxilla and mandible the
most freque n t s i tes. The polyostotic form accoun ts for 10 to 25% of cases and may
be un i- or bilateral. Craniofacial involvement is responsible for 40 to 60% of cases
and is also refered to as leo n tiasis ossea due to the characteris tic facial disfigure
men t. Albrigh t - McCune -Sternberg syndrome, the rarest form, accoun ts for 10% of
cases and consists of unilateral bone involveme n t associa ted with skin pigmentation
and precocious puberty. This form is seen almost exclusively in females.
Cherub ism is considered a special hereditary form of fibrous dysplasia exclusively
involving the jaws. The disease is usually symmetrical and tends to regress spon ta
neously after puberty.
I n the craniofacial form the bones most frequen tly involved include, in decreas
ing order of frequency, the sphenoid, frontal, maxillary, e thmoid, occipital. and tem
poral bone. The peak age for fibrous dysplasia is the second and third decades. The
disease tends to progress during the period of skeletal growth and to s tabilize once
growth ceases, a l though this is not always the case. Milder forms of fibrous dyspla-
CENTRAL SKULL BASEl

sia may manifest only in adulthood. The polyostotic forms tend to present earlier in
life and have a more aggressive course.

Pathophysiology
Pathologically, fibrous dysplasia is characterized by abnormal maturation of
medullary bone, which is replaced by mildly to highly cellular tissue composed of a
mixture of spindle-shaped fibroblasts and irregular trabeculae of woven bone with
out osteoblastic lining or progression to lamellar bone. The irregularly shaped bony
trabeculae have been compared to chinese characters. The "fibrous bone" merges
imperceptibly with normal surrounding bone. Unlike ossifying fibroma and fibrous
osteoma, these lesions are not encapsulated. The cortical bone is typically spared, al
though it may be thinned and transgressed due to marked expansion of the
medullary cavity.
Malignant transformation has a reported incidence of 0.5 % , with most of the
cases occuring after radiation therapy. The interval between diagnosis and malig
nant degeneration is usually more than one decade. Sarcomas are the most common
secondary malignancy, including osteo-, fibrous- and chondrosarcomas. Fifty per
cent of tbe sarcomas associated with the monostotic form occur in the craniofacial
region.
The etiology of fibrous dysplasia is unknown. Three different theories have been
proposed to explain its origin including developmental, traumatic and neoplastic. At
the present time the former is the most widely accepted .

Clinical Findings
Clinical findings largely depend on the extent and location of the disease. Patients
with craniofacial involvement present clinically with facial disfigurement, pain, or
functional problems including cranial nerve deficits.
Progressive, painless facial, or calvarial swelling is the most common presenta
tion. However, when severe bony expansion is present, sinonasal obstruction, mal
occlusion, exophthalmus and diplopia may ensue. Obstruction of sinus drainage pre
disposes these patients to mucocele formation. Cranial nerve deficits, due to stenosis
and obliteration of their natural skull base foramina, are a common complication.
Anosmia, visual impairment, diplopia, sensorineural hearing loss, and facial nerve
palsy are among the most common neurolog.ic deficits, although all cranial nerves
may potentially be involved. Physical exam of these patients should always include
a full ENT evaluation and assessment of the cranial nerves.

Imaging Findings
Imaging is important to establish the diagnosis and to evaluate the extent of the dis
ease. CT is the preferred modality. The M R T lacks specificity and on plain films; it
may be difficult to determine disease extent due to significant overlap of osseous
structures in the craniofacial region. The hallmark of fibrous dysplasia is expansion
of the medullary cavity by fibrosseous tissue, sparing the cortex and preserving the
original shape of the involved bone(s). The woven bone of the medullary cavity may
have a variety of patterns, depending on the amount of ossification of the fibrous
matrix. A hyperlucent cystic appearance results when trabecular bone formation is
poor or absent and is more common in the early stages of the disease. Progressive
ossification, with formation of irregular bony trabeculae intermingled with fibrous
matrix, leads to the classic "ground-glass" pattern. When bone formation and min
eralization predominates, the involved bone becomes densely sclerotic. These dif
ferent patterns may occur together or in isolation. Severely expansive lesions may
develop cortical breakthrough. Periosteal reaction is usually not seen except in
cases of pathological fracture or malignant transformation.
E F

Thin cr cction through the kull ba e are mandatory to detcct tenosi or oblit
eration of neural foramina. omplication uch a nasal ob truction or mucocele
formation are also adequately detected on cr cans. Tridimcn 'ional reconstructions
may provide useful information when urgical treatment is con idcrcd. Figure 3-D
hows a cr of the axial section of parallasal sinuses in anothcr patient. Figures 3-E
and 3-F arc coronal sections showing expansive cystic bony lesions which involve
the maxilla and mandible with multiple peck led calcification . TIle cortices of the
maxillary bones are thin ned with focal area of bony dehisccnce. -mere is marked

16
CENTRAL SKULL BASEl

displacement of tee th , particularly in the superior alveolus, w i th tee th seen adjacen t


PEARLSIPITFALLS to the orbi tal floors. The maxillary sinuses are completely obl i tera ted and there is
I t is a painless facial deformi ty also obli tera tion of the inferior aspect of the nasal cavi ty.
(leon tiasis ossea)/craoial nerve On M R I , fibrous dysplasia presen ts as low or i n termediate in tensity on T l W
palsy which can occur in a young images and bypoi n tensi ty on TIW images. Hemorrhage and cystic lesions may con
adolescen t. tribu te to signal h e terogenei ty. I n tense enhancemen t of the medullary cavi ty is the
rule.
Expansive cystic, sclerotic, or Paget disease, a p a thologic process wi th similar appearance, tends to involve the
ground-glass appearing lesions cortical bone and is more likely to cause bony deformi ty. Also, this disease occurs
involve the bone marrow, and later in life and, as opposed to polyosto tic fibrous dysplasia, is more predominan t
spare cortical bone. among males.
Ossifying fibroma may be difficul t to distinguish from fibrous dysplasia bo th
Axial cr sections show iIl radiographically and p a thologically. However, this lesion tends to be more focal and
defined margins wi th the abnor to have a more aggressive course.
mal bone merging imperceptibly Meningiomatosis may cause e x tensive bony sclerosis of the skull base mimicking
w i th normal bone. fibrous dysplasia. Post-con trast CT or M R I of the brain shows the e x tracranial tu
mor location and resolves this issue.
Unilateral polyosto tic fibrous
dysplasia in a young female usu
ally is accompanied by preco M anagement
cious puberty and pigme n ted Fibrous dysplasia is a benign disease tha t should be managed conservatively unless
cutaneous lesions (Albrigh t significant functional impairme n t or neurologic deficits ensue. Surgery for cosmetic
McCune-S ternberg syndrome). reasons should ideally be postponed u n til growth ceases and the lesions s tabilize.
When necessary, surgical resections in the craniofacial region should be l im i ted due
Fibrous dysplasia is l im i ted to
to the pote n tial surgical complica tions and the high recurrence rate. Recurrence oc
the jaw i n Cherubism.
curs in as many as 20 to 30% of cases wi thin 2 to 3 years after surgery.
The clinician may fail to recog
nize compromise of cranial Suggested Readings
nerves due to foraminal e n trap
ment. Ferguson, B.J. Fibrous dysplasia of the paranasal sinuses. A merican Journal of
Otolaryngology 15(3) :227-30, 1 994 May-Jun.
The clinician may fail to recog Simovic, S., Klapan, L., Bumber, Z., Bura, M. Fibrous dysplasia i n paranasal cavi ties.
nize mucocele formation. Journal of Oto-Rhino-laryngology and Its Related Specialties 58( 1 ) :55-8, 1 996 Jan
Feb.
Irradiating fibrous dysplasia le
sions may lead to a malignan t Van Rompaey, D., Schmel zer, B., Vers traete, W., Cammaert, T. Fibrous dysplasia in
transformation. the fron toe thmoidal complex: diagnosis and surgical aspects. Acta Oto-Rhino
Laryngologica Belgica 48( 1 ) :37--40, 1 994.
The clinician may mistake fi
brous dysplasia for a malignan t
process.
1 CENTRAL SKULL BASE

Case 4
Clinical Presentation
A 53-year-old male prese nted with worsening headache and diplopia.

A B

Radiologic Findings
I n an M R I of the skull base. axial post-contrast Tl WI (Fig. 4-A), a nd axial TIWI
(Fig. 4-B) show an e nh a nced lesio n i n the ce ntral skull base. ce ntered in the left lat
eral aspect of the clivus. TIle lesion is heteroge neous on the TIW images with sev
eral areas of hypoi nte nsity i nterspersed with areas of hyperi nte nsity. probaly repre
se nt i ng calcificatio ns. TIle left cavernous carotid artery is e ncased by tumor a nd
reduced i n caliber at the cervico-petrous j u nction. TIle mass protrudes posteriorly
i nto the left prel11edullary cistern.

Differential Diagnosis: Mineralized Lesions


of the Skull Base
Cho ndrosarcoma
Chondroma
Chondrol11yxoid fibroma
Chondroid chordoma
Osteosarcoma
Metastasis

118
CENTRAL SKULL BASEl

Diagnosis
Low grade chondrosarcoma of the skull base

Discussion
Epidemiology
Cartilaginous tumors of the skull base are relatively rare. The majority of these are
chondrosarcomas, which account for 0. 1 5 % of all intracranial neoplasms and for 5
to 10% of all skull base tumors. Only 5% of chondrosarcomas occur in the head and
neck region, the most common locations being the maxilla, mandible, skull base and
paranasal sinuses. They can occur at any age, but there is a peak incidence between
the 4th and 6th decades. There is no sexual predilection. Their benign counterpart,
chondroma, is even rarer and may occur in isolation or as part of Oilier's disease
(multiple enchondromatosis) or Maffucci 's syndrome (multiple enchondromas and
hemangiomas ).

Pathophysiology
Chondrosarcomas usually originate from skull base synchondroses. But they may
also arise from cartilaginous remnants left behind during embryologic development,
which may account for rare extraosseous locations. Development of chondroid tu
mors from multi potential mesenchymal cells or from chondral metaplasia of fibrous
tissue have also been postulated. Chondrosarcomas may arise de novo or result
from malignant degeneration of a pre-existing chondroma.
Paget 's disease, fibrous dysplasia and multiple enchondromatosis are all associ
ated with an increased incidence of chondrosarcomas. This incidence is even higher
when these conditions are managed with radiation therapy.
Pathologically, chondrosarcomas are divided into three different SUbtypes. The
conventional type is the most common and has a better prognosis. The mesenchy
mal and undifferentiated types show a more aggressive clinical course and bear a
worse prognosis. Conventional chondrosarcomas are further subdivided into types
1 through 4 reflecting an increasing grade of cytologic aggressiveness (increased cel
lularity, pleomorphism and mitotic rate). A simpler and more useful classification
system divides chondroid tumors into low, intermediate and high grade neoplasms,
taking into account the difficulty in differentiating benign from malignant tumors,
both pathologically and radiologically. Also, distinction between chondrosarcomas
and chordomas, particularly chondroid chordomas, may only be possible with im
munohistochemical analysis. I n contradistinction to chordomas, chondrosarcomas
do not stain for epithelial markers such as epithelial membrane antigen and keratin.
Chondromyxoid fibroma is a rare entity, that shares the same immunohistochemical
features with chondrosarcomas. However, on light microscopy this tumor shows an
important fibroblastic component made of stellate or spindle-shaped ceLis with
scarce eosinophiLic cytoplasm, not usually seen in chondrosarcomas.
Chondrosarcomas are locally invasive neoplasms, but rarely metastasize.

Clinical Findings
Signs and symptoms of skull base chondrosarcomas are non-specific and depend on
the location, extent, and relationship of the tumor to adjacent vital structures. The
nonspecific nature of the most frequent symptoms (headache and vagueness, wax
ing and waning neurologic deficits) delays the diagnosis. The mean time between the
onset of symptoms and diagnosis is between 18 to 60 months. Occulomotor disor
ders, decreased hearing, tinnitus, vertigo and pain, and hyperesthesia in the distri
bution of the Vth cranial nerve are among the most common presenting neurologic
I CENTRAL SKULL BASE

C D

deficits in these patients. They relate to the typical location of these tumors laterally
in the mid-skull base, and usually involve the cavernous sinus, petrous apex, and
cerebello pontine angle (CPA) cistern. Whenever a patient presents with unex
plainable multiple cranial neuropathies and headache, a skull base neoplasm should
be suspected and appropriate imaging studies requested.

Imaging Findings
Imaging evaluation of a patient with a suspected skull base neoplasm should include
both CT and MRI to delineate the bony and soft tissue changes. CT is particularly
valuable in the selling of chondroid tumors as it may show calcifications and tumor
mineralization. Chondroid type calcifications are present in 60 to 80% of tumors
and include complete and partial ringlets, as well as arciform and fine, punctiform
calcifications. The degree of mineralization appears to correlate well with tumor dif
ferentiation. Low grade tumors show heavy calcification and the more anaplastic
high grade tumors show little or no calcification of the tumor matrix. Bone destruc
tion is seen in 50% of cases and most frequently involves the clivus, petro us apex,
and posterior cranial fossa. In an MRI of another patient of the orbits axial T I W
(Fig. 4-C) and coronal post-contrast frequency selective fat suppression imaging
(FATSAT) TI W (Fig. 4-0) images show a heterogeneously cnhanced mass in the
lateral wall of the right orbit (greater wing of the sphenoid bone). This expansive
mass shows well defined margins and extends medially into the lateral aspect of the
extraconal compartment displacing, but not invading, the lateral rectus muscle. Di
agnosis: Low grade chondrosarcoma of the greater wing of the sphenoid bone.
Clival chondrosarcomas tend to occur off of the midline, an important distinctive
feature from chordomas, most commonly seen in the spheno-occipital or petrociival
synchondro is. The junction between the vomer and rostrum of the sphenoid is an
other possible location for this tumor. Chondrosarcomas, although locally invasive.
may show well-defined margins, almost geographic in appearancc, but without bony
sclerosis. Most tumors show marked contrast enhancement.
On M R I , tumors are of intermediate signal intensity on TI WI and fairly hyperin
tense on TIWI . Calcifications, depending on their size and composition, may present
as hypo- or hyperintense foci on M R l or may not be visible at all. Fat-suppressed,
contrast-enhanced sequences may be helpful in determining the amount of bone

I
CENTRAL SKULL BASEl

marrow involvement. Delineation of the neoplasm and its relationship to neurovas


PEA RLS/PITFALLS cular structures is also optimally achieved with M R I .
This diagnosis should be consid D u e t o the overlap o f the imaging features with other entities, particularly chor
ered for patients presenting with domas, chondromyxoid fibroma, osteosarcomas and mineralized metastasis, correct
headache and vague neurologic diagnosis requires pathologic sampling either by image-guided or open biopsy.
symptoms referable to cranial Immediate post-operative and subsequent follow-up imaging studies are manda
nerves. tory as residual and recurrent tumors are seen in a high percentage of cases.

A CT scan will show a mid -skull Management


base lesion located off midline
with ring or arciform-like calcifi The multidisciplinary approach to skull base lesions and the advent of image guided
cations. neuronavigation systems revolutionized the treatment of these tumors, allowing for
more radical resections. However, several authors recommend more conservative
Predisposing conditions include: surgical approaches with decompressive resections aimed at relief of symptoms and
Paget's disease, fibrous dysplasia repeated surgeries if the tumor recurs. The goal of surgical treatment should be to
and multiple enchondromatosis achieve maximal tumor resection with the least possible morbidity. Surgical
syndromes. approach depends on tumor location and extent, and may require both intra- and
extra-cranial approaches.
Results from immunohistochemi Radiation has been used both pre-operatively as adjuvant therapy, and as the sole
cal analysis show that chon treatment modality. The type of radiation with the highest success rates are charged
drosarcomas do not stain for ep heavy particles and stereotactic radiosurgery. The prognosis, five year survival rate,
ithelial markers. of chondrosarcomas (95% ) is better than for chordomas (62% ).
The clinician might fail to recog
nize a chondrosarcoma in the Suggested Readings
setting of an underlying diffuse
pathologic process, such as Crockard, A. Chordomas and chondrosarcomas of the cranial base: results and
Paget's or fibrous dysplasia. follow-up of 60 patients. Neurosurgery 38(2) :420, 1 996 Feb.
Keel, S.B., Bhan, A.K., Liebsch, N.I, Rosenberg, A.E. Chondromyxoid fibroma of
Chondrosarcomas may be indis the skull base: a tumor which may be confused with chordoma and chondrosarcoma.
tinguishible from other skull A report of three cases and review of the Literature. American Journal of Surgical
base neoplasms, particularly Pathology 2 1 (5):577-82, 1 997 May.
chordoma, chondromyxoid fi Korten, A.G., ter Berg, H .I, Spincemaille, G.H . , van der Laan, R.T., Van de Wei,
broma, osteosarcoma a n d skull A.M. I ntracranial chondrosarcoma: review of the literature and report of 15 cases.
base metastasis. Journal of Neurology, Neurosurgery and Psychiatry 65 ( 1 ):88-92, 1998 Jui.
Levy, R.A., Quint, D.I, Devaney, K.O. Unusual tumours of the skull and skull base:
a pictorial essay. Canadian Association of Radiologists Journal 47(6) :434-43, 1996
Dec.
McDermott, M.W., Gutin, P.H. Image-guided surgery for skull base neoplasms using
the ISG viewing wand: Anatomic and technical considerations. Neurosurgery Clin
ics of North A merica 7(2) :285-95, 1 996 Apr.
Muthukumar, N., Kondziolka, D., Lunsford, L.D., Flickinger, I e. Stereotactic radio
surgery for chordoma and chondrosarcoma: further experiences. international Jour
nal of Radiation Oncology, Biology, Physics 4 1 (2):387-92, 1998 May.
Rapidis, A.D., Archondakis, G., Anteriotis, D., Skouteris, e.A . Chondrosarcomas of
the skull base: review of the literature and report of two cases. Journal of Cranio
Maxillo-facial Surgery 25(6):322-7, 1 997 Dec.
Weber, A.L., Brown, E.W., Hug, E.B., Liebsch, N.J. Cartilaginous tumors and chor
domas of the cranial base. Otolaryngologic Clinics of North America 28(3):453-7 1 ,
1 995 Jun.
ENTRAL SKULL BASE

Case 5
Clinical Presentation
A 69-year-old female presented with headaches and a right X l l t h cranial nerve
palsy.

A B

Radiologic Findings
An axial T I W I ( Fig. 5-A) and agillal T2WI ( Fig. 5-B) through the the skull base
show a large soft tissue mass replacing the clivus. basioccipul. anterior arch of 1
and the dens. The mass is hypointense to the brain on T I W images and hyperi ntense
22
CENTRAL SKULL BASEl

on T2W I . There is inferior extension i n to the cervical spinal canal along the a n terior
epidural space, to the level of the mid-C2 vertebral body. There is spinal cord com
pression and kinking a t the cervico-medullary junction. The axial T l WI through the
oral cavi ty (Fig. 5-C) shows denervation a trophy of the righ t hemitongue subse
quent to invasion of the right hypoglossal canal by the tumor.

Differential Diagnosis: Destructive Mass in


the Clivus
Arising rrom the skull base: chordoma, chondroid tumors (chondroma/chon
drosarcoma), chondromyxoid fibroma, osteosarcoma, glomus j ugulare, me tastasis,
plasmacytoma/mul tiple myeloma, lymphoma, eosinophilic granuloma, fibrous
dysplasia
Arising above the skull base: meningioma (clivus/planum sphenoidale/tubercu
lum sella), craniopharyngioma, pi tui tary macroadenoma
Arising below the skull base: sphenoidal mucocele, squamous cell carcinoma of
the sphenoid sinus, nasopharyngeal carcinoma, nasopharyngeal rhabdomyo
sarcoma

Diagnosis
Clival chordoma

Discussion
Epidemiology
Chordomas are relatively rare tumors of the skull base, accounting for 1 % of all
i n tracranial tumors. Fifty per cen t of chordomas arise in the sacrococcygeal region.
O ther common locations include the clivus and the spine which comprise 35% and
1 5 % of all chordomas respe.ctively. I ntracranially, the most common locations are
the basisphenoid and basiocciput (near the spheno-occipi tal synchondrosis) and the
parasellar region. Rarely, chordomas may arise in the nasopharynx or within the
paranasal sinuses.
The incidence of chordomas vary according to their location. Clival chordomas
can occur at any age but show a peak incidence in the 3rd and 4 th decades. Sacro
coccygeal chordomas tend to prese n t later in the 40- to 60-year age group. There is
a predominance among males.

Pathophysiology
Chordomas are histologically benign, locally invasive, and slow growing neoplasms
that originate from the primi tive notochord. Because the embryonic notochord
terminates a t the sphenoid bone, immediately below the sella turcica, the midline
basisphenoid is the mos t common location for this tumor. However, due to the inti
mate rel a tionships of the n o tochord with several neuroectodermal, mesodermal and
endodermal derivatives, which give rise to the diencephalon, primi tive pharynx,
Ra thke's pouch, and facial bones, o ther "ectopic" locations including intradural,
nasopharyngeal, sellar and sinonasal regions are possible. Notochordal remnants in
the nasopharynx are believed to be responsible for the developme n t of Tornwaldt
cysts. Uncommonly, these n o tochordal remnants may give rise to a chordoma in
these locations.
Histologically, the tumor is composed of translucen t cells rich in mucin and glyco
gen, con taining large in tracy toplasmic vacuoles. These physaliforous cells are usu
ally arranged in cords or nests separated by thick fibrous sep ta. Macroscopically, the
tumor forms a whi te, soft, mul ti lobula ted mass surrounded by a pseudocapsule tba t
231
I CENTRAL SKULL BASE

is composed by compressed adjacent tissue. Gelatinous mucoid fluid, necrosis, hem


orrhage and sequestered bony fragments are common and contribute to the het
erogeneous imaging appearance of these neoplasms. Most chordomas are cytologi
cally benign. The high frequency of recurrence is explained by microscopic
extensions of tumor into the adjacent tissues which, together with a difficult surgi
cal access, makes complete resection problematic.
Highly malignant forms of chordoma are also seen containing undifferentiated
areas that may be confused with fibrosarcoma. Metastasis is seen in 7 to 14% of
cases, primarily to the lymph nodes, lungs and bone.
A variant rich in chondroid material, referred to as chondroid chordoma is a con
troversial pathologic entity, believed by some to represent a low grade chondrosar
coma. I m munohistochemical stains are very helpful in differentiating chordomas
from chondrosarcomas, which may have the same appearance on imaging studies.
Whereas chordomas are positive for epithelial markers such as cytokeratin and
epithelial membrane antigens, chondrosarcomas are negative for epithelial anti
bodies, but are positive for mesenchymal markers such as vimentin and protein
S- 1 00. Confusion between chordomas and epithelial metastasis to the skull base
may occur with immunohistochemical analysis.

Clinical Findings
The presenting symptoms depend on the tumor location, extent, and relationship
between the tumor and vital skull base structures. Most patients with clival chordo
mas present with headaches and ophthalmoplegia, usually due to impingement
upon the V l t h nerve in Dorello's canal. More extensive lateral extension into the
cavenous sinus results in cavernous sinus syndrome with deficits of cranial nerves
I I I , I V, V I and V2. Posterior and inferior extension into the jugular foramen
presents with palsy of cranial nerves IX, X and XI and involvement of the hy
poglossal canal results in X l lth nerve palsy. Destruction of the petrous apex and
involvement of the CPA cistern may cause V l l t h and VI 1 1th nerve deficits. Ante
rior growth into the sella turcica may lead to compression of the pituitary gland and
hypopituitarism. Symptoms of brainstem compression and increased intracranial
pressure signal advanced disease. Destruction of the occipital condyles and exten
sion of the tumor through the foramen magnum into the upper cervical spinal canal
may result in neck pain and quadraparesis.
Subarachnoid hemorrhage is a rare form of presentation of intradural tumors.

Imaging Findings
Radiologic evaluation of skull base tumors should include both CT and MRI to de
termine the full bony and soft tissue extent. Depending on their location within the
clivus, chordomas are classified as upper, middle or lower clival chordomas. Upper
clival chordomas occur rostral to the trigeminal nerve in the region of the dorsum
sella . Middle clival chordomas occur between the origin of the Vth and IXth cranial
nerves and chordomas of the inferior clivus below the origin of the glossopharyn
geal nerve. This classification along with evaluation of involvement of critical struc
tures (cavernous sinuses, circle of Willis and brainstem ) is mandatory in determin
ing the best management. Chordomas vary in size [rom I to 10cm but most are in
the 2 to Scm range. Typically, these tumors arise in the midline clivus near the
spheno-occipital synchondrosis. They may extend laterally into the cavernous sinus
and petrous apex, anteriorly into the sphenoid sinus. superiorly into the sellar and
suprasellar regions, inferiorly into the foramen magnum and/or posteriorly into the
j ugular foramen and prepontine cistern.
a should include post contrast axial and coronal sections through the skull base
and upper cervical spine with images filmed in both soft tissue and high resolution
bone windows. Chordomas are seen as iso- or hypodense soft tissue masses with
lytic bony destruction and no significant sclerosis. Bony destruction may be limited

I
CENTRAL SKULL BASE I

D E

to the clivus or involve the petrous apex, foramen j ugulare, occipital condyles and
upper cervical vertebra. Calcifications are present in 50% of cases. These are usually
large, distinctive from the typical small ring like or punctiform calcifications seen in
chondroid tumors, and may represent sequestered bony fragments or dystrophic
calcifications. After contrast administration, these lesions may show little to moder
ate enhallcemellt with non-enhancing areas corresponding to mucoid or cystic de
generation.
On M R I , the sigllal characteristics are quite variable depending on the presence
of tumoral calcification, hemorrhage or cystic degeneration. Usually they tend to be
iso- to hypointense on T l W I alld heterogeneously hyperilltense Oil TIWI . A lobu
lated, boneycomb appearance after gadolinium administration has been described
as a characteristic feature of chordomas. M R I is the optimal modality to detect
small intraclival tumors which manifest as an area o f T I W hypointensity within the
high signal intensity of fat. However, care should be taken to avoid mistaking areas
of hematopoietic bone marrow for a clival tumor. To avoid this confusion, one
should carefully look [or loss of sigllal void of the cortical margin of the clivus or
bony erosion on CT. Axial CT sectiolls througb the clivus in another patient (Fig.
5-D and 5-E) (bone willdows) , show a destructive mass centered ill the clivus and
eroding both petrous apices alld the allterior arch of the clivus. Diagnosis: Clival
chordoma.
M R I is also the modality of choice to evaluate the relationship of the tumor to the
structure of the cavenous sinus, j ugular foramen and posterior fossa. It is particu
larly useful for identifying the presence of compression or encasement of the basi
lar or cavernous carotid arteries.
The main differential consideratioll for dival chordomas are cbondroid tumors of
the skull base. The most useful clue is the topography of the lesion. Whereas chor
domas tend to arise in the midline, chondroid tumors are usually lateral in location
arising from the spheno-occipital or petroclival synchondrosis. However, this serves
ollly as a gelleral guidline, as chondroid tumors may be midline in location and chor
domas may locate more laterally due to terminal spliting of the embryonic noto
chord.
Craniopharyngiomas, in addition to their typical cystic appearance and charac
teristic linear, peripheral calcifications, are usually centered in the sellar region.
/cENTRAL SKULL BASE

They have a predilection for postero-superior extension into the interpeduncular


PEARLSIPITFALLS cistern.
The most common location for Clival meningiomas, show a broad dural base, tend to be isointense to the brain
this destructive mass is in the on all MR sequences, enhance homogenously and exhibit a dural tail .
midline clivus. The presence o f a rim of pituitary tissue surrounding a sellar lesion suggests the
diagnosis of a pituitary macroadenoma.
A characteristic feature of chor The signal characteristics of lymphoma, iso- to hypointense on T1 WI and low sig
domas is a multilobulated, hon nal intensity on T2WI due to hypercelluarity and low water content, usually allows
eycomb appearance on post distinction from chordoma.
contrast M R I . Mucoceles of the sphenoid sinus tend to cause bony expansion and cortical thin
ing. However, late in the course of the disease cortical breaching may be indistin
Because o f i t s embryonic origins, guishible from bony erosion.
ectopic locations can include in Squamous cell carcinoma of the sphenoid sinus, plasmacytoma, metastasis and re
tradural, sellar, nasopharyngeal current nasopharyngeal carcinoma may be indistinguishible from a chordoma.
and sino nasal regions.

When diagnosing this condition, Management


the clinician might fail to evalu
ate the fulJ extent of the tumor. Despite the continuous advances in skull base surgery, most chordomas cannot be
completely resected due to their tendency for microscopic invasion and to the prox
With MR imaging, care should imity of important structures such as cranial nerves and major intracranial arteries.
be taken to avoid mistaking ar Despite this fact, surgical excision should be attempted as radiation therapy is usu
eas of hematopoietic bone mar ally not effective in decreasing the bulk of the tumor. However, radiation using high
row in the clivus for a neoplasm. dose proton beam therapy can be successful in the treatment of a residual tumor.
The choice of the best therapeutic approach is based upon many factors including
the tumor location, the clinical condition of the patient, and any history of previous
surgery or radiation. Transphenoidal surgery may be used for upper and middle cli
val tumors with only minimal or no lateral extension. Large tumors usually require
extensive craniofacial and cervical approaches with increased morbidity and mor
tality. Imaging is used, both guiding surgery for small neoplasms, and in planning ra
diosurgery for these skull base tumors. Radiological fol low-up is mandatory due to
the high rate of recurrence of these neoplasms.

Suggested Readings
Casals, M.M., Hunter, S.B., Olson, J.1., Gussack, G., Blevins, L.S. Jr. Metastatic follic
ular thyroid carcinoma masquerading as a chordoma. Thyroid 5(3):2 1 7-2 1 , 1 995
Jun.
Doucet, v., Peretti-Viton, P, Figarella-Branger, D., Manera, L., Salamon, G. MRI of
intracranial chordomas. Extent of tumour and contrast enhancement: criteria for
differential diagnosis. Neuroradiology 39(8):571-6, 1 997 Aug.
Kaufman, B.A., Francel, pc., Roberts, R.L., Argemand, E., Park, TS., Dehner, L.P.
Chondroid chordoma of the lateral skull base. Pediatric Neurosurgery 23(3): 1 59-65,
1 995.
Keel, S.B., Bhan, A . K . , Liebsch, N.J., Rosenberg, A.E. Chondromyxoid fibroma of
the skull base: a tumor which may be confused with chordoma and chondrosarcoma.
A report of three cases and review of the literature. American lournal of Surgical
Pathology 2 1 (5):577-82, 1997 May.
Maira, G., Pallini, R., Anile, c., Fernandez, E., Salvi nelli, F., La Rocca, L.M., Rossi,
G.F. Surgical treatment of clival chordomas: the transsphenoidal approach revisited.
lournal of Neurosurgery 85(5):784-92, 1 996 Nov.
Tashiro, T , Fukuda, T, Inoue, Y, Nemoto, Y, Shakudo, M., Katsuyama, 1., Hakuba,
A., Onoyama, Y Intradural chordoma: case report and review of the literature. Neu
roradiology 36(4):31 3-5, 1994 May.
Weber, A.I., Brown, E.W., Hug, E . B., Liebsch, N.J. Cartilaginous tumors and chor
domas of the cranial base. OlOlaryngologic Clinics of Norrh America 28(3) :453-7 1 ,
1995 Jun.
CENTRAL SKULL BA EI

Case 6
Clinical Presentation
A 25-year-old male presented with headache, hoarseness, nasal regurgitation, and
difficulty swal lowi.ng.

A B

27 1
I CENTRAL SKULL BASE

Radiologic Findings
In an M R I of the skull base, axial T l W I , and coronal post-contrast T l WI show a de
structive mass in the posterior skull base centered at the right jugular foramen. This
enhancing mass invades the inferior aspect of the clivus, the right occipital condyle,
anterior arch of C l , and the right hypoglossal canal. The tumor extends medially
and posteriorly behind the right longus colli muscle and appears to transgress the
pre-vertebral fascia (Fig. 6-A) . The axial T l W image through the oral cavity shows
denervation atrophy of the right hemitongue and rightward deviation of the uvula
(Fig. 6-B). The coronal T l W image through the neck demonstrates fatty replace
ment and flacidity of the right lateral wall of the pharynx, con istent with involve
ment of cranial nerves IX and X (Fig. 6-C).

Differential Diagnosis: Destructive Lesion of


the Posterior Skull Base
Neoplasm:
Malignant:
Primary: chondrosarcoma, osteosarcoma, lymphoma, chordoma (midline )
Secondary: plasmacytoma, chloroma, metastasis
Benign: schwan noma of the lower cranial nerves, meningioma, chondroma, giant
cell tumor, brown tumor, hemangioma, eosinophilic granuloma, glomus jugulare
I n fectionlInflammation: osteomyelitis, radiation necrosis

Diagnosis
Choriocarcinoma metastatic to the skull base

Discussion
Epidemiology
Although relatively uncommon, metastasis should always be considered in the dif
ferential diagnosis of a central skull base mass. There is a peak incidence in the 5th
and 6th decades, but they may be seen in any age group. In the pediatric population
primary considerations include metastatic neuroblastoma, rhabdomyosarcoma.
choriocarcinoma, lymphoma, and chloroma. [ n adulthood the major considerations
are prostate, lung and breast carcinoma, and melanoma. Renal cell, thyroid and
gastro-intestinal tract neoplasms should also be considered. Skull base metastasis
usually occurs in the setting of a known malignancy but occasionally is the first sign
of an occult neoplasm. I n the latter circumstance differential diagnosis with primary
skull base tumors may be difficult. There is no gender predilection.

Pathophysiology
Metastatic disease to the skull base may occur via three different mechanisms.

( 1 ) The most common is direct extension from neoplasms arising in the head and
neck region, particularly nasopharyngeal, palatal. parapharyngeal, sphenoid
and temporal bone malignancies. Among these, squamous cell carcinoma of the
nasopharynx is the most frequent tumor presenting with invasion of the skull
base. Limitation of lateral spread by the pharyngobasilar fascia directs tumor
growth superiorly and posteriorly in the direction of the clivus and cavernous
sinuses. The tumor may also reach the central skull base through the Eustachian
tube.

1 28
CENTRAL SKULL BASEl

(2) The second most common mechanism is via perineural spread from head and
neck tumors, most frequently through branches of the second division of the
trigeminal nerve. Neoplasms particularly prone to this type of spread include
adenoid cystic carcinoma, squamous cell carcinoma, mucoepidermoid carci
noma, lymphoma, and melanoma.
(3) Finally, metastasis to the skull base may occur via hematogenous dissemination
from distant sites.

Direct and retrograde perineural spread of tumor to the skull base and intra
cranial compartment is a consequence of skull base anatomy and the multiple
foramina which allow for communication between the intra- and extracranial
compartments. Most head and neck spaces communicate freely with the intracranial
compartment t hrough one or more foramina. The main common pathway for in
tracranial spread of head and neck neoplasms is the pterygopalatine fossa, a
space limited anteriorly by the posterior wall of the maxillary antrum, bordered
posteriorly by the pterygoid plates and containing branches of the second division
of the trigeminal nerve, the pterygopalatine ganglion, vessels, and fat. Superiorly,
this space communicates with the inferior orbital fissure, medially with the posterior
nasal cavity through the sphenopalatine foramen, laterally with the infratemporal
fossa (masticator space) through the pterygomaxiUary fissure and inferiorly, with
the oral cavity through the pterygopalatine canals. Posteriorly, it communicates
with the central skull base via the vidian canal and foramen rotundum. The fora
men ovale opens into the masticator space and constitutes the second most com
mon site of perineural spread, along the third division of the trigeminal nerve.
Orbital malignancies may access the skull base through the orbital apex or orbital
fissures.
Perineural spread of parotid gland malignancies along the intra temporal facial
nerve is not uncommon and should always be sought. Metastatic disease to the pos
terior fossa may involve the jugular foramen and be confused with paragangliomas
or schwannomas, the most common tumors seen in this region.

Clinical Findings
Clinical presentation depends on the exact location of the lesion and on the skull
base structures involved. Usually symptoms include headaches and characteristic
neurologic syndromes. These syndromes were classified by Posner and Greenberg
into five different categories including orbital, cavernous/parasellar, middle cranial
fossa (Meckel's cave syndrome), jugular foramen, and occipital condyle syndrome.
Metastasis of the central skull base tends to involve the cavernous sinus and
Meckel's cave whereas metastasis to the posterior skull base usually involves the
jugular foramen and hypoglossal canal . The presence of one of these syndromes
should alert the clinician to the presence of a skull base lesion and prompt appro
priate imaging evaluation.

Imaging Findings
The central skull base, due to its anatomic complexity, is best evaluated by dedicated
cross-sectional studies allowing detection of subtle lesions. CT should include con
trast enhanced 3mm thick sections in axial and coronal planes, through the skull
base or helical aqusition in the axial plane, followed by coronal reformations. Skull
base M R I studies should include multiplanar thin sections including axial Tl W,
TIW, and axial and coronal frequency selective fat suppression imaging (FATSAT)
post contrast T l W images. These modalities are complimentary and should be used
in combination. I n another patient presenting with headache and hearing loss, an
ICE

F G

M R [ of the kull base. axial pre and post-contrast T l W image ( Fig. 6-0) and ( Fig.
6-E ) through the clivus show a large enhancing soft ti ue ma s replacing the the cli
val bone marrow and invading the petrou and ma toid portions of the right tem
poral bone. Diagno is: Skull base pia macytoma (courtesy of Dr. J.P. Wensel ) . [ n
another patient a n M R I o f the kull base, agiltal T l W (Fig. 6-F) and axial post con
trast T l W ( Fig. 6-G ) image show an enhancing mass in the left lateral aspect of the
sellar and supraseUar regions invading the left cavernou inus. This patient pre
sented with cavernou inus syndrome. Diagno is: Metastasi from brea t carci
noma.
CT may show subtle foraminal enlargement and ero ion of the cortical bone.
Detection of intratumoral calcification and recognition of specific pallerns of calci
fication may sugge t certain pathologic diagno e uch as chondroid tumors,
o teosarcoma, Paget's di ease, fibrou dyspla ia or 0 teobla tic meta ta is. TIle pre -
ence of bony remodelisg or sclerosis suggests indolent tumors such as meni ngiomas.
the Illost frequent benign tumor of the skull base.

/ 30
CENTRAL SKULL BASEl

M R I is very sensitive to bone marrow replacement and early perineural spread of


PEARLS/PITFALLS tumor, even before foraminal enlargement can be appreciated. Focal, segmental or
The presence of a headache asso patchy areas of enhancement along the course of a cranial nerve, replacement of the
ciated with a typical skull base fat hyperintensity near the foraminal openings and loss of TIWI hyperintensity
cranial nerve syndrome should within Meckel's cave are the typical M R I features of perineural spread of tumor.
prompt a search for a skull base Clival bone marrow replacement is usually easy to detect on the non-contrast Tl W
lesion. images as areas of loss of fatty signal intensity. However, incomplete replacement of
the hematopoietic bone marrow of the clivus is seen in 15% of the adult population
Metastasis to the skull base may and may mimic neoplastic infiltration.
be the first sign of an occult Highly vascular metastases such as renal cell carcinoma, choriocarcinoma,
malignancy. melanoma and thyroid carcinoma may show a "salt and pepper" appearance and
when located in the region of the jugular foramen are indistinguishible from para
cr and M R I should be used i n gangliomas. Melanoma metastases may show typical hyperintensity on Tl W I due to
conjunction for better assess the paramagnetic effect of melanin. However, this finding is non-specific and may be
ment of tumor extension. seen in hemorrhagic metastases, metastases containing mucinous material or cystic
metastases from thyroid malignancies which may have a high protein content.
It is possible to mistake metas Chondroid tumors tend to be heterogeneous in signal characteristics due to the
tases for primary tumors of the
presence of chondroid calcifications and tend to occur in a lateral location centered
skull base. upon the spheno-occipital or petro-clival synchondroses. The single most specific
feature of clival chordomas is their location in the midline. Nuclear medicine
The clinician may fail to recog
SPECT studies using 99mTc may be performed in symptomatic patients with
nize perineural spread of the tu
known malignancies, when cross-sectional imaging studies are negative.
mor.

It is possible to mistake a highly Management


vascular metastasis in the region
of the jugular foramen for glo Metastatic disease of the skull base is usually managed according to the cell type of
mus jugulare. the primary tumor. Radiation and/or chemotherapy may be used. Surgical resection
may be considered in individual cases, particularly those with occult primaries. The
surgical approach and completeness of resection depends on the location of the le
sion.

Suggested Readings
Ampil, FL., Hardin, IC, Peskind, S.P. , Stucker, FI Perineural invasion in skin can
cer of the head and neck: a review of nine cases. Journal of Oral and Maxillofacial
Surgery 53( 1 ):34-8, 1 995 Jan.
Jansen, B.P., Pillay, M., de Bruin, H . G., van den Bent, M.J., Vecht, CJ., Sillevis Smitt,
P. A . 99mTc-SPECT in the diagnosis of skull base metastasis. Neurology
48(5) : 1326-30, 1 997 May.
Ng, S.H., Chang, T.C, Ko, S.E . , Yen, P.S., Wan , YL., Tang, L.M., Tsai, M . H .
Nasopharyngeal carcinoma: M R I and CT assessment. Neuroradiology 39(10):741-6,
1 997 Oct.
Sakata, K, Aoki, Y, Karasawa, K , Hasezawa, K , Muta, N., Nakagawa, K.,
Terahara, A., Onogi, Y, Sasaki, Y, Akanuma, A . Wide variation of probability of lo
cal failure and distant metastasis among various stages of patients with nasopha
ryngeal carcinoma. Slrahlenlherapie und Onkologie 1 70(4):2 1 8-24, 1 994 Apr.
Sutter, CW., Masilungan, B.G., Shelton, D.K. An unusual first impression of skull
base metastasis. "The sunglasses illusion". Clinical Nuclear Medicine 2 1 (4):322, 1 996
Apr.

. l
II.

Larynx and Hypopharynx


LARYNX AND H Y POPHARYNXI

Case 7
Clinical Presentation
Adult male presented with pharyngitis and dysphagia.

Radiologic Findings
The lateral radiograph shows abnormal thickening of the epiglottis aryepiglottic
folds, and arytenoids (Fig. 7-A). There is no evidence of radio-opaque foreign body
or interstitial air.

Differential D iagnosis: Epiglottitis,


Supraglottitis
Squamous ceU carcinoma
Supraglottitis/epiglottitis
Atypical laryngocele
Post-traumatic deformity

Diagnosis
Epiglottitis, supraglottitis
I LARYNX AND H YPOPHA RYNX

Discussion
Epiglottitis, or as it is increasingly known, supraglottitis, is an inflammation of the
supraglottic larynx.

Epidemiology
The pediatric form is due to Haemophilus influenzae virus infection and occurs in
slightly older children. Its incidence in children is decreasing as childhood vaccina
tions are increasing.
The disease in adults is relatively uncommon but may be life-threatening. The
death of George Washington is believed by many authors to represent an early
recorded case of t he disease. Unlike the decreasing incidence of the pediatric form,
the incidence of the acute adult form remains stable. Only a minority of adult cases
are caused by H. influenza. More common pathogens are the staphylococcus and
streptococcus groups.
U nlike epiglottitis, croup manifests as a swelling of the subglottic larynx. trachea,
and bronchi due to type I parainfluenza virus or similar organisms. Patients with
croup are typically children 6 months to three years of age. Clinically. a characteris
tic barking cough and stridor are present.
The subglottic edema results in the signature radiographic appearance of the loss
of the normal shoulder shape of the subglottic angle. I nstead there is a church
steeple or wine-bottle appearance of this area on the frontal radiograph or coronal
vIew.

Clinical Fi ndings
Supraglottitis patients present with sore throat, dysphagia, drooling (due to failure
10 control secretions), and oflen, airway compromise. On examination, the supra
glottic larynx is swollen, often with a cherry red appearance. The radiographic find
ings of enlarged epiglottis on the lateral view are due to edema of this and other
supraglottic laryngeal structures. Close supervision of the patient with epiglottitis is

B
L A RYNX A N D H YPOPHARYNXI

required because acute airway closure may occur, and intubation or emergency tra
PEARLSIPITFALLS cheostomy may be necessary.
The epiglottis and supraglottis
can appear thickened on CT or Imaging Findings
MR imaging.
The classic appearance on the lateral radiograph is that of a thickened "thumb-like"
The organism responsible for pe epiglottis (Fig. 7-A). Normally, its appearance is a thin "shoe-horn" shape (Fig. 7-B).
diatric epiglottis is usually Generalized edema of the supraglottic larynx is another finding.
Haemophilus influenza. Although measurements are not necessary to make the diagnosis, some detailed
studies have been performed. For example, the ratio of the soft-tissue parameters to
The organism responsible for the anteroposterior width of the C-4 vertebral body yields three key parameters re
epiglottis in adults is usually a ported to be of high statistical significance in the diagnosis of adult epiglottitis.
member of the staphylococcus or
streptococcus groups. ( 1 ) The ratio of the width of the epiglottis to the anteroposterior width of C-4
should not be greater than 0.33 (sensitivity, 96% ; specificity, 100% ) .
Cross sectional imaging will help
(2 ) The ratio of the prevertebral soft tissue to C-4 should not exceed 0.5 (sensitiv
rule out complications.
ity, 37% ; specificity, 100% ) .
(3) The ratio o f the width of the hypopharyngeal airway to the width o f C-4 should
Epiglottitis is often a missed di
agnosis in patients with impend be less than 1 .5 (sensitivity, 44% ; specificity, 87% ).
ing acute airway compromise.
Because the symptoms may be nonspecific, in some cases CT or MRT may be use
ful to exclude other conditions with similar symptoms such as laryngitis, foreign
body, peritonsillar abscess, and tonsillitis. The most common CT/MRI findings of
epiglottitis are thickening of the epiglottis, aryepiglottic folds, false and true vocal
cords, obliteration of the preepiglottic fat, thickening of the platysma muscle, and
reticulation of the subcutaneous fat. Multiple loculated fluid-density collections
consistent with abscesses can be see!1. Although the diagnosis of supraglottitis is
generally made on the basis of the patient'S history, direct endoscopy, and plain
films, CT/MRJ may be used to confirm the diagnosis when an adequate clinical ex
amination cannot be performed. CompLications such as deep neck abscess also may
be identified easily with CT or M R I . Examinations requiring the patient to lie
supine should be performed with caution as this position can precipitate acute air
way closure.

Suggested Readings
Barrow, H.N., Vastola, A.P., Wang, R.c. Adult supraglottitis. OlOlaryngology and
Head and Neck Surgery 1 09(3 Pt 1 ):474-7, 1 993 Sep.
Dawson, K.P., Steinberg, A . , Capaldi, N. The lateral radiograph of neck in laryngo
tracheo-bronchitis (croup). Journal of QualilY in Clinical Praclice 1 4( 1 ):39-43, 1 994
Mar.
Frantz, T.D., Rasgon, B.M. Acute epiglottitis: changing epidemiologic patterns. 010-
laryngology and Head and Neck Surgery 109(3 Pt 1 ):457-60, 1 993 Sep.
John, S.D., Swischuk, L.E., Hayden, c.K. Jr., Freeman, D.H., Ir. Aryepiglottic fold
width in patients with epiglottitis: where should measurements be obtained? Radi
ology 190( 1 ): 1 23-5, 1 994 Jan.
Kass, E.G., McFadden, E.A., Jacobson, S., Toohill, R.l Acute epiglottitis in the adult:
experience with a seasonal presentation. Laryngoscope 103(8):84 1-4, 1993 Aug.
Nemzek, WR., Katzberg, R.W., Van Slyke, M.A., Bickley, r.S. A reappraisal of the ra
diologic findings of acute inflammation of the epiglottis and supraglottic structures
in adults. American fournal of Neuroradiology 1 6(3):495-502, 1 995 Mar.
Smith, M.M., Mukherji, S.K., Thompson, lE. Castillo, M. CT in adult supraglottitis.
American Journal of Neuroradiology, 1 7(7) : 1 355-8, 1 996 Aug.

I
I LARY X A N D H Y POPHA RYNX

Case 8
Clinical Presentation
A 77-year-old male presented with hoarsene s. On physical examination there was
a bulge in the raise cord level ( Fig. 8-A).

38
LARYNX AND H Y POPHARYNXI

Radiologic Findings
On a T l -weighted image (Fig. 8-B), there is an oval, sharply marginated abnormal
ity in the false cord obliterating the paralaryngeal fat. The mucosa can be seen along
the medial margin, indicating that this is in a submucosal position. ( Image courtesy
of Dr. Hugh D. Curtin.)

Differential Diagnosis: Paralaryngeal Cyst


Laryngocele
Congenital laryngeal cyst
Cyst of the epiglottis
Branchial cleft cyst
Neoplasm with necrosis simulating cyst
Thyroglossal duct cyst

Diagnosis
I n ternal laryngocele

Discussion
Epidemiology
Laryngoceles are usually unilateral, although they may be bilateral in 25% of cases.
Laryngoceles commonly present in adulthood and many are asymptomatic.

c
I LARYNX AND HYPOPHA RYNX

Pathophysiology
Laryngoceles are formed by a dilation of the small saccule (appendix) of the laryn
geal ventricle. The laryngeal saccule is a small diverticulum that passes superiorly
from the ventricle of the larynx. In so doing, it passes into the paraglottic space at
the level of the false cord. Some experts use the term saccular cyst if it is fluid filled
and laryngocele if it is air filled. For this discussion. either form will be referred to
as a laryngocele.
Sometimes this appendix expands superiorly in the paralaryngeal space, medial to
the thyroid cartilage, where it is known as an internal laryngocele and can present
as a submucosal supraglottic space mass. Figure 8-C shows a line drawing of a coro
nal view of the larynx with internal (right) and mixed/external ( left) laryngocele. An
axial Tl W image with contrast (Fig. 8-0 ) in another patient shows no enhancement
of a cystic paralaryngeal mass. Lateral displacement of the thyroid lamina can
occur.
Alternatively, if the dilated saccule extends through the thyrohyoid membrane
out into the soft tissues of the neck, it is a mixed (external) laryngocele. This form
may present as a soft tissue neck mass.
An unobstructed laryngocele is filled with air. When obstructed. high protein fluid
accumulates within the dilated appendix. H the obstructed fluid becomes infected. it
is referred to as a pyolaryngocele.
Sometimes a small lesion near the neck of the saccule causes the obstruction and
subsequent formation of a laryngocele. Laryngoceles may be associated with
chronic granulomatous disease, such as tuberculosis.
Laryngoceles are benign entities. However, they can result from a carcinoma of
the true cord or ventricle. The laryngeal saccule is normally collapsed. but if there is
a tumor obstructing its outflow into the ventricle, it can dilate, forming a laryngocele
or saccular cyst.
The differential diagnosis of a submucosal mass in the larynx should include var
ious benign and malignant lesions. The imaging appearances of these otlier entities.
however, are usually distinct from a typical laryngocele. Other cysts of the larynx
include congenital laryngeal cysts, which present in newborns as smooth. mucosal
LARYNX AND HYPOPHA RYNXI

covered, rounded masses of the false cords and aryepiglottic folds. Congenital cysts
PEARLS/PITFALLS in infants are similar to laryngoceles in adults in that both entities are lined with res
A laryngocele may be internal or piratory epithelium. They differ in that, unlike larygnoceles, congenital cysts do not
mixed (external), and it may be have a connection with the laryngeal lumen.
filled witb fluid or air. Cysts of the epiglottis are similar to congenital in that they do not communicate
with the laryngeal lumen but they are similar to laryngoceles in that tbey typically
Laryngoceles may be seen in occur in adults. All tbree of the above lesions may be associated with airway com
combination witb small glottic promise.
cancer.

The clinician must beware not to I m aging Findings


mistake tbe incidental laryngo In imaging studies, a laryngocele is a rounded well-circumscribed soft tissue mass or
cele for tumor extension in a air collection that extends superiorly from the laryngeal ventricle and false cord into
patient with known laryngeal the paralaryngeal space (internal laryngocele) or lateral to the thyrohyoid mem
cancer. brane (rnixed/external laryngocele). In mixed laryngoceles, both internal and exter
nal cystic features can be identified. Figure 8-E shows a coronal reformatted CT
In a patient with known laryngo
scan of a patient with a rnixed/external laryngocele. The signal intensity of the fluid
cele, it is important to determine
will depend on the protein content.
whether it is a small cancer tbat
A typical M R appearance depends on wbether the laryngocele is filled with air or
may be causing the laryngocele.
fluid. A signal void indicates that it is air filled whereas the intermediate signal is
An extemal laryngocele may be consistent with a saccular cyst. I n either situation the region of the ventricle should
mistaken for a branchial cleft be carefully examined to exclude a tumor. I ntravenous contrast may be helpful in
cyst or thyroglossal duct cyst. order to belp define the cystic nature of the lesion (Fig. 8-E).

Management
lnternal laryngoceles may be aspirated, incised, drained, and marsupialized. Exter
nal/mixed laryngoceles are often treated with an external surgical approach follow
ing any internal components for removal.

Suggested Readings
Curtin, H.D. Imaging of the larynx: current concepts. Radiology 1 73: 1-1 1 , 1 989.
Teresi, L.M., Lufkin, R.B., Hanafee, W.N. Magnetic resonance imaging of the larynx.
Radiologic Clinics of North A merica 27:393-406, 1 989.
ILARY

Case 9
Clinical Presentation
A 47-year-old female presented with per istent hoarseness (Fig. 9-A).

A B

Radiologic Findings
The left true vocal cord is partially abducted and appears slightly inferior to the
other cord on the axial image ( Fig. 9-B). There is asymmetry on a higher section due
42
LARYNX AND H Y POPHARYNXI

to medial positioning of the left aryepiglottic fold and resultant widening of the left
pyriform sinus (Fig. 9-C).

Differential Diagnosis: Vocal Cord Paralysis


Old trauma
Arytenoid dislocation
Laryngocele
Carcinoma

Diagnosis
Vocal cord paralysis

Discussion
The recurrent laryngeal nerve innervates all the intrinsic muscles of the larynx.
Paresis or palsy of this nerve usually results in a characteristic clinical and radi
ographic appearance of the larynx as well as hoarseness or weak voice in the pa
tient. Because of the long course of the left recurrent nerve around the ductus arte
riosus before its ascent up the tracheoesophageal groove to the vocal cord, it is
particularly susceptible to injury. Thus, vocal cord paresis may result from insult to
tbe brainstem, skull base, neck, or mediastinum.

D
I LARYNX AND HYPOPHARYNX

Pathophysiology
The vagus nerve exits the skullbase via the pars nervosa of the jugular foramen and
travels inferiorly j ust posterior to the carotid artery and jugular vein. Due to differ
ences in vascular embryology between the two sides, the recurrent nerve loops
around the subclavian artery on the right and under the aortic arch ductus arterio
sus on the left (Fig. 9-0, after Jacobs et al.). ll1Us, imaging studies for right sided
disease cover only to the level of the subclavian artery while evaluation of the left
recurrent nerve must include sections all the way down to the aortic arch. Both
nerves then ascend in the tracheoesophageal groove to the larynx.
As with denervation elsewhere in the body, there is a subsequent laxity of the
muscle groups following nerve injury. In the larynx, the most prominent muscle
group is the thyroarytenoid muscle, which includes the vocalis muscle. Denervation
results in medial rotation of the vocal process of the arytenoid cartilage and a para
median position of the muscle, which the inexperienced observer on radiographic
studies may mistake for a small vocal cord mass.
With paresis. in addition to the arytenoid cartilage and vocalis muscle rotating
into the midline in the adduction position, the structures also sag downward. This
causes ipsilateral widening of the laryngeal ventricle, which may be visible on coro
nal MRT imaging. Because the aryepiglottic fold is attached to the arytenoid carti
lage, this downward displacement also results in ipsilateral widening of the piriform
sinus. This is also visible on imaging studies. These findings strongly suggest recur
rent nerve paralysis and help distinguish this entity from other causes of vocal cord
fullness, which do not have these features.
Although nerve dysfunction may be related to tumors or vascular conditions, or
may be idiopathic, the vast majority of vocal cord paralysis is related to trauma dur
ing neck or thyroid surgery.
The superior laryngeal nerve exits the vagus nerve and innervates the cricothy
roid muscle, providing sensory innervation of the larynx. It is usually not possible to
diagnose paralysis from the radiological findings; however, due to subtle asymmetry
of the larynx, the diagnosis may sometimes be made clinically with unilateral in
volvemenL l maging plays a role in this case to evaluate the course of the nerve from
the larynx to the skullbase to exclude pathology. With symmetrical bilateral in
volvement, clinical or radiologic diagnosis is usually not possible. ll1is usually causes
adduction of the vocal cords and airway compromise.

Imaging Findings
Vocal cord thickening is a nonspecific finding. It does not necessarily imply tumor
or edema and may be secondary to paresis. The paramedian location of the true
vocal cord and the anteromedial rotation of the arytenoid process occurs due to
denervation of the cricothyroid muscle (which is a tensor of the true vocal cord).
The presence of ipsilateral piriform sinus and ventricle widening visible on imaging
studies helps to differentiate vocal cord fullness due to paresis from other vocal cord
masses.
With long-standing denervation, there may be atrophy as well as subtle ratty re
placement visible as increased signal on M R T l weighted images. ll1ere also may be
increased signal intensity on T2W images unrelated to fatty replacement. which may
be a change in water content related to loss of innervation.
The value of radiology in this condition is twofold. First, the astute radiologist
may make the diagnosis in the situation when the clinical picture is unclear and the
imaging findings are characteristic. Second, once the correct diagnosis of laryngeal
paralysis has been made, it is incumbent upon the radiologist to evalaute the entire
course of the recurrent nerves from the brainstem to mediastinum to look for pos
sible causes of dysfunction. Because of the course of the recurrent nerve, scans must
cover down to the aortic arch if the paralysis is left-sided, whereas it is only neces
sary to go down to the brachial artery if the paralysis is right-sided.
L A RY N X AND H Y POPHARYNXI

PEARLS/PITFALLS Suggested Readings


Radiologic findings include ipsi Decker, G.A.G., du Plessis, 0.J. Lee McGregor's synopsis of surgical anatomy. 12th
lateral widening of the piriform ed. Bristol: John Wright and Sons Ltd., 1986: 372.
sinus and ventricle. Later, atro Gacek, M . , Gacek, RR Cricoarytenoid joint mobility after chronic vocal cord
phy and fatty replacement are paralysis. Laryngoscope 106( 12 Pt 1 ) : 1 528-30, 1996 Dec.
denoted by increased 1'2 signal. Jacobs, c.J., Harnsberger, H . R , Lufkin, RB. Vagal neuropathy: evaluation with CT
and M R imaging. Radiology ] 64:97-1 02, 1987.
Scans must go down to the level
of brachial artery if the lesion is Tanaka, S., Hirano, M., Umeno, H . Laryngeal behavior in unilateral superior laryn
right-sided, and to the level of geal nerve paralysis. A nnals of Otology, Rhinology and Laryngology 103(2):
the aortic arch if the lesion is 93-7, 1 994 Feb.
left-sided.

The clinician must take care not


to mistake paralysis for vocal
cord mass.

It is important to evaluate the


entire course of the recurrent
nerve.
I LARY X AND H Y POPHARYNX

Case 10
Clinical Presentation
A 6 1 -year-old male complained of having weakness in his voice for the pa t everal
weeks. The patient denied dysphagia and hoar ene but did describe occasional
pain in the laryngeal area when speaking. He did not drink or smoke and could re
call no trauma to hi neck. Examination of the patient's head and neck revealed no
external neck masses and no gro pathology of tbe oral cavity, nasopharynx,
oropharynx, or nose. Vi ualization of the larynx showed a submucosal mass in the
region of the left false vocal cord ( Fig. l O-A).

A B

I
LARYNX A D HYPOPHARY xl

Radiologic Findings
A cr scan at the level of the false vocal cords shows the laryngeal "rna s" to be due
to a deformity of the underlying left thyroid cartilage (Fig. 1 0-B). 0 other soft ti -
sue rna es were noted. MR imaging at the same level also demon trates the " mas "
created by the laryngeal cartilage deformity (Fig. 1 0- ). The cr and MRI scans of
the larynx readily identify the cau e of the rna s to be due to di tortion and devia
tion of the left thyroid cartilage at the level of the false vocal cord . 0 associated
oft tissue mass or obliteration of fa cial plane is pre ent.

Differential Diagnosis: Abnormal Shape of


the Laryngeal Cartilage
Abnormal shape of laryngeal cartilage
hronic po ttraumatic deformity
Po tsurgical changes
Acute laryngeal trauma
hondroid neopla m of the larynx
Metastasi to the cartilage
ongenital deformity

Diagnosis
hronic posttraumatic deformity of the larynx

Discussion
Clinical Findings
Unlike acute laryngeal injury where the history of trauma is usually the presenting
finding. patient with chronic post-traumatic laryngeal deformities may present with
a variety of vague symptom and may deny any history of trauma.
Patient with chronic posttraumatic laryngeal deformitie may present to the clin
ician with a ymptomatic, muco ai-covered laryngeal "masses " long after the initial

47 1
I LARYNX AND HYPOPHARYNX

(and often forgotten) trauma. Hanson, et al ( 1 982) included six factors in their
symptom complex of old laryngeal injury: ( 1 ) voice fatigue, (2) sensation of food
"sticking" in the throat, (3) soreness in the side of the neck, (4) submucosal mass in
the supraglottic larynx, and (5) CT or (6) M R I evidence of trauma.

Imaging Findings
Posttraumatic deformity of the laryngeal cartilages can now be diagnosed by using
current CT and MR scanning techniques. This is of particular aid to the clinician
who is evaluating a laryngeal mass with no history of injury to the neck. CT and MR
imaging are both effective in this situation, and although M R imaging allows better
soft tissue contrast resolution and can produce coronal and sagittal views, CT is
probably superior for visualization of the ossified cartilage and associated changes.
The chronic findings of trauma of the larynx are soft tissue abnormalities due to
scarring and granulation and post-traumatic deformity of the laryngeal skeleton. I t
i s important t o recognize the appearance o f these chronic structural changes o n the
imaging studies as tbey may occur in individuals who do not recall the initial
trauma. Correct diagnosis may avoid an unnecessary biopsy or even more invasive
procedure.
Figure l O-D shows an M R I Axial T l W image in another patient with deformity
of the thyroid cartilage presumably due to prior trauma who presented with hoarse
ness and a "mass" on clinical examination. CT images (Fig. I O- E ) are shown for a
third patient with post-traumatic deformity of the thyroid cartilage causing a
pseudomass on the right.

Man agement
These patients usually need no specific management for the laryngeal deformity. If
there is airway compromise or voice changes, specific therapy may be necessary.
L A RYNX A N D HYPOPHARYNXI

PEARLSIPITFALLS References
Posttraumatic deformity of the Bent, IP. 3rd, Porubsky, E.S. 111 e management of blunt fractures of the thyroid car
larynx often presents as a submu tilage. Otolaryngology and Head and Neck Surgery 1 1 0(2): 1 95-202, 1994 Feb.
cosal "mass." ehui, L., Lufkin, R., Hanafee, W. The use of M R I in the identification of post
traumatic laryngeal deformities. Clinical Imaging 1 4 : 1 27-130, 1990.
Voice fatigue, a sensation of food
H a nson, D.G., Mancuso, A.A., Hanafee, W.N. Pseudomass lesions due to occult
sticking i n the throat, neck sore
ness, a submucosal mass in the trauma of the larynx. Laryngoscope 92:1 249-1 253, 1 982.
supraglottic larynx, and cr or
M R evidence are the derming
characteristics of posttraumatic
deformity of the cartilage.

Many patients do not recall any


history of laryngeal trauma.

If care is not taken, posttrau


matic deformity can be mistaken
for neoplasm or other pathology.
I LARYNX AND H Y PO PHARYNX

Case 11
Clinical Presentation
A 69-year-old male pre ented with right neck fullness and right otalgia.

B c

Radiologic Findings
Sagillal T l W (Fig. l 1 -A). axial T l W (Fig. I I -B), and axial TIW ( Fig. l l -C) MR
images through the supraglollic larynx show a I .S-cm rounded. well-defined cystic
50
LARYNX AND HYPOPHARYNXI

lesion in the left vallecula, displacing the the epiglottis posteriorly. This lesion is
homogeneously hypointense on Tl W images and hyperintense on T2W images and
does not enhance. There is slight impingement the tip of the epiglottis, which is dis
placed posteriorly. The glosso-epiglottic fold, base of the tongue, piriform sinuses,
and remainder of the larynx are unremarkable (Figs. l l -A-l l -C).

Differential D iagnosis: Vallecular Mass


Cystic: vallecular cyst; cyst in the base of the tongue; dermoid/teratoma; lym
phangioma/cystic hygroma; laryngocele
Solid: ectopic thyroid; hemangioma; squamous cell carcinoma

Diagnosis
Vallecular cyst

Discussion
Epidemiology
Vallecular cysts are lesions rarely seen in adulthood. In the pediatric age group,
supraglottic cysts at the tongue base and vallecula comprise the majority of laryn
geal cysts. Although anatomically the vallecula is not part of the larynx, pathologi
cally it may be considered as such. The age of presentation is variable but usually
vallecular cysts present in infants. There is no gender predominance.

Pathophysiology
Vallecular cysts are retention cysts that arise secondary to ductal obstruction of
mucous glands. Histologically, they are true epithelial-lined cysts. An association
with laryngomalacia, probably secondary to extrinsic compression by the cyst, has
been described. The condition consists of a redundant epiglottis with prolapse into
the laryngeal vestibule during inspiration. Removal of the cyst usually leads to res
olution.

Clinical Findings
Infants present clinically with feeding difficulty, failure to thrive, stridor or respira
tory distress. Early diagnosis and a high index of suspicion are required as these
lesions may cause life-threatening airway obstruction.
Adults may present with difficulty swallowing, fullness in the throat, referred ear
pain or stridor when the lesion is large and impinges on the laryngeal vestibule. On
direct laryngoscopy, these lesions manifest as a submucosal fullness, obliterating the
vallecula, but may be missed if the valleculae are overlooked. D ifferential diagnosis
from solid masses may not be possible on clinical exam and imaging studies are es
sential in determining the exact location, extent and nature of the lesion.

Imaging Findings
Lateral projection radiographs of the soft tissues of the neck are useful i n children
presenting with stridor. Supraglottic cysts manifest as an abnormal soft tissue
density, posterior to the tongue base that obliterates the vallecula and displaces the
epiglottis posteriorly.
A barium esophagram may be helpful and will show a filling defect in the vallec
ula and mass effect upon the supraglottic airway.
Sectional CT or M R imaging is the method of choice to evaluate the site of ori
gin, extent and nature of the lesion. On CT, a vallecular cyst manifests as a fluid
21J
I LARYNX AND H Y POPHARYNX

D E

density. non-enhancing lesion centered in the vallecula, posterior to the base of the
tongue. The degree of airway obstruction and displacement of the epiglottis are
adequately assessed and may alert the surgeon to the need for a tracheostomy
before any procedure is attempted.
Coronal or sagittal MR imaging may better delineate the cranio-caudal extent of
the lesion. Axial TI W (Fig. 11 - 0 ) and axial TIW (Fig. 1 1 -E) M R images through the
supraglottis in another patient show a 2-cm cystic lesion in the left vallecula and a
smaller, less than 5-mm, cyst in the contralateral side. This patient was diagnosed
with bilateral vallecular cysts.
When infected, vallecular cysts may demonstrate increased density and enhance
heterogeneously, making them indistinguishable from an abscess. On sectional
imaging, determination of the exact site of the lesion greatly assists in differentiat
ing vallecular cysts from other cystic lesions such as thyroglossal duct cyst (TGDC),
tongue base cyst and laryngocele. TGDC tends to be more midline and have a more
anterior location between the strap muscles and the hyoid bone or thyroid ala.
Internal laryngoceles grow superiorly in the paraglottic space and obliterate or
push the vallecula superiorly. However, their inferior extent and connection with
the laryngeal ventricle is adequately detected on imaging studies. Finally, dermoids
are usually located in the base of the tongue and floor of the mouth, and typically
contain fat. In children, ectopic thyroid tissue should be ruled out, and the presence
of a thyroid gland in normal anatomic position confirmed by a thyroid scan with
1 - 1 3 1 , before surgery is performed.

Man agement
Vallecular cysts may be managed by aspiration via an endoscopic approach. The
disadvantage of this procedure is the high rate of recurrence. Marsupialization
and laser ablation are the techniques of choice. Rarely. a supraglottoplasty may be
necessary to remove the cyst and correct redundant epiglottis and aryepiglottic
folds.
LARYNX AND HYPOPHARYNXI

PEARLS/PITFALLS Suggested Readings


An infant with a vallecular cyst Oluwole, M. Congenital vallecular cyst: a cause of failure to thrive. British Journal
often presents with feeding diffi of Clinical Practice 50(3): 1 70, 1 996 Apr-May.
culty and stridor. Wang, C.R., Lim, K.E. Vallecular cysts: report of two cases. Pediatric Radiology 25
Suppl 1 :S21 8-9, 1 995 Nov.
A cystic lesion often is centered
in the vallecula. Wong, K.S., Li, H . Y., Huang, T.S. Vallecular cyst synchronous with laryngomala
cia: presentation of two cases. Otolaryngology and Head and Neck Surgery
Patients with vallecular cysts 1 1 3(5):621 -4, 1 995 Nov.
should be evaluated carefully for
any degree of airway compro
mise.

A vallecular cyst may be mis


taken for other supraglottic
cystic lesions.

An abnormal supraglottic soft


tissue mass on lateral plain films
of the neck may be difficult to
recognize.
I LARYNX AND H Y POPHARYNX

Case 12
Clinical Presentation
A 40-year-old male presented with gradual onset dyspnea.

Radiologic Findings
The axial CT scan (Fig. 1 2-A) shows an expansile mass centered in the posterior
cricoid cartilage on the right. There are areas of speckled calcification within the
lesion.

Differential Diagnosis: Expansile Lesion of


the Laryngeal Cartilage
Chondroma
Chondrosarcoma
Metastasis
Post-traumatic deformity

Diagnosis
Chondroid tumor of the larynx (chondrosarcoma)

Discussion
Epidemiology
Chondroid tumors of the laryngeal cartilage are uncommon neoplasms. They are
found predominantly in men (3 : 1 ratio) in their 4th through 6th decades. This is
LARYNX AND HYPOPHARYNXI

similar to the age range of patients with cartilaginous tumors of the facial area.
Chondroid tumors of the larynx are unlike other cartilaginous neoplasms (espe
cially chondrosarcomas) of extralaryngeal origin, which tend to affect young and
middle aged adults.
Chondroid tumors are the most common mesenchymal tumor of the larynx. The
specific histology may range from chondroma to chondrosarcoma. Chondrosarcoma
is the most frequently encountered non-epithelial malignancy of the larynx.

Pathophysiology
The location of these tumors in over 75% of cases is the posterior lamina of the
cricoid cartilage. Histologically all documented cases have arisen from hyaline car
tilage with no evidence of elastic cartilage.
Biopsy may be difficult because of the firmness of the tumor. Interpretation of the
findings is also problematic. Chondroma and low-grade chondrosarcoma are diffi
cult to distinguish histologically. Some authors advocate that all laryngeal neo
plasms containing hyaline cartilage should be considered chondrosarcomas until
proven otherwise. They suggest that evidence of the lesion's benign nature is deter
mined by either: ( 1 ) its failure to metastasize or recur over a period of several years
follow-up or (2) pathologic examination of the lesion in its entirety.

Clinical Findings
The clinical findings are nonspecific and tend to be due to gradual encroachment
of the subglottic space. This can manifest as a neck mass or, if situated within the air
way, as a slowly progressive obstruction, hoarseness or dyspnea. Because most
lesions are below the vocal cords, hoarseness may be minimal, with dyspnea or stri
dor the more common symptom.

B
I LARYNX AND HYPOPHARYNX

C D

I maging Fi ndings
Chondroid tumors are characterized by focal expansion of the laryngeal cartilage.
They show typical mottled chondroid calcification in 80% of lesions. It is very diffi
cult to distinguish chondroma from low grade chondrosarcoma radiographically.
Higher grade chondrosarcomas may show more aggressive radiographic features.
cr is the preferred imaging technique because of its ability to define subtle calcifi
cations. On MRI these findings are less obvious. Figures l 2-8-l 2-F show five other
patients with chondrosarcoma of the larynx. The large lesion in Figure 1 2-8 is simi
lar to figure 1 2-A with significant airway obstruction. Figure 1 2-C shows a densely
calcified lesion. Figure 1 2-0 shows a smaller lesion with rare calcifications and Fig
ure 1 2-E shows a lesion with prominent central calcifications. The small lesion in
Figure 1 2-F appears as a slight widening of the cricoid cartilage without significant
airway compromise.
L A RYNX A N D HYPOPHARYNXI

PEARLS/PITFALLS Management

Chondrosarcoma presents as ex Although laryngeal chondrosarcoma reportedly can recur, local surgical excision
pansion of the laryngeal cartilage without radical margins and with long-term clinical follow-up is recommended. Al
with chondroid calcifications. though conservative therapy is acceptable i n most instances, total laryngectomy may
be performed in cases of extensive tumor, high-grade lesions, and recurrences. CO2
The cricoid cartilage is most fre laser excision for palliation has been shown to be a viable alternative in patients
quently affected, followed by the with high-operative risk.
thyroid and arytenoid cart i l ages.

Chondroma and chondrosar Suggested Readings


coma difficult to dist inguish.

Bogdan, C.l, Maniglia, A . I . , Eliachar, I . , Katz, R . L. Chondrosarcoma of the larynx:


The clinician must take care not challenges in diagnosis and management. Head and Neck 16(2): 1 27-34, 1 994 Mar
to mistake chondrosarcoma for Apr.
carcinoma of the larynx. Bough, I .DJ., Chiles, PI, Fratalli, M.A., Vernose, G. Laryngeal chondrosarcoma: two
unusual cases. American Journal of Otolaryngology 16(2): 1 26-3 1 , 1 995 Mar-Apr.
Browne, ID. Management of nonepidermoid cancer of the larynx. Otolaryngologic
Clinics of North A merica 30(2):2 1 5-29, 1 997 Apr.
Chiu, L.D., Rasgon, B.M. Laryngeal chondroma: a benign process with long-term
clinical implications. Ear, Nose, and Throat fournaI 75(8) :540-2, 544-9, 1996 Aug.
Lewis, IE., Olsen, K.D., Inwards, c.Y Cartilaginous tumors of the larynx: clinico
pathologic review of 47 cases. A nnals of Otology, Rhinology and Laryngology
106(2) :94-100, 1997 Feb.
I LARYNX A N D H Y POPHARYNX

Case 13
Clinical Presentation
A 59-year-old male presented with progressive hoarseness over the last 4 months.
Endoscopy (Fig. 13-A) shows an exophytic mass extending from the anterior com
missure to the posterior third of the vocal cord.

B c
LARYNX AND H Y POPHARYNXI

Radiologic Findings
An axial TZW MR image (Fig. 1 3-8) through the cords of a similar patient shows a
high intensity lesion invading the right true cord musculature involving the anterior
commisure. A coronal T l W MR scan of the same patient in Figure 1 3-C shows that
the lesion extends from the true cord to the right laryngeal ventricle. However, it
does not involve the false vocal cord. The para laryngeal space also appears to be
clear.

Differential Diagnosis: Vocal Cord Assymetry


Glottic carcinoma
Recurrent nerve paralysis
Teflon injection of vocal cord
Squamous cell carcinoma of the vocal cord

Diagnosis
Squamous cell carcinoma of the vocal cord

Discussion
Epidemiology
Glottic tumors (arising from the true cords) are the most common laryngeal can
cers, typically affecting males in their fifth to seventh decades. Of malignancies of

I
I LARYNX AND HYPOPHARYNX

Table 13-1 Tumor, Node, Metastasis (T M ) Staging Glottic Cancer

T l -Limited to vocal cords


T2-Extends to supraglottic and/or subglottic and/or with impaired cord mobility
TI-Limited to larynx with cord fixation
T4-I nvades thyroid cartilage and/or extralaryngeal spread

the larynx, 90% are squamous cell carcinomas and these tumors are usually well
differentiated. Glottic malignancies are slow-growing lesions that tend to metasta
size late as a result of the limited lymphatic drainage of the vocal cords. Initially
most patients complain of intermittent hoarseness that progressively worsens. These
tumors have been linked to smoking and radiation exposure.
Seventy-five percent of these tumors involve the anterior half of the true vocal
cord, as is seen in this patient. Early glottic lesions have an excellent prognosis; the
five year survival approaches 95% with surgery or radiation therapy. Cross sectional
imaging of these lesions may show thickening of the true cords or they may be nor
mal. I f the clinician has a clear view and can confirm that the lesion is an early T1
tumor, M R I is not necessary. With more advanced tumors and because the signals
from tumor and muscle differ, the distinction between exophytic lesions and lesions
infiltrating the true cord musculature is well demonstrated. True cord tumors spread
to involve the mucosa over the arytenoids or at the anterior commissure of the
cricoarytenoid joints. Figure 1 3-0 i llustrates a coronal view of the larynx with a car
cinoma shown as a mass in the region of the true vocal cord (speckled pattern).

Clinical Findings
Because glottic cancers involve the vocal cords, they also present very early with
hoarseness. Nodal spread is rare in the case of early lesions because there is an ab
sence of lymphatics along the free margin of the vocal cords. Advanced lesions with
a fixed cord, however, have a higher incidence of lymphatic involvement. Since
these tumors are readily accessible, the majority are diagnosed and management
planned by clinical examination using laryngoscopy and biopsy. The primary role of
CT and MR imaging, therefore, is to define the extent of the disease for appropri
ate management.

I m aging Findings
For all these reasons, despite the fact that squamous cell carcinomas are the most
common laryngeal cancers, they are not the most common laryngeal cancer in radi
ology departments because imaging studies add little to the diagnostic workup in
most cases. The very early true cord tumor can be readily evaluated on indirect
clinical examination and generally does not require an imaging study. The more
advanced lesions that show extension to the anterior commissure regions above or
below the vocal cord are quite a different matter. They require imaging techniques
to show deep infiltration and the full extent of the tumor.
Tl glottic lesions (Table 1 3-\ ) are confined to the true cords without affecting the
cord's mobility. CT scan of these lesions may how true cord thickening or they may
look normal. CT density differences between a nonenhancing tumor and muscle are
not great enough to allow one to predict invasion of the true cord musculature in
most cases. Because M R signals from tumor and muscle differ significantly, MR
imaging is the more sensitive imaging modality for determining muscle invasion.
The high fat content within the larynx makes T l -weighted images preferable to the
T2-weighted images in which the tumor may be obscured by the intensity of the are
olar tissue.
T2 lesions (Table 1 3-1 ) are defined as those that have spread to either the supra
or subglottic region and show normal or only slightly impaired mobility of the true
LA RYNX AND H Y POPHARYNXI

vocal cord. With CT, impaired mobility is implied in the scan by the diminished
PEARLS/PITFALLS motion artifacts on the involved side. In addition, the true cord typically takes a
An early symptom of squamous paramedian position and the arytenoid is displaced more medially. These changes
cell carcinoma is voice change. also are seen on MR i mages. True cord tumors may spread to involve the mucosa
over the arytenoids or at the anterior commissure. On CT and MR imaging any
Lesions are usually well differen more than 1 mm of thickness of the anterior commissure is considered abnormal
tiated and slow growing. and would require an "extended" hemilaryngectomy. One of the advantages of MRI
over CT is that sagittal M R images provide an excellent view of the anterior com
Spread to the lymphatics is usu missure and its relationship to the supraglottic and subglottic compartments.
ally limited. Tumors at the anterior commissure may follow the central tendon of the vocal
ligaments into the thyroid cartilage. This is very difficult to detect by clinical exam
Surgical resection or radiother and may be difficult to detect by CT as well. Cartilaginous destruction appears as a
apy are the preferred treatment moderate signal interrupting the normal outer rim of low signal, calcified cartilage
modalities. and/or as a focal area of lower signal in the high signal central marrow. Since focal
asymmetric areas of diminished density may be a normal variant in this region, an
Imaging studies have limited
area of demineralization i n the paramedian does not necessarily indicate cartilagi
value in this diagnosis. nous invasion. MR imaging is more accurate in diagnosing early cartilaginous inva
sion since the signal from the tumor differs dramatically from that of cartilage
Underestimating tumor exten
regardless of its state of m ineralization.
sion may result in undertreat
Vocal cord fixation comprises a T3 lesion (Table 13- 1 ) . Since cricoid cartilage is
ment.
almost invariably symmetric in its degree of ossification, any decrease in signal from
one side of the cricoid cartilage is evidence of a T3 lesion. Fixation is rarely due to
Squamous cell carcinoma of the
causes other than tumor, such as rheumatoid arthritis.
vocal cord should not be con
Cord fixation may be the result of several mechanisms. A tumor may infiltrate the
fused with paramedian cord due
intrinsic laryngeal musculature and fix the cord to the thyroid cartilage. A bulky
to recurrent nerve paralysis.
tumor may limit cord mobility by mass effect or may involve the cricoarytenoid
joint. Scans may show an arytenoid cartilage in the median or paramedian position.
MR imaging can show how the spread to the deep tissue planes and cartilage inva
sion lead to fixation. Only rarely is fixation due to causes other than tumor, such as
rheumatoid arthritis.
Mucosal spread to the false cord may not be appreciated with M R I . However,
extension across the ventricle to the false cord via the paralaryngeal space will
decrease the fatty high signal deep to the cord. Coronal sections are particularly
useful because bulky lesions occasionally bulge upward, distorting the false cords
rather than invading them. This may be difficult to appreciate on axial images.

Management
T1 and T2 lesions may be treated with surgery or radiation. Spread to the false cord
and significant subglottic spread may necessitate a vertical hemilaryngectomy. Fur
ther extension may fix the vocal cord to produce a T3 lesion, which may worsen the
prognosis and make selection of the appropriate therapy potentially more difficult.
Advanced lesions with cartilage invasion generally require surgery.

Suggested Readings
Katsounakis, 1., Remy, H., Vuong, T. , Gelinas, M., Tabah, R. I mpact of magnetic res
onance imaging and computed tomography on the staging of laryngeal cancer. Eu
ropean Archives of Oto-Rhino-laryngology 252(4):206-8, 1995.
Mukherji S.K., Castillo M., Huda w., Suojanen J., Kubilis P, Tart R.P, Dhillon G.
Comparison of dynamic and spiral CT for imaging the glottic larynx. Journal of
Computer Assisted Tomography 1 9(6):899-904, 1 995 Nov-Dec.
Teresi, L.M., Lufkin, R.B., Hanafee, W.N. Magnetic resonance imaging of the larynx.
Radiologic Clinics of North America 27:393-406, 1 989.
Weinstein, G.S., Laccourreye, 0., Brasnu, D., Yousem, D.M. The role of computed to
mography and magnetic resonance imaging in planning for conservation laryngeal
surgery. Neuroimaging Clinics of North America 6(2):497-504, 1996 May.
I LA RYNX A N D H Y POPHARY

Case 14
Clinical Presentation
A 70-year-old male presented with hoarseness.

A B

162
LARYNX AND HYPOPHARYNXI

Radiologic Findings
The axial T I weighted M R I shows an infrahyoid supraglottic mass replacing the
preepiglottic fat and narrowing the supraglottic airway ( Fig. 1 4-A). The 1'2 weighted
axial image shows similar findings with improved muscle/tumor contrast ( Fig. 1 4-B).
The sagittal Tl weighted image shows the mass extending inferiorly to the level of
the true vocal cords ( Fig. 1 4-C).

Differential Diagnosis: Supraglottic Mass


Carcinoma
Laryngocele
Post-traumatic deformity [pseudomass 1
Papilloma
Chondroma
Metastasis
Amyloid
Hemangioma
Lymphoma

Diagnosis
Supraglottic carcinoma of the larynx

Discussion
Epidemiology
Supraglottic tumors may arise anywhere [rom the false vocal cords to the epiglottis.
These malignancies comprise 20 to 35% of all laryngeal cancers. Unlike glottic can
cers, they tend to present later. They are also more poorly differentiated histologi
cally. Because of abundant lymphatics in the supraglottic region, these tumors tend
to metastasize early to lymph nodes high in the neck and involve the internal jugu
lar chain and often the jugulo-digastric node. Consequently, supraglottic cancers
often present as more advanced tumors than glottic lesions. Imaging studies are crit
ical in their workup. For tbis reason they are the often the most common laryngeal
cancer seen in many radiology departments.

Pathophysiology
Histologically, the vast majority of laryngopharyngeal cancers are of squamous cell
origin. Other types of malignancies found in the larynx and hypopharynx include
verrucous carcinoma. pseudosarcoma, adenocarcinoma, "spindle cell" carcinoma.
oat cell carcinoma, and basal cell carcinoma. Laryngeal malignancies of connective
tissue and hematopoietic elements have also been documented. Examples include
chondrosarcoma, fibrosarcoma, reticulosarcoma. and lymphosarcoma.

Clinical Fi ndings
Carcinoma of the supraglottic region can be subdivided into two groups: ( I ) ante
rior carcinomas which arise on the epiglottis and anterior false cords and (2) pos
terolateral carcinomas which arise from the medial surface of the aryepiglottic folds
and the para laryngeal spaces (marginal tumors). I n the anterior epiglottis group, the
lesions may be either above or below the level of the hyoid bone. Suprahyoid
epiglottic lesions show early invasion of the preepiglottic space, glosso-epiglottic,

I
I LARYNX AND H YPOPHA RYNX

and pharyngo-epiglottic folds. On T l W MR images the preepiglollic space i of high


intensity because of its high fat con ten!. Infiltration with intermediate-signal tumor
is readily identified. Advanced suprahyoid epiglottic cancers frequently cross the
midline with the paralaryngeal lymphatics and extend into the paramedian portions
of the thyroid cartilage, just above the anterior commissure level.
Tumors of the infrahyoid epiglottis rarely grow inferiorly to the laryngeal ventri
cle. If a supraglollic laryngectomy is to be successful, a free margin must be present
above the thyroepiglottic ligament with no cartilage invasion. Recalling that no ap
preciable tissue thickness should be visible at the anterior commissure, the vertical
extent of the lesion can be readily determined. Occasionally, the normal tissue thick
ness associated with the inferior pole of the epiglottic cartilage and the thy
roepiglottic ligament at the level of the false cord may be interpreted as tumor.
Here, sagillal images are particularly useful (Fig. 1 4-C).
Posterolateral (marginal) supraglottic tumors appear as thickened aryepiglollic
folds and as a mass in the paralaryngeal space. Their natural tendency is to grow
posteriorly and inferiorly to the arytenoids, rather than anteriorly to the preepiglot
tic space. I nvasion of the thyroid lamina occurs late and almost invariably along the
postero-Iateral-inferior margin. Tumors may also grow cephalad within the
preepiglottic space to invade the tongue base without distorting the mucosa in the
valleculae. Sagillal images will show spread to the tongue base to best advantage.
Marginal supraglollic cancers may also arise from the surface of the aryepiglottic
fold and spread to the infrahyoid epiglottis, false cord, and eventually to the
cricoarytenoid joint where invasion may cause cord fixation. [ t may be difficult to
distinguish an advanced piriform sinus carcinoma from a marginal supraglottic car
cinoma. Axial images clearly show the limits of the tumor relative to the preepiglot
tic space, tongue base, and hypopharynx. Lymph node metastasis is especially likely
with aryepiglottic fold tumors. Thyroid cartilage invasion is a rare finding unless the
tumor has become transglollic. The hyoid bone is usually displaced by tumor and
not destroyed.

Imaging Findings
Rarely does radiologic imaging play a significant role in reaching a diagnosis of ma
lignancy in the larynx and hypopharynx. These regions are so readily accessible to
clinical examination that the combination of cytology and visual inspection usually
strongly indicates the diagnosis of cancer. Therefore, the primary role of studies in
imaging the larynx and hypopharynx is to define the extent of the disease. While
laryngoscopy can show mucosal surfaces and masses involving the lumen, deep
extensions are difficult to detect clinically. However, these extensions can have pro
found implications for the management of disease. CT. and to an even greater
extent, M R [ can define this important deep anatomy.
Imaging studies may detect the deep extent of tumors that have normal overlying
mucosa and are not detectable on endoscopy. Sometimes large. bulky, proximal
lesions obscure the view of endoscopists, who may have difficulty in viewing the
infraglottic region and the apices (inferior tips) of the pyriform sinuses. Imaging is
also useful to detect clinically occult laryngoceles associated with a paraventricular
tumor.

M anagement
Frequency selective fat suppression imaging ( FATSAT) contrast-enhanced se
quences may be useful to answer the clinical questions such as the tumor extent into
the fat of the pre-epiglottic and paralaryngeal spaces. Additionally. these sequences
determine. to advantage, the presence of cartilage invasion, as is found in most adult
patients. The laryngeal cartilages are ossified and contain bone marrow. Using CT
and/or MRI for staging, a partial laryngectomy with preservation of glollic function
may be possible instead of a total laryngectomy. in some cases. In others, tumor

64
LARYNX AND HYPOPHARYNXI

invasion of the laryngeal cartilages, pre-epiglottic space, or infraglottic region may


PEARLS/PITFALLS exclude radiation therapy as the sole treatment.
Supraglottic carcinoma presents The loss of natural voice function resulting from a total laryngectomy can be dev
later, is more poorly differenti astating to many individuals. Less radical laryngeal resections that allow the preser
ated and grows faster tban glottic vation of natural speech and the protective sphincter mechanism of the larynx have
lesions. been developed for certain smaller laryngeal tumors. Planning for any of these con
servation laryngeal surgeries depends on an accurate pre-operative knowledge of
Lymphatic spread to upper cervi the precise extent of the disease within the larynx. All surgical techniques require
cal nodes may occur. an intact cricoid cartilage and at least one mobile arytenoid on which to construct
the functional voice box. cr and MRI can provide this essential information. To
I n certain cases, voice conserva plan this type of operation, the direct coronal and sagittal imaging capabilities of
tion surgery with partial laryn MR are an advantage over axial CT images, in their ability to define critical infor
gectomy is possible. mation regarding the cranial-caudal extent of the tumors.
The recognition of tumor spread to other area also markedly affects patient
I maging studies are very valu management. In the case of spread of the supraglottic tumor to involve the tongue
able in this diagnosis. base, a partial glossectomy may have to be performed in addition to the primary
surgery. In other areas, extraorgan spread may render the tumor unresectable.
I t is very difficult to recognize
tbe true extention of supraglottic
tumors.
References
Katsounakis, I . , Remy, H., Vuong, T., Gaelinas, M., Tabah, R. Impact of magnetic res
onance imaging and computed tomography on the staging of laryngeal cancer. Eu
ropean A rchives of Oto-Rhino-Iaryngology 252 4:206-8, 1 995.
Larsson, S. Y., Mancuso, A., Hoover, I., Hanafee, W. Differentiation of pyriform sinus
cancer from supraglottic laryngeal cancer by computed tomography. Radiol
ogy 1 4 1 :427-432, 198 1 .
Myers, E.N., Alvi, A . Management o f carcinoma o f the supraglottic larynx: evolu
tion, current concepts, and future trends. Laryn.goscope 1 06 5 Pt 1 :559-{j7, 1 996 May.
Thabet, H .M., Sessions, D.G., Gado, M.H., Gnepp, D.A., Harvey, J.E., Talaat, M.
Comparison of clinical evaluation and computed tomographic diagnostic accuracy
for tumors of the larynx and hypopharynx. Laryngoscope 106 5 Pt 1 :589-94, 1 996
May.
I LARYNX AND HYPOPHARYNX

Case 15
Clinical Presentation
A 62-year-old male complained of mild dysphagia of 6 weeks duration.

A B

c D

I
LARYNX AND H Y POPHARYNXI

Radiologic Findings
On axial T l -weighted images (Figs. 1 5-A and IS-B), a left-sided pyriform sinus mass
is seen, well delineated from the surrounding fat. Note that the t umor/muscle inter
face is indistinct. In contrast, on axial T2-weighted images (Figs. l S-C and 1 5-0), the
high signal intensity of the tumor allows excellent differentiation from pharyngeal
and prevertebral musculature but blends with fat. At the level of the false vocal
cords (Fig. IS-A), the bulky mass invades the posterolateral portion of the thyroid
cartilage.

Differential Diagnosis: Pyriform Sinus Mass


Pyriform sinus carcinoma
Post-operative scarring
Post radiation edema
Metastases
Mesenchymal tumor
Lymphoma

Diagnosis
Pyriform sinus carcinoma

Discussion
Pyriform sinus carcinoma is one of the most conUTIon cancers of the hypopharynx.
These tumors are usually well advanced on presentation. I nitial manifestations are
often subtle and include pain in the throat or referred pain to the ear, with later dys
phagia, otalgia, or an isolated lump in the neck. U n like the hoarseness tbat occurs
with vocal cord involvement, patients with pyriform sinus cancer often have a char
acteristic alteration in their vocalization described as a "potato voice".

Epidemiology
These lesions behave more aggressively than endolaryngeal lesions and comprise 1 0
t o 20% o f "laryngeal" cancers. They are actually cancers o f the inferior hypophar
ynx. Early nodal disease occurs because of the rich lymphatics anterior to the pyri
form si n uses.

Pathophysiology
Pathologically, the majority of these tumors are squamous cell carcinomas. They
tend to spread submucosally, either ( 1 ) circumferentially with extension into the
posterior pharyngeal wall or (2) posterolaterally with deep invasion and associated
cartilage destruction. The size of the tumor does not necessarily relate to the degree
of biological aggressiveness or the tendency for cartilage destruction.
Pyriform sinus tumors grow in two major patterns. ( 1 ) Tumors in the lateral wall
invade the thyroid cartilage and soft tissues of the neck forming bulky masses about
the pyriform sinus. (2) Tumors of the medial wall extend into the paralaryngeal
space and vocalis muscle, resembling marginal supraglottic lesions.
Despite the similarities, pyriform sinus tumors have several characteristics that
distinguish them from marginal supraglottic lesions. Pyriform sinus tumors fre
quently invade the thyroid cartilage, usually at its posterolateral margins. In addi
tion, pyriform sinus lesions tend to be unilateral and submucosal. If extensive, these
tumors may widen the space between the thyroid and cricoid cartilages (cricothy-
I LARYNX AND HYPOPHARYNX

roid notch). This is because the tough conus elasticus tends to direct the tumor pos
PEA RLSIPITFALLS terolaterally and inferiorly.
Cartilage invasion is a frequent
finding. I m aging Findings
It is unusual for these tumors to Clinical examination is often difficult and incomplete. Cross-sectional imaging is
cross the midline. necessary to evaluate the extent of submucosal spread.
M R I allows excellent soft tissue definition and is valuable in distinguishing a tu
The clinician should keep a sharp mor from surrounding structures. On T1 -weighted images, the primary tumor signal
eye so as not to overlook intensity differs sufficiently from fat so that its margins are well seen. On 1'2-
adenopathy. weighted images, the bright signal blends with fat but is clearly distinguished from
surrounding musculature. Thus, T1 -weighted sequences are better at determining
Pyriform sinus masses may be
tumor/fat contrast, whereas T2-weighted images are helpful in determining tu
mistaken for laryngeal cancer.
mor/muscle contrast.

Management
Surgical resection is the treatment of choice, often in conjunction with radiation
therapy. Nodal dissection is performed as necessary.

Suggested Readings
AlIal, A.S. Cancer of the pyriform sinus: trends towards conservative treatment. Bul
letin Cancer 84 7:757-62, 1997 Jul.
Elias, M.M., H ilgers, F.l., Keus, R.B., Gregor, R.T., Hart, A.A., Balm, AJ. Carcinoma
of the pyriform sinus: a retrospective analysis of treatment results over a 20-year pe
riod. Clinical Otolaryngology 20 3:249-53, 1995 Jun.
Larsson, S.Y., M ancuso, A., Hoover, L., Hanafee, W. Differentiation of pyriform si
nus cancer from supraglottic laryngeal cancer by computed tomography. Radiology
141 :427-432, 1 981 .

I
LARYNX AND HYPOPHARYNXI

Case 16
Clinical Presentation
A 5 1-year-old male presented with a right neck mass and hoarseness. Ten years ear
lier, the patient had undergone an above-the-knee amputation for an osteogenic
sarcoma of the right proximal tibia. Two years later, he was found to have a solitary
right pulmonary metastasis, which was resected without difficulty. Now, eight years
later, he has presented with the current lesion.

A B

c D
I LARYNX AND H Y POPHARYNX

Radiologic Findings
An axial T I -weighted image (Fig. 1 6-A) shows a low-intensity mass (arrowheads)
originating from the marrow space of thyroid cartilage. Cricoid cartilage (C) and the
left side of the thyroid cartilage (T) are preserved. A coronal T l -weighted image
(Fig. 1 6- 8 ) shows a 5-cm lesion on the right side of the larynx, which is displacing
paralaryngeal fat medially (arrowhead). A coronal T2-weighted image (Fig. 1 6-C)
shows the submucosal location of a high intensity mass (arrow) with preservation of
mucosal tissues medial to the lesion (arrowhead) . Fine-needle aspiration (Fig. 1 6-D)
demonstrated a group of round to oval cells embedded in the fibrous stroma
(H & E, original X 400). Nuclei are irregular and hyperchromatic, suggestive of a
metastatic tumor.

Differential Diagnosis: Destructive Laryngeal


Mass Centered in the Cartilage
Atypical squamous cell carcinoma of the larynx
Primary chondral tumor
Metastasis to the larynx

Diagnosis
Metastasis to the larynx (osteosarcoma)

Discussion
Epidemiology
Secondary laryngeal tumors are uncommon, accounting for approximately 0.02% of
all laryngeal malignancies. In 1 988, Ferlito et al reviewed the world literature, which
revealed 1 1 3 cases, and his series added another 7, for a total of 1 20 cases known at
that time.
The most common primary tumors metastasizing to the larynx are melanomas of
the skin (27 to 37% ), renal cell carcinomas ( 1 3 to 1 7% ), carcinomas of the breast
(9 to I I % ), lung carcinomas (8 to 1 1 % ), and adenocarcinomas of the prostate (6 to
7% ). Osteosarcoma metastatic to the larynx is rare although its appearance is simi-

E
LARYNX AND HYPOPHARYNXI

lar to other metastases to the larynx. A CT scan of a second patient with metastatic
PEARLS/PITFALLS renal cell carcinoma to the larynx (Fig. 1 6-E) shows similar findings.
In general, laryngeal lesions are
quite destructive. Pathophysiology
Metastases are usually centered Metastasis to the larynx is an uncommon event, and metastases occurring in the thy
over the laryngeal cartilage. roid cartilage are rarer still, contributing to 2.6% of secondary laryngeal tumors.
Metastases to the thyroid cartilage are possible only after endochondral ossification
Melanoma, renal cell, breast, and and development of a marrow space, which occurs by adulthood. Their most likely
lung primary carcinomas com route of spread is hematogenously, either retrograde through the valveless vertebral
monly metastasize to the larynx. veins, as described by Batson ( 1 940), or from the vena cava through the pulmonary
circulation to the laryngeal artery.
Metastases to the larynx are of
ten mistaken for primary squa
mous cell carcinoma or aggres Imaging Findings
sive chondrosarcoma.
The MR appearance of the entity is unlike the appearance of most primary squa
mous cell tumors of the larynx, which tend to arise from the mucosal surface rather
than centered over the cartilage as in this case. Unlike most primary chondral
tumors of the larynx (which are also centered over the cartilage), this lesion is very
aggressive and destroys the cartilage rather than expanding or remodeling it. There
is also no evidence of calcification to suggest a chondral tumor. There is preserva
tion of the right side of the thyroid cartilage and of the cricoid cartilage. On coronal
sections, the vocal cords appear displaced medially without gross invasion. Com
paring T I - and T2-weighted images, it appears that the lesion is submucosal (Figs.
1 6-B and 1 6-C).

Management
Metastasis to the larynx is almost always associated with long-standing, widely dis
seminated disease, and carries a poor prognosis. General treatment recommenda
tions for these lesions have emphasized a palliative approach, but there are reports
in the literature of long-term survivors. I n the case of metastatic osteosarcoma, there
are reports in the literature promoting metastatectomy in selected patients, espe
cially those with isolated pulmonary metastases. In those patients treated surgically
without evidence of disease, there is a 20 to 30% long-term survival. This case illus
trates the typical longstanding history of patients found to have metastases to the
larynx.

Suggested Readings
Batsakis, IF., Luna, M.A., Byers, R.M. Metastases to the larynx. Head and Neck
Surgery 7:458-60, 1 948.
Batson, O.V. Function of vertebral veins and their role in spread of metastases. An
nals of Surgery 1 12 : 1 38-49, 1 940.
Cavicchi, 0., Farneti, G., Occhiuzzi, I . , Sorrenti, G. Laryngeal metastasis from
colonic adenocarcinoma. Journal of Laryngology and Otology 104:730-2, 1 990.
Cullen, lR. Ovarian carcinoma metastatic to the larynx. Journal of Laryngology an.d
Otology 1 04:48-9, 1 990.
Ferlito, A., Caruso, G., Recher, G. Secondary laryngeal tumors. A rch.ives of Oto
laryngology and Head and Neck Surgery 1 1 4:635-9, 1 988.
Fields, lA. Renal carcinoma metastasis to larynx. Laryngoscope 76:99-10 1 , 1 966.
Grignon, 0.1., Ro, 1.y., Ayala, A.G. Carcinoma of the prostate metastasizing to vo
cal cord. Urology 36:85-8, 1 990.
Ikeda, M., Takahashi, H . , Karaho, T. Amelanotic melanoma metastatic to the
epiglottis. Journal of Laryngology and Otology 1 05:776-9, 1 99 1 .
I LARYNX AND HYPOPHARYNX

Szmeja, Z., Kruk-Zagajewska, A., Salwa-Zurawska, W. Metastases of renal


adenocarcinoma to the larynx and paranasal sinuses. Otolaryngol Pol 4 1 :221-7,
1 987.
Whicker I.H., Carder, G.A., Devine, K.D. Metastasis to the larynx, report of a case
review of the literature. A rch Olo/aryngoI 96: 182-4, 1 972.
III.

Nasopharynx and Parapharyngeal Space


NASOPHARYNX AND PARAPHARYNGEAL SPACEI

Case 17
Clinical Presentation
An adult male presented with headaches. A rounded midline, mucosal covered na
sopharyngeal mass was noted. He denied foul breath, nasal discharge. or muscular
spasm with head motion.

A B
I NASOPHARYNX AND PARAPHARYNGEAL SPACE

Findings
A 4mm rounded midline, mucosal covered nasopharyngeal mass was noted (Fig.
1 7-A) A 4 mm high signal intensity lesion on T l - ( Fig. 1 7-B) and T2-weighted ( Fig.
1 7-C) images can be seen in the nasopharyngeal midline. The surrounding soft
tissues are intact. The lesion is homogeneous in signal intensity. There is no com
munication with the intracranial space.

Differential Diagnosis: Cystic


Nasopharyngeal Mass
Tornwaldt cyst
Encephalomeningocele
Pharyngeal cyst
Atypical lymph node

Diagnosis
Tornwaldt (or Thornwaldt) cyst.

Discussion
Epidemiology
G.L. Tornwaldt was a German physician from the 1 9th century who described an
inflammation or abscess of an embryonic remnant cyst of the pharyngeal bursa
appearing at the posterior median wall of the nasopharynx. His name is spelled both
Tornwaldt and Thornwaldt in various publications although Tornwaldt appears to
be the preferred spelling. Tornwaldt's cysts are found in 4% of normal autopsy spec
imens. There is no gender predilection.

Pathophysiology
The nasopharyngeal bursa represents the site of the caudal-most end of notocord.
A Tornwaldt cyst results from an adhesion between the pharyngeal endoderm and
the notocord. The bursa may appear as a midline pit or closed over as a cyst.

D E
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

The cyst, which may have a canal that extends to the pharyngeal mucosa, is situ
PEARLSIPITFALLS ated beneath the adenoid, or its remnants, and extends backward and upward to the
A Tornwaldt cyst is a midline, periosteum of the occipital bone. This canal may become obstructed, resulting in
cystic thin walled nasopharyngeal infection and abscess formation.
mass.
Clinical Findings
It is an embryologic remnant.
Most Torowaldt's cysts are asymptomatic. When present, symptoms can include
A Tornwaldt cyst contains fluid, postnasal discharge, crusting, frequent colds, sneezing, hoarseness, bad taste or odor,
or occasionally air. coughing, or prevertebral neck pain with motion. Other symptoms may be nasal
speech, cervical adenitis, or symptoms relating to the ear such as vertigo, tinnitus,
It is usually asymptomatic.
earache, or deafness. With infection, prevertebral muscle spasm may occur.
Symptoms may include postnasal
discharge, bad taste or odor, Imaging Findings
coughing, or prevertebral neck
pain with motion. Tornwaldt cysts vary from 1 to 5 mm in diameter and are generally rounded. M R I
demonstrates high signal intensity both o n T l - and T2-weighted images, likely re
An encephalocele may be mis lated to either proteinaceous or hemorrhagic cyst contents. On CT they are rounded
taken for a Tornwaldt cyst. cysts. Figure 1 7-D shows a CT scan of another patient with this lesion. These cysts
may on rare occasions extend off the midline. They usually are fluid filled but occa
sionally contain air. A CT scan of another patient with an air-filled cyst can be seen
in Figure 1 7-E.
The integrity of the overlying bone should always be confirmed with sagittal,
coronal, or more cephalad axial views in order to rule out the uncommon possibil
ity of meningocele or encepalocele.

Management
Treatment consists of surgical drainage and excision for symptomatic lesions. As
symptomatic lesions are usually not treated.

Suggested Readings
Miyahara, H., Matsunaga, T. Tornwaldt's disease. A cta Oto-Laryngologica 5 1 7:36-9,
Supplement 1 994.
I NASOPHARYNX AND PA RAPHARYNGEAL SPACE

Case 18
Clinical Presentation
A teenage male complaiJ1Cd of nasal stuffiness for 2 years and several episodes of
spontaneous nose bleeds for 6 months.

A B

I
NASOPHA RYNX AND PARAPHARYNGEAL SPACEI

Findings
In the lateral radiograph of the midface, there is a soft tissue mass which bows the
posterior wall of the maxillary sinus anteriorly (Fig. IS-A). The axial CT with
contrast through the same level shows an enhancing mass arising from the region of
the pterygopalatine fossa and extending to the infratemporal fossa on the right (Fig.
1 S-B). An axial T l -weighted M R scan of a similar patient shows a large, intermedi
ate signal intensity lesion occluding the nasal cavity and nasopharynx with widening
of the left pterygopalatine fossa. There are multiple punctate areas of signal void
throughout the mass (Fig. I S-C).

Differential Diagnosis: Expansile


Nasopharyngeal Mass
Juvenile angiofibroma
Chordoma
R h abdomyosarcoma
Schwannoma of the 5th cranial nerve
Nasopharyngeal carcinoma
Angiomatous polyp

Diagnosis
Juvenile nasopharyngeal angiofibroma UNA)

Discussion
Epidemiology
Juvenile nasopharyngeal angiofibroma is a benign tumor of the nasopharynx com
posed of fibrous connective tissue and an abundance of endothelium-lined vascular

V - ./. r

o
I NASOPHARYNX AND PARAPHARYNGEAL SPACE

spaces. The name of the disease is derived from the fact tbat it occurs during ado
lescence, almost exclusively in boys. Some experts suggest that finding this lesion in
a female patient should be an indication for a DNA gender test.

Pathophysiology
Although the pathogenesis is not fully understood, the tumor is thought to be a mal
formation of vascular tissue. TIlese tumors are believed to originate in the postero
lateral wall of the nasal cavity near or within the sphenopalatine foramen. JNAs
tend to follow vessels as they grow through natural foramina and fissures, expand
ing as they grow. The vascular supply is primarily via branches of the external
carotid artery. The characteristic angiographic appearance is shown in Figure I S-D.
With intracranial extension, the internal carotid artery may also supply portions of
the mass. Extension may commonly be found in the infratemporal fossa (via the
pterygomaxillary fissure), orbital apex (via the inferior orbital fissure), and. less of
ten, into the middle cranial fossa via foramen rotundum.

Clinical Findings
The most common presenting complaint is nasal obstruction and epistaxis.

Imaging Findings
Figure lS-E is an axial MR image of a patient who has a large JNA with intracra
nial extension. The punctate and serpentine areas of signal void seen on MRI sug
gest a highly vascular lesion. Widening of the pterygopalatine fossa is perhaps the
most helpful distinguishing feature of JNA (occurring in over three quarters of pa
tients in one study). cr optimally demonstrates the bony changes usually with re
modeling and thinning of bone, without frank destruction. Diagnostic angiograms
are no longer routinely done. Preoperative embolization is performed on almost all
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

lesions. Accurate radiologic diagnosis is important since biopsy of a JNA can lead to
PEARLS/PITFALLS severe hemorrhage.
Nasal mass with nosebleeds is a
common symptom in a young Management
male with JNA.
Surgery is the treatment of choice for these lesions with preoperative embolization
JNA typically widens the ptery in nearly all cases. Radiation therapy is used in selected cases that are not amenable
gopalatine fossa. to surgical resection.

JNA may show a vascular pat


tern on imaging studies. Suggested Readings
The clinician must beware not to Antonelli, A.R., Cappiello, 1., Di Lorenzo, D., Donajo, CA., Nicolai, P, Orlandini, A.
fail to perform preoperative em Diagnosis, staging, and treatment of juvenile nasopharyngeal angiofibroma. Laryn
bolization. goscope 97: 1 31 9-1325, 1 987.
B rooker, D.S., Kenny, B., Gibson, R.G., Primrose, W.J. Juvenile nasopharyngeal
A biopsy of a JNA in uncon
angiofibroma in a static population: the implications of misdiagnosis. Clinical Oto
trolled fashion can lead to severe
laryngology 1 4:497-502, 1 989.
hemorrhage.
Economou, T.S., Abemayor, F., Ward, P.H . Juvenile nasopharyngeal angiofibroma:
an update of the UCLA experience, 1 960-1985. Laryngoscope 98: 1 70-175, 1988.
Jamal, M.N. Imaging and management of angiofibroma. European A rchives of Oto
Rhino-laryngology 251 (4):241-5, 1 994.
Lloyd, GA. Phelps, P.D. Juvenile angiofibroma: imaging by magnetic resonance, cr
and conventional techniques. Clinical Otolaryngology 1 1 :247-259, 1 986.
Ungkanont, K., Byers, R.M., Weber, R.S., Callender, D.L., Wolf, P.F., Goepfert, H.
Juvenile nasopharyngeal angiofibroma: an update of therapeutic management.
Head and Neck 18(1 ) :60-6, 1 996 Jan-Feb.
1 NASOPHARYNX AND PA RAPHARYNGEAL SPACE

Case 19
Clinical Presentation
A 50-year-old female patient with a known malignancy was referred for evaluation
of a new parapharyngeal space mass.

A B

182
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

D E

Findings
A mass is demonstrated on Tl and T2 weighted axial images (Figs. 19-A-19-B). I t is
located in the poststyloid compartment of the left parapharyngeal space with ante
rior displacement of the carotid arteries. There are many serpentine structures, seen
as signal void, within and around the mass suggesting hypervascularity. Because of
the question of metastasis, under M R I guidance a 22 g needle was passed into the
lesion and cells were obtained for cytologic study (Fig. 1 9-C).

Differential Diagnosis: Post-styloid


Parapharyngeal Space Mass
Glomus vagale
Glomus jugulare
Schwan noma
Meningioma
Metastasis

Diagnosis
Glomus vagale

Discussion
Paragangliomas and schwannomas are the two most common diagnoses for a post
styloid parapharyngeal space mass (Fig. 1 9-D-normal, Fig 1 9-E-cross hatched
83 1
I NASOPHARYNX AND PARAPHARYNGEAL SPACE

mass-after Dr. Hugh Curtin) Vagal paragangliomas represent approximately 1 0%


of incidence of all branchial paragangliomas and show a female dominance of 2.5 : l .
Vagal and carotid body tumors are more likely to be multifocal than paragan
gliomas in other head and neck locations.

Epidemiology
There is a tendency of multicentricity of tumors ( 1 0%), especially in a patient with
a carotid body showing familial tendency (26% ). The incidence 01' true malignancy
showing metastasis varies from 2-6% . The biologic behavior or natural course of the
tumors is not clearly correlated with histologic appearances.

Pathophysiology
A paraganglioma is a slowly growing neuroendocrine neoplasm that arises from
neural crest derivatives. T n the case of glomus vagale, this is usually from the nodose
ganglion of the vagus nerve. Chemodectoma, glomus tumor, nonchromaCfin para
ganglioma, and neurocristopathic tumor are other names given to this lesion.
Precapillary arteriovenous shunts and nonchromaffin cells are characteristic of the
histologic appearance of these tumors.
Paragangliomas of the head and neck can be classified by their locations. Tumors
of carotid and vagal locations are most frequent. Tympanic and jugular tumors are
less frequent. Histologically, sheets of tumor cells are often divided into ball-like
clusters ("Zelballen") separated by thin fibrovascular septa.

Clinical Findings
The most frequent presenting symptom is a mass in the cervical or pharyngeal area,
and 30% of patients have cranial nerve impairment that frequently manifests itself
by neck hypoesthesia and pharyngeal pain. A woman in her forties with a painful
pharyngeal mass, associated with Xth and X l l th nerve impairment has a very high
likelihood of having a vagal paraganglioma. This tumor may arise from paragan-

F
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

glions in two locations, intravagal and extravagal, and an intravagal tumor produces
PEARLS/PITFALLS rapid vagal impairment.
Glomus vagale has a "salt and
pepper" appearance on M R I . I m aging Findings
A mass i s located i n t h e post A vagal tumor is easily delineated on CT and M R I with its well-encapsulated and
styloid parapharyngeal space. hypervascular nature. The mass effect on adjacent vessels is quite different from that
of a carotid body tumor and may be a clue for differentation. The internal carotid
Performing a biopsy or surgery artery is displaced anteriorly by a vagal tumor (Fig. 1 9-F) whereas posterolateral
without preoperative emboliza displacement of the artery with separation from the external carotid artery is char
tion could cause bleeding. acteristic of a carotid body tumor. Conventional angiography is no longer routinely
used For diagnosis but is valuable for preoperative embolization. Some investigators
Synchronous or metachronous
are beginning to use color Doppler ultrasound to help characterize these lesions.
lesions may be detected in pa
Fine needle aspiration as demonstrated in this case is rarely used to make the diag
tients with glomus vagale.
nosIs.
A glomus vagale is generally located more cephalad than a carotid body tumor
which is characteristically located at the carotid bifurcation. U nlike glomus jugulare
tumors which are located at the jugular bulb, glomus vagale tumors are more
caudad.
Schwannomas may occur in a similar post styloid parapharyngeal space location
with similar displacement of the carotid vessels as a glomus vagale. However, they
are much less vascular without the "salt and pepper" appearance of the paragan
glioma.

Management
Surgery is the primary treatment for these lesions with radiation therapy used as an
alternative for complex cases. Preoperative embolization in order to control bleed
ing is the rule.

Suggested Readings
Batsakis, J.G Tumors of the head and neck: clinical and palhoiogical considerations,
2nd ed. Baltimore: Williams and Wilkins, 1 979.
Cole, J.M., Beiler, D. Long-term results of treatment for glomus j ugulare and glomus
vagale tumors with radiotherapy. Laryngoscope 1 04( 1 2 ) : 1 46 1 -5, 1 994 Dec.
Jansen, J.e., Baatenburg de Jong, R.J., Schipper, 1, van der Mey, A.G, van Oils, A.P.
Color Doppler imaging of paragangliomas in the neck. Journal of Clinical Vilra
sOllnd 25(9):481-5, 1 997 Nov-Dec.
Lasjaunias, P., Berenstein, A. Surgical neuroangiography, vol 2. Endovascl.llar lreal
men! of craniofacial lesions. New York: Springer-Verlag, 1 987.
Leverstein, H., Castelijns, lA., Snow, GB. The value of magnetic resonance imaging
in the differential diagnosis of parapharyngeal space tumours. Clinical OlOlaryngol
ogy 20(5):428-33, 1 995 Oct.
Som, P.M., Sacher, M., Stollman, A.L. Common tumors of the parapharyngeal space:
refined imaging diagnosis. Radiology 1 69:8 1 -85, 1 988.
Zak, EO., Lawson, W. The paraganglionic chemoreceplor syslem. Physiology, pmhol
ogy, and clinical medicine. New York: Springer-Verlag, 1 982.
D PA RAPHARYNGEAL PA E

Case 20
Clinical Presentation
A 45-year-old female who had an M R I for unrelated reasons.

A B

Findings
A well-circum cribed mas with a homogeneously hypointensc center i een in the
right poststyloid parapharyngeal space on the TI W images (Fig. 20-A). 11le TIW
images how a more heterogeneous interior. True flow void do not appear to be
present ( Fig. 20-B). The mass extends from the level of the soft palate to the mid
oropharynx. The rna di place the carotid artery medially and the medial ptery
goid muscle anteriorly. There i no evidence of the infiltration of the urrounding
struct ures.

Differential Diagnosis: Post-styloid


Parapharyngeal (Carotid) Space Mass
G lomu vagale
Glomu jugulare
Schwan noma
Meningioma
Metastasis

Diagnosis
chwannoma in the poststyloid compartment of the parapharyngeal pace.

Discussion
Knowledge of the contents of the para pharyngeal space is es ential in determining
the differential diagnosis of masses in this region. The parapharyngeal pace is lat-

86
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

C D

eral to the pharyngobasilar fascia that surrounds the nasopharynx. The parapharyn
geal spacc can be further subdivided into prestyloid and poststyloid compartments.
TIlese compartments are separated by fascia containing the tensor veli palatini mus
cle fibers. The medial pterygoid muscle and fascia form the lateral border of the
prestyloid compartment and separate the prcstyloid parapharyngeal space from the
masticator space. The prestyloid space contains mostly fat with occasional salivary
gland rests. Salivary gland tumors are rare in the poststyloid compartment, but are
the most common tumor found in the prestyloid compartment.
TIle poststyloid compartment contains the carotid artery, jugular vein, and the
lower four cranial nerves. Some experts refer to this area as the carotid space. Post
styloid lesions are generally related to the great vessels, cranial nerves (neural
sheath tumors and paragangliomas) , or lymph nodes. Paragangliomas and schwan
nomas are the two most common post styloid parapharyngeal space masses.

Epidemiology
Schwannomas are common, usually solitary, benign tumors that peak during the
third through sixth decades of life. Multiple lesions may be seen in patients with
neurofibromatosis.

Pathophysiology
Schwanomas are cncapsulated neoplasms of the Schwal1l1 cell. They are usually be
nign. Two tissue types predominate; Antoni A (cellular) and Antoni B (myxoid)
componcnts.
Malignant forms may occur. One malignant type. the melanotic schwannoma, has
cells that contain heavily pigmented cytoplasm. with abundant melanin pigment.
TIle effects of the paramagnetic melanin may be visible on MR images. Figures
20-C & 20-0 show a patient with a melanotic schwanoma of the poststyloid para
pharyngcal spacc. Thc TI W non contrast image (Fig. 20-C) shows TI shortening in
the mass. The TIW image ( Fig. 20- 0 ) also shows TI shortening due to the melanin.
Immunohistochemical stains are often positive for S- 1 00 protein and anti-human
melanoma HMB45 marker.
I NASOPHARYNX AND PARAPHARYNGEAL SPACE

Clinical Findings
PEARLS/PITFALLS
Schwannomas are one of the Symptoms are usually related to compression of the adjacent nerves and other
most common poststyloid para structures. It is common for these lesions to present as asymptomatic parapharyn
pharyngeal space masses. geal space masses.

Schwannomas show an interme Imaging Findi ngs


diate signal on T1 W and a high
signal on TZW M R I images. Both M R I and CT are excellent for delineating tumor extension. Schwannomas on
T l -weighted images are of intermediate to low signal intensity and enhance in
A schwannoma is a benign, slow tensely with contrast. They tend to have a heterogeneously high intensity signal on
growing mass. TI-weighted images. I f serpentine flow voids or a salt and pepper appearance is pre
sent, a glomus tumor (paraganglioma) should be considered.
A schwannoma can be confused
with a glomus tumor.
----
M an agement
Surgery is the treatment of choice for these lesions. To approach the parapharyngeal
space a transcervical approach is most often used. This minimizes the morbidity fol
lowing manipulation of the facial nerve that may be present with a transparotid ap
proach.

Suggested Readings
Curtin, H. Separation of the masticator space from the para pharyngeal space. Radi
ology ] 63: 1 95-204, 1 987.
Leverstein, H., Castelijns, 1. A. , Snow, G.B. The value of magnetic resonance imaging
in the differential diagnosis of parapharyngeal space tumours. Clinical Otolaryngol
ogy 20(5):428-33, 1 995 Oct.
Marco, v., Sirvent, 1., Alvarez Moro, 1., Clavel, M., Muntal, M.T., Bauza, A. Malignant
melanotic schwannoma fine-needle aspiration biopsy findings. Diagnostic
Cytopathology 18 4:284-6, 1 998 Apr.
Pensak, M.L., Gluckman, lL., Shumrick, K.A. Parapharyngeal space tumors: an
algorithm for evaluation and management. Laryngoscope I 04(9):1 1 70-3, 1 994 Sep.
Som, P.M., Curtin, H.D. Lesions of the parapharyngeal space. Role of MR imaging.
Otolaryngologic Clinics of North A merica 28(3):5 1 5-42, ] 995 Jun.
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

Case 21
Clinical Presentation
A 59-year-old male presented with a history of progressive hearing loss on the left
side. The patient also noted bilateral neck swelling.

A B

Findings
A T l -weighted axial sequence of the nasopharynx (Fig. 2 1 -A) shows diffuse soft
tissue infiltration of the vault of the nasopharynx with low to intermediate signal in
Tl -weighted images. There is obliteration of the fossae of Rosenmtiller and also
opacification of the mastoid air cells on the left side. Extension of this lesion beyond
the pharyngobasilar fascia is present on left. The longus colli muscle is invaded. Mul
tiple bilateral lymph nodes are presented in the neck (Fig. 2 1 -8). Incidental note is
also made of medial location of the carotid arteries.

Differential D iagnosis: Nasopharyngeal Mass


Carcinoma
Lymphoma
Rhabdomyosarcoma
Adenoidal tissue
Tornwaldt cyst
Retropharyngeal adenopathy

Diagnosis
Nonhodgkins lymphoma of the nasopharynx.
I NASOPHARYNX AND PARAPHARYNGEAL SPACE

c o

Discussion
Epidemiology
Nonhodgkin's lymphoma is the second most common histologic diagnosis (after
squamous cell carcinoma) made in nasopharyngeal malignancies. l1le lesion usually
appears as smooth. exophytic, submucosal. and non ulcerative. One should be aware
that extranodal lymphoma in the head and neck region increases the probability of
systemic disease below the clavicle or diaphragm. and the majority of patients have
involvement of cervical lymph nodes. The tonsils are most often involved, followed
by the nasopharynx and the lymphoid tissue of the base of the tongue.

Clinical Fi ndings
In the nasopharynx. symptoms related to the ear usually lead to early detection of
the tumor when it is fairly small because of the proximity of the lesion to the orifice
of the eustachian tube. Figure 2 1 -C shows a nonhodgkins lymphoma in another pa
tient. The T I W MR image depicts left mastoid nuid and left sided nasopharyngeal
mass invading the prevertebral muscles. A large right retropharyngeal node is pre
sent ( . Figure 2 1 -D is an MRI of a patient with non hodgkins lymphoma of the na
sopharynx. A bulky left sided nasopharyngeal mass is present with left sided ob
structive ear disease.

Pathophysiology
Of head and neck lymphomas 75% are non hodgkin and 25% are hodgk in. The
nodal form is most common for both types. l1le majority of cases of extranodal lym
ph om as of the head and neck are histiocytic lymphomas and lymphocytic lym
phomas of the diffuse type. Involvement of extranodal sites of the head and neck by
Hodgkin's disease is rare. Extranodal lymphoma may be either lymphatic
( Waldeyer's ring) or extra lymphatic. WaJdeyer's Ring of lymphoid tissue consists
of: ( I ) Adenoids ( nasopharyngeal lymphoid tissue). ( 2 ) Palatine tonsils. and ( 3 ) Lin
gual tonsils. Most of the patients with lymphoma limited to the head and neck are
in the fourth to eighth decades of life.
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

I maging Findings
PEARLSIPITFALLS
Lymphoma of the nasopharynx The pharyngobasilar fascia is the superior fascial continuation of the constrictor
shows up on imaging studies as a muscles of the pharynx. It separates the pharyngeal mucosa from the deeper mus
bulky mass with extensive culature and serves to hold the airway open as it attaches to the skull base. It is com
adenopathy. posed of the deep layer of the deep cervical fascia.
On MRI or contrast enhanced CT scanning it can often be identified by its "whale
Of head and neck lymphomas, tail" configuration. Figure 2 1-E depicts a right sided nasopharygeal lymphoma [> 1
75% are nonbodgkin type. in another patient seen on contrast enhanced CT. The pharygobasilar fascia is lat
erally violated by the tumor but is intact posteriorly [->1 and on the left. It pro
Chemotherapy and radiation vides an important imaging landmark to help differentiate benign from more ag
therapy are the primary modes gressive nasopharyngeal masses. Normal adenoidal lymphoid tissue does not cross
of treatment for lymphoma. this boundary while more malignancies such as carcinoma and lymphoma and ag
gressive infections do. Lymphoma of the nasopharynx may be indistinguishable on
Lymphoma of the nasopharynx imaging studies from nasopharyngeal carcinoma. A bulky nasopharyngeal mass
may be indistinguishable on with extensive adenopathy should suggest the diagnosis. Similar findings (na
imaging studies from carcinoma sopharynx mass and adenopathy) may be seen with H I V positive patients and in
of the nasopharynx. younger patients with infectious mononucleosis.

Care should be taken to avoid


mistaking a lymphoma for nor Management
mal adenoidal tissue.
Radiation therapy a n d chemotherapy are t h e primary modes of treatment for lym
phoma.

Suggested Readings
Braull, I . F. M R I of the nasopharynx. Radio Clin North Am 27:327, 1989.
Kieserman, S.P., Stern, 1. Malignant tran formation of nasopharyngeal lymphoid hy
perplasia. Otolaryngology and Head and Neck Surgery 1 1 3(4) :474-6, 1 995 Oct.
Yanagisawa, E., Citardi. M.1. Endoscopic view of malignant lymphoma of the na
sopharynx. Ear, Nose, and Throat iOLirnaI 73(8):5 14-6, 1 994 Aug.
D PA R A P H A RY N G E A L PA E

Case 22
Clinical Presentation
A 69-year-old female pre ented \ ith a rna in her right lateral neck. On phy ical
exam a oft. non tender. mobile rna was palpated underneath the right mandibular
angle and in the right ubmandibular reg.ion.

A B

c D

Findings
An M R I wa done of the neck. Figures 22-A and 22-8 show the a ial TI W I . Figure
22- depict the axial T2WI. aDd Figure 22-D how the enhanced axial TI W I . M R I

92
NASOPHARYNX AND PARAPHARYNGEAL SPACE!

of the neck shows a 6 em hour glass shaped mass centered in the right prestyloid
parapharyngeal space (PPS), extending posterolaterally into the parotid space
through an enlarged stylomandibular notch. Medially, it bulges into the right lateral
wall of the oropharynx. This well defined lesion causes mass effect but does not
appear to infiltrate the adjacent structures. The mass is predominantly homoge
neous except for the presence of l i near septations within the most lateral and infe
rior components. The signal follows that of the subcutaneous fat, although it appears
slightly hyperintense to the remainder of the subcutaneous fat on T2W images.
There was no evidence of cervical lymphadenopathy ( Figs. 22-A, 22-B and 22-C).

Differential Diagnosis: Fatty Lesions in


the PPS
Lipoma
Angiolipoma
Dermoid cyst/teratoma
Hibernoma
Lipoblastoma/Lipoblastomatosis (pediatric patients)
Benign symmetric lipomatosis (Madelung disease)
Liposarcoma

Diagnosis
PPS Liposarcoma

Discussion
Epidemiology
Fatty tumors are common in the body, but only l 3% occur in the head and neck re
gion. I n the head and neck, they can be subcutaneous or submucosal and the most
common location is in the subcutaneous soft tissues of the posterior neck.
They tend to grow with age and are more common in obese people. Although
they e nlarge with increasing body weight, they do not decrease in size with weight
loss. Age of presentation and location varies with gender. In males, they tend to
occur in the head and neck region after the 6th decade of life, whereas in females
they tend to present earlier (4th decade) and are usually found below the clavicles.
Liposarcomas are much less common than lipomas and are exceedingly rare in
the head and neck. The ratio of liposarcoma to lipoma has been estimated as 1 : 20.
The incidence of l iposarcomas above the clavicles is low, around 5 % , and there are
only 80 cases described in the literature. There is a slight male predominance with a
male to female ratio of 3 : 2. The age range is broad with a peak incidence in the 6th
decade.

Pathophysiology
Liposarcomas are malignant tumors which arise from lipoblasts or totipotential mes
enchymal cells, usually adjacent to muscle or fascia. They are much more common in
the deep-seated fat, rarely arising in the subcutaneous soft tissues. They do not origi
nate from pre-existing lipomas. The histologic classification of these tumors is con
troversial as the biological behavior of adipose tissue neoplasms depends not only on
morphology but also on location. According to some experts, fatty neoplasms arising
in deep fatty spaces tend to behave in a more aggressive fashion than their histologic
counterparts do in subcutaneous fat. Therefore, deep adipose tissue neoplasms should
be considered malignant even in the absence of cytologic features of malignancy.
Depending on their histologic features, liposarcomas are usually classified into
four categories: well-differentiated, myxoid, round cell and pleomorphic. Whereas
ASOPHARYNX A D PA RAPHARYNGEAL SPACE

the former two categories rarely metastasize and have a high five year survival rate,
the latter show a high recurrence rate and a high rate of distant metastasis.

Clinical Fi ndings
Lipomatous lesions in the PPS present as any other mass in the same location. They
cause few. if any symptoms until they become large and compress adjacent neu
rovascular structures or compromi e the airway. llley may grow laterally into the
parotid or submandibular spaces and present, as in this case, as a neck mass.
On physical exam, detection of a fatty PPS mass can be difficult as this neck space
usually contains primarily fat. Usually, a medial bulge of the lateral pharyngeal wall
is the only visible sign and often is only detected after the lesion has reached 2.5 to
3cm in size.
Earaches, tubal dysfunction, fullness or foreign body sensation in the oropharynx
may be the presenting symptoms and are nonspecific. When large enough, these le
sions may cause airway obstruction. When palpable, they manifest as soft. lobulated
masses that may or may not be mobile. Rarely, neurologic deficits due to compres
sion of neurovascular structures may ensue. Differential diagnosis of a PPS mass in
cludes abscess, atypical branchial cleft cyst, benign and malignant salivary gland rest
tumors, mesenchymal tumors, such as lipoma and hemangioma and, less commonly.
neural sheath tumors. Pseudomass, due to asymmetry of the pterygoid plexus.
should also be considered.

I magi ng Fi ndings
Imaging is crucial in the diagnosis of lesions seated in the deep spaces of the neck,
which are not accessible to clinical examination. It is common for a PPS mass to be
detected as an incidental finding in a study performed for unrelated reasons. Both
cr and MRJ are well-suited to detect masses in the PPS and may be diagnostic
when a lipomatous lesion is seen.
Although the imaging appearance of lipomas is pathognomonic on cr scans. li
posarcomas may be difficult or impossible to diagnose based on imaging, as they
may mimic any other soft tissue tumor (malignant fibrous h istiocytoma. lymphoma,
plasmocytoma, rhabdomyosarcoma). Depending on their histologic subtype they
may contain varying amounts of fat intermingled with other soft tissue components.
Therefore, the density of the lesion depends on its composition and vascularity. and
may be similar to that of muscle.
On contrast enhanced cr scans, liposarcomas often manifest as heterogeneous
masses containing a combination of fat and enhancing soft tissue elements. llle at
tenuation value of fat characteristically varies between - 65 and - 1 00 Hounsfield
units. It has been stated that the attenuation value of fat seen in liposarcomas is
higher than that of subcutaneous fat.
When liposarcomas are exclusively composed of fat, demonstration of contrast
enhancement is useful in differentiating them from lipomas, which usually do not
enhance. However, angiolipoma, another benign fatty tumor, may show contrast en
hancement and be indistinguishible from a liposarcoma. Fibrous septations may be
seen in both benign and malignant fatty lesions. I ll-defined margins and infiltration
of adjacent structures are the most reliable differentiating features, but are incon
sistent.
The MRI appearance of liposarcomas in the head and neck is not well docu
mented due to the rarity of this entity. However, it has been reported that the ap
pearance of liposarcomas differs from subcutaneous fat in that the signal character
istics of fat within a liposarcoma tends to be of lower signal intensity on T I W
sequences and higher signal intensity on TIW sequences.
M R I is less specific than cr in diagnosing fatty tumors, because the signal char
acteristics may overlap with those of mature hematomas. Several techniques have
been used to separate these entities including calculation of T l W and TIW relax-

194
NASOP HARYNX AND PARAPHARYNGEAL SPACEI

ation times, gradient echo imaging and several techniques of fat suppression. How
PEARLS/PITFALLS ever, even using these techniques, a definite diagnosis is not always possible because
Fatty tumors in the deep spaces hemorrhage within a fatty lesion is common. The most reliable technique to detect
of the neck should be considered fat on M R I is frequency selective fat suppression imaging ( FATSAT) in which a spe
to be liposarcoma until proven cific saturation pulse in the resonant frequency of fat is given.
otherwise. The advantages of M R I in the evaluation of liposarcomas include a better delin
eation of the neoplasm, and demonstration of the presence or absence of cleavage
Demonstration of contrast en planes between the tumor and adjacent structures, due to its multiplanar capability.
hancement and iU-defined mar Metastatic lymphadenopathy is rarely seen.
gins with infiltration of adjacent In the pediatric patient, lipoblastoma and Iipoblastomatosis should be consid
structures are the most reliable ered. These tumors have a similar imaging appearance as liposarcoma and, although
differentiating features, but are rare, are more common than liposarcomas in the pediatric age group. The differen
inconsistent. tial diagnosis is important as lipoblastomas are considered hamartomatous lesions
and should be managed accordjngly. Both discrete and diffuse forms have been de
Care should be taken not to con scribed. Other fatty tumors that show contrast enhancement and soft tissue compo
sider all fatty tumors with well nents include hibemomas, angiolipomas and teratomas.
defined margins as benign lipo
mas.
Management
Care should be taken not to ex
Liposarcomas of the head and neck should be managed surgically by wide local
clude liposarcoma from the dif
excision. Tn the PPS several approaches can be used depending on the size of the
ferential diagnosis when the tu
lesion and surgeon's preference. These include transcervical, submandibular.
mor is predominantly soft tissue
transparotid, transoral, and infratemporal approaches. In selected cases adjuvant
density/intensity.
radiotherapy may be of benefit. Palliative radiation therapy may prolong survival in
inoperable cases. Neck dissections are not performed routinely as cervical metasta
sis are rare.

Suggested Reading
Abdullah, BJ., Liam, C.K . , Kaur, B., Mathew, K.M. Parapharyngeal space lipoma
causing sleep-apnoea. British Journal of Radiology 70(838):1 063-5, 1997 Oct.
Collins, M.H., Chatten, 1. Lipoblastomallipoblastomatosis: a clinicopathologic study
of 25 tumors. American Journal of Surgical Pathology 2 1 ( 1 0): 1 1 3 1-7, 1 997 Oct.
Elango, S. Parapharyngeal space Ijpoma. Ear, Nose, and Throat Journal 74( 1 ):52-3,
1 995 Jan.
Kraus, M.D., Guillou, L., Fletcher, C.O. Well-differentiated infla=atory lipo
sarcoma: an uncommon and easily overlooked variant of a common sarcoma. Amer
ican Journal of Surgical Pathology 2 1 (5):518-27, 1 997 May.
Saddik, M., Oldring, OJ., Mourad, W.A. Liposarcoma of the base of tongue and ton
sillar fossa: A possibly underdiagnosed neoplasm. A rchives of Pathology and Labo
ratory Medicine 1 20(3):292-5, 1 996 Mar.
Stewart, M.G., Schwartz, M.R., Alford, B.R. Atypical and malignant lipomatous le
sions of the head and neck. A rchives of Otolaryngology and Head and Neck Surgery
1 20 ( 1 0 ) : 1 15 1-5, 1 994 Oct.
Case 23
Clinical Presentation
three-year-old female pre ented with history of chronic na al ob truction.

A B

c D

1 96
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

E F

Findings
An axial cr section through the basiphenoid (bone window) (Fig. 23-A) shows a
large soft tissue mass occupying the nasopharynx, nasal vault and nasal cavity, ex
tending through the left sphenopallatine foramen into the left pterygopallatine
fossa. There is bony destruction involving the left basisphenoid, medial wall of the
left maxillary sinus and nasal septum. An axial cr section through the low na
sopharynx (soft tissue window) (Fig. 23-B) shows a large left-sided retropharyngeal
nodal conglomerate that appears separate from the midline nasopharyngeal mass.
In an axial TIW M R image (Fig. 23-C) at tbe same level as the CT section showing
the large midline nasopharyngeal mass is seen as separate from bilateral retropha
ryngeal lymphadenopathy ( Fig. 23-0). The coronal enhanced T1 W MR image ( Fig.
23-0) shows the craniocaudal extent of the mass and homogeneous contrast en
hancement. There is no evidence of meningeal spread or intracranial invasion
through the cranial nerves foramina ( Fig. 23-E). The sagittal Tl W MR image (Fig.
23-F) shows a bulky soft tissue mass occupying the nasopharynx, nasal cavity, nasal
vault and oropharynx, obstructing the choana and oropharyngeal airway.

Differential Diagnosis: "Aggressive"


Nasopharyngeal Mass in a Child
Rhabdomyosarcoma (includes other soft tissue sarcomas such as fibrosarcoma,
angiosarcoma, mesenchymal chondrosarcoma, malignant mesenchymoma)
Lymphoma
Chloroma
Langerhans' cell tumor
Nasopharyngeal carcinoma
Metastatic neuroblastoma
Esthesioneuroblastoma
Hemangiopericytoma
Minor salivary gland malignancy
Aggressive infection (fungal, TB, Cocci)
I NASOPHARYNX AND PA RAPHARYNGEAL SPACE

Diagnosis
Nasopharyngeal rhabdomyosarcoma (embryonal type)

Discussion
Epidemiology
Rhabdomyosarcoma is the most common malignant soft tissue tumor in children
under 1 5 years of age. In the head and neck, it is the most common sarcoma and the
third most common malignancy following lymphoma and retinoblastoma. Two
peaks of prevalence are noted, one between 2 and 5 years of age and the other be
tween 1 5 and 20 years. Seventy percent of tumors occur before age 10. In the head
and neck, the most common location is the orbit followed in decreasing order of fre
quency by the nasopharynx, paranasal sinuses, and middle ear cavity.

Pathophysiology
Rhabdomyosarcoma is a malignant mesenchymal tumor that originates from undif
ferentiated mesenchymal cells, the precursors of skeletal muscle. The tumor was first
described in 1 854, by Weber and further characterized by Stout. who described the
histology of this tumor more precisely. 0 predisposing factors are recognized, but
a chromosomal translocation is noted in almost half of the children with rhab
domyosarcoma.
Pathologically, three main types of spindle cell tumor are recognized. The most
common is the embryonal type, which comprises 75% of all cases and is the form
most commonly encountered in the head and neck. This cell type is een in younger
children and carries a better prognosis, with a 5 year survival rate of 65% after com
bined treatment with radio- and chemotherapy. H istologically, the embryonal cell
type may be confused with anaplastic carcinoma, large cell lymphoma. esthesioneu
roblastoma and melanoma. Botryoid rhabdomyosarcoma is not histologically dif
ferent from the embryonal type and its name is derived from its macroscopic grape
like appearance. The second most common, the alveolar type ( I S% ). tends to occur
in older patients (second decade) . I t spreads to lymph nodes and carries a worse
prognosis with only a 2% S year survival rate. Finally, the least common form is
pleomorphic rhabdomyosarcoma, considered to be an adult form as only 6% of
cases occur before age 6. The most important diagnostic criterion is the identifica
tion of rhabdomyoblast . within the tumor. D i fferent stains help to characterize the
tumor, such as desmin, myoglobin and muscle specific actin.

Clinical Findings
Clinical presentation depends on the location of the tumor. asopharyngeal rhab
domyosarcomas tend to grow silently and present as large destructive masses at a
late stage. Very young children may present with difficulty breathing and eating and
failure to thrive. Older children present with sinonasal symptoms similar 10 those of
sinus infection which further delay the diagnosis. asal obstruction. rhinorrhea. and
epistaxis are the most common presenting symptoms. Hearing impairment due to
obstructive serous otitis is also common. Local pain appears late in the course of the
disease and usually indicates bony destruction. Cranial nerve and other neurologic
signs and symptoms imply skull base invasion with intracranial extension.
ENT examination usually discloses a nasopharyngeal mass which may extend
into the nasal cavity. Because the tumor itself is submucosal, the clinical findings are
non-specific and may mimic hypertrophic turbinates and hypertrophic adenoids.
common findings in pediatric patients.
Tumor subsite and tumor. node, metastasis (TN M ) staging have important impli
cations in management and prognosis. Subsites are classified into orbital. para-

198
NASOP H A RYNX AND PARAPHARYNGEAL SPACEI

meningeal, and miscellaneous tumors. The parameningeal group includes the na


sopharynx, paranasal sinuses, pterygopallatine fossa, infratemporal fossa, and tem
poral bone. The close proximity to the skull base and meninges makes this group of
tumors particularly prone to intracrarual extension and meningeal spread, worsen
ing the prognosis. The TNM classification for soft tissue sarcomas of the impacts and
Use Committee ( IUAC) divides these tumors into two groups according to their lo
cal invasiveness. Tl includes tumors confined to the muscle group of origin, 1'2 in
cludes tumors which have spread through the fascial planes outside the muscle
group of origin , and invading neurovascular structures or bone. Overall, lymph node
metastasis are seen in 50% of cases at presentation and distant metastasis in 58% .
The most common metastatic sites include the lungs, bone marrow and brain.

Imaging Findings
Imaging is crucial for the diagnosis, determination of local and distant tumor extent,
treatment planing and detection of local recurrence. Cross sectional imaging may
disclose the aggressive nature of a nasopharyngeal mass by showing deep invasion
and bone destruction, which can not be assessed on clirucal exam. This can differ
entiate adenoidal hypertrophy from more aggressive lesions. However, lack of
specificity in tissue characterization necessitates biopsy to confirm t he diagnosis.
On CT, the main findings include a bulky mass, isodense to muscle, with little to
moderate homogeneous contrast enhancement, expansion or erosion of skull base
foramina, or destructive bony changes of the skull base. The low yield of CT in soft
tissue characterization makes it difficult to separate tumor from post-obstructive
changes in the paranasal sinuses and mastoids, to detect meningeal spread, and sep
arate residual scar tissue from recurrent mass. Poor prognostic signs on CT include
skull base destruction and intracranial spread.
M R I is the modality of choice in evaluation of soft tissue tumors of the head and
neck. It allows multi planar delineation of the tumor, differentiation between the
tumor and post-obstructive inflammatory changes, and detection of meningeal and
cranial nerve spread in post-contrast images.
Rhabdomyosarcoma tends to be of intermediate signal intensity on Tl WI, hy
perintense on T2WI and enhances homogeneously. Cervical and retropharyngeal
lymphadenopathy should be surveyed.
Imaging follow-up should include a scan after completion of treatment, then
follow-up scans every 2 months during the first year, every 4 months for 2 years, and
once a year, thereafter. Imaging should also be performed any time symptoms ap
pear or a clinical exam disclnses new findings.
It has been demonstrated that the presence of a post-therapeutic residual mass
(defined as a region of soft tissue thickening at the original tumor site with en
hancement and absence of mass effect) is a poor prognostic sign heralding an in
creased probability of tumor recurrence. Differential diagnosis from other aggres
sive looking nasopharyngeal masses, such as lymphoma, Langerhans' cell tumor,
chloroma, nasopharyngeal carcinoma, metastasis from neuroblastoma and other
soft tissue sarcomas is not possible based solely on the imaging findings, and biopsy
is mandatory.

Management
The overall prognosis of rhabdomyosarcoma markedly improved, with the advent
of multidrug chemotherapeutic regimens and external beam radiotherapy, from a
55% to an 80% 5 year survival rate in patients with non metastatic disease. These
regimens had their major impact in the treatment of parameningeal tumors, not
amenable to curative surgery or requiring major craniofacial resections with inade
quate surgical margins. Surgery plays an important role in many patients. A sagittal
Tl W MR image (Fig. 23-F) shows the same patient after radiotherapy and
chemotherapy. There was complete resolution of the soft tissue mass.
ASOPHARYNX AND PARAPHARYNGEAL SPACE

PEARLS/PITFALLS Suggested Reading


A nasopharyngeal mass can of Gilles, R., Couanet, D., Sigal, R., De Baere, T. , Tardivon, A, Masselot, 1., Vanel. D.
ten be found in a child with Head and neck rhabdomyosarcomas in children: value of clinical and cr findings in
chronic sinus symptoms. the detection of loco-regional relapses. Clinical Radiology 49(6):41 2-5, 1 994 Jun.
Kowalski, L.P., San, c.l . Prognostic factors in head and neck soft tissue sarcomas:
Rhabdomyosarcoma can show analysis of 1 28 cases. Journal of Surgical Oncology 56(2):83-8, 1994 Jun.
signs of deep invasion.
Kraus, D.H., Saenz, N.C., Gollamudi, S., Heller, G., Moustakis, M., Gardiner, S., Ger
cr imaging may show bony de ald, w.L., Ghavimi, E, LaQuaglia, M.P. Pediatric rhabdomyosarcoma of the head
structive changes in the base of and neck. American Journal of Surgery 174(5):556-60, 1 997 Nov.
the skull and erosion of cranial Lee,J.H., Lee, M.S., Lee, B.H., Choe, D.H., Do, YS., Kim, K.H., Chin, S.Y, Shim, YS.,
nerves foramina. Cho, KJ. Rhabdomyosarcoma of the head and neck in adults: MR and CT findings.
American Journal of Neuroradiology 1 7 ( 10):1 923-8, 1 996 Nov-Dec.
Care must be taken not to mis
Lyos, A.T., Goepfert, H., Luna, M .A, Jaffe, N., Malpica, A Soft tissue sarcoma of the
take an aggressive nasopharyn
head and neck in children and adolescents. Cancer 77( 1 ): 1 93-200, 1 996 Jan I .
geal mass for adenoidal hyper
trophy. Odell, P.E Head and neck sarcomas:a review. Journal of Orolaryngology 25( 1 ):7-13.
1 996 Feb.
Care must be taken not to mis Park, Y W. Evaluation of neck masses i n children. Americall Family Physician
take post-obstructive changes i n 5 1 (8): 1 904- 1 2, 1 995 Jun.
the paranasal sinuses a n d mas Yang, W.T., Kwan, W.H., Li, C.K., Metreweli, C. Imaging of pediatric head and neck
toids for tumor extension. rhabdomyosarcomas with emphasis on magnetic resonance imaging and a review of
the literature. Pediarric Hematology and Oncology 14(3):243-57, 1 997 May-Jun.
Care must be taken not to miss
i n tracranial and meningeal
spread of the tumor.
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

Case 24
Clinical Presentation
A 42-year-old male presented with unilateral serous otitis media.

Findings
The axial Tl W MRI shows (Fig. 24-A) a right sided nasopharyngeal mass which in
vades the prevertebral musculature.

Differential D iagnosis: Nasopharyngeal Mass


Adenoidal tissue
Lymphoma
Carcinoma
Tornwaldt cyst

Diagnosis
Squamous cell carcinoma of the nasopharynx

Discussion
The nasopharynx remains an area that is occult to casual clinical examination. Its
proximity to the skull base makes cancers in this region particularly devastating.
ASOPHARYNX AND PARAPHARYNGEAL SPACE

Epidemiology
Malignancies of the nasopharynx can be divided into three groups. Seventy percent
are carcinomas and 20% are lymphomas. The remaining 1 0% are made up of a va
riety of lesions including adenocarcinoma, rhabdomyosarcoma, melanoma, adenoid
cystic carcinoma, and others.
Squamous cell carcinoma of the nasopharynx is a tumor not commonly seen in
the United States. However, in certain portions of the world, (Canton, China) it is
one of the most common cancers. Human leukocyte antigen A-2 and B-sin histo
compatibility loci have been identified as possible markers in the Chinese popula
tion for susceptibility to this disease. While the incidence of the disease in American
born Chinese decreases, it is still seven times higher than for other Americans.
Antibodies against Epstein-Barr virus have been associated with the undifferen
tiated form of nasopharyngeal carcinoma. Other risk factors for nasopharyngeal
carcinoma include nitrosamines (found in dry salted fish) and exposure to polycyclic
hydrocarbons (found in burned foods).

Pathophysiology
Carcinoma of the nasopharynx based on histopathology, is classified according to
the World Health Organization into three types. Type 1 is squamous cell carcinoma
which is similar to other lesions found in the upper aerodigestive system. Type 1 1
lesions have little or no keratin production and are referred to as a non keratinizing
carcinoma. Because of their similarity to urinary tract tumors they are sometimes
referred to as transitional cell carcinomas. Type I I I carcinomas include lymphoep
ithelioma, spindle cell and clear cell squamous carcinoma. While Type I and I I
lesions are usually found i n adults, type I I I lesions have a bimodal age distribution.

Clinical Findings
Nasopharyngeal carcinoma may present as a change in voice quality, impaired hear
ing, or a mass in the nasopharynx. Often the first sign of abnormality is the accu
mulation of fluid in the middle ear or mastoid caused by obstruction of the eu
stachian tube.

I J.Q?
NASOPHARYNX AND PARAPHARYNGEAL SPACEI

c D

Lymphadenopathy is also very common in nasopharyngeal carcinoma and is seen


in approximately 70% of patients at presentation. Nodal extent ion to the nodes of
Rouviere (lateral retropharyngeal nodes) is frequent. Figure 24-B shows large lat
eral retropharyngeal lymph nodes ( in a patient with squamous cell carcinoma of
the nasopharynx. A common presentation of nasopharyngeal carcinoma is a mass in
the nasopharynx and spinal accessory lymphadenopathy.

I m aging Findings
An M R I examination of the nasopharynx is indicated to: ( I ) delineate the extent of
a known malignancy, (2) search for the presence of the tumor in a patient with
symptoms or clinical findings, and (3) follow treated patients with a history of na
sopharyngeal cancer to rule out recurrence.
Figure 24-C shows a recurrent right sided nasopharyngeal carcinoma in a 21 year
old who had undergone radiation therapy for her original disease (note atrophic
changes on the contralateral side).
Anatomical routes hy which tumors extend from the nasopharynx to the middle
cranial fossa may be shown by imaging studies. By following the eustachian tube,
nasopharyngeal carcinoma can pass through the tough pharyngobasilar fascia at its
only natural defect, the sinus of Morgagni. From this point, a tumor may enter the
parapharyngeal space and extend to the floor of the middle cranial fossa by ex
tending along the course of the eustachian tube in the sphenoidal sulcus, following
it to the region of the foramen lacerum and ova Ie, and then extending into the cav
ernous sinus. Figure 24-0 shows a large nasopharyngeal carcinoma extending
through a widened foramen ovale to invade the middle cranial fossa. Note that the
internal carotid artery is encircled by the mass on this section.
Tumors high in the nasopharynx may also gain access to the middle cranial fossa
via either the pterygoid canal or sphenopalatine foramen. Through the pterygoid
canal, tumors may extend directly to the foramen lacerum, or erode through the thin
lateral wall of sphenoid sinus, entering the cavernous sinus. Through the
sphenopalatine foramen, tumors may extend directly to the pterygopalatine fossa,
inferior and superior orbital fissures, and into the cavernous sinus. I n cases of na-

1 03 1
I NASOPHARYNX AND PARAPHARYNGEAL SPACE

sopharyngeal carcinoma with neck metastases, MR images may show an extranodal


PEARLSIPITFALLS tumor abutting and surrounding the carotid artery and jugular vein.
The clinicians should search for
an occult nasopharyngeal mass Management
in patients presenting with
nodal extension to the posterior The role of surgery in this disease is limited to biopsy and staging. Radiation ther
trangle. apy is the standard treatment for nasopharyngeal carcinoma. Advanced lesions may
also require chemotherapy.
The clinician should identify in
tracranial extension and
adenopathy which may have Suggested Readings
treatment and prognostic signifi
cance. Teresi, L.M., Lufkin, R.B., Hanatee, W. MR imaging of the nasopharynx and floor of
the middle cranial fossa. Part 1 . Normal anatomy. Radiology 1 64:8 1 1 , 1 987.
Care should be taken not to miss Teresi, L.M., Lufkin, R.B., Hanatee, W. MR imaging of the nasopharynx and floor of
a nasopharyngeal mass in adult the middle cranial fossa. Part I I . Malignant tumors, etc. Radiology 164:81 7, 1 987.
patients presenting with unilat
eral serous otitis media.

Care should be taken not to con


fuse carcinoma with adenoidal
enlargement.
IV.

Neck, Thyroid, Parathyroid


ECK, THYROID, PA RATHYROI DI

Case 25
Clinical Presentation
An adult male patient presented with painles midline swelling adjacent to the hy
oid bone.

Radiologic Findings
CT cans revealed a thin walled midline cy tic mass adjacent to the hyoid bone
(Fig. 25-A).

Differential Diagnosis: Anterior Cystic


Neck Mass
Branchial cleft cyst
External laryngocele
Thyroglossal duct cy t
Necrotic lymph node
Ab ce s
ySlic hygroma
Dermoid
llHombosed anterior jugular vein
Aneurysm

Diagnosis
Thyroglossal duct cyst

Discussion
11lyroglossal duct cy ts are more frequently found in children. but they also may
present in adulthood. They can be found anywhere along the thyroglo sal tract, ex-
1 07 1
I NECK, THYROID, PARATHY ROID

pyramidal lobe thyroid

tending from the foramen cecum to the thyroid gland, and most frequently present
as anterior, midline or paramedian neck masses. Although they are reported most
commonly in the low to mid-neck, up to 20% occur above the hyoid bone. They may
be seen as a mass at the root of the tongue. The cyst is often associated with the body
of the hyoid bone, and in order to avoid a surgical recurrence, the body of the hyoid
bone should be removed along with the cyst.

Pathophysiology
The thyroglossal duct is an epithelial lined tubular structure extending from the
foramen cecum, around the inferior border of the hyoid bone to the pyramidal lobe
of the thyroid bed (Fig. 25-B). It is formed by the 3rd week of gestation and nor
mally involutes by the 1 0th week.
If any portion fails to involute, the secretory epithelial lining may cause a cyst to
be formed. Although these are congenital lesions, they often do not present until
there is inflammation or blocked drainage.

Clinical Findings
Most patients present with asymptomatic neck masses within the anterior triangle.
Fistulas are uncommon but can occur related to surgery or complicated infection.
Most cysts ( >75% ) are midline and are most common at or near the level of the
hyoid bone.

Imaging Findings
Thyroglossal duct cysts typically appear as a cystic thin-walled midline mass on CT
or M R T scanning. They may be intimately associated with the hyoid bone. Tby-

1 1 08
c o

roglossal ducI cy t' appear on as ell-circums ribed fluid-den it tructures in


\

the midline. The cy I are u ually large I at the b oid level but aJ lrack inferiorly
0

adjacent to tbe laryngeal kelelon Fig. 25- ).


On M R I tbe nuid content may be expected to demon trate tbe same anatomic
and gro morph logic features. yst nuid ill how both long Tl and 1'2 in appro
\

priately weighted pin echo image The ignal of the nuid depend on its content
(e.g protein. hemorrhage). The cy t rim ill be nonenhancing on
. \ or M R l un Ie
inflammati n i pre ent. which i often the ca e on mplomatic Ie ions. Perhap the
mo t significant morphol gic feature to be determined by or M R I i evidence of
internal or external fi tulae. adherence I underlying lruclure or lobulati n ince
the e eem to be the mo t important cau e f urgical recurrences. agina! TI WI
in another patient (Fig. 25-D) how a characteri lie cyst (arrow). n axial T2WI
( Fig. 25- ) in another patient show cy tic signal (arrow) adjacent to the hyoid bone.

Management
When pos. ible, surgery is the treatment of choice. The goal or the surgery is to
remove all the epithelial lining. Becau e of their e tent, complete rem val rna be

1 09
/ NECK, THYROID, PARATHYRO I D

difficult and may recur. Extensive resection (Sistrunk procedure), which removes
PEARLS/PITFALLS the entire duct, midportion of the hyoid bone and a portion of the tongue base, has
A thyroglossal duct cyst appears decreased the recurrence rate from 50% to 20% .
as a thin walled midline cystic
mass on cr and M R I scanning.
Suggested Readings
A t hyroglossal duct cyst is lo
cated along path of thyroglossal Johnson, u., Smith P., Akintunde, M.O., Robson, A.K., Stafford, F. W. Assessment of
duct. pre-operative investigations of thyroglossal cysts. fournal of the Royal College of
Surgeons of Edinburgh 41 ( 1 ):48-9, 1 996 Feb.
A thyroglossal duct cyst has a Kawanaka, M., Sugimoto, Y., Suehiro, M . , Fukuchi, M. Thyroid imaging in a typical
characteristic appearance on cr case of acute suppurative thyroiditis with abscess formation due to infection from a
and M R images. persistent thyroglossal duct. A nnals of Nuclear Medicine 8(2) : 1 59-62, 1 994 M ay.
Lim-Dunham, lE., Feinstein, K.A., Yousefzadeh, D.K., Ben-Ami, T. Sonographic
Surgical removal is the treatment
demonstration of a normal thyroid gland excludes ectopic thyroid in patients with
of choice.
thyroglossal duct cyst. A merican fournal of Roentgenology 1 64(6) : 1 489-9 1 , 1 995
Care should be taken not to mis Jun.
take a thyroglossal duct cyst for a McHenry, c.R . , Danish, R . , Murphy, T., Marty, 1.1. Atypical t hyroglossal duct cyst: a
thin walled abscess or necrotic rare cause for a solitary cold thyroid nodule in childhood. American Surgeon
node. 59(4):223-8, 1 993 Apr.
Urao M . , Teitelbaum, D.H., Miyano, T. Lingual thyroglossal duct cyst: a unique sur
gical approach. fournal of Pediatric Surgery 3 1 ( 1 1 ): 1 574-6, 1 996 Nov.

/2 2 9
E K, THYRO I D PA R ATH Y RO I DI

Case 26
Clinical Presentation
A 36-year-old male presented with a history of progressive swelling in his neck.
Phy ical examination revealed a round. painless. mass just inferior to the mandible
on the left.

A B

Findings
There is a well-circum cribed cystic rna in the right lateral neck ( Figs. 26-A, 26-6,
& 26- ). TIle submandibular gland is displaced anteriorly. The sternocleidomastoid

111
I NECK, THYRO I D, PARATHYROID

muscle is displaced posterolaterally, and the carotid artery and jugular vein are dis
placed medially. (Case courtesy of Dr. Andrew Berger.)

Differential Diagnosis: Cystic Neck Mass


Thyroglossal cyst
Branchial cleft cyst
Cystic hygroma
Necrotic lymph node
Laryngocele
Sebaceous cyst
Jugular vein
Lipoma

Diagnosis
Second branchial cleft cyst.

Discussion
Epidemiology
Second branchial cleft cysts are the most common congenital cystic neck masses.
These cysts usually present during the second to fifth decades of life. Second
branchial cleft anomaly comprises a spectrum of cysts, sinus tracts, and fistulae that
are caused by incomplete obliteration of the branchial apparatus.

Pathophysiology
Branchial cleft cysts represent a congenital cystic anomaly of the branchial appara
tus (Fig. 26-0, 5-week embryo). TIle branchial apparatus begins to develop in the
third week of gestation, surrounding the primitive pharynx. The branchial appara-
N ECK, THYRO I D, PARATHYROIDI

tus consists of five transverse mesodermal ridges, separated by clefts. Each cleft is
met by an outpouching of the pharynx, called the pharyngeal pouch, with a thin
membrane separating the structures at their interface. The branchial apparatus de
velops into the main structures of the face, nasopharynx, oropharynx, and neck.
The first branchial arch ( I in Fig. 26-0) gives rise to the maxilla and mandible, as
wel l as the facial artery and mandibular and maxillary divisions of the trigeminal
nerve. The first cleft pinches off and gives rise to the external auditory canal, with
the thin membrane separating it from the pharyngeal pouch, becoming the tym
panic membrane. Cysts associated with this cleft are uncommon and occur in the re
gion of the external auditory canal.
The second branchial arch ( I I in Fig. 26-0) forms, among other structures, the sty
loid process, anterior base of the tongue, and posterior belly of the digastric muscle.
The fate of the second branchial cleft is controversial, but in conjunction with rem
nants of the second pharyngeal pouch, it is believed to be responsible for 95% of
branchial cleft cysts.
Second branchial cleft cysts occur via the persistence of the cleft as an epithelium
lined tract ( * in Fig. 26-0) that courses between the second and third branchial arch
( I l l in Fig. 26-0). If the tract exists in its entirety (i.e., there is a complete fistula), its
internal opening usually lies in the tonsillar fossa. From there, the tract runs beneath
tbe stylobyoid muscle and posterior belly of the digastric muscle, and above the
glossopbaryngeal and hypoglossal nerves. Further on, it crosses between the inter
nal and external carotid arteries (see Fig. 26-D). Below the carotid bifurcation, it
courses along the carotid sheath a nteromedial to the sternocleidomastoid muscle.
The external opening appears along the anterior edge of the sternocleidomastoid,
usually in its middle third. If the tract is incomplete, it may present as an isolated
cyst anywhere along tbe course of the tract, or as a cyst in communication with an
internal or external fistula.
There is a spectrum of cysts, sinus tracts, and fistulae that arise from incomplete
obliteration of this branchial apparatus. Cysts are much more common than fistulae
or sinuses.

Clinical Findings
The second branchial cleft cyst most often presents as a smooth, nontender, fluctu
ant mass located along the upper third of the sternocleidomastoid. Wben not in

E F
I NECK, THYRO I D, PARATHYROID

communication with an external fistula, the cyst may go unnoticed until the second
PEARLS/PITFALLS to fourth decades. The discovery of the cyst is often related to an upper respiratory
Second branchial cleft cysts are infection, which causes transient enlargement of the cyst. Cysts may become in
the most common congenital cys fected and progress to abscess formation, with the possibility that spontaneous rup
tic neck masses. ture or incision and drainage will lead to a permanent fistula. Patients with a
branchial fistula may present with persistent mucous discharge from a skin opening
Second branchial cleft cysts may in the neck.
have internal oral or external
lower neck fistulae.
I maging Findings
Ninety five per cent of second The introduction of ultrasonography, CT, and M R I has improved the accuracy of di
branchial cleft cysts are from sec agnosis of these lesions. The course and extent of a fistulous tract may be demar
ond branchial cleft. cated by fistulography, which may be followed by CT scanning. The cysts are well
demonstrated on cr and M R I scanning. On cr, they are usually sharply mar
A second branchial cleft cyst
ginated, centrally hypodense masses with thin enhancing capsule.
can pass between the internal
On M R I a noninfected second branchial cleft cyst presents as a homogeneous,
and external carotid arteries.
low to intermediate signal intensity cystic mass on T l -weighted images (Fig. 26-E;
Care should be taken not to mis s-submandibular gland, m-branchial cleft cyst, sm-sternocleidomastoid muscle:
take a second branchial cleft cyst image courtesy of Dr. E l len K. Tabor). They are usually high signal on T2 weighted
for an abscess. images (Fig. 26-F; image courtesy of Dr. E l len K. Tabor). The lesion is well circum
scribed and thin walled. If there has been previous infection, there will be varying
degrees o[ wall thickening, increased signal intensity and obliteration of the sur
rounding [at planes.
The radiographic differential diagnosis includes neck abscesses, branchiogenic
carcinoma, lipoma, tuberculous adenitis, cystic hygroma, paraganglioma, and
metastatic neoplasm. The benign, thin walled, cystic appearance, classic location,
and T l and T2 parameters usually allow correct radiographic diagnosis, with differ
entiation from an abscess presenting the greatest difficulty. Thyroglossal duct cysts
tend to be close to midline and laryngoceles usually have an obviolls association
with the larynx.

Management
Complete excision under general anesthesia is the treatment of choice. The proce
dure is associated with a low incidence of local complications and neurologic
sequelae. The overall recurrence rate is less than 5% after a follow-up of 2 years.

Suggested Readings
Agaton-Bonilla, F.e., Gay-Escoda, e. Diagnosis and treatment of branchial cleft
cysts and fistulae. A retrospective study of 1 83 patients. lnternatio ll al Journal of Oral
and Maxillofacial Surgery 25(6):449-52, 1 996 Dec.
Batsakis, J.G. Tumors of the head and neck: clinical and pathological considerations,
2nd ed. Baltimore: Williams and Wilkins, 1 979;5 1 4-520.
Chandler, J.P, Mitchell, B. Branchial cleft cysts, sinllses, and fistulas. OlOlaryngologic
Clinics of North America 1 4: 1 75-1 86, j 98 1 .
De, P.R., Mikhail, T. A combined approach excision of branchial fistula. Journal of
Laryngology and Otology 1 09( 1 0) :999-1 000, 1 995 Oct.
De Schepper, A.M., Monheim, P, Degryse, H . R . , Van de Heyning, P. cr of second
branchial cleft cysts and fistula: comparison with M R I in three cases. Annals of Ra
diology 3 ] : 1 4 1-1 48, 1 988.
Reynolds, J.H . Y, Wolinski, A.P Sonographic appearance of branchial cysts. Clinical
Radiology 48(2): 1 09-10, 1 993 Aug.
ECK, T H Y ROID, PA RATHYRO I DI

Case 27
Clinical Presentation
An adult female pre ented with a painless lower neck mass.

A B

Findings
A homogeneous mass is pre ent in the posterior triangle. It conforms to surround
ing oft tissue structures ( Figs. 27-A and 27-B). (Case courtesy of Dr. Andrew
Berger.)

Differential Diagnosis: Cystic Neck Mass


Branchial cleft cyst
Lymphangioma
Thyroglossal duct cyst
External laryngocele
Necrotic lymph node
Lipoma

Diagnosis
Lymphangioma

Discussion
Lymphangiomas are benign malformations of the lymphatic sy tern.
CK, T H Y RO I D. PARATH Y RO I D

Epidemiology
Mo t lymphangioma occur during childhood with 60% present at birth. and 0%
presenting by 2 years of age. This corre pond to the period when the greate t de
velopment of the lymphatic ystem OCCUJ . There i no gender predominance. orne
of tho e that occur in adult may be of traumatic rather than developmen tal origin.

Pat hophy iology


Lymphangioma are nonencap ulated lesions of lymphoid ti ue. Mo t researcher
in thi area feel the e Ie ion a re malformation rather than true neopla m . For the
record, they are cia sified into three group , ba ed on the size of the I mphatic
pace within the rna : ( I ) lymphangioma implex are compo ed of thin walled cap-
il lary sized channel , (2) cavernou lymphangioma have more dilated lymphatic
with fibrous adventitia and (3) cy tic hygroma have large cy ts up to everal cen-

c D

11 16
N ECK, THYRO I D, PARATHYROIDI

timeters in diameter. I n practice, the radiographic appearance of these types over


PEARLS/PITFALLS lap and, in fact, all three tissue types may occur in the same lesion.
Most lymphangiomas occur dur
ing childhood with 60% present Clinical Findings
at birth, and 80% presenting by
2 years of age. Lymphangiomas are generally painless, compressible neck masses. Cystic hygromas
classically occur in the posterior triangle but may extend more anteriorly.
A lymphangioma is a cystic neck
mass that classically occurs in the
Imaging Findings
posterior triangle.
U ltrasound is valuable in imaging these lesions especially in the perinatal period.
A lymphangioma is a non en CT and M R I are useful for defining the full extent of the lesions. Accurate delin
hancing, soft pliable mass. eation of lesion extension is important for preoperative diagnosis, surgical planning,
and assessing recurrence.
Care should be taken not to mis
Lymphangiomas are characteristically soft and conform to existing tissue spaces
take a lymphangioma for a
rather than distorting surrounding structures. Because of their large cystic spaces,
necrotic lymph node.
they do not typically enhance with contrast. They are low signal on Tl WI (Fig.
Care should be taken not to fail 27-C) and high signal on TIWI ( Figs. 27-D and 27-E). With infection or hemorrhage,
to recognise the full extent of the these signal characteristics may change.
lymphangioma for adequate sur
gical planning. M anagement
Surgery is the therapy of choice for most patients with this condition. Imaging is es
pecially valuable to define the full extent of the lesion to allow full excision at
surgery.

Suggested Readings
Borecky, N., Gudinchet, E, Laurini, R., Duvoisin, B., Hohlfeld, 1., Schnyder, P. Imag
ing of cervicothoracic lymphangiomas in children. Pediatric RadioLogy 25 2 : 1 27-30,
1 995.
D ubois, 1., Garel, L., Grignon, A., David, M., Laberge, L., Filiatrault, D., Powell, 1.
Imaging of hemangiomas and vascular malformations in children. A cademic Radi
ology 5 5:390-400, 1 998 May.
Fung, K., Poenaru, D., Soboleski, D.A., Kamal, I . M . I mpact of magnetic resonance
imaging on the surgical management of cystic hygromas. Journal of Pediatric
Surgery 33 6:839-41 , 1 998 Jun.
Vazquez, E., Enriquez. G., Castellote, A., Lucaya, J., Creixell, S., Aso, C, Regas, 1. US,
CT, and MR imaging of neck lesions in children. Radiographies 15 1 : 1 05-22, 1 995
Jan.
I NECK, THYRO I D, PARATHYRO I D

Case 28
Clinical Presentation
An adult female was brought to the clinic after sustaining a fall and complai ning of
neck pain and a sore throat. Physical examination demonstrated slight muscle
spasm in the cervical region without point tenderness. An exudative pharyngitis was
also present. There was no evidence of a pulsatile neck mass.

A B

Radiologic Findings
Cervical radiographs (Fig. 28-A) demonstrated prevertebral soft tissue widening.
1l1ere was no evidence of cervical fracture. Prevertebral vascular calcifications were
not present. Because of the radiographic and clinical findings, an MRI was per
formed of the upper neck (Fig 28-B). There was no evidence of abscess. A tubular
structure with signal void on all sequences was seen in the prevertebral soft tissues.

Differential Diagnosis: Prevertebral Soft


Tissue Widening
Abscess
Neoplasm
Post-traumatic change
Lymph fluid
Tortuous carotid artery

Diagnosis
Medially deviated (tortuous) carotid artery
N ECK, THYROI D, PARATHYROID1

Discussion
The cervical prevertebral soft tissues are made up of the prevertebral space and the
retropharyngeal space.
The prevertebral space is bounded posteriorly by the anterior longitudinal
ligament of the vertebral column, and anteriorly by the prevertebral fascia (deep
layer of deep cervical fascia.) This space extends the entire length of the vertebral
column.
The retropharyngeal space is bounded posteriorly by the prevertebral fascia and
anteriorly by tbe buccopharyngeal fascia (middle layer of the deep cervical fascia.)
The retropharyngeal space extends from the skull base to the level of tbe sixth tho
racic vertebral body. The retropbaryngeal space normally contains only lymph
nodes. Several other spaces border the retropharyngeal space. The pbaryngeal mu
cosal space of the nasopharynx, oropharynx, and hypopharynx are located anteri
orly.
The carotid space (i.e., poststyloid portion of the parapharyngeal space) is located
lateral to the retropbaryngeal space. It contains the carotid artery, internal j ugular
vein, the lower four cranial nerves, as well as most of the external carotid artery
branches.

Epidemiology
Congenital tortuosity of the internal carotid artery has an estimated incidence of
< 1 % of the population. This abnormal course results from failure of uncoiling of an
embryological bend present at the junction of tbe embryonic dorsal aorta and the
third aortic arch. The aberrant vessel will course adjacent to the tonsiLlar capsule of
the pharyngeal wall in close apposition with the superior constrictor muscle.
Medial deviation of the carotid artery also is seen with normal aging as an inci
dental finding. Redundency of the carotid arteries also has been related to a de
crease in cerebral flow with rotational motion of the neck and stroke.

Pathophysiology
The retropharyngeal space is an area that is frequently involved by pathologic
processes arising from contiguous spaces or primarily in that space. A large number
of abnormalities involving the retropharyngeal space have been reported, such as
abscess, benign and malignant neoplasm, lymphedema, post-traumatic processes,
and ectatic carotid arteries.
Tortuous carotid arteries and congenital aberrant course of the carotid arteries
are relatively less well known. Awareness of these entities becomes significant be
cause of procedures that may be performed sucb as oropharyngeal intubation,
biopsy, abscess drainage, or other surgical procedures. All of these procedures could
result in vascular injury, witb potentiaLly disastrous results.

I maging Findings
The presence of this finding in older patients may sometimes be associated with vas
cular calcifications on lateral and frontal cervical radiographs. Calcified carotid
arteries in normal position overlie the vertebrae on the lateral radiograph and can
be seen lateral to the vertebrae on tbe frontal views. In patients with medial devia
tion of the carotid arteries, the calcified vessels are anterior to the vertebrae on the
lateral view and are usually not visible or superimposed upon the vertebrae on the
frontal view.
CT or M R I scans with or without intravenous contrast enhancement may be
needed for proper localization of the medial position of the carotids. This is espe
cially true when vascular pulsations are felt on pbysical examination, but no definite

1 191
INECK, THYROID, PARATHYROID

c D

calcifications are seen on plain radiographs. Prior to biopsy, an M R I of another pa


tient with metastatic lung carcinoma to the right medial pterygoid muscle and pha
ryngeal wall (Fig. 28-C, arrow) revealed a carotid artery adjacent to the mass, in
denting the posterior pharyngeal airway. Figure 28-0 shows an enhanced view of a
CT scan of another patient with a tortuous carotid artery (arrow). A reformatted
coronal enhanced CT scan in another patient (Fig. 28-E) shows the medial position
of the vessel.

Suggested Readings
Cheung, Y.K., Sham, J.S.T., Chan, F.L., Leong, L.L.Y., Choy, D. Computed tomogra
phy of the paranasopharyngeal spaces: normal variations and criteria for tumor ex
tension. Clinical Radiology 45: 109-1 13, 1 992.
Daniello, N.J., Goldstein, S. l . Retropharyngeal hematoma secondary to minor blunt
head and neck trauma. Ear, Nose, and Throat lou.rnaI 73:4 1-43. 1 994.
N ECK, THYROID, PARATHYROml

Davis, W.L., Harnsberger, H.R, Smoker, W.R.K., Watanabe, A.S. Retropharyngeal


PEARLS/PITFALLS space: evaluation of normal anatomy and diseases with CT and MR imaging. Radi
A tortuous carotid artery can ology 1 74:59--64, 1 990.
show up as a pre vertebral soft Gianoli, GJ., Espinola, T.E., Guarisco, J.L., Miller, R H . Retropharyngeal space in
tissue mass on radiographic fection: changing trends. Otolaryngology and Head and Neck Surgery 1 05:92-100,
imaging. 1 99 1 .
Poe, L.B., Manzione, 1. Y. , Wasenko, J.1., Kellman, R M . Acute internal j ugular vein
A tortuous carotid artery bas a
thombosis associated with pseudoabscess of the retropharyngeal space. A merican
characteristic cr and MRI ap
Journal of Neuroradiology 1 6:892-896, 1 995.
pearance.
Silver, A.J., Mawad, M.E., Hilal, S.K., Sane, P., Ganti, S.R . Computed tomography of
Do not biopsy! ! ! ! ! ! the carotid space and related cervical spaces. Radiology 1 50:723-728, 1 984.
Som, P.M., B iller, H.F., Lawson, W., Sacher, M., Lanzieri, c.F. Parapharyngeal space
masses: an updated protocol based upon 1 04 cases. Radiology 1 53 : 1 49-156, 1 984.
Wong, Y K., Novotny, G.M. Retropharyngeal space-a review of anatomy, pathol
ogy, and clinical presentation. Journal of Otolaryngology 7:528-535, 1 978.
E K, T H Y RO I D. PA RATH Y RO I D

Case 29
Clinical Presentation
A 56-year-old female with nonHodgkin lymphoma presented with discomfort and a
palpable mass in the right anterior neck.

A B

Radiologic Findings
An axial IT section at the in fragloltic level (Fig. 29-A) shows an enlarged right
mass lacking enhancement. The central portion i hypoden e with a surrounding rim
of enhancement. An axial ection at the supraglottic level ( Fig. 29- B ) hows a
peripheral area of enhancement. There are no inflammatory change urrounding
the mass. The carotid arteries and left internal jugular vein ( U V ) are unremarkable.

Differential Diagnosis: Anterolateral


Neck Mass
Pseudomasses: hypertrophy of the sternocleidoma lOid muscle, status poSt unilat
eral radical neck di section
Vascular masses: asymmetry of the j ugular veins, jugular vein ectasia, aneurysm of
the jugular vein, jugular vein thrombosis (bland, septic or metastatic clot).
aneurysm of the carotid artery, arteriovenous fistula. hemangioma. lymphan
gioma
y tic ma es: branchial cleft cy t, external laryngocele. cy tic adenopathy. thy
roid cysts
olid masses: adenopat hy. paraganglioma, schwannoma, ab ce s, neurofibroma.
thyroid tumors, lipoma

Diagnosis
(' mernal jugular vein thrombo is

1 22
N ECK, THYROID, PA RATH YRO l Dl

Discussion
There is a long List of differential diagnoses for a patient presenting with an antero
lateral neck mass. Although these masses are easily amenable to diagnostic FNA
due to their superficial location, care should be taken to avoid puncturing a normal
vessel or a vascular mass. As such, a thorough physical examination and diagnostic
imaging should be performed before more invasive maneuvers are undertaken.
Asymmetry of the venous drainage of the neck is fairly common either in the
number or size of the vessels. The most common and striking of these normal vari
ants is asymmetry in the size of the IN's. I n 80% of cases the right IJV is larger than
the left. The IJV can be congenitally absent or there may be more than one vessel
on the same side.

Epidemiology
In the preantibiotic era, septic [JV t hrombosis (lJVT) was a frequent complication
of local and regional infectious processes occuring in the head and neck. Lemierre's
syndrome, also known as necrobacilosis, was a well known lethal entity referring to
suppurative thrombophlebitis of the TlV, secondary to an oropharyngeal infection.
With the widespread use of antibiotics, vascular involvement from an infectious
process of the head and neck has became rare. However, there has been a resur
gence of IJV thrombophlebitis in the past few decades, due to the more aggressive
and invasive care of critical patients and the increasing number of intravenous drug
abusers. At the present date, the more frequent causes of IJV thrombophlebitis are
placement of central venous lines and puncture of the lJV by intravenous drug
abusers. The incidence of jugular vein thrombosis i n patients with indwelling
catheters is as high as 60% in most series and this number is thought to be an un
derestimate, as the diagnosis may be easily overlooked.

Pathophysiology
TIle pathogenesis of IJVT involves several factors.
Causes relating to endothelial damage include placement of indwelling catheters,
central venous lines, direct puncture of the IlV (usually by intravenous drug
abusers), surgical damage during head and neck procedures, cervical trauma (open
or blunt trauma), radiotherapy and infection. An infection may result from direct in
noculation or from hematogeneous or lymphatic spread through the vasa vasorum,
which ultimately leads to intimal disruption. In cases of an infected thrombus, the
entity is referred to as thrombophlebitis and has more ominous implications. Ton
sillitis, otomastoiditis and odontogenic infections are the leading causes of IJV
thrombophlebitis.
Causes related to hypercoagulable states include malignancies, paraproteinemias,
connective tissue disorders (systemic lupus erythematosus), pregnancy, and defects
in the coagulation cascade (e.g., protein C and protein S deficiency, deficency of
antithrombin TIl, etc. ). The final factor potentiating thrombosis is blood stasis, which
is present in states of dehydration, hypotension and prolonged imobilization. lJVT
can result from extension of a thrombus arising in intracranial venous sinuses, par
ticularly the sigmoid and transverse sinuses.
Pathologically a thrombus seen within the LJV may be bland, infected or neo
plastic in nature. Clinical history and imaging findings usually allow [or discrimina
tion of these entities.

Clinical Findings
The clinical presentation of IJVT may be quite nonspecific and not infrequently
asymptomatic and detected as an incidental finding. Neck discomfort, pain and a
I NECK, THYRO ID . PARATHYROID

cervical mass are the most frequent presenting symptoms. When there is associated
infection. symptoms of sepsis may dominate the clinical picture. Physical exam usu
ally reveals a palpable mass along the anterior border of the sternocleidomastoid
muscle which may be firm and tender ("cord sign ) . I nflammatory changes in the
"

overlying skin indicate thrombophlebitis. In such cases, fever, elevated white count
and elevated sedimentation rate are usually present. Complaints of headaches and
other neurologic signs suggest intracranial deep venous thrombus propagation with
impending risk of hemorrhagic infarction. Fundoscopic examination will reveal pa
pilledema.
Complications of IJVT include pulmonary and systemic thromboembolism, su
perior vena cava syndrome, and chylothorax.

I maging Fi n dings
The diagnosis of IJVT is most often established based on imaging findings. Imaging
is also mandatory for defining the extent of the thrombus and identifying any asso
ciated complications.
U ltrasound is the modality of choice in the initial evaluation of a patient pre
senting with a lateral neck mass. 11lis modality will rapidly determine the location
of the mass and reveal whether it is vascular, cystic or solid. Sonographic signs of
thrombosis include the presence of an incompressible, dilated vessel with filling of
the lumen by hypoecboic material and loss of respiratory phase changes in the cal
iber of the vessel. Using Doppler, it is possible to make the diagnosis and to identify
any residual flow suggestive of partial thrombosis or recanalization. Collateral cir
culation can also be depicted. Limitations of US include the inability to evaluate the
vessel above the mandiblular angle and below the supraclavicular notch. and less
conspicuity of the surrounding soft tissues and vessel wall.
CT with and without contrast enhancement has been advocated as the preferred
noninvasive diagnostic modality. The CT appearance depends on the age of the clot
and whether the clot is infected or contains a tumor. Acute thrombus ( less than 24
hours) is hyperdense relative to flowing blood and may go undetected if precontrast
images are not obtained as it blends with the contrast enhanced blood. Subacute
and chronic bland clots appear as hypodense material partially or completely filling
the lumen. A surrounding hyperdense rim is frequently noted corresponding to
enhancement of the vessel wall . lllis is due to dilation of the vasa vasorum, although
local inflammation may also contribute to this phenomenon. Neoplastic clots usu
ally cause irregular vessel dilatation due to invasion of the vessel wall. In the
enhanced scan enlarged venous collaterals are easily depicted. CT adequately
determines the full extent of the thrombus and in some cases also reveals the un
derlying cause such as an abscess or tumor of the head and neck. The presence of an
ill-defined vessel wall witb surrounding inflammatory changes (e.g., soft tissue thick
ening, fatty stranding and loss of fascial planes) is diagnostic of thrombophlebitis.
Less commonly, a fluid collection can be seen within or surrounding the vessel wal l
representing an abscess. Enlargement of the retropharyngeal space by hypodense
material is sometimes seen and is thought to be related to interstitial edema. This
should not be mistaken for a retropharyngeal abscess as the cause of the venous
thrombosis.
Care should be taken with the timing of the contrast bolus in order to avoid a
false-negative result. Vascular flow phenomena result in asymmetric and heteroge
neous enhancement of the IJV, which may be mistaken for thrombosis or may make
it impossible to ensure an accurate diagnosis. This problem became more frequent
after the introduction of power injectors and faster scaning times (helical CT).
Whenever the diagnosis is in doubt the study should be repeated with a longer de
lay between the bolus injection and image acquisition. Another potential pitfall for
the inexperienced radiologist is mistaking IJVT for a necrotic lymph node. The ver
tically tubular nature and smooth peripheral enhancing rim seen with the former

1 1 24
ECK, TH Y RO I D, PA RATH Y RO I DI

hould preve nt thi confusion. Disadvantages of cr include radiation exposure and


the need for intravcnou contra t admini tration.
An en hanced cr scan of the neck (axial ection at the level of the hyoid bone) in
a patient with thromboph lebitis of the left IJV demonstrate a lack of enhancement
of the left IJ V, which show a central area of hypodensity surounded by a rim of pe
ripheral enhancement (Fig. 29- ) . The wall of the vessel i indistinct and there i oft
ti sue den ity and fany tranding encircling the vein. The fa cial plane with the ip i
lateral sternocleidomastoid muscle is indistinct. The left common carotid artery is
smaller than the right. but patent. There are no abnormalitie in the right jugular
vein.
On M R I . J VT may be more difficult to detect because slow flow or transient sta
is in the ve el may mimic clot as they both appear as intraluminal hyperinten ity.
The signal intensity depends on the age of the thrombu becau e of the biochemi
cal changes undergone by clotted blood. The multiplanar capability and high oft tis
ue re olution make M R I an ideal tool to evaluate the extent of the thrombu and
any a ociated soft tis ue changes.
M R venography with a two dimensional time of flight gradient echo acquisition
has hown excellent correlation with Doppler US and conventional venography in
the detection of venous thrombosi . onventional venography wa abandoned as a
diagno tic tool due to the inva ive nature of the procedure and the potential for
complication related to embolization of the clot.

M anagement
IJVT is managed with ant icoagulant therapy, primarily for prevention of pulmonary
thromboembolic events. When ant icoagulants are ine ffective. intravascular proce
dure may be attempted to remove the clot. In the ca e of an infected clot. broad
spectrum antibiotics should be init iated after blood culture are pcrformed. 111c
presence of purulent thrombophlebitis with intraluminal absces formation man
date' excision of the I1V to prevent serious complications. oncomitant i n fected
collections in the head and neck region hould be debrided and appropriately
drained. eopla tic thrombus is usually resistant to oral anticoagulation but may re-
pond to hcparin the rapy. However, primary thcrapy should target the malignancy
itself with chemotherapy or radiation therapy.

1 25 1
INECK, THYRO I D, PARATHYROLD

PEARLS/PITFALLS Suggested Readings


An INT appears as a "cord D uffey, D.C, Billings, K.R., Eichel, B.S., Sercarz, 1.A. Internal jugular vein throm
like" mass in the anterolateral bosis. A nnals of Otology, Rhinology and Laryngology 104( 1 1 ):899-904, 1 995 Nov.
neck on radiographic imaging. Finn, 1.p., Zisk, 1.H., Edelman, R R, Wallner, B.K., Hartnell, G.G .. Stokes, K.R ..
Longmaid, H.E. Central venous occlusion: MR angiography. Radiology 1 87( 1 ):
Predisposing factors to INT in 245-5 1 , 1 993 Apr.
clude indwelling catheters, cen
Gudinchet, F., Maeder, P., Neveceral, P., Schnyder, P. Lemierre's syndrome in chil
tral venous Lines, i ntravenous
dren: high-resolution cr and color Doppler sonography patterns. CheST 1 1 2( I ):
drug abusers, head and neck in
27 1 -3, 1 997 Jul.
fections, cervical trauma, and hy
percoagulable states. Kalan, A., Tariq, M., Harar, R P.. Gatland, D. Spontaneous internal jugular vein
thrombosis and recurrent laryngeal nerve palsy: a rare simultaneous presentation of
E nhanced cr shows an enlarged an occult malignant neoplasm. Joumal of Laryngology and OTology 1 1 0( 1 2): 1 1 66-8,
vessel, lacking contrast enhance 1 996 Dec.
ment. Leontsinis, TG., Currie, A.R., Mannell, A. Internal jugular vein thrombosis follow
ing functional neck dissection. Laryngoscope 1 05(2): 1 69-74, 1 995 Feb.
Care should be taken not to per
form an FNA before excluding a Poe, L.B., Manzione, 1. Y., Wasenko, 1.1., Kellman, RM. Acute internal jugular vein
vascular origin for a lateral neck thrombosis associated with pseudoabscess of the retropharyngeal space. Americall
mass. Journal of Neuroradiology 1 6(4 Su p pl ) 892-6 , 1 995 Apr.
:

Sakai, 0., Nakashima, N., Shibayama, C, Shinozaki, T , Furuse. M. Asymmetrical or


Care should be taken not to mis heterogeneous enhancement of the internal jugular veins in contrast-enhanced CT
take asymmetric and heteroge of the head and neck. NeLiroradiology 39(4):292-5, 1 997 Apr.
neous enhancement of the IJV
Stocks, RM., Milburn, M., Thompson, 1. Unusual neck masses secondary to jugular
for thrombosis.
venous abnormalities: case report and discussion. American Surgeon 63(4):305-9,
1 997 Apr.
Care should be taken not to mis
take IIV thrombosis for necrotic Terada, Y. , Mitsui, T , Jikuya, T , Kaminishi, Y., Watanabe, K. Infected throm
nodes. bophlebitis of the right internal jugular vein. Journal of Vascular Surgery 24(6):
1 066-7, 1 996 Dec.
A clinician should take care not Tovi, F., Fliss, D.M., Noyek, A.M. Septic internal jugular vein thrombosis. Journal of
to perform an i.ncomplete study Otolaryngology 22(6):41 5-20, 1 993 Dec.
which does not not include the
full extent of the clot.

1 1 26
NE K, THYROID, PA RATHY ROIDI

Case 30
Clinical Presentation
A 65-year-old male presented with a painle s mass in the left po terior neck. On
clinical exam a oft, mobile mass was palpated in the left occipital region.

A B

Radiologic Findings
An enhanced cr scan of the neck ( Fig. 30 A ) hows a 2 x 1 .5 cm well-circum
-

scribed, fat density mass in the subcutaneous soft tissues of the left occipital region.
A thin enhancing rim is noted. There a re no areas of central enhancement and no
internal septations. The attenuation value within the lesion was -85 HOllnsfield
units ( H U ) the same as subcutaneous fat. Figure 30-B is a photograph of the surgi
cal pecimen from the patient.

Differential Diagnosis: Fatty Tumors of


the Neck
Solitary fatty tumor
With no evidence of contra t enhancement: lipoma, dermoid cyst

With contrast enhancement: angiolipoma, hibernoma, lipobla tomaJlipoblas

tomato i , liposarcoma
Multiple fatty tumors
Benign symmetric lipomatosis ( Madelung disease)

ushing syndrome

Diagnosis
Posterior neck lipoma

1 27
1 N ECK, THYROID, PARATHYROID

Discussion
Epidemiology
Lipomas are benign, encapsulated, mesencbymal tumors. Although highly prevalent
in the body, only 13% of lipomas occur in the head and neck, most frequently in the
subcutaneous soft tissues. Submucosal lipomas also have been reported in the phar
ynx, oral cavity and larynx, but are much rarer. The most frequent location is the
posterior neck. There is a male predominance, with a peak incidence in the 7th
decade.
Lipomas are more frequent in obese people. ll1ese tumors tend to increase in size
with age and body weight, but do not decrease in size with weight loss.

C D

1 1 28
NECK, THYRO I D, PA RATH YROIDI

Pathophysiology
Pathologically, lipomas are encapsulated, benign tumors composed of adult fat cells,
separated into lobules by septae of connective tissue ( fibrolipomas). When highly
vascularized, these lesions are classified as angiolipomas. Angiolipomas may be of
the infiltrating or non-infiltrating sUbtype. The latter may infiltrate adjacent muscles
and neurovascular bundles, demonstrating a more aggressive appearance. However.
angiolipomas are rare in the head and neck.
Another histologic subtype of lipoma is the spindle cell lipoma, which is charac
terized by a mixture of fat cells and spindle-shaped fibroblasts in a collagenous ma
trix. This subtype of tumor, has a more aggressive cytologic appearance than the
conventional lipoma, with considerable nuclear pleomorphism, and may be con
fused with a well-differentiated liposarcoma.
Submucosal Lipomas may arise within a muscle or from intermuscular fascial
septa. These deep seated lipomas may be sessile or pedunculated.
Although benign, these lesions may recur when not completely resected. Malig
nant degeneration of a lipoma has never been fully proved. Most experts agree that
liposarcomas arise de novo and not from pre-existing lipomas.
Lipomas may be congenital or acquired, and are thought to arise from a multi po
tential mesenchymal cell. TIlere are several congenital and acquired diffuse, 110n
encapsulated lipomatoid lesions which are not true neoplasms. These include,
among others, lipoblastomatosis, which is a growth of embryonal lipoblasts seen in
children, and benign symmetric lipomatosis, which is a disease of middle-aged men
characterized by multiple symmetric accumulations of fatty tissue in the neck.

Clinical Findings
Neck lipomas usually present as subcutaneous soft, non-tender, freely movable
masses that may have a lobulated surface. TIle most common location is the poste
rior neck, but other subcutaneous locations are possible. Axial TI W (Fig. 30-C) and
T2W ( Fig. 30-D) images in another patient with a subcutaneous lipoma show a large

F G
1 NECK, THYROID, PA RATHY RO I D

mass in the anterolateral and inferior aspect of the right neck, insinuating between
the thyroid gland and the strap muscles and splaying these structures. There is also
posterior deviation of the neck vessels and leftward deviation of the airway. The
mass shows well defined margins and follows the signal intensity of subcutaneous fat
on both sequences. Coronal T l W image (Fig. 30-E) shows the craniocaudal extent
of the mass in the right cervico-thoracic j unction. There are linear areas of hy
pointensity within the mass representing fibrous septa.
Patients usually present for cosmetic reasons, very rarely with compressive symp
toms. Even submucosal lipomas are frequently asymptomatic. The presence of
symptoms depends on tumor size and location. They are usually related to airway
obstruction, compression of the pharynx or visceral space of the infrahyoid neck,
and include dysphagia, dysphonia, stridor, sleep apnea, foreign body sensation in the
throat, and irritating paroxysmal cough.
On clinical exam, submucosal lipomas are non-specific in appearance and mani
fest as any other submucosal mass bulging the walls of the pharynx or larynx. Coro
nal M R I of the larynx of another patient with submucosal lipoma of the larynx (Fig.
30-F) shows a submucosal mass centered in the right paraglottic space. The mass has
the same signal intensity as the subcutaneous fat and insinuates itself through the
adjacent laryngeal structures, including the ipsilateral true vocal cord. An axial Tl W
image at the supraglottic level (Fig. 30-G) shows a decrease in the transverse diam
eter of the airway caused by this submucosal mass.
I n the oral cavity, lipomas may cause discomfort during speech or mastication,
and manifest as a yellowish submucosal mass located in the tongue or cheek.
Multiple lipomas may be seen in association with several syndromes, such as mul
tiple familial lipomatosis, Birt-Hogg-Dube syndrome, Gardner's syndrome, multiple
familial and nonfamilial spindle cell lipomas, etc.

Imaging Findings
Fat has unique characteristics on both CT and M RI allowing for the diagnosis of
most lipomatoid masses. The diagnostic role of imaging is especially important in
submucosal lesions, which are non-specific on clinical exam, and have a vast differ
ential diagnosis. I maging is also necessary to evaluate the extent of the tumor, which
may be transpatial, and to look for any aggressive features.
On CT, lipomas manifest as well-circumscribed fat density masses with -65 to
- 1 00 HU, with a thin and smooth e n hancing capsule and, eventually, thin intratu
moral septations.
M R l gives better delineation of the tumor, showing to advantage any infiltrative
behavior or transpatial extent. Lipomas follow the signal characteristics of fat, hy
perintense on Tl WI and intermediate signal intensity on T2WL When the diagnosis
is in doubt additional fat-suppression sequences may be used. Characteristically,
lipomas do not show contrast enhancement. This is an important distinguishing fea
ture from liposarcoma. Lipomas also tend to displace and compress rather than in
filtrate adjacent structures. Lipomas also may be an incidental finding.

M anagement
Surgical excision is the treatment of choice, when warranted. Care should be taken
to do a complete resection due to the high rate of recurrence. The rate of recurrence
is believed to be secondary to microscopic foci of lipomatous inflltration of adjacent
soft tissues. The recurrence rate of infiltrating lipomas is as high as as 62% . Lipo
suction assisted excision of lipomas may be used in cervico-facial lesions to improve
the cosmetic result and the technique can be repeated as needed in case of recur
rence, with no increasing risk. Submucosal lesions, depending on their size and lo
cation, may be removed endoscopically, or via an external approach.

1 1 30
NECK, THYROID, PA RATHYROIDI

PEARLSIPITFALLS Suggested Readings


A lipoma presents as a soft, non Abdullah, B.1., Liam, C.K., Kaur, H., Mathew, K.M. Para pharyngeal space lipoma
tender mass in the posterior causing sleep apnoea. British fournal of Radiology 70(838 ) : 1 063-5, 1 997 Oct.
neck. Calhoun, K.H., Bradfield, 1.1., Thompson, C. Liposuction-assisted excision of
cervicofacial lipomas. Otolaryngology and Head and Neck Surgery 1 1 3(4):401-3,
0.n cr and MR i mages a lipoma 1995 Oct.
can be seen as a homogeneous,
Eckel, H.E., JungehUlsing, M. Lipoma of the hypopharynx: pre-operative diagnosis
well-defined, fat density or fat
signal intensity mass. and transoral resection. fournal of Laryngology and Otology 1 08(2): 1 74-7, 1 994
Feb.
Care should be taken not to fail Elango, S. Parapharyngeal space lipoma. Ear, Nose, and Throat fournaI 74( 1 ) :52-3,
to evaluate the full extent of the 1995 Jan.
tumor. Kransdorf, M.J., Benign soft-tissue tumors in a large referral population:
distribution of specific diagnoses by age, sex, and location. A merican fournal of
The clinician should take care
Roentgenology 164(2) :395-402, 1995 Feb.
not to mistake diffuse lipomatous
processes with true, encapsulated Murty, K.D., M urty, P.S., George, S., Balakrishnan, R., Mathew, K.I., Varghese, G.
lipomas. Lipoma of the larynx. A merican fournal of Otolaryngology 1 994 Mar-Apr,
1 5(2):149-5l .
Tsunoda, A . Lipoma in the peri-tonsillar space. fournal of Laryngology and Otology
108(8):693-5, 1 994 Aug.
Worsey, 1., McGuirt, W., Carrau, R.L., Peitzman, A.B. Hibernoma of the neck: a rare
cause of neck mass. A merican fournal of Otolaryngology 1 5(2):1 52-4, 1 994 Mar
Apr.
Zeiger, B.w., Zeiger, B.G., Plorer, A., Steiner, H., Fritsch, P.o. Dermal spindle cell
lipoma: plexiform and nodular variants. Histopathology 27(6):533-40, 1995 Dec.

I
INE K . THYRO I D. P RATHYRO I D

Case 31
Clinical Presentation
A 66-year-old male presented with a 5-year history of progre ive generalized neck
welling and a recent onset oC hoarsne and nonproductive cough. The patient a lso
complained of facial congestion upon exertion. On clinical exam there was a disfig
uring oft. non-tender. non-mobile, generalized neck swelling with a lobulated ur
face. 0 discrete masses were palpated.

A B

Radiologic Findings
cr of the neck demonstrates massive accumulation of homogeneou' fat-density tis
'ue in all the infrahyoid compartments of the neck, the submandibular and sub
mental spaces. TIlere is no demarcation from the subcutaneous fat and no encap u
lated areas or fibrous septalions. Thi fally tissue infiltrate the visceral pace
involving the glollic and upraglollic larynx. with definite infiltration of the true vo
cal cord . TIlere is also infiltration of the retropharyngeal and prevertebral space by
thi fat tissue. TIle ternocleidomastoid muscle and trap mu des are displaced and

1 132
NECK, THYROID, PARATHYROI DI

compressed. The neck vessels are displaced posterolaterally. There is mediastinal ex


tent of this increased fat which displaces the thoracic vessels laterally and com
presses the mediastinal trachea. The tranverse caliber of the trachea is slightly de
creased at this level due to extrinsic compression ( Figs. 3 1 -A, 3 1 -B and 31-C).

Differential Diagnosis: Diffuse Lipomatosis


of the Neck
Without inflammatory signs: benign or multiple symmetric lipomatosis, multiple
familial lipomatosis, morbid obesity, Cushing's disease/syndrome, lipoblastomato
sis (children), liposarcoma
With inflammatory signs: Weber-Christian's disease (painful adipositis), Dercum's
disease (acute panniculitis), progressive nodular lipomatosis

Diagnosis
Benign symmetric lipomatosis (Madelung disease)

Discussion
Epidemiology
Multiple symmetric lipomatosis is a rare lipodystrophy characterized by the pres
ence of multiple non encapsulated fatty infiltrative lesions that occur in a symmetric
distribution. The disease has several eponyms including Madelung disease, madelung
collar, lipomatosis of Launois-Bensaude, and benign symmetric lipomatosis.
Men are affected more often than women with a male-to-female ratio of 1 5 : 1 . For
unclear reasons Mediterranean males are predominantly afflicted.
The fatty deposits usually appear after 20 years of age and the peak age at pre
sentation is between the 4th and 6th decades. Typically, there is rapid progression of
the lipomatous tissue over a variable period of time foLlowed by a period of stabi
lization or slow progression over many years.
A familial incidence has been described with an autosomal dominant mode of in
heritance, but most cases are sporadic.

Pathophysiology
The etiology of Madelung disease is unknown and various theories have been pos
tulated to explain its pathophysiology. There is an unequivocal relationship with al
coholism, with 65 to 90% of cases seen in patients with a prolonged history of
ethanol abuse and some with chronic liver disease. The accumulation of fat is ex
plained by the lipogenic and antilipolytic effect of alcohol, through a decrease in B
adrenergic receptors.
Another theory is based on a genetic defect in catecholamine-stimulated lipoly
sis, which has been associated with hypertrophy of adipocytes in animal models and
related to functional sympathetic de nervation. The mechanism of denervation may
be related to the alcohol-related neuropathy or to other unknown factors. However,
the defect appears to be at the post-receptor level, because the lipolytic response to
cyclic AMP is preserved. Finally, another theory places Madelung disease in the
group of the phakomatoses.
Several metabolic defects have been associated with this disease including hyper
lipidemia, glucose intolerance, diabetes mellitus, hyperinsulinism, hyperuricemia,
hypertension, hypothyroidism, polyneuropathy, and abnormalities in liver [unction
tests. Some of these biochemical defects may be associated with alcoholism itself, al
though most have been reported i n patients with Madelung disease who have no
history of ethanol abuse. The frequent association with upper aerodigestive tract
neoplasms leads some experts to consider this disease a paraneoplastic syndrome.
PatllOl gically, the fat depo its ar nonencapsulated and compo ed of mall. ma
ture adipo e cell with a light increa e in fibro a cular troma. 0 cellular pleo
m rphi m or atypia are een. There i only one rep rt in the ngli h literature of
malignant degeneration of the fatty ti' ue in Madelung di ea e. Once pre nt. the
fat dep its do not spontaneousl re 01 e.

li nical fi nding
Most patient pre ent ith cervical welling ovcr a peri d of ears taU ing disfig
\

uremcnt. re triction of motion and. ometime I cal pre ure mpt m "Madelung
collar" r "bo e collar" were the term used to de crib the appearance pr duced
by large p terior cervical lump extending int the anterior neck, ubmandibular.
ubmental. parotid and upracla icular region When there i in olvcmenl of th
deep and vi ceral pace of the ncck. patient may pre ent with dy pnea. hoar ene
or d phagia. Media tinal involvement ha been a iated ilh uperi r vena cava
\

yndrome.
On phy ical exam, tbe fat dep it are nontender and nonencap ulated. NT ex
amination ma di clo e ubmuco al bulge in the pharynx and/or larynx due to ub
muCO 31 infiltration and extrin ic compre ion of the e ructure
linicall . the main differential diagno i i made with ma ive lymphadenopathy.
According to the di lributi n f fat dep it Madelung di ea.e i di ided into two
ubI pe In 1 pe I, the fatt dep it are focally circum cribed and di tributed ym
metricall in the upper part of the b d , paring the extremitie There may be me-

D E

1 1 34
NECK, THYROID, PA RATHY R O I DI

diastinal extention and involvement of the deep spaces of the neck, including the
upper aerodigestive tract. Type I l-disease is characterized by a more diffuse in
volvement with accumulation of lipomatoid tissue in the entire subcutaneous fat
layer, mimicking simple obesity.

Imaging Findings
The role of imaging is three-fold: ( 1 ) distinguishing this disease from other causes of
diffuse neck swelling when the clinical findings are not clear, (2) evaluating the ex
tent of fat accumulation and its relationship to adjacent neurovascular structures for
therapy planning, and (3) identifying complications due to compression.
CT and M R I are the preferred methods for evaluation of this disease and are
both diagnostic, demonstrating nonencapsulated deposits of fatty tissue involving
several compartments of the neck. The density and signal intensity of the fat de
posits are similar to that of subcutaneous fat with no demarcation between the two.
The most common sites of accumulation of fat tissue are the subcutaneous soft tis
sues of the posterior and lateral neck, paraspinal region, supraclavicular fossa, an
terior neck, prevertebral space, parotid region, submandibular and submental re
gions, and mediastinum. Submucosal fat deposits also may be detected in the
visceral space of the neck, even when not clinically apparent. Sectional imaging de
tects compression and displacement of adjacent structures and allows quantification
of airway compromise. M R I , having the advantage of multiplanar capability, better
depicts the extent of the fatty deposits and compression of adjacent structures.
An enhanced axial CT scan of the neck of another patient with Madelung disease
at the glottic (Fig. 3 1 -D ) and supraglottic (Fig. 3 1 -E ) levels show diffuse accumula
tion of fatty tissue in the anterior and lateral compartments of the neck, sub
mandibular space, prevertebral, and visceral spaces of the neck. The submandibular
glands are displaced anteriorly and compressed, and the sternocleidomastoid mus
cle and neck vessels are displaced posteriorly by these fat deposits. The supraglottic
larynx and true vocal cords are infiltrated by fat. Direct laryngoscopy (Fig. 3 1 -F) of
the same patient shows fullness of the paraglottic spaces bilaterally with a smooth
yellowish surface.
The main distinguishing features from other forms of diffuse lipomatosis include
the absence of encapsulation, absence of inflammatory signs in the adjacent soft tis
sues, and the distribution of fatty deposits. According to some experts fat suppres
sion and chemical shift imaging can be used to distinguish encapsulated lipomas that
are seen in multiple hereditary lipomatosis, and progressive nodular lipomatosis
from Madelung disease.
Imaging may also be used to monitor medical therapy and to detect complications
of surgical procedures, such as hemorrhage and infection.

M anagement
1l1erapy may be required for cosmesis, for functional problems, or to alleviate com
pressive symptoms. Correction of metabolic imbalances and alcohol abstinence may
help to control the disease, but do not reduce the fatty deposits. Medical therapy us
ing a B2 agonist (salbutamol) has been used with variable success and may help
maintain a certain degree of normal lipolysis.
Liposuction is tbe preferred treatment for subcutaneous involvement as it can
safely be performed under local anesthesia. 1l1is is an important advantage in this
patient population, usually poor surgical candidates, due to respiratory distress and
chronic liver disease. Also, tbe amount of scarring is much less when compared to
open lipectomy, allowing repeated procedures for recurrent disease. Both open and
closed resection of these fat deposits have a high recurrence rate and are considered
palliative. Open lipectomy is the modality of choice for deep-seated fatty deposits,
and when the proximity of the airway or major vascular structures makes liposuc
tion unsafe.
INECK, THYROID, PA RATH YROI D

PEARLSIPITFALLS Suggested Readings


Madelung disease often presents Borges, A., Torrinha, E, Lufkin, R.B., Abemayor, E. Laryngeal involvement in mul
with diffuse neck swelling and tiple symmetric lipomatosis: the role of computed tomography in diagnosis. Ameri
the appearance of a "horse col can Journal of Otolaryngology l 8(2): l 27-30. 1 997 Mar-Apr.
lar." Chung, J.Y., Ramos-Caro, EA.. Beers. B.. Ford, M . I .. Flowers, E Multiple lipomas,
angiolipomas, and parathyroid adenomas in a patient with Birt-Hogg-Oube syn
Madelung disease is most often
drome. Il11ernational Journal of Dermatology 35(5):365-7, 1 996 May.
seen in middle-aged males with
history of chronic alcohol intake. Feldman, D.R .. Schabel. S.1. Multiple symmetrical lipomatosis: computed tomo
graphic appearance. SoLt/hern Medical Journal 88(6):68 1-2, 1 995 Jun.
Accumulation of non-encap u Kitano, H., akanishi, Y.. Takeuchi, E., agahara. K. Multiple symmetrical lipo
lated fat i n the subcutaneous soft matosis: no longer just a Mediterranean disea e? Journal of Oto-Rhino-Iaryngology
tissues, deep spaces of the neck, and Its Related Speciallies 56(3): 1 77-80, 1 994 May-Jun.
visceral space, or mediastinum is Martin, OS, Sharafuddin, M., Boozan, 1., Sundaram, M., Archer, C MUltiple sym
commonly seen in Madelung dis metric lipomatosis (Madelung's disease). Skeletal Radiology 24( I ):72-3. 1 995 Jan.
ease.
Murty, K . D., Murty, P.S., George, S., Balakrishnan, R., Mathew, K.I., Varghese. G.
Care should be taken not to Lipoma of the larynx. American Journal of Otolaryngology 1 5(2): 1 49-5 1 , 1 994 Mar
mistake the disease for simple Apr.
obesity. Parmar. C. Blackburn, C Madelung's disease: an uncommon disorder of unknown
aetiology? British JOllrnal of Oral and Maxillofacial Surgery 34(5):467-70. 1 996 Oct.
Failure to recognize submucosal
deposits of fat can cause airway
compromise or other compres
sive symptoms (superior vena
cava yndrome).

There is sometimes insuffi c ient


coverage of the neck and medi
astinum, when imaging this dis
ease.

1 36
NE K, T H Y RO I D, PA RATH Y RO I D1

Case 32
Clinical Presentation
A 30-year-old male pre ented with malai e. dy phagia. and neck pain and stiffnes .
linical exam revealed a eptic patient with erythema of the po terior pharyngeal
wall and no definite mass. The neck was painful with motion. l1le patient's prior clin
ical history was uneventful.

A B

Findings
Lateral plain films of the cervical region how marked full ness of the prevertebral
soft t issues, which measure approximately 20 mm in lhickness at the level of C2. The
airway is di placed anteriorly, but appear adequate in caliber. No soft t issue em
physema i noted. There is a l 0 mild traightening of the cervical pine with 10 s of
tbe normal lordosis. The cervical vertebral bodies and di k paces showed no ab
normalities ( Fig. 32-A). An enhanced cr scan performed the same day shows a hy
poden e mass with an enhancing wall extending fTom the level of the nasopharynx
to the post-cricoid region. No mediast inal exten ion wa noted ( Fig. 32- 8 ) .

Differential Diagnosis: Enlargement of the


Prevertebral Soft Tissue
Ma s within the prevertebral space
M ass in the retropharyngeal pace: adenit is/celluliti . ab cess, edema, hematoma,
lipoma, metastatic lymphadenopat hy, direcat invasion from na opharyngeal or
oropharyngeal squamous cell carcinoma, Kawa aki' disease. atypical third
branchial cleCt cyst, hemangiomallymphangioma.

Diagnosis
Retropharyngeal absces

1 37 1
1 NECK, THY RO I D, PA RATHYROID

Danger S pa c e

M i d d l e L a y e r of t h e Deep C e r vi c a l Fascia
( B u c c o - P h a r y ng e a l F a s c i a )

Ret rophar yngea l A l ar F a s c i a

Space (Deep L a y e r of t h e Deep C e r v i c a l F a s c i a )

Pre- Vertebral Fasci a


J u gul a r Vei n (Deep L a y e r of t h e Deep C e r v i c a l F a s c i a )

P r e - Vert ebral Space

C Caroti d Artery

Discussion
The retropharyngeal space ( RPS) is a midline space in the deep neck bounded an
teriorly by the middle layer of the deep cervical fascia (buccopharyngeal fascia) and
posteriorly by the deep layer of the deep cervical fascia (prevertebral fascia). This
space extends from the base of the skull to the level of the body ofT4 and is a route
for disease extension into the mediastinum. Lymph nodes and fat are the only con
tents of the retropharyngeal space and, therefore, primary disease processes of this
space are uncommon. A midline raphe separates the right and left chains of lymph
nodes lying in this space (nodes of Rouviere). Posterior to the RPS lies a virtual
space between two slips of the deep layer of the deep cervical fascia, often referred
to as the danger space. This potential space can serve as a conduit for spread of dis
ease to the level of the diaphragm, where these two leaflets join together. The most
posterior midline space is the prevertebral space which extends from the skull base
to approximately T3 (Fig. 32-C).
It has been argued that the retropharyngeal nodes regress by the age of 6 years,
which would account for the higher incidence of RPS infection in children. How
ever, retropharyngeal adenopathy is commonly seen in older patients with head and
neck malignancies, especially nasopharyngeal carcinoma.

Epidemiology
The incidence of retropharyngeal space infections dropped dramatically during the
antibiotic era. However, there has been a recrudescence of this disease in the past 3
decades due to an increasing population of immunocompromised patient . Children
are particularly prone to retropharyngeal abscesses due to the spread of infection
from adenoidal, tonsillar or middle ear disease. Some series report 95% of retropha
ryngeal infections occurring before the age of 6 years. In adults. males are more fre-

1 1 38
N ECK, THYRO I D, PARATHYROIDI

quently affected then women (2 : 1 ) and predisposing factors should be carefully


searched.

Pathophysiology
A retropharyngeal infection can result from spread via lymphatic drainage, direct
extension of prevertebral space infection or from direct inoculation related to pen
etrating trauma. I n fection of the retropharyngeal space may also be iatrogenic (sec
ondary to surgery), may result from foreign body penetration, or from esophageal
perforation.
The areas whose primary lymphatic drainage is the retropharyngeal lymph nodes
include Waldeyer's ring, the sinonasal tract, the oral cavity and the middle ear. I n the
pediatric population upper respiratory infections involving these sites are very com
mon and may extend into the deep spaces of the neck when untreated. Other pos
sible etiologies in children include blunt trauma to the oropharynx and impacted
foreign bodies. Toddlers are especially prone to oropharyngeal trauma due to
unsteady gate and tendency to walk with sharp objects in their mouth. Trauma may
result in undetected oropharyngeal hematoma which may secondarily become
infected.
The organisms most commonly cultured from these deep space infections are
group A beta-hemolytic streptococci, Staphylococcus aureus and Hemophilus in
fluenza.
I n adults spread of infection to the deep spaces of the neck is quite infrequent in
the absence of predisposing factors. Odontogenic infection due to poor oral hygiene
and spread from a peritonsillar abscess are the most common associations. Under
lying illnesses such as diabetes mellitus, chronic alcoholism, malignancy, H I V infec
tion, and other causes of immunocompromise are usually present.
Spread from cervical osteomyelitis is not uncommon and mycobacterial and fun
gal infections should be considered in the differential diagnosis. I n the iatrogenic
group the most frequent causes are endoscopy and orotracheal intubation which
may lead to pharyngeal mucosal tears and subsequent infection. Impaction of for
eign bodies and esophageal perforation also should be considered i n an adult
patient.
A great variety of organisms are cultured from retropharyngeal abscesses in
adults, depending on the cause of infection and underlying clinical status. These
range from Gram negatives to anaerobic organisms and acid fast bacilli.

Clinical Findings
Symptoms vary with the patient's age although in most cases patients are septic at
presentation. I nfants and young children usually present with difficulty feeding and
drooLing. I n older children, obstructive symptoms, particularly stridor, are the most
typical presentation. Gastrointestinal symptoms such as nausea and vomiting also
may occur and may draw the attention of the clinician away from the head and neck
region. The onset of symptoms may be insidious and the patient may not come to
medical attention until there is impending airway obstruction.
Adults usually present with sore throat, odynophagia, dysphagia and neck pain.
On clinical exam there may be inflammatory changes in the pharyngeal walls and
a pharyngeal bulge, although these findings are inconsistent. Therefore, a normal
ENT examination does not exclude the diagnosis of a retropharyngeal abscess.
Fever, leucocytosis and elevated sedimentation rate are the rule.

I maging Findings
A lateral plain film of the neck should be performed in any patient with suspected
retropharyngeal abscess. Fullness of the prevertebral soft tissues is the hallmark of
K. T H Y ROID. PA RAT H Y RO I D

this condition. As a rough guideline. there i prevertebral oft ti ue welling when


the distance bet' een the anterior a pect of and the posterior pharyngeal wall is
greater than 7 mm for adult and children or when the same distance at 7 mea-
urc greater than 22 mm in adult or 14 mm in children. I nterst itial air may be een
within the prevertebral soft tissue and, although infrequent, is a valuable diagnos
tic sign. The film should be obtained during quiet respiration becau e with forced
expiration (especially i n children). the thickne s of the pharyngeal oft tis ues i

D E

1 1 40
NECK, THYROID, PA RATHYROIDI

overestimated. Plain films of the neck are also useful to rule out other pathologic
processes with similar clinical presentations such as supraglottitis and cervical os
teomyelitis.
Cross sectional imaging is the only way to distinguish between retropharyngeal
and prevertebral space infections, and this distinction is based upon the location of
the mass or fluid collection with respect to the prevertebral fascia. Retropharyngeal
masses displace the fascia and prevertebral muscles posteriorly. Conversely, prever
tebral masses displace these structures anteriorly. Another distinguishing feature of
retropharyngeal space masses is that they tend to be lateral in position due to the
presence of the median raphe as opposed to prevertebral masses, which are usually
midline. However, inflammatory processes may violate fascial planes, making it im
possible to accurately determine the site of origin.
CT is superior to M R I in the evaluation of infectious processes in the head and
neck region because it is more accurate in distinguishing adenitis and phlegmon
from a true abscess. For this purpose contrast administration is mandatory. Adenitis
presents as enlarged lymph nodes, which may or may not show a central area of
necrosis and ill-defined margins, suggesting extracapsular spread. A phlegmon
appears as an ill defined enhancing soft tissue density, whereas a hypodense lesion
with an enhancing rim defines an abscess. Enhanced axial CT at the level of the
oropharynx in a patient with retropharyngeal abscess due to a fungal infection (coc
cidioidomycosis) (Fig. 32- 0 ) shows a large bilobed mass in the retropharyngeal
compartment, displacing the longus colli muscles posteriorly and the airway anteri
orly. This well circumscribed mass is cystic and shows a thick enhancing wall. The
largest component of this mass lies on the left side and compresses the airway at this
level. Axial T 1 WI (Fig. 32-E) and axial TIWI (Fig. 32-F) of the same patient at the
level of the nasopharynx show the superior extent of this lesion, which involves the
left longus colli muscle. The signal characteristics are those of a fluid collection. TIlis
distinction has important consequences in the management of these patients, be
cause only an abscess is amenable to surgery. Air-fluid levels and the presence of gas
pockets are also more easily detected with CT. Figure 32-0 depicts an enhanced
axial CT of the neck at the glottic level in a patient with retropharyngeal abscess
that shows retropharyngeal fullness with interstitial gas. The fascial planes between
the retropharyngeal space and the carotid sheath are obscured bilaterally. Anaero
bic organisms were isolated on culture.
Another important consideration for surgical planning is the delineation of the
full extent of the lesion. Therefore, a cross sectional study should be performed from
the skull base to the diaphragm. Extension of an infection into the mediastinum is
not uncommon and can be accompanied by pleural and pericardial effusions. Eval
uation of airway patency is also mandatory and may alert the clinician as to the need
for tracheostomy placement. A CT may localize the primary site of infection, detect
radiopaque foreign bodies and localize esophageal perforations (using oral contrast
administration).
I nvolvement of the carotid space by infection may have disastrous complications
such as jugular vein thrombosis and spasm or thrombosis of the internal carotid
artery. TIl is is another reason to perform an enhanced CT study.
I n children, helical CT may circumvent the need for sedation.
It should be kept in mind that a CT is not 1 00% accurate in the evaluation of deep
space infections. A recent study showed a significant rate of false-positive results for
the presence of abscess ( 13% ) and proposes that the decision for surgery should be
based on both clinical and imaging data.
M R I is especially useful when the primary site of infection is the prevertebral
space, as it is the method choice to detect epidural abscesses and to evaluate the in
tegrity of the spinal cord.
On MRT, a retropharyngeal abscess appears hypointense on TI WI (Fig. 32-E ) and
hyperintense on TIWl (Fig. 32-F). When intravenous contrast is used. ring en
hancement is seen. Gas and foreign bodies are more difficult to detect and the im
ages more difficult to interpret due to susceptibility artifacts.

1 41 I
ECK, THYROID, PARATHYROID

M R I is the only imaging method that adequately depicts the layers of the deep
PEARLSIPITFALLS cervical fascia allowing a more precise localization and better anatomic delineation
A retropharyngeal abscess is of the lesion ( Figs. 32-E and 32-F).
more frequently found in chil
dren and immunocompromised Management
patients.
The management of retropharyngeal infections depends on both the patient's clini
Do not exclude a retropharyn cal status and the imaging findings. Adenitis and phlegmons are managed conserva
geal abscess on the basis of a tively with antibiotics and antiinflammatory drugs. When the clinical response is not
normal ENT/clinical exam. adequate, tissue should be obtained for culture and sensitivity tests. When the pres
ence of an abscess is certain, the lesion should be drained surgically or under imag
A lateral plain film of the neck ing guidance. Complex, multi loculated abscesses and those with extremely viscous
should be the first imaging study. secretions should preferably be managed surgically, because they would require
multiple drains and longer hospitalization. Surgery is also preferred when patients
An enhanced cr scan is manda
are severely septic or when there is mediastinal extension.
tory to: (1) distinguish retropha
ryngeal from prevertebral space
infections, (2) distinguish adeni
tis, flegmon and non-infected
Suggested Readings
fluid collections from abscess, Babl, EE., Pascucci, R. I mages in clinical medicine. Retropharyngeal abscess. New
and (3) evaluate the full extent England Journal of Medicine 337(7):472, 1 997 Aug 14.
of the inflammatory process. el-Sayed, Y. , al Dousary, S. Deep-neck space abscesses. Journal of OIO/aryngology
25(4):227-33, 1 996 Aug.
Care should be taken to perform
a complete study (including the Gidley, P w. , Ghorayeb, B.Y., Stiernberg, CM. Contemporary management of deep
mediastinum). neck space infections. OlOlaryngology and Head and Neck Surgery 1 16( 1 ): 1 6-22.
1 997 Jan.
Care should be taken to not con Goldenberg, D., Golz, A, Joachims, H.Z. Retropharyngeal abscess: a clinical review.
fuse adenitis or phlegmon with Journal of Laryngology and Otology 1 1 1 (6):546-50, 1 997 Jun.
an abscess. Hewel, K., Kioumehr, E, So, G., Wang, M . Infected third branchial cleft cyst:
retropharyngeal extension to the superior mediastinum. Canadian Association of
Care should be taken for the
Radiologists Journal 47( 2 ) : 1 1 1-3, 1 996 Apr.
clinician to recognize vascular
complications, especially throm Karkanevatos, A, Beasley, N.1., Swift, A.C Acute non-tuberculous retropharyngeal
bosis of the ICA abscess in an adult. A case report and review of the literature. Journal of Laryngol
ogy and Otology 1 1 1 (2) : 1 69-7 1 , 1 997 Feb.
Lazor, 1.B., Cunningham, M.J., Eavey, R.D., Weber. A.L. Comparison of computed
tomography and surgical findings in deep neck infections. Otolaryngology and Head
alld Neck Surgery 1 1 1 (6):746-50, 1 994 Dec.
Marra, S., Hotaling, A1. Deep neck infections. A merican Journal of Otolaryngology
1 7(5):287-98, 1 996 Sep-Oct.
Nokes, S.R., Adametz, J., Gardner, G., Beaton, J.N. Radiological case of the month.
Blastomycosis osteomyelitis with epidural and retropharyngeal abscess. Journal of
the A rkansas Medical Society 92(5):253-4, 1 995 Oct.
Sakaguchi, M., SalO, S., [shiyama, T., Katsuno, S., Taguchi, K. Characterization and
management of deep neck infections. in ernational Journal of Oral and Maxillofacial
Surgery 26(2 ) : 1 3 1 -4, 1 997 Apr.

1 1 42
NECK, THYROID, PARATHYROIDI

Case 33
Clinical Presentation
An adult male presented with a 5-year history of an asymptomatic left cervical mass,
which was non tender and without bruit. The patient denied dysphagia, stridor, and
neurologic symptoms.

Radiologic Findings
An axial T l W image ( Fig. 33-A ) through the level of the valleculae shows a left
sided mass with a heterogeneous "salt and pepper" appearance. The internal and ex
ternal carotid arteries are displaced anteromedially and splayed.

Differential D iagnosis: Neck Mass


Schwannoma
Carotid body tumor
Adenopathy
Branchial cleft cyst
Lymph node

Diagnosis
Carotid body tumor
ECK, THYRO I D, PARATHYROID

Discussion
Epidemiology
Paragangliomas are benign tumors arising from paraganglion cells. The most com
mon paraganglioma in the head and neck is a carotid body tumor. An increased
incidence of carotid body tumors has been found in people living at high altitudes.
The carotid body tumor arises at the bifurcation of the common carotid artery. As
it grows it becomes adherent and difficult to separate from the major vessel.
There is a tendency to a multicentricity of tumors ( 10% ) , especially in a patient
with a positive familial history (26% ). The incidence of true malignancy showing
metastasis is higher in a carotid body tumor ( 1 0% ) than the overall incidence, vary
ing from 2%-6% . The biologic behavior or natural course of the tumors is not
clearly correlated with histologic appearances.

Pathophysiology
A paraganglioma is a slowly growing neuroendocrine neoplasm that arises from
neural crest derivatives. Chemodectoma, glomus tumor, nonchromaffin paragan
glioma, and neurocristopathic tumor are the most common names given to this
lesion. Precapillary arteriovenous shunts and nonchromaffin cells are characteristic
of the histologic appearance of these tumors.
Paragangliomas can be classified by their locations: tympanic and jugular tumors
are less frequent; tumors of carotid and vagal locations are more frequent. Sheets of
tumor cells are often divided into ball-like clusters ("Zelballen " ) separated by thin
fibrovascular septa.

Clinical Fi ndings
Paragangliomas often present as slowly growing and compressible masses, or cranial
nerve paralysis, according to the location. A vertically fixed and laterally movable
mass is a characteristic finding in a carotid body tumor.

B c

1 44
NECK, THYRO I D, PARATHYROIDI

o E

Imaging Findings
Radiological diagnosis of this tumor is based on location and characteristic byper
vascularity. M R I has some advantages over CT in delineating and characterizing the
lesion witb its superb soft tissue contrast. The hypervascular nature of the tumor can
be demonstrated by MR with signal void of flowing vessel. MR angiography can
show the major vascular structures adjacent to the tumor as well as tumor vascular
ity. The characteristic splaying of the carotid bifurcation is characteristic of a carotid
body tumor.
The most characteristic findings on an M R I are multiple low signal intensity ar
eas that represent vascular flow voids throughout the tumor. H igh and low signal
intensity regions on MR images are called a "salt and pepper" appearance. This
appearance is not specific for paraganglioma but may be seen with a number of
hypervascular lesions.
Figures 33-B and 33-C show another patient witb a less common appearance of a
carotid body tumor. The mass is circumferentially located around the common, in
ternal and external carotid arteries. Figure 33-B is through the common carotid
artery and Figure 33-C is slightly more cephalad through the level of the hyoid bone.

M an agement
Surgery is the tberapy of choice for most patients with this condition. While con
ventional angiography is generally no longer used for diagnosis, preoperative em
bolization is used in most cases to limit bleeding at surgery. The arterial pbase of an
angiographic study in a patient with a carotid body tumor (Fig. 33-0) shows the typ
ical appearance of the displacement of the internal and external arteries by the mass
at the bifurcation. During the capillary phase (Fig. 33-E) dense contrast enbance
ment is noted. Radiation therapy is used in some patients who are not surgical can
didates.
I N ECK, THYRO I D, PA RATHYROID

PEARLS/PITFALLS Suggested Readings


The carotid body tumor is a mass Batsakis, J.G. Tumors of the head and neck: clinical and pathological considerations,
tbat is centered at tbe bifurcation 2nd ed. Baltimore: Williams and Wilkins, 1 979.
of the carotid arteries. Olsen, WL., Dillon, WI', Kelly, WM. MR imaging of paragangliomas. American
Journal of Neuroradiology 7: 1039-1 042, 1 986.
A carotid body tumor has a "salt
Shugar M.A., Mafee M.F. Diagnosis of carotid body tumors in dynamic computer
and pepper" appearance on MRl.
ized tomography. Head Neck 5urg 4:5 1 8-521 , 1 982.
Do not biopsy or perform Vogl , T., Bruning, R., Schedel, H. Paragangliomas of the jugular bulb and carotid
surgery without a preoperative body: MR imaging with short sequences and GD-DTPA enhancement. AJNR
embolization. 1 0:823-827, 1 989.

Care must be taken to detect


synchronous or metachronous
lesions.
Case 34
Clinical Presentation
53-year-old female presented with a painlc w grov ing rna in the right lal-
eral neck. n clinical exam the rna i firm, non-lender and non-pul atile.

A B

147 1
I NECK, THYROID, PA RATHYROID

Radiologic Findings
A sagittal T l W image ( Fig. 34-A) shows a 2.5 X 1 cm well-circumscribed, oval
mass, with its long axis parallel to the neck vessels. The mass is located within the
right carotid sheath, displacing the common carotid artery and jugular vein anteri
orly. The axial T2W image (Fig. 34-B) shows heterogeneous T2W hyperintensity and
anterior displacement of the neck vessels. No relationship with the cervical nerve
roots is noted. Coronal post-contrast Tl W image (Fig. 34-C) shows intense, homo
geneous enhancement.

Differential Diagnosis: Solid Mass in the


Carotid Sheath
Inflammatory/Infectious: lymphadenopathy, traumatic neuroma
Neoplasm
Benign: nerve sheath tumor (schwannoma, neurofibroma, ganglioneuroma.

ganglioblastoma, ganglioneuroblastoma), paragangLioma (glomus vagale,


carotid body tumor, glomus jugulare), bpoma, hemangiolymphangioma, granu
lar cell tumor (rhabdomyoblastoma), thyroid and parathyroid tumors
Malignant: metastatic lymphadenopathy, lymphoma

Diagnosis
Vagus nerve schwannoma

Discussion
Epidemiology
Twenty-five to 45% of nerve sheath tumors occur in the head and neck region. [ n
the neck, these tumors may arise from the vagus nerve, sympathetic chain o r cervi
cal nerve roots, including the brachial plexus. Often the nerve of origin is difficult to
determine preoperatively.
Benign peripheral nerve tumors include two basic tumors with different patllo
logic features: schwannomas and neurofibromas. Over the past decades, these tu
mors have been known by a multitude of names, such as neurilemoma, neurinoma,
perineural fibroblastoma, fibroma of the nerve, peripheral glioma, and gliofibrosar
coma.
Schwannomas may present at any age although a peak incidence is noted between
the 4th and 6th decades. Neurofibromas tend to occur earlier in life (2nd and 3rd
decades), especially when associated with neurofibromatosis type [ (NF- [ ) . Neuro
fibromas may occur as a solitary lesion or, most often, be multiple. Only 1 0% of pa
tients with neurofibromas have N F type I. Schwannomas may also be multiple.

Pathophysiology
Nerve sheath tumors originate from Schwann cells or neuroectodermal perineural
cells. Pathologically, there are two distinct types of nerve sheath tumors with distinct
histologic patterns and clinical behavior. Schwannomas or neurilemomas are well
encapsulated tumors characterized by the presence of two different types of tissue:
Antoni A, which is the cellular component made by Schwann cells and fibroblasts,
and Antoni B, which is composed of myxoid elements. Hemorrhage and cystic de
generation may be seen in larger tumors.
Neurofibromas are well circumscribed nonencapsulated nerve sheath tumors
composed of Schwann cells, fibroblasts and swirls of neuronal elements running
through the bulk of the tumor. Plexiform neurofibromas are a histologic variant,
pathognomonic of NF I, with a more aggressive behavior. Whereas schwannomas
N ECK, THYROID, PA RATHYROIDI

rarely, if ever, undergo malignant degeneration, neurofibromas show a 5% to 15%


rate of malignancy, which is higher in patients with N F type 1.

Clinical Findings
Symptoms and signs depend on the location of the tumor and nerve of origin. The
most common clinical finding is a painless, slowly growing mass in the anterolateral
neck. Neurologic symptoms may arise from direct involvement of the nerve by tu
mor or from adjacent nerves due to compression and ischemia. Involvement of the
cervical sympathetic chain may infrequently present with Horner's syndrome
(myosis, ptosis and anhydrosis). Vagus nerve tumors may present with dysphonia,
dysphagia, hoarseness and arrhythmia. H owever, the presence of these symptoms
does not allow for topographic localization of the tumor, because the same symp
toms may result from compression by an adjacent tumor. Tumors located higher up,
near the skull base may involve the jugular foramen causing neuropathy of the
lower cranial nerves.
Nerve sheath tumors arising in the cervical nerve roots present with sensory and
motor deficits in the distribution of the involved root, usually paresthesia and mo
tor weakness.
Pain may occur with large tumors, particularly when there is significant compres
sion and bone remodeling.
On physical exam, the mass is usually non-tender. A sensation of electric shock
may be elicited by palpation. The mass is often mobile laterally but not along the
axis of the nerve. Pulsation and bruits should be sought to exclude highly vascular
lesions, such as a carotid artery aneurysm and a paraganglioma. A biopsy should not
be performed before imaging studies rule out these diagnostic possibilities. A biopsy
of nerve sheath tumors may be hazardous, causing excruciating pain or nerve
damage.
A complete physical exam, including a thorough neurologic examination, is rec
ommended for all patients, especially when neurofibromatosis is a consideration.
Electromyographic testing with evaluation of nerve conduction may be helpful in
the diagnosis and localization of lesions involving the cervical nerve roots.

Imaging Findings
The main purpose of imaging masses in the anterolateral compartment of the neck
is to distinguish between solid and cystic lesions and to detect vascular or highly vas
cularized lesions. These goals may be achieved by using ultrasound with Doppler or
cross-sectional imaging. Once the solid nature of the lesion is established, several
imaging features should be assessed to narrow the differential diagnosis. These in
clude the exact location of the lesion, the level of the lesion within the neck, direc
tion of displacement of adjacent structures (especially the neck vessels), and the de
gree of vascularity.
Schwannomas of the vagus nerve present as fusiform, well-circumscribed soft tis
sue masses, located in the carotid sheath, dorsally to the internal carotid artery
( lCA), displacing the neck vessels anteriorly. The long axis of the mass parallels the
course of the n erve. On CT, schwannomas are typically iso- to hypodense to muscle,
depending on tumor cellularity, degree of Antoni B myxoid tissue and presence of
hemorrhage or cystic degeneration. In addition, the degree of enhancement is vari
able. Although most lesions show little to moderate enhancement, one third of
schwannomas are highly vascularized and may mjmjc the appearance of a paragan
glioma. Contrast enhancement appears to be related to extravascular leakage with
stagnation and pooling of contrast into the tumor bed. Dynamic CT and M R I stud
ies usually show a flat, hypovascular curve contrasting with the early arterial peak
of paragangliomas.
When the lesion is centered at the skull base or extends superiorly into the jugu-
ECK, THYROID. PARATH Y RO ID

lar foramen, it may cau e calloping of the bone, a opposed to the permeative
change usually een with paraganglioma.
M R I bener demon trate the fusiform morphology of the tumor on sagillal and
coronal sections. Schwannomas are usually hypointense to muscle on T l W images.
bright on T2W image and how intense and uniform enhancement. Rarely these tu
mors show How voids. In the rare instance where a schwan noma is highly vascular
and how How void or a small. Ie s than 2 cm, glomu tumor contains no How voids.
differential diagnosis between these lesions may not be po ible. However, the man
agement is the same, with a t ranscervical urgical approach and preoperative em
bolization, if required.
Large chwannomas may undergo significant cystic degeneration cau ing diag
nostic problem with other acquired or congenital cy tic lesions of the neck.
Glomu jugulare tend to be centered in the region of the jugular foramen and
only rarely extend below t he level of the hyoid bone. Permeative bony changes of
the jugular foramen are helpful in the differential diagno is. arotid body tumors
have a typical location, centered in the carotid bifurcation and splaying apart the
I A and external carotid artery (E A).
Ultrasound is useful in differentiating cervical nerve sheath tumors from lym
phadenopathy. Ultrasonic features that favor a neural origin include the pre ence of
a olitary, oval shaped hypoechoic lesion with po terior enhancement and ab ence
of a fally hilum.
Exophytic thyroid nodules and parathyroid adenomas are al 0 a con ideration in
the differential diagnosis. Parathyroid adenoma tend to be located more medially
in the tracheoe ophageal groove.
eurofibromas are u ually indi tinguishable from chwannomas with imaging,
but a low, almost water den ity lesion is characteri tic of neurofibromas. Typically,
the plexi.form variant of this tumor has a central area of low density/intensity that
correspond to the central fibrou core. Fatty degeneration i also a classic feature
of neurofibroma . When extensive, it may mimic a lipoma.
The pre ence of aggressive imaging features such as ill-defined margin, bony de
struction, or the presence of distant spread, indicates a malignant nerve sheath tu
mor (neuroectodermal sarcoma). However, the ab ence of these aggressive features
does not confirm a benign nature.
Nerve sheath tumors arising from the cervical sympathetic chain are indistin
guishible from vagus nerve tumors, as the sympathetic chain is located posterome
dially to the ICA and tumors in this location also displace the vessels anteriorly.
Glomus vagale tend to occur within the carotid sheath at the skull base.

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N ECK, THYROID, PA RATHYROIDI

Peripheral nerve sheath tumors arising from the cervical nerve roots or brachial
'pEARLSIPITFALLS plexus manifest as soft tissue masses in the lateral neck, retroclavicular area, or ax
A vagus nerve schwan noma is a illary region. The typical imaging appearance of a cervical nerve root tumor is a
painless, slow-growing mass in dumbbell-shaped lesion that bridges and scallops the neural foramen, with in
the carotid sheath, that displaces tradural and extraforaminal components. The extraforaminal component may ex
the neck vessels anteriorly. tend between the anterior and middle scalene muscles into the posterior cervical
space and axillary apex. Axial postcontrast T1 W (Fig. 34-D) and TIW (Fig. 34-E)
The typical imaging appearance M R r of the neck of a patient with solitary neurofibroma of the right C5-6 nerve root
of a cervical nerve tumor is a through the level of the true vocal cords show a fusiform mass posterolaterally to
dumbbeU-shaped mass bridging the right carotid sheath, along the course of the right C5-6 nerve root. There is no
and scalloping the cervical neural evidence of intracanalar or intraforaminal components.
foramina. Imaging also is required for surgical planning and follow up of asymptomatic pa
tients treated conservatively. Tumor growth and interval appearance of aggressive
A dynamic cr or M R I study features should prompt intervention.
shows a flat arterial curve.

Care should be taken not to mis Management


take a nerve sheath tumor for
Nerve sheath tumors are managed surgically. Although most schwannomas may be
lymphadenopathy.
resected without damaging the nerve of origin, resection of neurofibromas is more
If the clinician fails to recognize hazardous and may result in severe morbidity. When it is not possible to preserve
multiple lesions the diagnosis of the nerve of origin the surgeon should be prepared to perform an end-to-end anas
neurofibromatosis may be tomosis or interpose a nerve graft. Several experts advocate conservative manage
missed. ment of asymptomatic lesions and imaging follow up to evaluate for growth. The
transcervical approach is the most commonly used for lesions located in the lateral
neck. When the pathologic diagnosis reveals a malignant nerve sheath tumor, post
operative radiation should be offered.

Suggested Readings
Catalano, P, Fang-Hui, E., Som, P.M . Fluid-fluid levels in benign neurogenic tumors.
American Journal of Neuroradiology 1 8(2):385-7, 1 997 Feb.
Furukawa, M., Furukawa, M.K, Katoh, K, Tsukuda, M. Differentiation between
schwannoma of the vagus nerve and schwan noma of the cervical sympathetic chain
by imaging diagnosis. Laryngoscope 1 06( 1 2 Pt 1 ) : 1 548-52, 1 996 Dec.
Ganesan, S., Harar, R.P., Owen, R.A., Dawkins, R.S., Prior, A.J. Horner's syndrome:
a rare presentation of cervical sympathetic chain schwan noma. Journal of Laryn
gology and Otology 1 1 1 (5)493-5, 1 997 May.
George, B., Lot, G. Neurinomas of the first two cervical nerve roots: a series of 42
cases. Journal of Neurosurgery 82(6):91 7-23, 1 995 Jun.
Kumchev, Y, Kalnev, B. A neurofibroma affecting the first right cervical sympathetic
ganglion and entering the jugular foramen of the skull base. Folia Medica
39(2) : 15-9, 1 997.
Sairyo, K, Henmi, T., Endo, H. Foramen magnum schwannoma with an unusual clin
ical presentation: case report. Spinal Cord 35(8):554-6, 1 997 Aug.
Sheridan, M.F., Yim, D.W. Cervical sympathetic schwannoma: a case report and re
view of the English literature. Otolaryngology and Head and Neck Surgery
1 17(6):S206- 1 0, 1 997 Dec.
INECK. T H Y R O I D. PA RATH Y R O ID

Case 35
Clinical Presentation
A middle-aged male with a history of tongue carcinoma pre ented with a new neck
mas on the right.

A B

1152
N ECK. TH YROID , PA RATHYROIDI

Radiologic Findings
An axial TI W M R image ( Fig. 35-A) shows bilateral masses posterior to the sub
mandibular giands.llley appear nearly isointense to muscle on this pulse sequence.
Following contrast administration there is enhancement of both lesions with a cen
tral area of nonenhancement on the right ( Fig. 35-B). The T2WI ( Fig. 35-C) shows
the central area to be high signal.

Differential Diagnosis: Neck Mass with


Central Lucency
Branchial cleft cyst
Lymphangioma
ecrotic lymph nodes
Abscess
Benign node with fally degeneration

Diagnosis
Necrotic lymph node from squamous cell carcinoma metastases

Discussion
Epidemiology
Squamous cell carcinoma is the most common malignant neoplasm of the upper
aerodigestive tract. One of the most important factors that influences therapeutic
outcome in the management of patients with this disease is the presence of cervical
lymph node metastases. A single nodal metastasis reduces the patient's survival by
approximately 50% , regardless of location. or size of the primary tumor in the head
and neck.

D
I NECK, THYROI D, PARATHYROID

Table 35-1 Correlation between the Rouviere. UICC, and Numeric classifications.

Rouviere U ICC Numeric

Submental Submental Level I


Submaxillary Submandibular Level l
Lateral cervical group Superior jugular nodes Level I I
Lateral cervical group Midjugular nodes Level I I I
Lateral cervical group Inferior jugular nodes Level IV
Lateral cervical group Spinal accessory nodes Level V
Anterior cervical group Prelaryngeal nodes Level VI
Anterior cervical group Para tracheal nodes Level V I I
Retropharyngeal nodes Retropharyngeal nodes
Parotid and facial nodes Parotid and buccal nodes
Mastoid and occipital nodes Retroauricular and occipital nodes

Clinical Findings
Before the development o f CT, assessment o f cervical nodal metastasis was based
completely on palpation of lymph nodes as detected by physical examination. TIle
inaccuracy of the physical examination has been documented in a number of stud
ies. False-negative and false-positive rates of the physical examination are 1 5-20%
and 30-50% , respectively. CT and M R I have improved the accuracy of nodal stag
ing over physical examination. Both techniques allow visualization of nonpalpable
nodes deep into the sternocleidomastoid muscle or in the retropharyngeal space.

Pathophysiology
Clinically, occult normal-sized nodes with central necrosis or with extracapsular
spread can be identified as metastases by CT or MR. Borderline-sized nodes with
out necrosis or extracapsular spread remain indeterminate by both CT or MR. Size
has been a widely used criterion to determine the presence of nodal metastases.
Because size data are a continuum, the relative sensitivity and specificity of any size
criteria can be adjusted by changes in the threshold dimensions, depending on a
clinical setting. Small metastatic nodes can be missed. Conversely, large reactive
nodes cannot be differentiated consistently from metastatic nodes.
Lucency in lymph nodes by CT can sometimes be seen with normal fally hilus or
with fatty replacement of nodal parenchyma following inflammation or irradiation.
Post treatment fally density signal is usually peripherally located. Central necrosis
also can be seen with lymphoma, papillary thyroid carcinoma. and tuberculous
adenitis (Fig. 35-0).
The nomenclature of cervical nodes has been complicated by variations among
several different nodal classification systems. Cervical nodes were initially catego
rized by Rouviere into one of 1 0 principal groups based on their precise anatomic
location (Table 35- 1 ). Today the most widely accepted classification has been that of
the Union I nternational Contre Ie Cancer ( International Union Against Cancer;
U lCC) , organized by the American loint Commillee on Cancer (AlCC), wherein
cervical lymph nodes are subclassified into 1 2 regions. Head and neck surgeons
have increasingly used a more simplified numeric classification based on physical
examination, which was established and adopted by the Academy of Otolaryngol
ogy-Head and Neck Surgery, in 1 99 1 . This simplified classification aims to eliminate
misinterpretation due to variation in terminology of cervical nodes as well as to pro
vide standardization of variable modification of neck dissection. Major cervical
nodal groups are classified as level I through level V I . This classification is
useful for understanding recent applications of conservative or selective neck dis-

I
NECK, THYRO I D, PARATHYROIDI

section, in which a part of the cervical nodal groups are removed. Some nodal
groups, such as retropharyngeal nodes and intraparotid nodes. are not included in
this numeric classification and must be referred to by their U I CC classification.

Imaging Findings
[ n the early era of CT, size criteria for nodal metastases were established by inves
tigating the range of normal variation in size of lymph nodes in subjects without
cancer. The initial criterion was that "discrete lymph nodes larger than 1 .5 cm are
considered metastases." More recently, size criteria have been reassessed with ex
tensive pathologic analysis.
One study suggested that a minimum (short) axial diameter of 1 1 mm for the
j ugulodigastric nodes and 10 mm for all other nodes more accurately reflects the
presence of metastases. Sensitivity and specificity of the size criteria in this study
were 89% and 73% per specimen and 4 1 .7% and 99.3% per node, respectively.
These results indicate that more than one-half of metastatic nodes are smaller than
10 mm. Small metastatic nodes missed by this simple size criterion are seen fre
quently in a specimen that already contains a large metastatic node. Considering
partial voluming effects and the limits of spatial resolution in cross-sectional imag
ing, there are a substantial number of indeterminate "borderline-size" lymph nodes.
Another multicenter study analyzing nodal size in 1 00 neck dissections revealed
that 46% of pathologic nodes were less than 1 em and 22% of pathologic nodes
were between 1 and 1 .5 cm in diameter. Another pathologic analysis of 750 lymph
nodes in patients with head and neck cancer demonstrated that most normal nodes
are small (less than 5 mm in diameter) but that metastatic nodes vary in size with
out having any peak in size distribution. Thus, a significant number of metastatic
nodes are missed (false-negative) by simple size criteria.
In patients with squamous cell carcinoma, central nodal low attenuation with ring
enhancement is likely to represent the presence of necrosis or tumor itself, within
metastatic nodes. Tumor cells replacing the medulla of lymph nodes generally show
less contrast enhancement than the remaining nodal cortex, resulting in a heteroge
neous appearance. This finding is highly characteristic of metastatic squamous cell
carcinoma. In a large clinical series, 74% of metastatic nodes contained necrosis or
cystic areas on pathology, whereas central necrosis was visualized only in 32% of
metastatic nodes on imaging studies. Therefore, a homogeneous appearance of
lymph nodes on CT or MRI does not exclude the presence of nodal metastasis.
Moreover. the incidence of central necrosis in positive nodes was reported to be di
rectly proportional to the size of the nodes. Even though small metastatic nodes
may contain necrotic foci, central necrosis is seen more commonly in larger nodes,
which usually are diagnosed as metastases by size criteria alone. Normal fat deposi
tion. as well as abscess formation in patients with infectious disease, may mimic cen
tral necrosis of lymph nodes.
Spread of the malignancy beyond the lymph node "capsule" has a strong negative
impact on patient prognosis. One study reported that the presence of extracapsular
spread reduces the 2-year survival of patients by approximately 50% . They con
cluded that histologic evidence of extracapsular spread is a more sensitive prognos
tic indicator than is the stage of the head and neck cancer. I ndeed. when extracap
sular spread is pathologically evident. postoperative radiation therapy is mandatory.
CT and MRI findings indicative of extracapsular spread include irregular nodal
boundaries and obliteration of the adjacent fat planes, if there has been no recent
infection, radiation therapy, or surgical intervention in this region. All of these
processes cause irregular nodal borders and obliteration of fat planes.

Management
A variety of cervical nodal dissection procedures have been developed for treat
ment of nodal metastases in head and neck cancer. Radical neck dissection (RND),
I NECK, THYROID, PARATH YROID

involves en bloc removal of aU lymph nodes from level I through level V along with
PEARLS/PITFALLS the sternocleidomastoid muscle, internal jugular vein, and spinal accessory nerve.
Large size lymph nodes with cen Significant morbidity is associated with RND, uch as shoulder dropping and pain .
tral necrosis, and extracapsular The idea of a less invasive neck dissection has been developed to minimize func
extension all suggest metastatic tional and cosmetic deficits while maintaining adequate disease control. As more
involvement. conservative neck dissections have become available without sacrificing prognosis,
more accurate and tissue specific presurgical imaging studies are necessary. Radia
These findings can also be found tion therapy is added in selected cases.
with infiammatory disease.

Care should be taken not to mis Suggested Readings


take metastasis for a benign con
dition. Anzai, Y, Brunberg, J.A., Lufkin, R. Imaging of nodal metastases in the head and
neck. Journal of Magnetic Resonance Imaging 7:774-783, 1 997.
Don, D., Anzai, Y, Lufkin, R.B., FU, Y S., Calcaterra, T., Lufkin, R. Evaluation of cer
vical lymph node metastases in squamous cell carcinoma of the head and neck.
Laryngoscope 1 05:669-674, 1 995.
Som, P. M . Detection of metastases in cervical lymph nodes; CT and MR criteria and
differential diagnosis. American Journal of Roentgenology 1 58:961 -969, 1992.
NECK, THY ROID, PARAT H Y ROIDI

Case 36
Clinical Presentation
A 58-year-old female presented with a rapidly enlarging neck ma s, progre ive
hoar enes, in piratory dyspnea and dysphagia. On phy ical exam a hard woody
ma wa palpated in t he right lateral and anterior neck, and was fixed to the sur
rounding structures. On laryngoscopy right vocal cord paraly i wa noted.

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1 N ECK. THYROID, PARATHYRO I D

Radiologic Findings
An MRI of the neck shows an asymmetric thyroid gland due to enlargement of the
right thyroid lobe, which extends posteriorly into the retrotracheal region. The mar
gins of this lobe are ill-defined and there is obscurity of the fascial planes with the
right sternocleidomastoid muscle, strap muscles, anterior aspect of the right internal
jugular vein and prevertebral muscles. The retrotracheal component obliterates the
tracheo-esophageal groove and blends with the proximal esophagus, which cannot
be separated from this mass. A nodular area of hyperintensity is noted in the medial
aspect of the right thyroid lobe, adjacent to the trachea, possibly representing a pre
existing nodular goiter with high protein content or hemorrhage. The remainder of
the right lobe is markedly hypointense compared to the contralateral side. The tra
chea is deviated anteriorly and to the left, and its tranverse lumen is decreased (Fig.
36-A). The cranio-caudal extent of this lesion is best appreciated in the coronal sec
tion ( Fig. 36-B). The axial section at the supraglottic level shows right vocal cord
paralysis (Fig. 36-C).

Differential Diagnosis: Ill-defined


Enlargement of the Thyroid Gland with
Extrathyroid Extension
Infectious/Inflammatory: acute suppurative thyroiditis ( bacterial or fungal),
Riedel's thyroiditis (also called invasive fibrous thyroiditis)
eoplastic: anaplastic thyroid carcinoma, thyroid lymphoma
Iatrogenic: postsurgical changes, postradiation therapy ( radiation induced thyroiditis)

Diagnosis
Riedel's thyroiditis

Discussion
Thyroiditis is a general term to designate a variety of inflammatory conditions
occurring in the thyroid gland. They are classified according to their clinical presen
tation into acute, sub-acute and chronic forms, and further subclassified according to
etiology or pathology. The acute form is usually associated with an infectious
process due to bacterial or fungal agents and is also called acute suppurative thy
roiditis. The subacute category comprises two different conditions: subacute granu
lomatous thyroiditis (Quervain's thyroiditis), which is thought to result from a viral
infection. and subacute lymphocytic thyroiditis, which likely represents an autoim
mune entity. It differs from Hashimoto's thyroiditis by the fact that it is self-limited
and does not tend to cause irreversible hypothyroidism. This entity may be seen in
the postpartum period and tends to reccur in subsequent pregnancies. Chronic thy
roiditis includes Hashimoto's (chronic lymphocytic thyroiditis), an autoimune
process that usually results in irreversible hypothyroidism, and Riedel's thyroiditis
( invasive fibrous thyroiditis).
With the exception of acute suppurative thyroiditis, all other forms of thyroiditis
are limited to the thyroid gland and do not commonly cause vocal cord paralysis.
although they can cause compressive symptoms due to gland enlargement. For these
reasons they are excluded from the current differential diagnoses. The main clinical
and imaging diagnostic mimic of Riedel's thyroiditis is anaplastic thyroid car
cinoma, which may present in the same age group and with an indistinguishable
clinico-radiologic picture.
Thyroid lymphoma with a florid reactive desmoplastic reaction. and the fibrosing
variant of Hashimoto's thyroiditis may also be very difficult to distinguish from

1 1 58
N ECK, THYROID, PARATHYROID1

Riedel's thyroiditis pathologicaUy. However, thyroid lymphoma does not tend to


have a hard consistency on palpation and Hashimoto's thyroiditis, even the scleros
ing variant, does not cross the boundaries of the thyroid gland.

Epidemiology
Riedel's thyroiditis was first described by Bernhard Riedel in 1883. It is best de
scribed as a benign fibrotic process with aggressive features, partially or completely
involving the thyroid gland and extending beyond the confines of the gland into the
soft tissues of the neck. Since first described, multiple terms have been used to refer
to this entity such as Riedel's struma, struma thyroiditis and invasive fibrosing thy
roiditis.
It is a rare condition, with an estimated incidence of 0.05% determined in the
largest series of thyroid resections reported to date. The female-to-male ratio varies
between 3 to 5 : 1 and the age group most commonly affected is between the fourth
and seventh decades, with a peak incidence in the late forties. The disease may be
uni- or bilateral. There is a well known association with other fibrosclerosing dis
eases including retroperitoneal and mediastinal fibrosis, orbital pseudotumor (scle
rosing variety) and Addison's disease.

Pathophysiology
The etiology of Riedel's thyroiditis is unknown. There are two main theories, one
suggesting an autoimune process, which may be the same or different from the one
responsible for Hashimoto's thyroiditis, and the other suggesting a localized or sys
temic fibrosclerosing inflammatory process. Evidence to support an autoimmune
pathophysiology includes the presence of thyroid autoantibodies (anti thyroglobulin
and antimicrosomal), the coexistence of histologic features of both Riedel's thy
roiditis and Hashimoto's in the same gland, and some land, and some isolated re
ports of other autoimune diseases, such as pernicious anemia associated with
Riedel's thyroiditis. Therefore, some experts characterize Riedel's thyroiditis as the
end spectrum of chronic lymphocytic thyroiditis. The association with and similari
ties to other fibrosclerosing diseases support a primary systemic fibrosclerosing

1 59 1
INECK, THYROID, PARATHYROID

process. Recently, an autoimune mechanism. mediated by eosinophils and their


highly fibrogenic degranulation products has been proposed as the basis of this sys
temic fibrosclerosing process. Potential mechanisms for eosinophilic recruitment
have been proposed such as adhesion molecules expressed by the vascular en
dothelium, connective tissue cells and thyroid follicular cells after local stimulation
by inflammatory mediators.
Macroscopically, Riedel's thyroiditis is characterized by a tony-hard fibrous mass
effacing the normal glandular architecture. Microscopically, there is extensive re
placement of the normal thyroid tissue by paucicellular bands of dense fibrous tis
sue, with bundles of collagen fibers and a hyalinized matrix invading the capsule and
extending into the surrounding tissues. A scarce inflammatory infiltrate may be
seen, primarily composed of lymphocytes, plasmocytes and polymorphonuclear
(PMN) leukocytes. Recently, attention has been put on the presence of eosinophilic
infiltrates in fibrotic regions and in the perivascular spaces. Histologic section of the
same patient (Fig. 36-0) shows compressed and atrophic follicles surrounded by
dense collagenous bands and scarce inflammatory infiltrate.
It is important to note that fibrotic tissue may be found in a variety of other con
ditions, including other forms of thyroiditis (radiation-induced and Hashimotos).
nodular goiter, desmoplastic reaction associated with lymphoma, post-trauma. scle
roderma, amyloid deposition. and neoplastic processes such as undifferentiated thy
roid carcinoma.
Therefore, the presence of three diagnostic criteria is required to make the diag
nosis of Riedel's thyroiditis. These include the presence of fibrosis of all or part of
the thyroid gland, near complete destruction of the involved portion of the gland,
with absent or destroyed follicules, and extension beyond the capsule.
The major pathologic differences between Riedel's and Hashimoto's disease is
the absence of lymphoid germinal centers, the presence of extra thyroid extension.
vascuJar changes, and loss of thyroid lobulation in the former.
FNA results are often nondiagnostic due to the increased consistency and
hypocellularity of the gland. They also may be misleading with a few described cases
of false-positives for carcinoma. Surgical biopsy is usually required for accurate di
agnosis.

Clinical Findings
Patients with Riedel's thyroiditis typically present with a rapidly enlarging neck
mass, causing compressive symptoms such as hoarseness. inspiratory dyspnea, stri
dor and dysphagia. Hoarseness may result from direct laryngeal extension. but more
commonly results from involvement of the recurrent laryngeal nerve in the tracheo
esophageal groove. Mediastinal extent may occur and be responsible for venous hy
pertensive yndromes such as superior vena cava syndrome. The disease may appear
first in an otherwise normal gland or a preexisting goiter. Rarely. the gland may be
normal in size and show a single nodular lesion. Symptoms and signs of hypothy
roidism should be sought, although these tend to appear in the later phases when
there has been extensive replacement of the thyroid gland parenchyma by fibrotic
tissue.
On clinical exam, the thyroid gland is diffusely or focally enlarged, with a rock
hard consistency and most likely a lobulated surface. Typically, the gland is fixed to
surrounding structures, including the skin, and may be impossible to mobilize. Un
like anaplastic carcinoma, lymphadenopathy is not a feature of Riedel's thyroiditis,
an important distinguishing feature. Laryngoscopy may disclose vocal cord paraly
sis or submucosal laryngeal infiltration. stenosing the laryngeal lumen. Esoph
agoscopy may demonstrate esophageal stenosis due to extrinsic compression.
On clinical history, it is important to ask for symptomatology related to other fi
brosclerotic processes, such as renal problems, hypertension, visual deficits or jaun
dice.
Laboratory tests may be useful in the differential diagnosis. They should include

1 1 60
NECK, THYROID, PARATHYROIDI

erythrocyte sedimentation rate, evaluation of thyroid function and detection of an


tithyroid antibodies. In some cases, there is an elevated ESR. Euthyroidism or hy
pothyroidism is the rule and a positive reading for antithyroid antibodies occurs in
as many as 67% of cases. Further diagnostic tests should include imaging studies and
FNA or surgical biopsy to make a definitive diagnosis and, especially, to exclude
malignancy.

Imaging Findings
I n addition to providing useful diagnostic information, imaging studies are required
to determine the extent of disease and to exclude other sites of systemic involve
ment. Cross-sectional imaging of the orbits, mediastinum or abdomen may be re
quired in selected cases.
Plain films of the neck and chest may disclose enlargement of the thyroid gland,
thyroid calcifications and airway deviation or stenosis. However, cross-sectional
imaging better depicts the presence of extrathyroid extension, which is the hallmark
of this disease.
Ultrasound is the optimal screening test for thyroid disease. Typically, the in
volved portions of the gland are homogeneously hypoechoeic, but a nodular ap
pearance, indistinguishable from multinodular goiter, may also be seen. US may also
detect extra thyroid fibrotic masses and exclude the presence of lymphadenopathy.
Limitations of this modality include the evaluation of mediastinal extent and inva
sion of air-containing structures such as the larynx, esophagus and trachea.
Due to its multiplanar capability, MRI adequately depicts the extent of the lesion
and does not necessitate intravenous contrast. The gland may be partially or dif
fusely enlarged and the involved portions tend to be hypointense on both T l W and
T2W images. These are the signal characteristics of fibrotic tissue, although amyloid
deposits and hemochromatosis may share these same features on MRI. Addition
ally MR1 may allow distinction from Hashimoto's thyroiditis, which tends to be
markedly hyperintense on T2WI . Less commonly, the gland is heterogeneous in sig
nal intensity, especially when the disease is superimposed upon a pre-existing goi
ter. Solitary or multiple nodules may also be present, as in the case presented (Figs.
36-A and 36-8). Extracapsular extension manifests as an irregularity of the margins
of the gland, obscurity of the fascial planes, and obvious invasion of the surround
ing structures. When the isthmus of the gland is involved, infiltration of the strap
muscles and skin may be seen. Other structures frequently invaded include the ster
nocleidomastoid muscle, carotid space, tracheoesophageal groove and postcricoid
and retropharyngeal regions. Airway deviation and stenosis is a frequent finding, as
well as indistinctness of the esophagus. Vocal cord paralysis, when present, manifests
as a paramedian vocal cord, ipsilateral anteromedial deviation of the aryepiglottic
fold and enlargement of the pyriform sinus (Fig. 36-C). The presence of cervical
lymphadenopathy favors malignancy, most likely anaplastic thyroid carcinoma. As
recurrence is seen in a high percentage of cases, imaging follow up is usually re
quired.
CT requires intravenous contrast administration and the only advantage over
MRI is the detection of dystrophic calcification, an inconsistent and nonspecific
finding in Riedel"s thyroiditis. Scintigraphic studies with Tc pertechnetate show non
specific decreased activity in the affected portions of the gland.

Management
Surgery may be required to alleviate compressive symptoms and for cosmetic rea
sons, or to make the definitive diagnosis when the FNA is nondiagnostic or doubt
ful. Tracheostomy may be required for life threatening airway compromise, includ
ing cases of bilateral vocal cord paralysis. When malignancy is excluded, surgery
should be limited, to avoid complications resulting (rom attempted dissection of the
invasive fibrotic tissue from surrounding structures. Potential complications include
E K, TH YROID, PA RATHY ROIDI

Case 37
Clinical Presentation
A 72-year-old male with a long tanding anterior neck mas pre ented with recent
onset of dysphagia, hoar ene s and stridor.

Radiologic Findings
Coronal Tl WI (Fig. 37-A) and axial T2Wl (Fig. 37-B) of the neck show multinodu
lar, diffuse enlargement of the thyroid gland extending into the superior and ante-
163
ECK, THYROID, PARATHYROID

rior mediastinum and compressing and displacing the trachea and esophagus. TIlere
is a dominant nodule in the left thyroid lobe extending posteriorly into the left
tracheo-esophageal groove. The signal characteristics of these nodules are heteroge
neous, which is consistent with the presence of cystic degeneration, calcification and
hemorrhage. The gland is lobulated in contour, but shows well-defined margins with no
evidence of invasion of the adjacent neck structures. No lymphadenopathy is apparent.

Differential Diagnosis: Multinodular Thyroid


Gland Enlargement
Multinodular goiter
Thyroiditis (subacute, Hashimoto's, Quervain's, post-partum)
Primary or secondary lymphoma
Metastatic disease
Undifferentiated/anaplastic carcinoma
Thyroid sarcoma
Graves disease

Diagnosis
Multinodular goiter

Discussion
Epidemiology
Goiter is a nonspecific term meaning diffuse enlargement of the thyroid gland,
which may be seen in a large variety of pathologic processes affecting the thyroid
gland. However, it is commonly used as a synonym [or nontoxic or euthyroid goiter.
Endemic goiter, defined as goiter in at least 10% of the population, is the most
common cause of goiter worldwide. Seventy five per cent of cases occur in third
world countries, which lack iodine prophylaxis, and the remainder in mountainous
regions of Europe and Asia. In the United States, endemic goiter is virtually non
existent and most cases of goiter are termed sporadic goiters. The incidence of goiter
in iodine-replete populations has been estimated between 4 and 6% . Non-endemic
goiter is more common in women and in the elderly. The incidence of malignant de
generation varies from I to 4% and increases with age and prior exposure to radiation.

Pathophysiology
The pathophysiologic mechanism common to all causes of goiter is an excessive
stinlUlation of the epithelial follicular cells leading either to excessive replication
and formation of hyperplastic [ollicules, or to hyperfunction with excessive produc
tion and accumulation o[ colloid material. I n endemic goiter, the gland hypertro
phies in an attempt to produce more thyroid hormone with less available iodine.
Most goitrogens act through the hypothalamic-hypophyseal loop, producing an
increase in thyroid stimulating hormone (TSH). Another common mechanism is the
stimulation by thyroid antibodies that act directly on the TSH receptors in the
follicular cells such as long acting t h yroid stimulator (LATS) in Graves' disease.
These thyroid antibodies can also damage the thyroid cells as seen in HashinlOto's
thyroiditis. Goiter may also result from congenital enzymatic deficiencies impairing
iodine uptake, or organification, or be part o[ congenital syndromes such as Pan
dred's syndrome (congenital deafness and goiter). Common goitrogens include
iodine rich foods (soy beans, sea weeds), iodine containing drugs (amiodarone, con
trast material, antiseptics), and drugs containing salts, which compete with iodine for
thyroid absortion (lithium). The typical progression of goiter is from diffuse
enlargement of the thyroid gland to increasing nodularity.

11 64
NECK, THYROID, PARATHYROIDI

Pathologically, goiter is characterized by follicular hypertrophy and hyperplasia


with accumulation of colloid, resulting in cystic degeneration. When the expanding
gland outgrows the blood supply, follicular necrosis and hemorrhage may occur.
Dystrophic calcification and psammous calcification of stagnant colloid material
are also typical features of goiter. Nodularity results from the presence of small
subsets of follicular cells with abnormal growth potential at different sites through
out the gland. These autonomous replicating follicular cells are thought to be rem
nants of fetal thyroid tissue or to result from somatic mutations resulting in cell
lines with a preferential growth advantage accounting for the formation of a few
well-encapsulated nodules. Mutations in the TSH receptor gene account for the
development of hyperfunctioning adenomas. It should be stressed that autonomous
growth and autonomous function are separate features of follicular cells further
contributing to the heterogeneity of multinodular goiter. Depending on its mor
phologic and functional characteristics, goiter is classified into diffuse or nodular
and euthyroid, hypothyroid or hyperthyroid respectively.
Cytologically, it is impossible to separate follicular adenomas from carcinomas.
1l1erefore, the general term "follicular tumor" is commonly used. Malignancy can
only be diagnosed in the presence of vascular or capsular invasion which requires
evaluation of the histologic specimen.

Clinical Findings
Seventy five percent of patients who have a goiter present with a chronic asympto
matic anterior neck mass and seek medical attention for cosmetic reasons. Most
commonly, goiters are detected by physicians during routine physical examination.
A small percentage present with compressive symptoms, symptoms of thyroid dys
function (either hypo- or hyperthyroidism) or sudden growth associated with pain.
Compressive symptoms include dyspnea, stridor, dysphagia and hoarseness and are
seen more often in patients with substernal or intrathoracic goiter. These patients
may rarely present with superior vena cava syndrome and rupture of esophageal
varices. Although vocal cord paralysis usually signals the presence of malignancy, it
may occasionally result from benign compression of the recurrent laryngeal nerve
when the goiter grows posteriorly and medially into the tracheo-esophageal groove.
Even though 80% of patients with goiter are euthyroid at presentation, subclinical
hypo- or hyperthyroidism may be present and therefore thyroid function tests
should be performed in the initial evaluation. Sudden enlargement and pain are
usually associated with hemorrhage into a follicular cyst, but also may signal malig
nant transformation. The clinical history should address not only the possible causes
of goiter (recent viral infection, pregnancy or recent delivery, autoimune disorders
or exposure to goitrogens) but also the risk of malignancy (rate of growth, family
history of thyroid cancer, presence of MEN syndromes, etc). The nationality and res
idence of each patient should be ascertained to rule out the possibility of iodine
deficiency. On physical exam the volume, mobility, and consistency of the gland, and
the presence of any dominant nodules or cervical and supraclavicular lym
phadenopathy should be evaluated.1l1e volume of the gland should be recorded in
the patient's clinical chart to allow for future comparison and determine the success
of therapy. Auscultation of the thyroid gland may detect the presence of a bruit, in
dicating increased vascularity, typical of Graves' disease. Direct or indirect laryn
goscopy also should be performed to evaluate vocal cord mobility. Examination of
other major organs and systems (particularly the cardiovascular system, skin and
eyes) is important to determine the presence of clinical thyroid dysfunction. Bio
chemical analysis including determination of blood levels of TSH, free triiodothy
ronine (T3) and tetraiodothyronine (T4), and antithyroid antibodies may be re
quired. After an adequate history is taken and physical examination and
biochemical testing are performed, most patients do not require further diagnostic
tests. When the goiter is multinodular at palpation, FNA of any dominant or en
larging nodules is advised to exclude malignancy.
ECK, THYROI D, PARATHYROID

Imaging Findings
Imaging of a goiter has five major goals: ( 1 ) to provide an accurate assessment of
glandular volume and morphology; (2) to evaluate compression of adjacent struc
tures including the airway; (3) to determine the functional status of clinically suspi
cious nodules; (4) to detect imaging features suggestive of malignant degeneration;
and (5) to direct FNA. However, the cost-effectiveness of imaging and the preferred
modalities for addressing these problems are the subject of controversy. Surgical
candidates should have a cross sectional imaging study to determine the anatomy of
the gland and its relationship to adjacent structures. MRI is preferred over CT as it
does not involve ionizing radiation, does not require contrast administration, and al
lows for multiplanar evaluation. The use of iodine based contrast materials on CT
not only may precipitate a hyperthyroid crisis but it will interfere with subsequent
scintigraphic studies, laboratory tests and radioiodine therapy. TIle only advantage
of CT is better sensitivity in detection of thyroid calcifications that, although non
specific, may be of diagnostic value. M R I gives optimal delineation of the size and
configuration of the gland. TIle coronal and sagittal images are particularly helpful
in assessing the cranio-caudal extent of the gland, detecting the presence of any me
diastinal components (plunging goiter) and assessing the status of the airway and
esophagus.
The use of scintigraphic studies to evaluate the functional status of thyroid nod
ules is questionable as FNAB is more accurate than scintigraphy in the assessment
of thyroid nodules. Scintigraphic studies may be performed with 1 1 23 or Tc99m. The
former is used to evaluate glandular function whereas the latter is used to evaluate
glandular morphology. According to the amount of radiotracer uptake, nodules may
be classified as hot (hyperfunctioning), warm (normally functioning), or cold (hy
pofunctioning). The importance of this classification lies in the fact that 10 to 25%

11 66
NECK, THYROID, PARATHYROIDI

D E

of cold nodules are malignant whereas the risk of malignancy in warm or hot nod
ules is 9% , and I to 4% , respectively. Scintigraphy also may be helpful in patients
with hyperthyroid goiter as it differentiates Graves' disease (characterized by a dif
fuse increase in iodine uptake), toxic multinodular goiter, (presenting with multiple
areas of increased uptake), and Plummer's disease (manifesting as a single toxic
nodule causing near total suppression of the remainder of the thyroid gland). How
ever, scintigraphy shows a lot of overlap between benign and malignant nodules and
is not sensitive to nodular lesions that are smaller than 1 cm.
Ultrasound is the optimal imaging modality for evaluation of the thyroid gland.
I t is widely available, rapid, and inexpensive. Ultrasound is very sensitive in the de
tection of focal thyroid lesions and may show additional lesions in a patient with a
single thyroid nodule on palpation. 11 is also very accurate in distinguishing solid
and cystic lesions and can provide guidance for FNAs of any suspicious nonpalpa
ble lesions. Although early studies suggested a higher risk of malignancy in solitary
thyroid nodules, most recent series do not confirm these data. [n patients with non
diagnostic "blind" FNAs, ultrasound may assist in directing the needle to solid com
ponents of the lesion and increase diagnostic accuracy. The major drawback of US
is an inability to evaluate substernal goiters, requiring further assessment with cross
sectional imaging.
Plain films of the chest or soft tissues of the neck may give a gross evaluation of
the extent of the gland and detect airway compromise. The imaging features of a
goiter include an enlarged heterogeneous thyroid gland with single or multiple nod
ules and areas of cystic degeneration, hemorrhage and calcification. A lateral plain
film of the soft tissues of the neck in a patient with papillary carcinoma arising
within a multinodular goiter, (Fig. 37-C) shows a large soft tissue mass in the ante
rior neck extending inferiorly into the mediastinum. This mass displaces the cervi
cal trachea posteriorly and decreases the anteroposterior diameter of the trachea. A
large nodular area with multiple punctiform calcifications is noted bulging anteri
orly in the soft tissues of the anterior neck. Larger, coarse calcifications are also
noted projecting posteriorly to this mass.
Cystic lesions may show fluid-fluid levels due to intracystic hemorrhage or debris.
A longitudinal section of a thyroid ultrasound in a patient with colloid goiter (Fig.
37-D) demonstrates a large predominantly cystic nodule in the inferior aspect of the
right thyroid lobe with some posterior acoustic enhancement. Echogenic material is
seen layering in the dependent portion of the lesion, probably representing debris
or hemorrhage. The margins of the nodule are well defined. A transverse section of
the right thyroid lobe in another patient (Fig. 37-E) shows an US-guided FNA of a
dominant solid nodule in a multinodular goiter that had recently increased in size.
The small hyperechoic foci with acoustic shadowing represent calcification. The
margins of the nodule are well defined, surrounded by a hypoechoic halo. The mar-
1 NECK, THYROID, PARATHYROID
gins of the gland are usually lobulated but well defined with no evidence of invasion
PEARLS/PITFALLS of adjacent structures or regional lymphadenopathy. Graves' disease manifests as an
A goiter can presen t as a long enlarged thyroid gland. Sonographically, the gland shows lobulated margins, a dif
standing, asymptomatic anterior fusely hypoechoic texture and increased vascularity better appreciated on Doppler
neck mass. US (thyroid inferno).
Differential diagnosis of a goiter includes several other lesions that present with
The imaging features of a goiter an enlarged heterogeneous gland. These include several forms of thyroiditis,
include a diffuse, heterogeneous anaplastic thyroid cancer, lymphoma and metastatic thyroid disease. In the acute
enlargement of the thyroid gland inflammatory phase Hashimoto's thyroiditis and Quervain's thyriditis may be par
with mUltiple nodules and areas ticularly difficult to differentiate from a goiter, requiring adequate clinical and
of cystic degeneration, calcifica biochemical correlation.
tion or hemorrhage.
Possible mediastinal extension Management
causing compressive symptoms Therapy of a goiter should be directed to its cause. Therefore, it may include dietary
can be seen in a small percentage iodine supplements, elimination of goitrogens, anti-infammatory drugs and anti
of patients. thyroid drugs. Treatment should also address problems associated with hyper
thyroidism including management of hypertension. arrythmia and ophthalmologic
FNA studies should be per

problems.
formed in patients who have Most patients with euthyroid goiters can be managed conservatively with clinical
dominant or rapidly enlarging monitoring of the volume of the gland. However, surgical treatment may be re
nodules.. quired for cosmesis, alleviation of compressive symptoms or malignant degenera
A clinician shouLd take care not
tion.
to fail to recognize a malignant Decrease in glandular volume may be achieved with antithyroid drugs such as
nodule within a goiter. levothyroxin, radioiodine or surgery. US guided ethanol ablation of hyperfunction
ing thyroid nodules has also been used with high success rates.
A clinician shouLd take care not
to fail to evaluate the full extent
of the gland. Suggested Readings
It's important not to mistake Daniels, G.H. Thyroid nodules and noduJar thyroids: a clinical overview. Compre
hensive Therapy 22(4):239-50, 1996 Apr.

other diffuse thyroid processes


such as thyroiditis, thyroid lym Dworkin, H.J., Meier, D.A., Kaplan, M. Advances in the management of patients
phoma or anaplastic carcinoma with thyroid disease. Seminars in Nuclear Medicine 25(3):205-20, 1 995 Jul.
for goiter. Hurley, D.L., Gharib, H. Evaluation and management of multinodular goiter. 010-
laryngologic Clinics of North America, 1 996 Aug, 29(4):527-40.
Naik, K.S., Bury, R.F. l maging the thyroid. Clinical Radiology 53(9):630-9, 1998 Sep.
Petrone, I.R. A primary care approach to the adult patient with nodular thyroid dis
ease. Archives of Family Medicine 5(2):92-100, 1 996 Feb.
Singh, B., Lucente, F.E., Shaha, A.R. Substernal goiter: a clinical review. American
Journal of Otolaryngology 1 5(6):409-16, 1994 Nov-Dec.

1 1 68
N ECK, THYROI D, PARATHYROIDI

Case 38
Clinical Presentation
A 76-year-old female with a long history of neck swelling reported a rapidly en
larging right neck mass.

Radiologic Findings
An axial CT through the level of the cricoid ring (Fig. 38-A) shows a large inhomo
geneous mass within the right lobe of the thyroid gland. This mass is predominantly
1 NECK, THYROID, PARATHYROID
hypodense and shows a gross calcification. TIle margins of the gland are indistinct
with no cleavage plane between the mass and the strap muscles. Extension into the
postcricoid region also is seen with leftward deviation of the in(raglottic larynx and
cervical esophagus. Laterally, the mass surrounds and distorts the internal jugular
vein, which is otherwise patent. An axial view at the level of the proximal trachea
(Fig. 38-B) shows inhomogeneity of both lobes of the thyroid gland, an annular cal
cification in the right lobe, and two hypodense lymph nodes in the right internal
jugular chain. An axial section at the level of the thoracic inlet (Fig. 38-C) clearly
shows the strap muscle infiltration. An axial section at the level of the glottis (Fig.
38 D) demonstrates right vocal cord paralysis, with the right vocal cord paramedian
-

in position and a patulous piriform sinus. There is no evidence of cartilaginous in


vasion.

Differential Diagnosis: An Aggressive


looking Thyroid Mass
Infection: Acute suppurative thyroiditis
Inflammatory: Reidel's thyroiditis
Malignant tumors: anaplastic carcinoma, lymphoma, sarcoma, squamous cell car
cinoma, metastasis

D iagnosis
Anaplastic thyroid carcinoma

D iscussion
TIle differential diagnoses of an enlarged thyroid gland include diffuse and multin
odular goiters, infectious and inflammatory thyroiditis and benign and malignant
tumors (Table 38- 1 ). Rapidly enlarging thyroid masses are usually associated with
signs of compression and pain, and are due to acute infection/inflammation (acute
thyroiditis), hemorrhage in a benign or malignant lesion, or aggressive malignancies
such as anaplastic carcinoma or lymphoma. The presence of aggressive features in a
thyroid mass, as seen in this case, suggests malignancy, one exception being Reidel's
thyroiditis, which is a benign condition with malignant features. Clinically aggressive
lesions manifest rapid growth, hoarseness, dysphagia and dyspnea. Characteristic
imaging features of these lesions include ill-defined margins, invasion of adjacent
structures with loss of cleavage planes between muscles, airway and the carotid
sheath, vocal cord paralysis and associated adenopathy.
A variety of histologic types are seen with thyroid malignancy including. in order
of decreasing frequency, papillary, follicular, medullary, anaplastic, lymphoma, epi
dermoid, sarcoma and metastasis. Although these cannot be differentiated clinically
or on the basis of imaging alone, certain features can suggest the correct diagnosis.
In the present case, the patient's age (seventh decade), previous history of long
standing goiter, rapid growth and the presence of a ill-defined mass with aggressive
features in a background of an heterogeneous, enlarged gland and nodal metastasis
point to anaplastic carcinoma. Other thyroid carcinomas should be considered in
the differential diagnosis although most tend to occur in younger age groups.
Follicular tumor also can be associated with goiter and is usually unifocal. Nodal
involvement is rare but the remaining features are indistinguishable from the other
forms of malignancy.
Papillary carcinoma occurs most frequently in the fifth decade and generally pre
sents as a unifocal lesion with well- or ill-defined borders (40 and 60% of cases re
spectively). This lesion can show cystic degeneration, hemorrhage and calcification.
Nodal spread is very common and can display the same features as the primary tumor.
Medullary carcinoma usually presents with benign features and most are sus
pected clinically due to an elevated calcitonin level. These tumors are easily distin-

1 .129
NECK, THYROID, PARATHYROIDI

guished from the other differentiated thyroid cancers on the basis of their lack of
iodine uptake on scintigraphic studies.
Lymphoma can present as a diffusely enlarged gland, mUltiple modules or, more
commonly, as a solitary mass (80% of cases). It also may behave as an aggressive/
infiltrative mass and can have associated adenopathy. A previous history of
Hashimoto's thyroiditis is the rule, and the presence of necrosis or calcification is
rare (less than 7%).
Sarcomas are rare tumors i n the thyroid gland and are not associated with a prior
history of goiter. The most common variants are angiosarcoma and fibrosarcoma.
Imaging findings do not allow distinction from other tumors.
Metastases to the thyroid are usually not clinically apparent and are a late event
in a patient with known history of cancer. The most frequent presentation is multi
ple discrete lesions and the most common tumors that metastasize to the thyroid are
breast, lung, renal cell carcinoma and melanoma.
Reidel's thyroiditis is a rare chronic inflammatory condition that is characterized
by extensive fibrosis of the gland infiltrating the capsule and adjacent structures. The
clinical presentation can be similar to that of a malignant condition and imaging fea
tures can also be misleading. Enlarged nodes are not usually present. M RI can be
helpful in differentiating this condition from malignancy due to the typical signal
intensity of fibrous tissue (hypointense on T1 W and TIW images).
Acute thyroiditis is usually clinically apparent with signs and symptoms of in
flammation being prominent features. It is usually unilateral, limited to one thyroid
lobe. When there is abscess formation and rupture, indistinctness of the adjacent
structures and nodal involvement can be confused with malignancy.
Anaplastic carcinoma of the thyroid can be indistinguishable from an exten
sive esophageal carcinoma in a patient presenting with dysphagia. This is often
the case when the bulk of the tumor grows posteriorly and involves the cervical
esophagus.

Epidemiology
Anaplastic thyroid carcinoma is a rare tumor ( 1 to 10% of all thyroid malignancies)
and is one of the most lethal tumors of the head and neck region. I n areas of en
demic goiter the incidence is considerably higher with some series reporting an
i ncidence of 30% . I n addition, patients with longstanding goiter have an increased
susceptibility to this tumor. Coexistence with other histologic variants, particularly
with follicular and papillary tumors, suggest that anaplastic carcinoma can result
from dedifferentiation of these well-differentiated tumor types. It tends to occur in
older patients than other thyroid gland tumors and a slight female predominance is
consistently reported in large series. Association with radiation exposure has never
been proven.

Clinical Findings
The most common and specific clinical presentation is a rapidly growing thyroid
mass i n a patient with previous stable thyroid gland enlargement. Other symptoms
such as dyspnea, dysphagia and hoarseness are equally frequent in patients with
simple goiter or other thyroid masses, both benign and malignant. Weight loss is a
frequent associated symptom. On physical examination the most frequent finding,
aside from the neck mass, is vocal cord paralysis. Mediastinal extension can also be
responsible for superior vena cava syndrome.

I maging Findings
A thyroid mass or enlargement should initially be assessed by ultrasound. Ultra
sound delineates the gland's morphology, identifies focal lesions and their charac
teristics, detects enlarged nodes, and allows assessment of patency of the neck ves-
I NECK, THYROID, PARATHYROID

sels using Doppler mode. Anaplastic carcinomas usually present as ill-defined


hypoechoic masses within an inhomogeneous and enlarged gland.
CT or M R I should be used to determine invasion of adjacent structures, medi
astinal extension and to further characterize enlarged lymph nodes looking for
evidence of necrosis, calcification and extra-capsular invasion. CT easily detects cal
cifications which are common in this tumor. Internal areas of necrosis are commonly
seen and present as ill-defined regions of hypodensity on CT and areas of hy
pointensity on T1 W and hyperintensity on T2W M R images. The pattern of spread
is easily recognized with both imaging techniques. Initially, there is capsular invasion
with infiltration of the extraglandular fat, followed by involvement of the strap mus
cles and sternocleidomastoid muscle and carotid sheath. Invasion of the larynx and
trachea are not uncommon and mediastinal extension is also a possibility.
On M RI there are no specific signal characteristics to suggest the diagnosis.
Anaplastic tumors present as masses hypointense on Tl and hyperintense on T2W
images and show no specific pattern of enhancement. However, MRI can be useful
in distinguishing anaplastic carcinoma from Reidel's thyroiditis and lymphoma. In
Reidel's, fibrosis of the gland makes it hypointense on both T1 and T2W images. The
high cellularity of lymphoma makes it less hyperintense on T2W images than other
thyroid malignancies. Recent studies using MRI with gadolinium enhancement
showed that another distinguishing feature of primary thyroid lymphoma when
compared to other thyroid tumors is poor enhancement. Scintigraphic studies show
no evidence of radioactive iodine uptake which is not specific for this tumor.
Although clinical and imaging features can suggest the diagnosis of anaplastic
tumor, histologic confirmation is mandatory. Tissue can be obtained from FNAB or
from open biopsy. FNAB is a safe procedure with high accuracy rates and should be
preferred to open biopsy whenever surgery is not considered the treatment of
choice. Several samples should be obtained, preferably with imaging guidance to
avoid areas of necrosis or hemorrhage.

Manage ment
The treatment and prognosis of thyroid cancers depend on the extent of the disease.
The staging systems utilized for well differentiated tumors are not very helpful in
anaplastic carcinoma that usually presents with more aggressive features. Prognos
tic factors that proved significant in anaplastic carcinoma include gender, tumor size
and complete surgical resection. Female gender and tumor size less than 6 cm are
associated with a better prognosis. Tumors that are amenable to complete surgical
resection also carry a better outcome.
Although there is no consensus as to optimal therapy for this type of tumor, ag
gressive treatment seems to be associated with a slightly better mean survival rate.
Surgery should be offered to all patients amenable to this type of therapy and
should be used as palliation of respiratory and upper digestive symptoms. Com
bined treatments with chemotherapy and hyperfractionated radiotherapy with or
without debulking surgery also have a role in those patients with extensive locally
invasive lesions. The mean survival rate for this tumor varies between 4 and 1 2
months and the 5 year survival rale ranges from 1 to 7 . 1 'Yo . Death usually results
from respiratory failure or inanition because of invasion of the tumor into the up
per aerodigestive tract or pulmonary metastasis.

Suggested Readings
Brooke, P.K., Hameed, M., Zakowski, M.E Fine-needle aspiration of anaplastic thy
roid carcinoma with varied cytologic and histologic patterns: a case report. Diag
nostic Cytopathology 1 1 ( 1 ) :60-3, 1994.
Greval, R.S., Goyal, S.c., Goyal, R. Chemotherapy and combination therapy in
anaplastic thyroid carcinoma. indian Journal of Medical Sciences 47( 1 1 ):269-71 ,
1993 Nov.
NECK, THYROID, PARATHYROIDI

Hadar, T., Mor, c., Shvero, 1., Levy, R., Segal, K. Anaplastic carcinoma of the thyroid.
European Journal of Surgical Oncology 1 9(6):5 1 1 -6, 1993 Dec.
Levendag, P.c., De Porre, P.M., van Putten, W.L. Anaplastic carcinoma of the thy
roid gland treated by radiation therapy. International Journal of Radiation Oncol
ogy, Biology, Physics 26(1 ): 1 25-8, 1 993 Apr 30.
Mathai, v., I dikula, 1., Fenn, A.S., Nair, A. Do long-standing nodular goitres result in
malignancies? Australian and New Zealand Journal of Surgery 64(3):180-2, 1 994
Mar.
Melliere, 0.1., Ben Yahia, N.E., Becquemin, 1.P., Lange, F., Boulahdour, H. Thyroid
carcinoma with tracheal or esophageal involvement: limited or maximal surgery?
Surgery 1 13(2):166-72, 1993 Feb.
Nakahara, H., Noguchi, S., Murakami, N., et al. Gadolinium enhanced M R imaging
of thyroid and parathyroid masses. Radiology 202(3):765-72, 1 997 Mar.
Peters, 1., O'Reilly, S., Barragry, 1.M. Anaplastic carcinoma of the thyroid following
radio-iodine therapy. Irish Journal of Medical Science 1 62(1 ):3-4, 1 993 Jan.
Russell, P., Lean, c.L., Delbridge, L., May, G.L., Dowd, S., Mountford, C.E. Proton
magnetic resonance and human thyroid neoplasia. I : Discrimination between be
nign and malignant neoplasms. A merican Journal of Medicine 96(4):383-8, 1 994
Apr.
Salabe, G.B. Aetiology of thyroid cancer: an epidemiological overview. Thyroidol
ogy 6(1):1 1-9, 1 994 Apr.
Staunton, M.D. Thyroid cancer: a multivariate analysis on influence of treatment on
long-term survival. European Journal of Surgical Oncology 20(6):613-21, 1 994 Dec.
Tan, R.K., Finley, R.K. 3rd., Driscoll, D., Bakamjian, v., Hicks, w.L. Jr., Shedd, D.P
Anaplastic carcinoma of the thyroid: a 24-year experience. Head and Neck
17(1):41-7, discussion 47-8, 1 995 Jan-Feb.
Tennvall, 1., Lundell, G., Hallquist, A., Wahlberg, P. , Wallin, G., TibbLin, S. Combined
doxorubicin, hyperfractionated radiotherapy, and surgery in anaplastic thyroid car
cinoma. Report on two protocols. The Swedish Anaplastic Thyroid Cancer Group.
Cancer 74(4): 1348-54, 1 994 Aug 15.
I N ECK, THYROID, PARATHYROID

Case 39
Clinical Presentation
A 47-year-old female presented with fatigue, weakness and diffuse bone pain. Bio
chemical analysis showed hypercalcemia.

A B

Radiologic Findings
Axial T1 W (Fig. 39-A) and TZW (Fig. 39-B) coronal (Fig. 39-C) poslconlrasl TJ W
sections show a small, well-circumscribed, homogeneously enhancing lesion in the
left tracheo-esophageal groove. (Case courtesy of Dr. Fernando Torrinha.)
NECK, THYROID, PARATHYROIDI

Differential Diagnosis: D iscreet Mass in the


Tracheosophegeal Groove
Exophytic thyroid nodule
Lymphadenopathy
Parathyroid adenoma/ carcinoma/ cyst
Nerve sheath tumor (cervical roots/ vagus nerve)

Diagnosis
Parathyroid adenoma

Discussion
Epidemiology
Primary hyperparathyroidism has a prevalence of 0.037% , with 100,000 new cases
diagnosed annually in the United States. The disease is more prevalent in post
menopausal women with a peak incidence between the fifth and sixth decades.
Patients with MEN syndrome tend to present earlier.

Clinical Findings
Most cases of primary hyperparathyroidism are diagnosed in asymptomatic pa
tients. When symptoms occur, the most common complaints are fatigue, bone pain,
and recurrent renal stones. The classic association of "stones" (renal calculi),
"groans" (abdominal cramps), "bones" (osteopenia and arthritides), and "moans"
(psychiatric disturbances) is rarely seen.
Although hypercalcemic crisis and compression symptoms may occur with large
parathyroid adenomas, paticularly when they undergo hemorrhage or necrose, they
are more commonly associated with parathyroid carcinoma. Hoarseness, dyspnea,
stridor and dysphagia are the most common symptoms, resulting from compression
of adjacent structures.

Pathophysiology
Primary hyperparathyroidism results from increased secretion of parathyroid hor
mone (PTH) and subsequent hypercalcemia. Parathyroid adenoma acounts for
most of the cases of primary hyperparathyroidism (80 to 85% ), followed by parathy
roid hyperplasia (15%), parathyroid cysts 5%), and parathyroid carcinoma
1 % ). Multiple adenomas are present in 2 to 3% of cases. Ectopic adenomas are
not uncommon, representing 10% of all cases.
To localize the lesion, it is important to know the anatomy and embryology of the
parathyroid glands. Normally, four parathyroid glands are present in each individual
(two superior and two inferior) but, supranumerary glands, up to six in number, are
seen in 13% of people. The superior parathyroids originate from the endoderm of
the fourth pharyngeal pouch along with the thyroid gland. Because their descent to
the normal position is minimal and intimately related to the thyroid gland, their
position behind the upper lower third of the thyroid is relatively constant with only
2% of ectopic locations. These include ectopic locations at or above the superior
thyroid pole, posterior and medially in the retropharyngeal region and below the
inferior thyroid artery. Mediastinal locations are rare and usually in the posterior
mediastinum.
The inferior parathyroid glands originate from the 3rd pharyngeal pouch in close
relationship to the thymic anlage. Because they migrate a long distance along with
the thymus, ectopic locations are more frequent (50% ). The most common anatomic
location for the inferior parathyroid glands is lateral to the lower pole of the thy-
E K, T H Y RO I D. PARATHY R O I D

roid. Ectopic location may be anywhere from the angle of the mandible to the low
anterior media tinum. I ntrathyroid and intrathymic locations are een in 2% of
cases. Histologically, parathyroid adenoma are rather monomorphic, compo ed of
chief cells and oxyphil cells embedded within fibrou and adipose ti ue.

Imaging Findings
The optimal imaging modality [or asse sment of primary hyperparathyroidism, and
whether imaging is necc ary in all patients, are topic of debale. Because the diag
nosis is u ually made on the basis of clinical and biochemical dala, Ihe primary role
of imaging i to localize the di ease pre-operatively, and detect any other head and
neck pathology that may be t reated simultaneou ly. However. becau c most
parathyroid adenomas are peri thyroidal in location, surgical treatment al ne with
out preoperative localization i ucces ful in 70 to 90% . depending on the erie , and
experience of the surgical team. Although pre-operative imaging improves this uc
ce s rate to 97%, opponent of thi approach argue that it doc not significantly
change surgical morbidity or operative time. I n addition. some studie suggest that
the co t of preoperative imaging outweighs its benefits. Arguments to support pre
operative imaging include the need for only unilateral cervicotOmy when a single
adenoma is delected, identification of ectOpic adenomas. and reduction in operating
r om time. Therefore. the approach should be tailored to each particular in titution
depending on the experience of bOlh the head and neck surgeon and radiologi I ,
and availability of imaging modalities. Longitudinal U of the neck i n a patient wilh

E F

1 76
NECK, THYROID, PARATHYROIDI

parathyroid adenoma (Fig. 39-0; courtesy of Dr. Fernando Torrinha) shows a fairly
homogeneous, hypoechoic mass posterior to the right thyroid lobe. The mass is
oblong and shows increased through transmission. Two small hypoechoic lesions are
noted in the inferior third of the right thyroid lobe.
The options for imaging the parathyroid glands are multiple and include US, CT,
M R I and nuclear medicine stidies. Studies comparing the accuracy of these imaging
modalities alone and in several different combinations show conflicting results in
different series. Far from being consensual, general guidelines suggest the use of a
functional study (parathyroid scintigraphy) in association with an anatomic study
(cross-sectional imaging). In the newly diagnosed patient, US should be used as the
screening modality because it is the least expensive and most widely available of all
modalities, followed by MRI, when the results are negative or doubtful. The ap
proach for residual and recurrent hyperparathyroidism includes M R I and parathy
roid scintigraphy. For difficult, conflicting cases intraoperative US and invasive stud
ies such as angiography and venous sampling for PTH levels, are recommended.
On US, using a linear array 7.5 to 10 Mhz transducer, parathyroid adenomas man
ifest as round or oblong well circumscribed lesions, peri thyroidal in location.
Arathyroid adenomas are usually small, ranging from a few millimeters to 2 cm in
size, and homogeneously hypoechoic due to their hypercellularity and cellular
monomorphism. Increased through-transmission of the US beam is common due to
the lack of acoustic interfaces. Limitations of this modality include inability to de
tect ectopic adenomas in the mediastinum or near the airway or osseous structures,
and low accuracy in differentiating adenomas from exophytic thyroid nodules and
cervical lymphadenopathy. The use of color Doppler may be useful in distinguishing
parathyroid adenomas from thyroid nodules, because the former lack Doppler sig
nal. In addition, the presence of a bean-shaped lesion with a central area of hyper
echogenicity is more likely to be a lymph node.
CT has the advantage of allowing simultaneous imaging of the neck and the me
diastinum and allows for the detection of most ectopic adenomas. Parathyroid ade
nomas present as well-margined lesions, hypodense to the thyroid gland, enhancing
in 25% of cases. Limitations of CT include the need for intravenous contrast ad
ministration to differentiate parathyroid adenomas from adjacent vessels, artifacts
from breathing and swallowing, and beam hardening artifacts, particularly from the
shoulder. The inexperienced radiologist may also be confused by tortuous vessels or
a tortuous esophagus. Differentiation between adenoma and enlarged lymph nodes
may be imposible.
MRI is the most accurate of the cross-sectional modalities in detecting eutopic or
ectopic parathyroid adenomas. These lesions are of intermediate signal intensity on
Tl WI, hyperintense on TIWI and show intense, homogeneous enhancement in
most cases. However, highly cellular lesions may be of higher signal intensity on
Tl WI and show intermediate signal intensity on TIWI due to a low water content.
The most common M R l pitfall is confusion with an enlarged lymph node.
Parathyroid adenomas can be heterogeneous with areas of cystic degeneration
and hemorrhage, causing further confusion with exophytic thyroid lesions. Calciii
cation of a parathyroid adenoma is, however, uncommon. Concomitant thyroid le
sions are seen in 41 % of patients diagnosed with primary hyperparathyroidism. Ill
defined margins, invasion of adjacent structures and the presence of cervical
lymphadenopathy are all suggestive of malignancy.
The diagnosis of parathyroid hyperplasia is based solely on enlargement of the
parathyroid glands, and the imaging diagnosis is difficult. Normal parathyroid
glands usually measure 5 x 3 x 1 mm. When parathyroid hyperplasia is present,
MEN syndrome should be considered as it is the underlying cause in 30% of cases.
Parathyroid cysts are rarely the cause of primary hyperparathyroidism. Although
congenital epithelial parathyroid cysts may occur, most are thought to represent cys
tic adenomas. When present, they manifest as peri thyroidal or mediastinal cystic le
sions and the differential diagnosis includes congenital cysts such as third and fourth
branchial clefts, bronchogenic and thymic cysts. A variety of scintigraphic studies are
1 NECK, THYROID, PARATHYROID
available for parathyroid imaging. The most accurate include technetium 99m and
PEARLS/PITFALLS thallium 201 subtraction studies and technetium sestamibi studies with or without
Primary hyperthyroidism is most thallium 201 or 1 131 subtraction. The most recent literature reports high accuracy
prevalent in post-menopausal for the sestamibi study using delayed (2 bour) images, precluding the need for imag
woman. ing subtraction, which can be a source of error and technical difficulty. The increased
fixation of thallium 201 and sestamibi in parathyroid adenomas results from the
On US, parathyroid adenomas high level of mithochondria in the oxyphilic cells. Limitations of tbe scintigraphic
manifest as small, homogeneous, studies include poor detection of small lesions, particularly those smaller then 1 cm,
well circumscribed, perithyroidal even wben associated with SPECT imaging. Detection of parathyroid hyperplasia
or mediastinal in location. and intrathyroidal parathyroid adenomas is also problematic. When evaluating a pa
tient with residual or recurrent primary hyperthyroidism of the parathyroid glands
Imaging evaluation with US and aU imaging modalities are less accurate due to post-operative changes, including dis
possibly M RI should be used in tortion of anatomy, presence of post-inflammatory granulation tissue and reactive
the newly diagnosed patient. lymphadenopathy. Enhanced axial CT section througb the superior mediastinum of
a patient with ectopic Parathyroid adenoma (Fig. 39-E; courtesy of Dr. Fernando
Evaluation with MRI and Tc ses Torrinba) shows a well-circumscribed enhancing mass in the prevascular compart
tamibi studies sbould be used for ment. Enhanced axial CT section in a patient with parathyroid carcinoma (Fig.
the palient with residual or re 39-F; courtesy of Dr. Fernando Torrinha) shows an ill-defined mass in the left tra
current disease. cheoesophageal groove infiltrating the left thyroid lobe. No clear cleavage plane
Consider MEN II (Sipple's syn with the esophagus is noted.
drome) as a possible diagnosis in
patients with parathyroid hyper Management
plasia.
The gold standard for treatment of parathyroid adenoma is surgical resection. When
Care should be taken not to mis a single lesion is successfully localized preoperatively, a unilateral cervicotomy is
take enlarged lymph nodes, ves performed. However, some experts recommend intra-operative determination of
sels, or tortuous esophagus or ex- PTH levels to exclude the possibility of a second missed lesion. When no presurgi
0phytic tbyroidal lesions for cal imaging is performed, bilateral cervicotomies are routinely performed and, if no
parathyroid adenoma. lesions are found, an anterior cervicotomy or mediatinostomy may be required. For
high surgical risk patients or patients who refuse surgery, parathyroid adenomas
Care should be taken not to fail may be managed by image guided therapies such as ethanol ablation, laser ablation
to recognize ectopic adenomas or electrocautery. Parathyroid carcinoma is managed by radical surgery followed by
due to limited imaging studies. radiation therapy.
Care should be taken not to fail
to recognize multiple lesions.
---
Suggested Readings
Hodin, R.A., Silen, W.W. Detection and management of parathyroid tumors. Cur
rent Opinion in Oncology 9( 1):75-8, 1997 Jan.
Hopkins, CR., Reading, CC Thyroid and parathyroid imaging. Seminars in Ultra
sound, Ct and Mr 1 6(4):279-95, 1995 Aug.
Loevner, L.A lmaging of the parathyroid glands. Seminars in Ultrasound, Ct and M,.
17(6):563-75, 1 996 Dec.
Norton, I.A Reoperation for missed parathyroid adenoma. Advances in Surgery
31 :273-97, 1997.
Santos, E., Higgins, CB., Clark, O. Clinical image. Recurrent hyperparathyroidism
caused by a paratbyroid cystic adenoma: localization by MRI. lournal of Computer
Assisted Tomography 20(6):996-8, 1996 Nov-Dec.
Shah a, AR., Sarkar, S., Strashun, A., Yeh, S. Sestamibi scan for preoperative local
ization in primary byperparatbyroidism. Head and Neck 19(2):87-91, 1 997 Mar.
Shen, w., DUren, M., Morita, E., Higgins, C, Duh, O.Y., Siperstein, AE., Clark, O.H.
Reoperation for persistent or recurrent primary hyperparathryoidism. Archives of
Surgery 131(8):861-7, discussion 867-9, 1996 Aug.
Yousem, D.M., Scbeff, A.M. Thyroid and parathyroid gland pathology. Role of imag
ing. Otoiaryngologic Clinics of North America 28(3):621--49, 1995 Jun.

1 1 78
NECK, THYROID, PARATHYROIDI

Case 40
Clinical Presentation
A 49-year-old female presented with hemoptysis and cough. After clinical examina
tion, a CT scan was requested to evaluate a possible mass.

A B

Radiologic Findings
Axial CT images through the level of the upper trachea (Fig. 40-A) and slightly
lower (Fig. 40-B) show a right sided posterolateral soft tissue mass which extends
into the lumen and causes some airway compromise. There is no evidence of calci
fication. The mass appears to erode the cartilage and extend into the adjacent tra
cheal bed.

Differential Diagnosis: Primary


Tracheal Carcinoma
Papilloma
Granuloma
Foreign body
Primary tracheal carcinoma
Secondary invasion from adjacent neoplasm
Amyloid
Ectopic thryoid

Diagnosis
Primary tracheal carcinoma
1 NECK, THYROID, PARATHYROID

Discussion
Epidemiology
Primary neoplasms of the trachea are rare, especiaUy when compared with neo
plasms of the larynx or lung. Depending on the series reported, there is approxi
mately one carcinoma of the trachea for every 20 carcinomas of the larynx, and for
every 1 80 lung carcinomas. Why this is so remains unclear.
Secondary involvement of the trachea by carcinomas is much more common with
1 0-20 cases for every case of primary tracheal carcinoma.

c D

1 1 80
NECK, THYROID, PARATHYROIDI

Pathophysiology
PEARLSIPITFALLS
A primary tracheal carcinoma Primary tracheal tumors in the pediatric age group are distinctly uncommon with
appears as an invasive tracheal 90% of tumors occurring in adults. In the pediatric population, less than 10% of
mass which narrows the tracheal tumors are malignant. In adults, 50% of primary tracheal neoplasms are malignant.
lumen. Of these most are carcinomas. Half of these carcinomas are the familiar squamous
cell carcinoma. It should also be noted that in patients with primary tracheal carci
In patients with primary tracheal noma, 20% will manifest synchronous or metachronous tumors.
carcinoma, 20% may have Benign nonneoplastic soft tissue masses such as granulomas may occur in the tra
metacbronous or synchronous chea but they do not tend to have an aggressive radiographic appearance.
tumors.
Hemoptysis is a presenting symp Clinical Findings
tom in half the patients with pri The clinical manifestations of tracheal tumors are primarily through the effects of
mary tracheal carcinoma. partial airway obstruction. Cough is present in half the patients and dyspnea and
wheezing are common. Approximately 25% of the tracheal lumen must be occluded
Care should be taken not to mis before obstructive symptoms become dominant. Hemoptysis is present in 10% of
take primary tracheal carcinoma patients with benign tracheal tumors, and in over 50% of those with malignant tu
for a more benign tracheal mors.
process.
Imaging Findings
The diagnosis is suggested by the presence of a destructive mass narrowing the tra
cheal lumen. Secondary invasion by malignancies of adjacent structures may have a
similar appearance and are much more common than primary tracheal carcinoma.
MR images in another patient with thyroid carcinoma (Figs. 40-C and 40-0) show
secondary tracheal invasion. The axial T l WI (Fig. 40-C) shows the destructive thy
roid mass invading and narrowing the tracheal lumen. The coronal T1 WI (Fig.
40-0) shows similar findings. With a more benign appearing tracheal mass, other
considerations such as granuloma or papilloma should be considered. When tra
cheal lumen narrowing is due to cartilage changes without a discrete mass, chon
dromalacia should be considered. An axial CT in another patient with chondroma
lacia of the trachea (Fig. 40-E) shows angular narrowing of the lumen and the
absence of a soft tissue mass.

Suggested Readings
Holbert, I.M., Strollo, O.c. Imaging of the normal trachea. lou al of Thoracic
rn

Imaging 10(3): 1 7 1 -9, 1 995 Summer.


Weber, A.L. Radiologic evaluation of the trachea. Chest Surgery Clinics of North
America 6(4):637-73, 1 996 Nov.
v.

Orbit and Visual Pathways


O R B I T A D V1SUAL PATHWAYSI

Case 41
Clinical Presentation
A 96-year-old female presented with left orbital pain, swelling and decrea ed vi ion.
On phy ical exam there was swelling and erythema of tbe periorbital soft ti sue
proptosis and chemo i . Fundo copic examination bowed papilledema.

B c

Radiologic Findings
Axial cr cans of t he orbit (Fig. 4 1 -A and 4 1 -8) show marked swelling and soft
tissue thickening of the pre eptal compartment of the left orbit. There i also soft
tissue thickening and enhancement surrounding the globe. Fatty stranding i noted
in the extra- and inlraconal compartments. No fluid collections are een. Coronal
section (Fig. 4 1 -C) how enlargement of the left lateral rectus mu de with ill
defined margin . Also note the stranding of the retrobulbar fat.

1 85
I ORBIT AND VISUAL PATHWAYS

Differential D iagnosis: O rbital Swelling


Orbital infection (bacterial, viral, fungal)
Orbital inflammation: orbital pseudotumor; granulomatous diseases (sarcoid,
Wegener's granulomatosis); vasculitides, connective tissue disorders

Diagnosis
Orbital cellulitis (serratia isolated on culture)

Discussion
Epidemiology
Although the incidence of infections has markedly decreased with the availability
of antibiotic therapy, it is still the most common primary orbital disease process,
accounting for more than 50% of orbital pathology. It may affect all age groups,
although children are especially susceptible.

Pathophysiology
Orbital infection may occur by three different mechanisms, which include in de
creasing order of frequency, spread from adjacent structures, penetrating trauma
and hematogenous spread. Sinusitis is the most frequent cause of orbital cellulitis,
accounting for two thirds of all cases of orbital infection, usually spreading from the
ethmoid sinus. Spread of infection occurs via the thin, often dehiscent, bony orbital
walls and natural foramina. Other sources of direct spread include infection of the
facial soft tissues, pharynx and teeth, which may extend into the orbit by means of
interconnecting venous plexuses. Orbital involvement also may result from infec
tion arising in the lacrimal apparatus (lacrimal sac and lacrimal gland). Penetrating
trauma, particularly in the presence of an intraorbital foreign body, is the second
most common cause of orbital cellulitis. Hematogenous spread of infection to the
orbit may be a late event in patients with septicemia.
Orbital infections may be acute, subacute or chronic. The majority of acute infec
tions are bacterial in origin and the most frequent agents include staphylococcus,
streptococcus, hemophilus, neisseria and pseudomonas. Viral infections can also pre
sent acutely and are usualy due to herpes viruses (herpes simplex and herpes
zoster). Mycobacterial, fungal and parasitic infections tend to be subacute or
chronic at onset. Immunocompromised and diabetic patients are especially prone to
fungal infections. Mucor and aspergillus species are the most common opportunis
tic agents. They may involve the orbit by direct extension from the nasal cavity and
paranasal sinuses, through bone destruction. Pathologically, acute infection is char
acterized by polymorphonuclear leucocytes infiltration, leading to necrosis and
rapid destruction of the tissue planes. In chronic infections a mononuclear infiltrate
predominates with or without associated multinucleated giant cells. Mycobacterial
infection is characterized by the presence of caseating granulomas. Angioinvasion
and infarction are the hallmarks of fungal infection.

Clinical Findings
Presenting symptoms depend on the severity and extent of the orbital involvement.
Orbital infections usually spread in a fairly constant fashion from periorbital in
flammatory edema to pre-septal cellulitis, followed by retroseptal involvement. Sub
periosteal abscess, orbital cellulitis and, eventually, an intraorbital abscess may en
sue. Acute inflammation is characterized by rapid onset of orbital pain and
periorbital soft tissue swelling and redness involving the preseptal compartment.
The orbital septum, a fibromembranous structure within the eyelids and contiguous
ORBIT A N D V ISUAL PATHWAYS1

with the periorbita, prevents spread of infection into the retroseptal compartment
in the early stages. Retroseptal involvement presents with more striking symptoms
such as loss of visual acuity, diplopia and exophthalmus. A mass in the superotem
poral aspect of the orbit suggests the presence of dacryoadenitis, which may be the
origin of orbital infection.
Physical exam may disclose chemosis, ophthalmoplegia, papilledema, and inflam
matory signs in the periorbital soft tissues. Signs and symptoms of sinusitis or other
head and neck infections are usually present. In children, orbital cellulitis may be
the first sign of sinus infection. History of prior trauma, or the possibility of an
intraocular foreign body should be elicited. Complications of orbital cellulitis in
clude thrombosis of the superior orbital vein and cavernous sinus and intracranial
spread of infection, with development of epidural, subdural or intra parenchymal
abscesses.

Imaging Findings
Imaging studies are performed to determine the etiology, extent and complications
of the disease. One of the major goals is to differentiate between pre- and retrosep
tal involvement. which have different prognoses and management. CT is the modal
ity of choice, as it nicely depicts cortical bony changes and radiopaque intraorbital
foreign bodies, and differentiates phlegmon from drainable fluid collections. Thin,
3mm axial and coronal sections should be obtained through the orbits and paranasal
sinuses after intravenous contrast administration. M R I is useful in the evaluation of
intracranial complications and magnetic resonance venography ( M RV) may dis
close thrombosis of the superior ophthalmic vein and cavernous sinus without the
need for intravenous contrast administration.
Imaging cannot differentiate between inflammatory periorbital edema and pre
septal cellulitis, as they both present with soft tissue thickening of the periorbital
soft tissues and stranding of the subcutaneous fat. These inflammatory changes
involve the eyelids and adjacent soft tissues. There may be associated uveoscleral
thickening and enhancement. Progression to orbital cellulitis manifests as stranding
of the intra- and extraconal fat, proptosis and, eventually, as enlargement of the
extraocular muscles (EOMs). Subperiosteal phlegmon or abscess is not uncommon
when infection spreads from the ethmoidal sinuses. Accumulation of inflammatory

o E

1 87 1
I ORBIT AND VISUAL PATHWAYS

tissue and edema beneath the periorbita manifests on cr as a soft tissue density
located between the lamina papyracea and the medial rectus muscle. Displacement
of a thickened periorbital membrane and medial rectus into the orbit is usually
seen. cr of the orbits of another patient ( Fig. 4 1 -0) shows soft tissue thickening and
fatty stranding limited to the preseptal and periorbital soft tissues on the left. These
inflammatory changes extend to the medial canthus and prezygomatic soft tissues.
111is patient was diagnosed with orbital cellulitis secondary to ethmoidal sinusitis.
Progression to a central area of hypodensity surrounded by an enhancing rim
indicate abscess formation. This is usually accompanied by increasing mass effect
upon the EOMs and optic nerve. Enlargement of the EOMs with ill-defined mar
gins also may be depicted on cr. Enhancement along the optic nerve sheath indi
cates perineuritis. An intraconal abscess is rarely seen. Thrombosis of the superior
ophthalmic vein and cavernous sinus manifest on CT by e nlargement and absence
of contrast enhancement. The hallmark of fungal infection is the presence of bone
destruction and rapid progression, whjch can be noted in Figure 4 1 -E. This axial
T2W image from another patient hows a crescentic region of hyperintensity in the
preseptal compartment of the right orbit, extending laterally into the suprazygo
matic masticator space. The right globe is slightly proptotic. 111ere is diffuse hyper
intensity of the intra- and extraconal fat. The right ethmoidal air cells are opacified
and there appears to be bony disruption of the medial wall of the right orbit. The
diagnosis in this case was mucormycosis of the paranasal sinuses invading the orbit
in a diabetic patient. (Case courtesy Dr. J.P. Wensel. )

Management
Medical therapy with antibiotics should be directed against the causal agent.
according to the results of orbital cultures and antibiograms. Retroseptal involve
ment may require intravenous therapy, especially when the optic nerve is in danger.
Subperiosteal and intraorbital abscesses should be drained surgically. Early surgical
debridement is also required in cases of fungal infection to avoid the devastating
consequences of intracranial extention.

Suggested Readings
Davis, P.C.. Newman, .1 . Advances in neuroimaging of the visual pathways. Amer
.

ican Journal of Op/lIhalmology 1 2 1 (6):690--705. 1 996 Jun.


Haugen, J.R., Ramlo, J. H., Serious complications of acute sin usitis. Posigraduare
Medicine 93( 1 ): 1 1 5-8, 1 22, 1 25, 1 993 Jan.
Klapper. S.R., Patrinely. J.R., Kaplan, S.L., Font, R.L.. Atypical mycobacterial infec
tion of the orbit. Op/1fhalmology 102( 1 0) : 1 536-4 1 . 1 995 Oct.
Mauriello. lA. Jr .. Yepez. .. Mostafavi, R .. Barofsky, J.. Kapila. R .. Baredes. S., or
ris, J. Invasive rhinosino-orbital aspergillosis with precipitous visual loss. Canadian
Journal of Ophlhalmology 30(3): 1 24-30, 1 995 Apr.
Mitchell, C.S., Nelson. M.D. Jr., Orofacial abscesses of odontogenic origin in the pe
diatric patient. Report of two cases. Pedialric Radiology 23(6 ):432-4. 1 993.
Moll, G.W. Jr .. Raila, EA .. Liu. G.c.. Conerly. A.W. Sr. Rhinocerebral mucormycosis
in 1 0 0 M. Sequential magnetic resonance imaging of long-term survival with inten
sive therapy. Diabeles Care 1 7( I I ): 1 348-53, 1 994 Nov.
Sullivan, T.l, Patel, B.c., Aylward, G.W., Wright, J.E. Anaerobic orbital abscess sec
ondary to intraorbital wood. Auslralian and New Zealand Journal of Ophthalmol
ogy 2 1 ( 1 ):49-52. 1 993 Feb.
ORBIT A N D V ISUAL PATHWAYSI

Case 42
Clinical Presentation
The patient is an adult male who presented with new onset proptosis on the right.

A _........
.
I ORBIT AND V I S UA L PATHWAYS

Radiologic Findings
An axial T l W, noncontrast MR image through the orbit (Fig. 42-A) shows a ho
mogenous enlargement of the right lacrimal gland. There is no evidence of bony in
vasion. The above findings were confirmed in the coronal ( Fig. 42-B) and axial TIW
(Fig. 42-C) images.

Differential Diagnosis: Lacrimal Gland Mass


Lymphoid/I nflammatory: acute (viral, bacterial, orbital pseudotumor, lymphoma)
or chronic (sarcoid, Wegener's, thyroid opthmalopathy, Sjogrens's syndrome,
Mickulicz syndrome)
Epithelial neoplasms: pleomorphic adenoma or malignant tumors (adenoid cystic
carcinoma, mucoepidermoid carcinoma, adenocarcinoma)

Diagnosis
Lacrimal gland enlargement due to acute dacroadenitis

Discussion
Pathophysiology
One useful approach to diagnosing masses of the lacrimal glands is to consider that
approximately 50% are of the Iymphoidlinflammatory type and 50% are neoplasms
of epithelial origin. Dermoid cysts are not included in this scheme. This is because
although dermoid cysts may arise near the lacrimal fossa, they are not true lacrimal
gland lesions, but, instead, arise from embryological rests in the orbit. Metastases to
the lacrimal gland have been reported, but they are rare.
The lesions in the lymphoid/inflammatory group run the spectrum from mild in
flammatory conditions all the way to lymphoma (usually nonhodgkins). Lesions in
this group tend to result in diffuse enlargement of the gland and usually show no
bone erosion.
It is useful to group these lesions according to their time course. The acute group
includes bacterial and viral dacroadenitis and tends to involve younger age groups.
Orbital pseudotumor and lymphoma can also present with this picture. Entities in
the acute group can also have a more chronic course.
Other more chronic conditions to be considered are sarcoid, Wegener's, thyroid
opthmalopathy, Mickulicz, and Sjogrens's syndromes. Mickulicz's syndrome is non
specific lacrimal (and salivary) gland swelling associated with leukemia, lymphoma,
TB, syphilis, or sarcoid.
Sarcoid Sjbgrens's disease is lymphocytic infiltration and enlargement of lacrimal
(and salivary) glands associated with connective tissue diseases. This infiltration may

1 1 90
ORBIT A N D VISUAL PATHWAYSI

be associated with decreased lacrimation (xerophthalmia) and xerostomia (dry


PEARLS/PITFALLS mouth).
Lacrimal gland enlargement is The other 50% of lacrimal gland masses tend to be epithelial cell tumors. Radi
often a non-specific radiographic ographically this group is more likely to show aggressive behavior and occasionally
finding. bone involvement. Fifty percent of the tumors in this group are pleomorphic ade
nomas. A reformatted sagittal CT scan in another patient with a pleomorphic ade
Half the cases are lymphoid/in noma of the lacrimal gland is seen in Figure 42-D.
flammatory and half are epithe The remaining 50% include adenoid cystic carcinoma, mucoepidermoid carci
lial neoplasms. noma, adenocarcinoma, and others.

Epithelial tumors tend to be


more aggressive. Imaging Findings
Enlargement of the lacrimal gland is usually a nonspecific radiographic finding that
Dermoid cysts may occur in this
may be due to either inflammatory (dacroadenitis) or neoplastic conditions. The
location but also occur through
lacrimal glands may be involved with a variety of disease processes. However,
out the orbit and in other loca
because of the histologic similarities of the lacrimal gland with salivary gland tissue,
tions in the head and neck.
these two groups of glands share many of the same pathologies. Although the radi
Avoid mistaking other (non ologic findings are usually nonspecific, the clinical setting may help suggest the
lacrimal) masses for lacrimal diagnosis.
gland enlargement.
---
Management
Clinical management of lacrimal gland masses depends on the pathology involved.
While a specific tissue diagnosis is often not possible based on the CT or M R I find
ings, these studies provide valuable information about the extent of disease and
involvement of adjacent structures.

Suggested Readings
Bilaniuk, L.T., Farber, M . Imaging of developmental anomalies of the eye and the
orbit. American loumal of Neuroradiology 1 3(2):793-803, 1992 Mar-Apr.
Carmody, R.F., Mafee, M . F., Goodwin, lA., Small, K., Haery, C. Orbital and
optic pathway sarcoidosis: M R findings. American lournal of Neuroradiology
1 5(4):775-83, 1 994 Apr.
Krzystolik, M., Warner, M.A. Orbit and adnexal neoplasia. Current Opinion in Oph
thalmology 6(5):78-85, 1995 Oct.
Shields, c.L., Shields, lA. Lacrimal gland tumors. International Ophthalmology
Clinics 33(3): 1 8 1 8, 1 993 Summer.
10RBIT A

Case 43
Clinical Presentation
A 9 1 -year-old female pre ented with acute left orbital pain. On clinical exam, there
was no proptosis. No inflammatory changes were noted in the anterior orbital com
partment.

A B

1 1 92
ORBIT AND VI SUAL PATHWAYSI

Radiologic Findings
An axial enhanced TI W image with fat-saturation (Fig. 43-A) shows enlargement of
the lateral and medial recti, with involvement of the anterior tendinous insertion on
the globe and abnormal enhancement of the intraconal fat. Coronal sections (Figs.
43-B and 43-C) also show generalized enlargement of the extraocular muscles
(EOMs) which have ill-defined margins and enhancement of the retrobulbar fat.
Abnormal enhancement also is seen along the optic nerve sheath. These changes are
more pronounced near the orbital apex. There is no evidence of extension into the
optic tracts or cavernous sinus.

Differential Diagnosis: E nlargement of the


E xtraocular Muscles
I n fection (bacterial, viral or fungal)
Thyroid orbitopathy
Inflammatory conditions: orbital pseudotumor, Sjiigrens's syndrome, Mickulicz's
syndrome, granulomatous conditions (sarcoid, Wegener's granulomatosis), con
nective tissue disorders (rheumatoid arthritis, lupus, scleroderma), retained for
eign bodies
Vasculitides (PAN, giant cell arteritis)
Tumors: hemangioma, rhabdomyoma, plasmacytoma, lymphoma, leukemia, rhab
domyosarcoma
Amyloidosis

Diagnosis
Orbital pseudotumor

Discussion
Epidemiology
The term orbital pseudotumor was first used by Birsch-Hirschfeld in 1 905 to de
scribe an idiopathic inflammatory condition involving the orbit. This entity is the
third most common ophthalmologic disease after thyroid orbitopathy and Iympho
proliferative disorders, and is the most frequent cause of an intraorbital mass in
adults. [t is the underlying cause of unilateral exophthalmos in 25% of patients. Only
5 to 15% of cases of orbital pseudotumor occur in the pediatric age group.

Pathophysiology
Orbital pseudotumor defines a clinically and histologically heterogeneous group of
lesions for which an identifiable cause cannot be found. I t is a diagnosis of exclusion,
after other local or systemic disease processes with similar c1inico-radiographic find
ings have been ruled out.
Proposed etiologic factors include an autoimune process and Iymphoproliferative
disease.
Histologic changes define two different types of orbital pseudotumor and may
vary in the course of the disease. In the acute inflammatory type there is infiltra
tion of the orbit by polymorphous acute inflammatory cells dispersed in a matrix
of granulation tissue, whereas in the chronic sclerosing form, inflammatory changes
are replaced by fibrotic tissue with subsequent stretching and deformity of the
intraorbital structures. There is also a predominantly lymphocytic variety of pseudo
tumor, thought to be a prelymphomatous condition. The presence of germinal
follicles and increased vascularity helps in differenting this condition from true
lymphoma.

1 93
1 0RBIT AND VI SUA L PATHWAYS

Clinical Findings
The symptoms in orbital pseudotumor depend on the degree of inflammatory re
sponse, the stage (acute, subacute or chronic), and on the affected orbital structures.
The most common symptoms in the acute inflammatory form of disease include
abrupt onset of orbital pain, swelling of the periorbital tissues, double vision and,
occasionally, decreased vision. This form of presentation is easily confused with
orbital or periorbital infection, and it is common for patients to be treated initially
with antibiotics for several days. On physical exam, there may be chemosis, sclero
uveitis, papillitis and exudative retinal detachment. The chronic sclerosing form has
a more indolent course. Patients typically present with ophthalmoplegia, diplopia,
proptosis and progressive visual loss.
Several clinico-radiographic classifications based on the involvement of different
orbital structures have been proposed, but these normally overlap. The most fre
quently used separates orbital pseudotumor into six different categories: acute and
subacute idiopathic anterior orbital pseudotumor, acute and subacute idiopathic
diffuse orbital inflammation. myositic form, apical orbital inflammation (Tolosa
H unt syndrome), idiopathic dacryoadenitis, and idiopathic perineuritis. The Tolosa
Hunt syndrome is a variant of pseudotumor characterized by an inflammatory infil
tration of the orbital apex, with extention into the superior orbital fisure and
cavernous sinus. Clinically, it manifests with painful ophthalmoplegia involving cra
nial nerves I l l, I V and VI and hyposthesia of the periorbital tissues due to involve
ment of the first division of the trigeminal nerve.
Isolated involvement of the lacrimal gland may be acute or chronic, presenting as
either a painful or painless mass in the superolateral orbit. Differentiation from
dacryoadenitis, lymphoma or other lacrimal gland tumors may require tissue sam
pling.
Perineuritis presents with orbital pain on extraocular movement, decreased vision
and papilledema. I ncreased pain upon retrodisplacement of the globe and the pres
ence of proptosis differentiates perineuritis from true optic neuritis. I rreversible
ophthalmoplegia and amaurosis are dismal complications when the disease is not
promptly recognized and treated.

Imaging Findings
I maging of orbital pseudotumor, although nonspecific, is useful in defining the ex
tent of the disease and excluding true orbital tumors. When the differentiation from
orbital tumor is not possible, FNA may be required. CT and M R I are best suited for
these goals, with M R I being superior to CT in the evaluation of the optic nerve, cav
ernous sinus and globe. Fat-saturated TI W sequences after contrast enhancement
are required for evaluation of inflammatory changes in the retrobulbar fat, optic
nerve and cavernous sinus. Ocular ultrasound is a good method to evaluate the
globe, but much less accurate for assessment of the retrobulbar structures.
The structures involved by orbital pseudotumor are, in decreasing order of fre
quency, retrobulbar fat (76% ), extraocular muscles (57% ), optic nerve (38% ), uvea
and sclera (33% ), and lacrimal gland (5% ) .
I n the anterior variety o f pseudotumor, the inflammatory changes are seen i n the
preseptal orbital compartment and in the globe itself. There is swelling, thickening
and enhancement of the preseptal soft tissues. Ocular findings include uveoscleral
thickening, blurring of the ocular-optic nerve junction and accumulation of fluid in
Tenon's capsule. An accumulation of fluid beneath Tenon's capsule. anechoic on
ultrasonography (T sign), appears as a crescentic area of hyperintensity on TIW
MR images. Retinal detachment may also be present.
Diffuse orbital pseudotumor, also referred to as a tumefactive or infiltrative type
of pseudotumor, is characterized by increased soft tissue density and abnormal
enhancement of the retrobulbar fat, which molds itself around the globe without
distorting the surrounding structures. The globe may be slightly proptotic, but is nor-

1 1 94
ORBIT AND V ISUAL PATHWAYSI

mal in shape. I n the sclerosing variety of pseudotumor, the extensive desmoplastic


changes manifest as regions of bypointensity on both Tl W and T2W images and a
greater amount of distortion and retraction of the orbital structures is seen. These
findings harbor a poor prognosis. The absence of mass effect and bony invasion are
the main diagnostic clues to differentiate pseudotumor from a true orbital tumor,
although distinction from orbital lymphoma may be impossible based on imaging
alone.
In the myositic form, the inflammatory changes involve one or more EOMs on
the same side. Bilateral muscular involvement is less common and, when present,
may be confused with thyroid orbitopathy. The main characteristics are enlargement
of the muscle(s), involving both the belly and tendinous insertions, and ill-defined
margins. The snperior muscle complex and the medial rectus are the most com
monly affected.
Apical orbital inflammation presents as irregular, infiltrative, enbancing soft tis
sue involving the orbital apex, which may compress the muscle cone and optic
nerve, leading to ischemic neuropathy. Extension of this abnormal soft tissue into
the cavernous sinus and superior orbital fissure is a hallmark of the Tolosa-Hunt
syndrome.
Lacrimal adenitis manifests as a nonspecific and diffuse enlargement of the
lacrimal gland, with ill-defined margins, and associated inflammatory changes in the
surrounding soft tissues. The morphology of the gland is usually preserved.
Idiopathic perineuritis usually occurs in association with other inflammatory find
ings, but occasionally as an isolated finding. The hallmark is enlargement and en
bancement of the optic nerve-sheath complex, which may show ill-defined margins.
I n general terms, the most important imaging features in differentiating pseudo
tumor from true neoplastic disease are the absence of mass effect, confinement to
tbe space of origin and the lack of bony destruction. However, there are a few
reported cases of extraorbital extension of pseudotumor, including intracranial
extension. As opposed to lymphoma, orbital pseudotumor shows intense enhance
ment and is hyperintense on T2W images (except for the sclerosing variety). When
tbe diagnosis is in doubt, imaging guided FNA, followed by flow cytometry or
Southern blot analysis of tbe cytopatbologic specimen, usually allows differentiation
between the lymphocytic variety of pseudotumor and true lymphoma.
M R I of the orbits in another patient (enhanced Tl W images with fat-saturation,

D E
( ORBIT AND VI SUAL PATHWAYS

Fig. 43-D) show intense enhancement in the region of the orbital apex with indis
PEARLS/PITFALLS tinctness of the muscular cone, intraconal fat and optic nerve sheath. This patient
Sudden onset of unilateral was diagnosed with orbital pseudotumor (apical variety).
painful proptosis or ophthalmo For another patient with an orbital pseudotumor (anterior variety), an axial CT
plegia in an otherwise healthy of the orbits (Fig. 43-E) demonstrates swelling and soft tissue thickening of the pre
patient suggests an orbital septal compartment of the left orbit. There is thickening and abnormal enhance
pseudotumor. ment of the ocular layers, representing uveo-scleritis. The retrobulbar fat adjacent to
the globe also shows stranding, consistent with inflammatory change.
Rapid and lasting response to An enhanced frequency selective fat suppression imaging ( FATSAT) T1 W, coro
steroids usually indicates a nal M R image shows enlargement and enhancement of the medial, inferior, and
pseudotumor. lateral recti of the left orbit. The muscles show ragged margins with slight enhance
ment of adjacent fat. Note also enhancement surrounding the optic nerve. This
Imaging shows abnormal en patient has orbital pseudotumor (myositic variety with associated perineuritis).
hancing soft tissue. with ill
defined margins, confined to the Management
space of origin and little or no
mass effect. Orbital pseudotumor usually has a dramatic and rapid response to steroids. Thus.
not infrequently, a course of steroids is attempted when the diagnosis is in doubt.
Do not mistake an orbital However, several other diseases, including tumors, may have a partial response to
pseudotumor for a neoplastic steroids. Close follow-up of patients is mandatory whenever the diagnosis is in
process. doubt. The acute inflammatory variety of the disease is particularly sensitive to
steroids. TIle subacute and chronic forms. including the sclerosing variety. are unre
Partial response to steroids does sponsive. Radiation therapy and immunosuppressors may be used in patients unre
not exclude the possibility of an sponsive to steroids, but are not successful in treating fibrosclerosing lesions. When
orbital neoplasm! the optic nerve is in danger of compressive ischemic neuropathy, surgical decom
pression should be attempted.

Suggested Readings
Atabay, c., Tyutyunikov, A., Scalise, D., Stolarski, c., Hayes. M.B .. Kennerdell, 1.S.,
Wall, 1. Serum antibodies reactive with eye muscle membrane antigens are detected
in patients with nonspecific orbital inflammation. Ophthalmology 1 02( 1 ): 1 45-53,
1 995 Jan.
Berger, 1.W, Rubin. P.A .. Jakobiec, F.A. Pediatric orbital pseudotumor: case report
and review of the literature. international Ophthalmology Clinics 36( I ): 1 6 1 -77, 1 996
Winter.
Char, D.H., Miller, T., Orbital pseudotumor. Fine-needle aspiration biopsy and re
sponse to therapy. Ophthalmology 1 00( 1 1 ): 1 702-10, 1 993 Nov.
de Jesus, 0., Inserni. 1.A., Gonzalez, A., Colon, L.E. Idiopathic orbital inflammation
with intracranial extension. Case report. Journal of Neurosurgery 85(3):5 1 0-3, 1 996
Sep.
Mombaerts, I.. Goldschmeding, R., Schlingemann, RO., Koornneef, L. What is or
bital pseudotumor? Survey of Ophthalmology 4 1 C I ):66-78, 1 996 Jul-Aug.
Mombaerts, 1 . , Schlingemann, R.O., Goldschmeding, R, Koornneef, L. Are systemic
corticosteroids useful in the management of orbital pseudotumors? Ophthalmology
1 03(3):52 1 -8, 1 996 Mar.
Mombaerts, I . , Schlingemann, Ro., Goldschmeding, R., Koornneef, L. Idiopathic
granulomatous orbital inflammation. Ophthalmology 1 03 ( 1 2):2 1 35-4 1 , 1 996 Dec.
Notter, M., Kern, T., Forrer, A., Meister, F; Schwegler, N. Radiotherapy of pseudo
tumor orbitae. Frontiers of Radiation Therapy and Oncology 30: 1 80-9 J , J 997.
Osguthorpe, 1.D.. Hochman, M. l nflammatory sinus diseases affecting the orbit. 010-
laryngologic Clinics of North America 26(4):657-7 J . 1 993 Aug.
Weber, A.L., Jakobiec, F.A., Sabates, N.R Pseudotumor of the orbit. Neuroimaging
Clinics of North America 6( 1 ):73-92, 1 996 Feb.

( 1 96
ORBIT A N D VISUAL PATHWAYSI

Case 44
Clinical Presentation
A 5 1 -year-old female presented with progressive bilateral painless exophthalmus.

A B

Radiologic Findings
An axial CT through the optic nerve canal (Fig. 44-A) shows bilateral enlargement
of the medial recti and crowding of the orbital apices. The lateral recti appear nor-
I ORBIT A N D VISUAL PATHWAYS

mal in thickness. A mild degree of proptosis is also noted. Coronal sections ( Figs.
44-B and 44-C) show bilateral enlargement of the extraocular muscles (EOMs) with
relative sparing of the lateral recti. The section near the orbital apices ( Fig. 44-C)
again shows crowding, which is more pronounced on the right side. Note that the
right optic nerve is surrounded only by a thin rim of fat.

Differential Diagnosis: Extraocular


Muscle E nlargement
Endocrine
Thyroid ophthalmopathy

Acromegaly

I nflammatory

Pseudotumor

I n fection: bacterial, fungal, parasitic (cysticercosis, trichinosis)

Plasma cell granuloma

Sarcoid

Neoplastic

Benign: rhabdomyoma

Malignant: lymphoma, rhabdomyosarcoma, metastatic disease

Vascular

Carotid cavernous fistula

Extradural arteriovenous fistula

Deposition diseases: amyloid

Diagnosis
Thyroid orbitopathy

Discussion
Enlargement of the EOM may result from three basic mechanisms: infiltration by
abnormal cells (inflammatory or neoplastic), deposition of abnormal material (amy
loid, mucopolysaccharides), or edema from vascular congestion.
The extent of involvement, and un i- or bilaterality of the process are valuable
clues in the differential diagnosis. While neoplastic tumors vascular processes and
pseudotumors tend to be unilateral, endocrine diseases tend to affect both orbits.
When the clinical and imaging findings are non diagnostic, fine needle aspiration
(FNA) guided by imaging is a valuable tool for therapy planning.

Epidemiology
Thyroid orbitopathy is also referred to as thyroid associated ophthalmopathy, au
toimmune thyroid disease, endocrine exophthalmus and Graves' ophthalmopathy.
It is the most common orbital disorder, affecting approximately 0.5% of the US
population. Thyroid orbitopathy is the most frequent cause of exophthalmus in
adults, representing 80% of bilateral and 15 to 28% of unilateral exophthalmus. It
usually presents in adulthood with a peak incidence in the fourth and fifth decades
of life. Only 5% of cases occur before age 1 5 . Females are more frequently affected.
The EOMs are the most common orbital structures to be affected. The involve
ment is bilateral in 70 to 85% of cases, although asymmetric in 20 to 30% . Isolated
involvement of a single EOM is much less common, only seen in 1 0% of patients.
Hyperthyroidism is present in the majority of patients (70% ), with increased lev
els of triiodothyronine (D) and tetraiodothyronine (T4). However, thyroid oph
thalmopathy may be the first sign of Graves' disease and preceed any hormonal
changes by a year or more. TIle incidence of proptosis varies widely in different se
ries, ranging from 35 to 93% .

1]2?
ORBIT A N D V ISUAL PATHWAYSI

Table 44-1 Werner's Modified Staging System for Thyroid Orbitopathy

I-Asymptomatic eyelid retraction


I I-Symptomatic eyelid retraction
I II-Proptosis greater than 22 mm without diplopia
I V-Proptosis greater than 22 mm with diplopia
V-Corneal ulceration
VI-Loss of vision

Pathophysiol ogy
Thyroid ophthalmopathy is due to deposition of abnormal material in the orbital
contents, predominantly involving the EOMs and retrobulbar fat. This process
is thought to have an autoimune origin due to cross-reactivity between the eye
muscle and thyroid autoantigens. Several autoantibodies have been implicated,
including long acting thyroid stimulating factor (LATS) and thyroid stimulating
immunoglobulins (TSI), which are now recognized as antibodies against the thy
rotropin receptors of the thyroid gland.
In the early acute i nflammatory stage of the disease, there is deposition of hygro
scopic mucopolysaccharides and glycoproteins, and infiltration by inflammatory
cells (lymphocytes and mast cells). With disease progression, there is replacement
by collagenous tissue, necrosis of muscle fibers and fatty degeneration. These
changes result from chronic increase in orbital pressure, which leads to ischemia and
subsequent edema and fibrosis of the EOMs. The fibrotic phase of the disease is
usually irreversible. Several factors contribute to the development of proptosis, in
cluding enlargement of the EOMs increased volume of retrobulbar fat and vascular
congestion. Ischemia of the optic nerve, due to compression by the enlarged mus
cles in the orbital apex, may result in optic neuropathy.
The degree of orbital involvement does not correlate with the degree of thyroid
dysfunction or the levels of circulating LATS. Thyroid orbitopathy also has been
associated with hypothyroidism and Hashimotos' thyroiditis.

Clinical Findings
The most common presenting symptoms are proptosis and double vision. On clini
cal exam, lid lag due to retraction of the upper eyelid, restricted ocular motility and
loss of sight may be noted. In the acute phase of the disease periorbital swelling and
chemosis are common. Blindness from ischemic optic neuropathy is a dismal conse
quence of thyroid orbitopathy if early decompression is not undertaken. Corneal ul
ceration, due to exposure keratitis, is another possible complication of the disease.
A family history should be elicited, because 30% of patients have other familial
autoimune disorders.
Staging systems to evaluate the severity and progression of the disease have been
devised. The most commonly used is based on clinical findings and classifies patients
into six different stages (Table 44- 1 ) .

I maging Findings
U l trasonography, CT and M R I may be used to evaluate orbital involvement in
Graves' disease. As orbital involvement may be subclinical, all patients should have
an imaging study as a screening test for thyroid orbitopathy. Imaging is useful for
detection and serial follow up of proptosis and is crucial for detection of optic nerve
compression. The degree of proptosis is measured in the axial plane at the level of
the lens, from the interzygomatic line to the anterior margin of the globe. Another
important role of imaging is providing guidance for FNA of undeterminate masses.
IORBIT A

12 00
ORBIT AND V ISUAL PATHWAYSI

Ocular US is well-suited for orbital screening in patients with Graves' disease as it


is innocuous, inexpensive and rapidly performed.
The major finding on cross sectional imaging is enlargement and enhancement of
the EOM, which is usually bilateral and asymmetric. Typically, the muscles most fre
quently involved are, in decreasing order of frequency, the inferior, medial, superior,
and lateral rectus. In patients with isolated involvement of a single muscle the su
perior rectus and levator palpebrae complex is most commonly affected. Classically,
the swelling is maximal in the belly of the muscle, sparing the tendinous attachment
to the globe, and the margins of the involved muscles are smooth. These are impor
tant distinguishing features from orbital pseudotumor, which usually involves the
entire length of the muscle and shows ill-defined margins. Other CT findings include
increased volume and increased density of the retrobulbar fat and uveoscleral thick
enmg.
Crowding of the orbital apex is usually associated with dilatation of the superior
orbital vein and increased diameter of the optic nerve sheath due to CSF trapping
in the subarachnoid space.
On M R imaging the enlarged muscles are hyperintense on TIW images due to
edema and inflammation. Fat-saturated enhanced Tl W images are very sensitive for
detection of muscle enlargement due to the increased vascularity of the muscle. The
better contrast resolution of M R I makes it the best method to evaluate for optic
nerve compression. A "tram track" sign on TIW images may be seen representing
dilatation of the subarachnoid space surrounding the optic nerve and is suspicious
for optic nerve compression. Differential diagnosis of this sign includes increased
intracranial pressure and pseudotumor cerebri.
Scintigraphic studies using I I 11n labelled octeotride have proven helpful in iden
tifying and following patients with inflammatory eye disease as the tracer accumu
lates in the retrobulbar space. TIle radiotracer fixates in the cell membrane of
activated lymphocytes expressing somatostatin receptors.
Axial CT of the orbits in another patient (Fig. 44-0) shows symmetric bilateral
enlargement of the medial and lateral recti, more prominent at the muscular belly
and tapering toward the tendinous insertion on the globes. The margins of the mus
cles are well-defined. The volume of retrobulbar fat appears increased and shows
scanty linear hyperdensities consistent with increased vascularity. There is bilateral
proptosis more prominent on the right side.
Axial (Fig. 44-E) and coronal (Fig. 44-F) CTs of the orbits in another patient show
the appearance of the orbits after decompressive surgery. There has been bilateral
resection of the medial wall of the orbits and the markedly enlarged medial recti are
seen to herniate through the surgical defect, decompressing the optic nerves.
Axial (Fig. 44-G) and sagittal TIW (Fig. 44-H) M R images of the orbits show en
largement of the muscular bellies of the medial, superior and inferior recti, sparing
the tendinous insertions in the globe. There is some crowding of the orbital apices
but a thin rim of fat is seen surrounding both optic nerves. There has been prior or
bital decompression with partial resection of the lamina papyracea bilaterally.

Management
Left to its natural course, the disease resolves spontaneously in the majority of
patients. However, in 1 0% of cases the progression of orbital involvement leads to
recurrent corneal ulcerations and loss of vision. Some local measures may relieve
orbital symptoms, particularly in the acute phase of the disease. TIlese include head
elevation at night, and lubricating eyedrops and ointments. Steroids and radiation
therapy are also useful in the acute inflammatory stage and should be administered
to patients with progressive disease that is unresponsive to local measures. Im
munosuppressive drugs have been used with varying success rates, although the in
herent risks of this kind of therapy limit their use in this benign condition. Surgery
is the only successful therapy in the chronic fibrotic stage of the disease and may be
used to correct eyelid retraction or to decompress the optic nerve. Surgical proce-
I ORBIT AND VI SUAL PATHWAYS

dures attempt to increase the bony orbit in order to decompress the orbital con
PEARLS/PITFALLS tents. One or more orbital walls may be ressected depending on the degree of com
Bilateral or unilateral painless pression.
proptosis is one of the most com
mon presenting symptoms of
thyroid orbitopalhy. Suggested Readings
Cangiarella, J., Cajigas, A., Savala, E. et al. Fine needle aspiration cytology of orbital
M R images of the orbit show
masses. Acta Cytological 40(6): 1205- 1 2 1 1 , 1 996 Nov-Dec.
EOM enlargement involving the
muscular belly, sparing the tendi Kao, S.C, Kendler, D.L., N ugent, PA., Adler, J.S., Rootman. J. Radiotherapy in the
nous insertions on the globe. management of thyroid orbitopathy. Computed tomography and clinical outcomes.
Archives of Ophthalmology 1 1 1 (6):8 1 9-23, 1 993 Jun.
cr findings include an increased McNab, A.A. Orbital decompression for thyroid orbitopathy. Australian and New
volume of the retrobulbar fat. Zealand Journal of Ophthalmology 25( 1 ):55-6 1 , 1 997 Feb.

Do not exclude the diagnosis in Postema, P.T., Krenning, E.P., Wijngaarde, R., Kooy, P P., Oei, H.Y., van den Bosch,
euthyroid patients! WA., Reubi, J.C, Wiersinga, WM .. Hooijkaas, H . , van der Loo T. et al. 1 1 1 -
.

In-DTPA-D-Phe l octreotide scintigraphy in thyroidal and orbital Graves' disease:


Do not fail to recognize muscle a parameter for disease activity? Journal of Clinical Endocrinology and Metabolism
enlargement (when bilateral and 79(6): 1 845-5 1 , 1 994 Dec.
symmetric). Rosen, CE., Kennerdell, J.S. Extreme eyelid swelling as an unusual presentation of
dysthyroid orbitopathy. Journal of Nellro-Ophthalmology 1 5(2):84-9, 1 995 Jun .
Avoid failing to recognize optic Sendrowski, D.P. Hyperthyroidism. Optometry Clinics 3(4):87-97, 1 994.
nerve compression.
Shokeir, M.O., Pudek, M.R., Katz, S., Rootman, J., Kendler, D.L. The relationship of
thyrotropin receptor antibody levels to the severity of thyroid orbitopathy. Clinical
Biochemistry 29(2):1 87-9, 1 996 Apr.
Villadolid, M.C, Yokoyama. N., Izumi, M. et al. Untreated Graves' disease in pa
tients without clinical ophthalmopathy demonstrate a high frequency of extraocular
muscle enlargement, JOllrnal of Clinical Endocrinilogy and Metabolism. 80(9):2830-
2833, 1 995.
Weber, A., Dallow, R., Sabates, N. Graves' disease of the orbit. Nellroimaging Clin
ics of North America 6( 1 ):6 1 -72. 1 996 Feb.
Wilson, WB., Prochoda, M . Radiotherapy for thyroid orbitopathy. Effects on
extraocular muscle balance. Archives of Ophthalmology 1 13( 1 1 ): 1 420-5, 1 995 Nov.

1202
O R B IT D VISUAL PATHWAY

Case 45
Clinical Presentation
A 58-year-old female presented with nonspecific left orbital pain. The ophthalmo
logic exam was normal.

A B

Radiologic Findings
Axial ( Fig. 45-A) and coronal ( Fig. 45-B) fat- uppre ed TI WI M R I cans how an
ill-defined area of hyperinten ity in the medial and inferior aspect of tbe left orbit.
involving both the extra- and intraconal fal. There is no ignal abnormality or mass
effect on the adjacent extraocular mu c1es ( EOM). I ncidentally, prior cataract
urgery is noted bilaterally.

Differential Diagnosis: Diffuse


Hyperintensity in the Extra- and Intraconal
Fat on FATS AT Tl WI
Artifactual: art i fact due to failure of fat suppression
Pathologic: orbital p eudotumor. orbital cellulitis. orbital neoplasm

Diagnosis
Artifact due to a ymmetric failure of fat-suppres ion

Discussion
Epidemiology
Artifacts associated with M R fat uppre sion imaging are relatively frequent in
orbital studies and may mimic orbital pathology. These include complete or partial
failure of fat suppression. which may be symmetric or asymmetric.

Pathophysiology
Fat uppressioo M R J techniques provide a method of improving patial resolution
by decreasing t he 110rmal hyperinten ity of the orbital fal. which may ob cure
203 1
/ORBIT AND VISUAL PATHWAYS

equally hyperintense lesions on enhanced or nonenbanced T l WI sequences. 1l1cre


are several techniques of fat suppression available on M R I scanners which are
based on frequency selective pulses ( FATS AT) phase difference descrimination.
,

saturation of signal from short Tl relaxation times (STIR) and variation of phase
encoding gradients. The former technique, FATS AT, is the most commonly used and
is subject to this artifact.
The FATSAT sequence consists of delivery of a 90 degree radiofrequency ( RF)
pulse, followed by a RF pulse at the resonant frequency of fat to selectively suppress
the fat signal. When two substances with a large difference in magnetic susceptibil
ity, such as air and fat, share an interface, the main magnetic field ( BO) around this
interface is distorted due to a different amplitude of magnetization on each side of
the border. This results in a shift of the resonant frequency of the fat protons near
the interface toward that of water protons. The result is failure of the presaturation
pulse to suppress fat protons.

Imaging Findings
Failure of fat-suppression manifests as ill-defined regions of hyperintensity. ll1e
magnitude of these areas of fat suppression failure are known to depend on the

c D

/204
ORBIT AND VISUA L PATHWAYSI

geometry of the interface and its orientation in regard to the main magnetic field,
PEARLS/PITFALLS being maximal where the interface is perpendicular to BO.
I ll-defined area of hyperintensity A FATSAT artifact is not difficult to recognize when there is complete or sym
on FATSAT images could be a metric failure of fat suppression. However, it may be misleading when asymmetric.
result of an artifact due to fat Asymmetry of this artifact is seen in any circumstance that alters either the geome
suppression. try or the orientation of fat-water or fat-air interfaces. This includes physiologic
asymmetry of the paranasal sinuses or configuration of the orbits, asymmetric dis
When an artifact is present, there ease of the paranasal sinuses (with soft tissue or fluid within the sinus) or asymmet
is an absence of mass effect or ric positioning of the patient'S head in the scanner.
signal abnormalities on otber se Differential diagnosis from orbital pseudotumor, orbital cellulitis and orbital neo
quences. plasm in clinically doubtful cases may be a challenge. Distinguishing features of the
artifact vs pathology include a location near fat-air interfaces, ill-defined margins
Take care not to mistake fat sup
with fading of the hyperintensity towards fat-water interfaces. absence of mass
pression failure for orbital
effect on adjacent structures and absence of signal abnormalities on SE T l W and
pathology.
T2W images.
Both orbital cellulitis and orbital pseudotumor are inflammatory conditions that
are typically hyperintense on T2WI. Orbital neoplasms tend to be more focal and,
in general, are also hyperintense on T2WI.
To avoid misinterpretation of a FATSAT artifact for orbital pathology, it is im
portant to correlate the imaging findings with clinical examination. It may also be
necessary to use an additional technique of fat suppression, less sensitive to suscep
tibility artifacts such as STIR, repeat tbe same sequence, changing the orientation of
the patient's head in the scanner, and correlate the FATSAT images with T l W and
T2W SE sequences.
Coronal FATSAT enhanced Tl WI (Fig. 45-C) shows an ill-defined region of
hyperintensity along the medial and inferior aspects of the orbits bilaterally. The
hyperintensity is maximal near the interfaces with the ethmoid and maxillary
sinuses and fades gradually towards the center of the orbit.
Axial (Fig. 45-D) and coronal (Fig. 45-E) FATSAT contrast enhanced T l WI show
hyperintensity of the intraconal fat in the left orbit, along with marked enlargement
of the extra-ocular muscles.

Suggested Readings
Anzai, Y., Lufkin, R . B., labour, B.A, Hanafee, W.N. Fat-suppression failure artifacts
simulating pathology on frequency-selective fat-suppression MR images of the head
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Barakos, J.A Advances in magnetic resonance imaging of the head and neck. Top
ics in Magnetic Resonance Imaging 6(3): 1 55-65, 1 994 Summer.
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Borges, A.R., LufkiJ1, R.B., H uang, A.Y., Farahani, K., Anold. A.C. Frequency
selective fat suppression MR imaging. Localized asymmetric failure of fat suppres
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sion recovery sequence for fat-suppressed M R l : theory and experimental valida
tion. Magnetic Resonance Imaging 1 1 (3):34 1 -55, 1 993.
Lee, D.H., Simon, J.H . , Szumowski, J., Feasby, T.E., Karlik, S.1., Fox, AJ., Pelz, D.M.
Optic neuritis and orbital lesions: lipid-suppressed chemical shift MR imaging.
Radiology 1 79(2):543-6, 1 99 1 M ay.
LUdeke, K.M., Rbschmann, P., Tischler, R. Susceptibility artefacts in NMR imaging.
Magnetic Resonance Imaging 3(4):329-43, 1 985.
Mintz, E., Kline, L.B., Duvall, E.R. Diagno tic misinterpretation of fat suppression
I ORBIT AND VI SUAL PATHWAYS

orbital magnetic resonance scanning [ letter1 American Journal of Ophthalmology


1 1 5(2):262, 1 993 Feb 1 5 .
Simon, J., Szumowski. 1., Totterman, S.. Kido, D., Ekholm, S., Wicks, A., Plewes, D.
Fat-suppression M R imaging of the orbit. American Journal oj' Nellroradiology
9(5):96 1 -8, 1 988 Sep.
Tien, R.D .. Chu, P.K., Hesselink, J. R., Duberg, A., Wiley, C. Intracranial cryptococ
cosis in immunocompromised patients: CT and M R findings in 29 cases. American
Journal of Nellrorndiology 1 2(2):283-9. 1 99 1 Mar-Apr.
ORBIT AND V1SUAL PATHWAYS1

Case 46
Clinical Presentation
A 48-year-old female with a hi tory of prior surgery and radiation therapy for
a left frontal lobe neoplasm, now pre ents with a new onset of decrea ed vi ual
acuity.

A B

207 1
I ORBIT AND VISUA L PATH WAYS

Radiologic Findings
Coronal noncontrast Tl WI ( Fig. 46-A), coronal post-contrast frequency selective fat
suppression imaging (FATSAT) T l WI ( Fig. 46-B), and axial post-contrast FATSAT
T I WI ( Fig. 46-C) show enlargement and abnormal enhancement of the retrocanalic
ular segment of the optic nerve and optic chiasm, with the left greater than the right.

Differential Diagnosis: Optic Nerve


E nlargement and E nhancement
(Optic Neuritis)
'
Demyelinating disease : multiple sclerosis
Inflammatory: collagen vascular disease, orbital pseudotumor, sarcoid
I nfectious: H I V-related optic neuropathies, tuberculosis, fungal infection, toxo
plasmosis, syphilis
'
Neoplastic : optic glioma, optic nerve meningioma, metastasis, leptomeningeal
carcinomatosis
'
Vascular : ischemic neuropathy (severe hypertension, systemic vasculopathies)
Others: radiation induced optic neuropathy, optic nerve trauma
'
Note: indicates that the process is usually unilateral.

Diagnosis
Radiation-induced optic neuropathy

Discussion
Epidemiology
Optic neuritis is a general term used to describe an acute inflammatory condition
involving the optic nerve. usually presenting as rapid onset of visual loss. While it
occurs as an isolated entity, its greater significance results from its association with
mUltiple sclerosis (MS). In fact, the term optic neuritis is often misused as a syn
onym of optic nerve involvement in MS.
Optic neuritis is a relatively common disease affecting young adults (mean age of
30 years). There is a slight female predominance. The disease is rare in children.

Pathophysiology
Optic nerve inflammation may be due to mUltiple pathologic processes including
demyeLinating disease, infection, systemic inflammatory processes. optic nerve
ischemia and toxic insults. Because the optic nerve is an extension of the CNS, con
sisting of bundles of myelinated axons, it is not surprising that it is affected by the
same demyelinating diseases as the brain. Approximately 35 to 40% of patients with
MS develop optic neuritis and in 15 to 20% optic neuritis is the initial manifestation
of MS, meaning that 20% of patients with optic neuritis will develop MS over a short
or longer term. Concurrent optic neuritis and cervical myelitis is known as Devic's
syndrome, and is uncommonly seen in patients with MS.
Infectious processes involving the CNS and meninges may spread to the optic nerve.
The conduit for spread may be the nerve itself (e.g., neurotropic viruses such as H IV)
or the CSF in the optic nerve sheath. Basilar meningitides such as tuberculosis and fun
gal meningitis are the most common infectious processes spreading to the optic nervc.
Systemic vasculopathies of small and medium sized vessels may also be responsi
ble for optic neuritis.
The optic nerve is fairly sensitive to radiation injury, and radiation necrosis of the
optic nerve is a well known complication when the optic nerve is included in the

1208
O R B IT AND V I SUAL PATHWAYSI

radiation field. This is usually encountered after radiotherapy for orbital, sinonasal,
sellar and orbitofrontal neoplasms. 1l1e effects of radiation are dependent on the
total dose and the amount of fractionation.

Clinical Findings
Optic neuritis may be asymptomatic, although typically, patients present with sud
den loss of visual acuity, pain with eye movement, and dyschromatopsia. The visual
loss tends to be of sudden onset, but may be more gradual, progressing over a
period of 1 to 2 weeks. Slow recovery over a period of 6 to 1 2 weeks is the rule. A
rare chronic progressive unremitting variant of optic neuritis has also been de
scribed, eventually leading to blindness. Symptoms are unilateral in 70% of cases.
Diagnosis is based on clinical history and ophthalmologic evaluation, including
assessment of visual acuity, color vision, and visual fields. Fundoscopic examination
may show optic disc swelling.
A thorough clinical history and neurologic exam should be performed in order to
exclude MS. Even when optic neuritis is seen in isolation, patients should be closely
followed because a significant percentage will eventually develop MS.
Radiation induced optic neuropathy has no specific clinical features. Ophthalmo
logic evaluation may show disc swelling, prepapillary exudates, hemorrhage, and
subretinal fluid. The onset of symptoms is usually remote from the time of radiation
therapy, and may be confused with recurrence of the primary neoplasm.

I m aging Findings
Abnormal imaging findings are not required for the diagnosis of optic neuritis and,
when present, the findings are nonspecific. The main role of imaging is to exclude
other pathologic processes and to detect other white matter abnormalities in the
eNS, making the diagnosis of MS more likely. I maging is also important in the eval
uation of treatment efficacy and in long-term follow-up.
1l1e best imaging modality to evaluate the visual pathway is M R l . The study
should include the orbits and brain. Postcontrast fat-suppressed axial and coronal

D E
I ORBIT AND VISUAL PATH WAYS

T I W images of the orbits are mandatory. The most common findings are optic nerve
PEARLS/PITFALLS enlargement and optic nerve enhancement, which may be focal. segmental or dir
Typically, young adult patients fuse. Single or multiple discontinuous lesions may be seen. More nodular enhance
with optic neuritis present with ment suggests neoplastic involvement, although sarcoid and some infectious
sudden onset of visual loss. processes (such as tuberculosis and fungal infection) may have a similar appearance.
M R I may also show T2W hyperintensity in the optic nerve, optic chiasm and in the
MR1 of the orbits including post parenchymal white matter.
contrast FATSAT Tl W sequence T2W abnormality and/or optic nerve enhancement is present in 56 to 72% of pa
is mandatory. tients with isolated optic neuritis and in 90 to 98% of patients with definitive MS.
The diagnostic yield is increased with the use or inversion recovery or FATSAT
It is important to perform M R I postcontrast sequences and a surface coil. The number, site and size of the lesions
of t h e brain to look for otber evi should be reported. These data are useful to evaluate the success of treatment. The
dence of demyelinating disease. retrobulbar segment of the optic nerve is the most commonly involved segment of
the optic pathway in MS.
The most common MR1 findings CT imaging may also detect optic nerve enlargement and enhancement, but has
for optic neuritis are smooth en a lower accuracy when compared to MRI.
largement and enhancement of Radiation necrosis manifests as a focally cxpanded. enhancing optic nerve and
the optic nerve.
optic chiasm. Contrast enhancement is thought to be due to vascular injury result
ing in increased permeability of the blood-brain barrier.
Avoid mistaking non-neoplastic
Axial T2WI in a 40-year-old woman with lupus (Fig. 46-0) shows diffuse en
optic neuritis for neoplastic
largement and hyperintensity of the intraorbital segment of the left optic nerve.
processes.
FATSAT post-contrast T l WI (Fig. 46-E) shows optic nerve asymmetry due to en
I t is important to confirm radia largement of the left optic nerve. 1l1ere is no apparent abnormal enhancement seen .
tion necrosis of the optic nerve
with recurrence of the primary Manage ment
tumor.
Steroids are used as the "gold standard" treatment for optic neuritis, regardless of
Avoid failing to evaluate tbe cause. Several studies have shown a quicker recovery of vision and a transient pro
brain and miss tbe diagnosis tective effect from development of MS with the use of intravenous methyl pred
of MS. nisolone.

Suggested Readings
Cornblath, W.T., Quint, OJ. M R I of optic nerve enlargement in optic neuritis. Neu
rology 48(4):82 1-5, 1 997 Apr.
Kortvelesy, S. Recent advances in the management of optic neuritis. Hawaii Medical
Journal 56( 1 0):28 1 , 1 997 Oct.
Villablanca, P., Curran, J., Arnold, A .. Lufkin, R. Orbit and optic nerve. Topics in
Magnetic Resonance Imaging 8(2):87-1 1 0. 1 996 Apr.
Weber. A.L., Klufas, R., Pless, M. Imaging evaluation of the optic nerve and visual
pathway including cranial nerves affecting the visual pathway. Neuroimaging Clill
ics of North America 6( 1 ): 1 43-77, 1 996 Feb.
O R B IT AND V ISUAL PATHWAYSf

Case 47
Clinical Presentation
A 64-year-old male presented with gradual proptosis of the right globe over several
years.

A B

Radiologic Findings
A well-defined mass in the intraconal space is present 011 the right ( Fig. 47-A). The
mass effect causes a slight proptosis. The mass is homogeneous on all sequences. On
the TI-weighted sequence, it is hyperintense (Fig. 47-B) ( Image courtesy Drs.
Andrew Berger and Ed Helmer). Figure 47-C shows it to be clear of the optic nerve.
1 0RBIT AND VI SUAL PATH WAYS

Differential Diagnosis: Retrobulbar


Intraconal Mass
Hemangioma
Schwanoma
Metastasis
Pseudotumor
Meningioma
Hemangiopericytoma
Lymphangioma
Varix
Hematic cyst

Diagnosis
Cavernous hemangioma

Discussion
Epidemiology
A cavernous hemangioma is one of the most common tumors of the orbit and the
most common benign primary orbital tumor in the adult. There is a female prepon
derance, and it usuaLly presents in the third to fifth decade of life. The majority of
cavernous hemangiomas are solitary.

Pathophysiology
Cavernous hemangiomas are round or oval in shape and may have a slightly nodu
lar surface. Macroscopically, they are purplish-red in color, well circumscribed, and
are encapsulated by a fine fibrous capsule. Microscopically, cavernous hemangiomas
have numerous blood-filled vascular channels that are lined with flattened en
dothelial cells that are surrounded by smooth muscle. The vascular channels are of
ten separated by fibrous septa. Rarely, phleboliths may be present. Intramuscular
hemangiomas may also occasionally occur.

Clinical Fi ndings
1lle clinical course is characteristic: a slow, progressive enlargement with findings
related to displacement or compression of orbital structures. This is in distinction
to capillary hemangiomas which tend to decrease in size over time. The sign/
symptom at the time of diagnosis is usually proptosis. I f the tumor is in the orbital
apex, it may produce diplopia and optic nerve compression. Typical M RI and CT ap
pearance is that of an oval or round, well-defined enhancing tumor in the intraconal
space. The mass is soft and conforms to the shape of the orbit without distorting that
structure. Occasionally, a small portion of the lesion may extend into the extraconal
space.
On MRI it is usually homogeneous in intensity on T l W sequences and heteroge
neous in appearance on T2W images. On a T I -weighted image. the intensity is
greater than muscle and globe and much less than fat. On a T2-weighted image it is
more intense than any other tissue. The marked hyperintensity on T2 weighting is
due to the high fluid content of the numerous blood-filled microscopic spaces. 1llis
intensity component predominates over that of the fibrous and supporting tissues
and results in marked hyperintensity. Enhancement with contrast agents occurs dif
fusely and moderately. A cavernous hemangioma grows insidiously and may be
come so large that it can expand and remodel the orbital walls.

1212
ORBIT AND VISUAL PATHWAYS(

Management
PEARLS/P ITFALLS
A cavernous hemangioma pre Surgical excision is the treatment of choice. The encapsulated tumor can easily be
sents as a retrobulbar, intraconal totally removed. Cavernous hemangiomas generally do not recur, even after in
mass. complete excision.

Typical MRl and cr appearance


is of a well-defined, contrast en Suggested Readings
hancing tumor. Bilaniuk, L.T., Rapoport, R.1. Magnetic resonance imaging of the orbit. Topics in
Magnetic Resonance Imaging 63: 1 67-8 1 , 1 994 Summer.
Take care not to mistake a cav
ernous hemangioma for a more Forbes, G. Vascular lesions in the orbit. Neuroimaging Clinics of North America
aggressive process. 6 1 : U 3-222, 1 996 Feb.
Mukherji, S.K., Tart, R.P., Fitzsimmons, 1. , Belden, C, McGorray, S., Guy, 1., Man
Avoid mistaking a cavernous cuso, A.A. Fat-suppressed MR of the orbit and cavernous sinus: comparison of fast
hemangioma for a varix of the spin-echo and conventional spin-echo. American Journal of Neuroradiology
ophthalmic veins. 1 59: 1 707- 14, 1 994 Oct.
Sweet, C, Silbergleit, R., Mehta, B. Primary intraosseous hemangioma of the orbit:
CT and M R appearance. American Journal of Neuroradiology_ 1 82:379-8 1 , 1 997
Feb.
IORBIT A

Case 48
Clinical Presentation
47-year-old female pre ented with progre ive loss of vi ion in her left eye. Phy -
ical examination revealed propto is and impaired mobility of the left globe. Fundo
scopic examination wa unremarkable.

A B

12 1 4
O R B IT AND V ISUAL PATHWAYS/

Radiologic Findings
Axial T l W (Fig. 48-A), axial frequency selective fat suppression (FATSAT) T1 W
(Fig. 48-B) images after gadolinium enhancement, and coronal FATSAT T I W im
age after gadolinium enhancement (Fig. 48-C) show a round, weLl-circumscribed
intraconal mass in the left orbit. This mass is intimately related to the optic nerve,
which is displaced inferiorly and abuts the posterior aspect of the globe anteriorly.
The lesion is isointense to the gray matter and shows vivid enhancement after con
trast administration. There is no evidence of posterior extension to orbital apex and
the extraconal muscles are also uninvolved by this mass. A mild to moderate degree
of proptosis is noted (Courtesy Dr. Howard Krauss).

D ifferential D iagnosis: E nlargement of the


Optic Nerve or Sheath
Tumors: optic nerve glioma, optic nerve meningioma, optic nerve neuroma, lym
phoma, leukemia (chloroma), metastasis (hematogeneous: breast and lung, direct in
vasion: retinoblastoma, uveal melanoma), hemangioblastoma, hemangiopericytoma
I nflammatory lesions: orbital pseudotumor, optic neuritis, sarcoidosis
Others: pseudotumor cerebri

D iagnosis
[ntraorbital meningioma

Discussion
In the assessment of orbital lesions accurate localization is critical. In the intraconal
compartment, lesions can arise from the globe, extraocular muscles, intraconal fat,
optic nerve or sheath. This distinction significantly reduces the list of differential
diagnoses.
The optic nerve is enveloped by the meninges from the optic chiasm to the pos
terior aspect of the globe where they merge with the sclera. This meningeal enve
lope provides an anterior extension of the subarachnoid and subdural spaces. I n the
superior intracanalicular portion of the optic nerve, the pia mater, arachnoid mem
brane, and dura fuse with each other and with the optic nerve, providing an attach
ment to the optic canal.

Epidemiology
Optic nerve meningiomas comprise 5 to 7% of aLl primary orbital tumors. Females
are more frequently affected than males (4 : 1 ) and, although there is a broad range,
a peak incidence is seen in the fourth and fifth decades. I ntraorbital meningiomas
can also occur in children, usually in the first decade of life. TIlese meningiomas
show more aggressive behavior than the adult form. Bilateral meningiomas are a
diagnostic criteria for neurofibromatosis type 1 .

Pathophysiology
I ntraorbital meningiomas may originate within the orbit or on rare occasion, result
from direct extension of an intracranial tumor usually arising from the sphenoid
ridge. Intraorbital meningiomas can arise from the meningeal sheath of the optic
nerve, or from remnants of meningoepithelial cells left behind during embryologic
development. The most common location is the optic nerve sheath and the orbital
apex. However, meningiomas of the extraconal compartment can be seen, usually
arising from the periosteum of the orbital walls. Intracranial meningiomas gain ac-
1 0RBIT AND VISUAL PATHWAYS

cess to the orbit either by extension along neural and vascular foramina, or through
invasion of the bony walls of the orbit.
The most frequent pathologic varieties of intraorbital meningiomas are menin
goepithelial and transitional cell types. Pathology may be useful in distinguishing
the rare angioblastic meningioma from hemangiopericytoma.
As the clinical and imaging findings are often nonspecific, FNA should be per
formed prior to therapy planning. Because most of the lesions are not readily
accessible, imaging guidance is usually necessary.

Clinical Findings
The clinical presentation of intraorbital meningiomas is non-specific, similar to any
other intraorbital mass. The most frequent presenting symptoms include decreased
vision, proptosis and constriction of the visual field. However. the clinical presenta
tion largely depends on the size and location of the tumor. Small meningiomas aris
ing along the optic nerve sbeath may be asymptomatic for long periods before they
come to clinical attention. Clinical examination may reveal papilledema, optic atro
phy, scotomata, and decreased motility of the extraocular muscles. Canalicular and
chiasmal meningiomas are usually not associated with significant proptosis and
often present as central scotomata.
Clinically, there are a few findings that may help to distinguish a mass arising i n
the optic nerve itself from the optic sheath and other i ntraorbital locations. A sig
nificant visual impairment associated with mild proptosis is more likely to be related
to an optic nerve lesion. Conversely, extrinsic lesions tend to produce significant
proptosis before compromising visual acuity. It is also typical for primary lesions of
the optic nerve to produce transient visual obscurity in the extremes of gaze, or with
change in head position due to vascular compression.

Imaging Findings
CT and M R I have complementary roles in the evaluation of intraorbital masses.
Although M R I has superior soft tissue contrast resolution, in the case of an intra
orbital meningioma, CT is superior in detecting intratumoral calcification and asso
ciated bony changes which may be important clues in the differential diagnosis.
Beam hardening artifact due to adjacent bone is one limitation of CT, particularly
in the evaluation of small perioptic meningiomas.
On CT, a meningioma of tbe optic nerve may appear as a tubular thickening, an
eccentric mass, or a fusiform enlargement of the optic nerve-sheath complex. Stip
pled or ring shaped calcifications, although rare, may differentiate these tumors
from optic nerve gliomas. Typical associated bony findings include sclerosis and scal
loping of adjacent bony structures, seen primarily in intracanalicular meningiomas
and in those arising from the sphenoid ridge and secondarily invading the orbit.
A spongy appearance of the bone around the optic canal, sphenoid wing and chias
matic sulcus may be the only finding when the tumor is too small to be detected
radiographically.
Diagnostic dilemmas such as confusion with fibrous dysplasia and Paget's disease
occasionally occur. Meningiomas near tbe orbital apex, however, tend to cause
demineralization and enlargement of the optic canal. Another finding is the associ
ation with pneumosinus dilatans, usually of the sphenoid sinus. The cause of this as
sociation is controversial. Some experts believe meningiomas produce irritation of
adjacent bone and cause progressive thinning of the bony walls of the sinus. which
then expand. Others suggest that pneumosinus dilatans is the primary process which
stimulates the growth of arachnoid cap cells to produce a meningioma.
Meningiomas show moderate to marked homogeneous enhancement. The classi
cal "tram track" sign consists of linear enhancement along the nerve sheath sepa
rated by a central area of hypodensity which corresponds to the optic nerve.
ORBIT AND V I SUAL PATHWAYSl

D E

However, this sign is not specific for meningioma and can be seen in a variety of in
flammatory conditions including optic neuritis and pseudotumor.
M R I is more accurate in the detection of optic nerve/sheath complex enlarge
ment, especially when enhanced coronal T l W images with fat saturation are per
formed. This sequence provides better contrast resolution between the enhancing
lesion and the intraconal fat (also hyperintense on TI W I ) and is crucial in the diag
nosis of small perioptic tumors. Orbital meningiomas are usually slightly hy
pointense to the brain parenchyma on botb T I W and TIW images and enhance
vividly and homogeneously, being clearly distinguishable from the optic nerve.
Coronal images are particularly well-suited for evaluation of the optic canal, the
optic chiasm and the precise relationship between tbe lesion and the optic nerve
(Fig. 48-C).
Meningiomas are well circumscribed unless tbey breach the dural Lining and
infiltrate the surrounding fat. This is seen more frequently in pediatric patients and
represents more aggressive behavior. Associated bony sclerosis may be depicted on
M R I as an enlarged area of absent signal corresponding to the enlarged cortical
bone.
Dilatation of the subarachnoid space, usually due to increased intracranial pres
sure, may appear as a halo surrounding the optic nerve, following the signal charac
teristics of CSF. This should not be confused with a tumor as the post-contrast
images will show no evidence of enhancement.
Diffuse enhancement along the optic nerve is more likely to be related to an
inflammatory process, altbough sometimes it is impossible to distinguish from a
tumor.
Ultrasound is a good screening test for detection of intraorbital lesions because it
is readily available and easy to perform. Although the findings are nonspecific, US
may be used in FNA guidance and Doppler US may be belpful in the diagnosis of
vascular lesions.
Axial FATSAT T l W images of the orbit after contrast enhancement (Figs. 48-D
and 48-E) show a large hourglass-shaped mass with a larger component centered in
the left cavernous sinus and a smaller component in the intraorbital component. The
mass extends into the orbit through the optic canal which is markedly enlarged. The
cavernous segment of the ICA is medially displaced and compressed by this
I ORBIT AND VISUAL PATHWAYS

lesion. The lesion extends laterally into the medial cranial fossa compressing and
PEARLS/PITFALLS displacing the medial aspect of the temporal lobe.
Optic nerve meningiomas are
seen most often in middle aged Management
women.
The management of lesions involving the anterior optic pathways is controversial.
The most frequent presenting TIle treatment of intraorbital meningiomas depends upon their location, extent and
symptoms include proptosis and associated symptoms. As opposed to nerve sheath meningiomas, extradural or "ec
slowly progressive loss of vision. topic" meningiomas are usually easy to resect without endangering the optic path
way. Intracanalicular and nerve sheath meningiomas, with some rare exceptions, are
An optic nerve meningioma ap not amenable to complete resection without considerable risk of optic nerve dam
pears as a tubular, fusiform or age due to the close proximity of the ophthalmic and central retinal arteries. There
eccentric enhancing lesion along fore, the decision to perform surgery depends upon the severity of symptoms and
the optic sheath without involve disease progression. Surgery is indicated when there is loss of visual acuity, in
ment of the nerve itself. creased proptosis, pain or radiographic evidence of further growth. Intracranial le
sions with intraorbital extension require a multidisciplinary approach with surgery
Perform enhanced M R l of the
performed by a team of neurosurgeons and skull base surgeons. Lesions involving
orbits with fat saturated Tl W
the chiasm carry a significant risk of postsurgical blindness. Radiotherapy may be
images to get a more accurate
considered in cases of chiasmatic involvement and for recurrence.
picture of the optic nerve/sheath
complex.

Take care not to miss involve Suggested Readings


ment of the chiasm. Biesman, B.S.. Heilman, C. Surgical management of lesions affecting the anterior
optic pathways. Seminars in Ophrhalmology 1 0(3):260-4, 1 995 Sep.
Avoid misdiagnosing for an optic
Delfini, R., Missori, P., Tarantino, R., Ciapetta, P., Cantore. G. Primary benign
nerve glioma.
tumors of the orbital cavity: comparative data in a series of patients with optic nerve
Take care not to confuse dilation glioma, sheath meningioma, or neurinoma. SlIrgical Neurology 45(2): 1 47-53. dis
of the subarachnoid space cussion 1 53-4, 1 996 Feb.
around the optic nerve for a Gunalp, I.. Gunduz. K., Duruk, K .. Kanpolat. Y. Neurogenic tumors of the orbit.
tumor. Japanese Journal of Ophrhalmology 38(2): 1 85-90, 1 994.
Hashimoto, M., Tomura. N., Watarai. 1. Retrobulbar orbital metastasis mimicking
Differentiation from inflamma
meningioma. Radiarion Medicine 1 3(2):77-9. 1 995 Mar-Apr.
tory conditions is not always
possible. l ng, E . B .. Garrity, 1.A .. Cross, S.A., Ebersold, MJ. Sarcoid masquerading as optic
nerve sheath meningioma. Mayo Clinic Proceedings 72( I ):38-43. 1 997 Jan.
Johnson, T.E . , Weatherhead, R.G., NasI', A.M .. Siqueira, E.B. Ectopic (extradural)
meningioma of the orbit: a report of two cases in children. Journal of Pediarric Oph
r/1almology and Srrabisl11us 30( 1 ):43-7, 1 993 Jan-Feb.
Miller, N.R .. Golnik, K.C., Zeidman, S.M., North, R.B. Pneumosinus dilatans: a sign
of intracranial meningioma. Surgical Neurology 46(5):471 -4, 1 996 Nov.
Reid, D.. Ngo, H . H . , Lamarche, 1.B. Primary post-traumatic intraorbital menin
gioma. JOllrnal of Orolaryngology 23(4):298-30 I . 1 994 Aug.
Rose, G.E. Orbital meningiomas: surgery, radiotherapy, or hormones? 8riri.l'/1 Jour
nal of Ophrhalmology 77(5):3 13-4, 1 993 May.
Ruscalleda. 1., Feliciani, M., Avila, A., Castaner. E., Guardia, E .. de Juan. M. Neuro
radiological features of intracranial and intraorbital meningeal hemangiopericy
tomas. Neurodiology 36(6):440-5, 1 994 Aug.

I
ORBIT A N D V I S UAL PATHWAYSI

Case 49
Clinical Presentation
A 65-year-old male consulted his ophthalmologist because of a 2-week history of
decreased vision in his right eye. Opthalmic examination revealed a right choroidal
mass.

A B

Radiologic Findings
There is a choroidal mass in the right that is seen on the axial CT image (Fig. 49-A).
The lesion is hyperintense (arrowhead) on TI W images both before ( Fig. 49-B) and
after ( Fig. 49-C) contrast. The contralateral globe is unremarkable.
I ORBIT A D V I SUAL PATHWAYS

Differential Diagnosis: Choroidal Mass


Melanoma
Hemangioma
Hemorrhage
RetinaUchoroidal detachment
Choroidal metastasis
Sarcoidosis
Retinal cyst
Retinoblastoma
Retinal gliosis
Nevi
Leiomyoma
Schwanoma
Neurofibroma

Diagnosis
Choroidal (uveal) melanoma

Discussion
Uveal melanomas are the most common primary ocular neoplasm in adults with an
annual reported incidence of seven case per million. There is no gender predilec
tion. It is more common in individuals witb light skin coloring and is almost always
unilateral.

1 220
ORBIT A D V I SUAL PATHWAYSJ

Pathophysiology
The tumor arises from malignant transformation of melanocyte in the uveal tract,
which is made up of the ciliary body. iris, and choroid. Melanomas in the ciliary body
and choroid are believed to originate from preexisting nevi. Choroidal nevi are con
genital lesions, usually recognized late in the first decade of life and most commonly
located in the posterior third of the choroid. TIley are usually not visible with high
resolution CT or M R I techniques.

Cli nical Fi ndings


Diagnosis is usually accomplished using the standard opthalmologic examinations
of opthalmo copy, sonography and/or f10 urescein angiography. CT and more often
M R I are valuable, however, to evaluate extracapsular extension, which may occur
in 1 0 to 1 5% of patients and may be difficult or impossible to evaluate with the prior
techniques. Direct opthalmologic visualization may be impo sible in patients with
"opaque media" (i.e. cataract, vitreous hemorrhage).

Imaging Fi ndings
The CT and M R I appearance of choroidal melanoma is that of an enhancing
choroidal mass ( Figs. 49-A-49-C). I nitially there is a smooth contour as the mass
elevates Bruch's membrane (lamina vitrea; Fig. 49-D left). With more growth and
rupture of the membrane, the tumor assumes a more characteristic mushroom
shape (Fig. 49-D. right). TIle adjacent retina also becomes elevated (detached) on
either side of the tumor. These fluid collections can usually be differentiated from
the primary mass on T2W M R I . The imaging appearance of the melanoma may be
mimicked by retinoblastoma, choroidal metastases. hemangioma. or intrachoroidal
hemorrhage.
Electron-spin resonance studies have shown that melanin produces a stable, free
radical signal. These radicals cause a proton relaxation enhancement that shortens
both T l and 1'2 relaxation time values. Damadian, et al ( 1 974), reported that, unlike
other tumors, melanomas have a short T l value, which they attributed to paramag
netic proton relaxation enhancement by a stable radical in melanin. Because of the
relatively shortened TI and 1'2 relaxation time values, owing to paramagnetic prop
erties of melanin. uveal melanomas may appear relatively hyperintense (to vitre
ous) on T l W, proton-weighted MR images, and become hypointense on T2W MR
scans.
On M R I . associated retinal detachment can be easily differentiated from the
uveal melanoma. Choroidal hemorrhage may be mistaken for malignant uveal
melanoma on clinical examination as well as on various imaging techniques. On
M R I , the acute choroidal hemorrhage appears a a moderately hypointense image
on T2W MR scans. Subacute choroidal hemorrhage may be seen as an area of het
erogeneous signal intensity on both T l - and T2-weighted MR images. Chronic
choroidal hemorrhage appears as hyperintense areas in both TI W and T2W M R
scans. Uveal metastases may b e very difficult t o differentiate from uveal melanoma.
Most metastases, however, often appear hyperintense on T2W MR images. Metas
ta es from primary mucin-producing carcinoma may be very difficult to differenti
ate from uveal melanomas.
Although T l and 1'2 shortening has been seen in some MRI studies of patients
with melanoma, the finding is neither particularly sensitive nor specific in the diag
nosis of melanoma. It is insensitive because not all melanomas contain large
amounts of melanin (amelanotic melanomas). The finding is nonspecific because
other tissues such as blood breakdown products can also cause relaxation enhance
ment.
I ORBIT AND VISUAL PATHWAYS

Management
PEARLSIPITFALLS
Tl shortening for melanoma is Treatment is based on the size. location and extent of the tumor. Brachytherapy with
neither particularly sensitive or or without local excision. enucleation. orbital exenteration. or percutaneous proton
specific. irradiation are all treatment options.

Amelanotic melanomas may


have relatively low levels of the Suggested Readings
paramagnetic stable free radical. Damadian. R .. Zaner. K . . Hor. D.. DiMaio. T. H uman tumors detected by nuclear
Subacute hemorrhage with magnetic resonance. Proceedings or the ational Academy of Sciencc SA 7 1 :
methemoglobin is paramagnetic. 1 4 7 1 - 1 473, 1 974 .
Gomori. JM. et al. Choroidal melanomas: correlation of NMR spectroscopy and
Proteinaceous fluid may cause T l MR imaging. Radiology 1 58:443-445, 1 986.
shortening. Mafee. M.E Malignant uveal melanoma and simulating lesions. MR imaging evalu
Care should be taken not to con ation. Radiology
1 60:773-780. 1 986.
fuse a choroidal melanoma with Potter, P. O., Shields. C.L .. Shields, JA., Flanders. A.E. Thc role of magnctic reso
choroidal metastasis or other nance imaging in children with intraocular tumors and simulating lesions. Oplulwl-
mimics. 111010gy 1 03 1 1 : 1 774-83. 1 996 Nov.
Romani. A .. Baldcschi. L., Genovesi - E be rt F. . G re mi g ni . E .. Ragone. M.e.. Rizzo. S ..
.

ardi, M. Sensitivity and specificity of ultrasonography. fluorescein vidcoangiogra


phy indocyanine green videoangiography. magnetic resonance and radioim
.

munoscintigraphy in the diagnosis of primary choroidal malignant melanoma. Oph


thalmologicnl 2 1 2 Suppl 1 :44-6, 1 998 .
Scott, l .u.. Murray, T.G . Hughes. JR. Evaluation of imaging techniques for detec
.

tion of extraocular extension of choroidal melanoma . A rchives of Ophthalmology


1 1 6 7:897-9, 1998 luI.

222
Case 50
Clinical Presentation
n J -month-old child pre ented with leukocoria and trabi mus.

Radiologic Findings
An axial nonenhanced cr of the orbit (Fig. 50-A) hows a gros ly calcified retro
lental oft ti ue mass in the po teromedial a pect of the left globe. There is no
evidence of extraocular exten ion along the optic nerve or into the retrobulbar fal.

Differential Diagnosis: Intraocular Mass


in a Child
eoplastic conditions: retinoblastoma and retinocytoma -, uveal melanoma,
metastases (neurobla IOma, mo t common), p eudoglioma (retinal a trocytoma
or a lrocytic hamartoma)
onneopla tic conditions: per i tent hyperpla tic primary vitreous (PHP ) .

retinopathy of prematurity or retinal dysplasia ( Ropf, Coat's disease. toxocari


a is or larval granulomato is, chronic retinal detachment, uveiti
ote: The pre encc of calcification in an intraocular mas further limit t he diagnostic
po ibilities. The lesions most likely to calcify are marked with .

223
I ORBIT A A L PATHWAY

Diagnosis
Retinoblastoma

Discussion
eparating retinobla toma ( R B ) from benign nonneopla tic condition . uch a
tho e Ii ted above, i one of the major challenge in pediatric ophthalmology. In
orne ca e . thi differentiation is impo ible both clinically and radiologically, lead
ing to delay in t he treatment of this highly malignant tumor and. ometimes, to
ocular enucleation for benign pathologie
Differentiation of RB from it more benign variants ( retinocytoma) and from
uveal melanoma is not mandatory a patient management i the arne. Ocular
meta ta e u ually appear long after detection of the primary tumor making the
diagno i obviou .
Retinal a trocytoma or astrocytic hamartoma is commonly a ociated with tuber
ou c1ero i and may be the pre enting ign of the di ea e.
hronic retinal detachment can appear a a dense retrolental rna s on , which
may show dystrophic calcification, mimicking R B. However, this p eudoma s d e
not cros the optic nerve head, and i limited anteriorly by the ora serrata. sually,
the clinical hi tory and the presence of a predi po ing factor for retinal detachment
allow for the correct diagno is.
Mo t benign congenital ocular di ease are a socia ted \ ith malformed globe . mi
crophthalmia and hallo\ anterior chamber nlike RB. they rarely calcify and do
so only in the late stage of the disease (dystrophic calcifications in phthisis bulbi ).

B c

224
ORBIT AND VISUAL PATH WAYSI

Retinopathy of prematurity or retinal dysplasia is associated with prematurity.


low birth weight and exposure to supplemental oxygen therapy. which may be
elicited from the clinical history. It is typicaUy a bilateral and asymmetric condition
which rarely calcifies. With progression of the disease. the globes become micro
phthalmic with shallow anterior chambers.
Persistent hyperplastic primary vitreous is the second most common cause of
leukokoria in the pediatric population and may pose diagnostic problems with RB.
Distinguishing features include the presence of microphthalmia at birth and a more
anterior location within the globe, sometimes manifesting as a triangular shaped
mass extending [rom the posterior aspect of the lens toward the optic di c (along
the Cloquet's canal). It does not usually calcify.
Coat's disease is more common in males and tends to present later in life.
between 4 and 1 0 years of age. Like retinoblastoma the globe is normal in mor
phology and size. On US, a definite solid mass is usually not identified. and on M R I
the more solid component of the lesion i s located anterior t o t h e detached enhanc
ing leaves of the retina, while in RB the tumor arises from the retina and projects
into the subretinal effusion. The subretinal effusion in Coat's disease are hyperin
tense on both T l W ( Fig. 50-B) and T2WI due to its lipoproteinaceous content (Fig.
50-C).
Ocular toxocariasis or sclerosing endophthalmitis may be radiologically indistin
guishable from RB, presenting as solitary or multiple calcified ocular masses (Fig.
50-D). Involvement of other organs and systems and a positive enzyme-linked
immunoadsorbent assay (ELISA) for Toxocara canis usually allows an accurate
diagnosis.

Ep idemiology
Retinoblastoma is a rare congenital intraocular malignancy with a reported world
wide incidence varying between I : 15,000 and 1 : 34.000 live births, and comprising
approximately I % of all pediatric malignancies. It is the most common intraocular
malignancy in children and the most frequent cause of leukocoria, responsible for
60% of cases. Although congenital in origin, the median age of presentation
depends largely on the efficiency of pediatric health care systems. In the United
States, the median age of presentation is 18 months and 98% of all cases are diag
nosed before age 5 years. There is no gender predominance. Among all forms of RB.
65 to 75% are unilateral and 25 to 33% are bilateral. In the presence of a positive
familial history of RB the incidence of bilaterality increases to 65% .

Pathophysiology
Retinoblastoma is a primary malignant congenital neoplasm that arises from prim
itive embryonal retinal cells that are located in the nuclear layer of the retina. These
embryonal cells are primitive neuroectodermal derivatives and. therefore, RB is
classified within the primitive neuroectodermal tumor (P ET) group.
According to their etiology, four different groups of RB are recognized. Non
hereditary forms comprise 60% of aU cases and result either from a sporadic postzy
gotic mutation in the retinoblasts or from anomalies involving chromo ome 1 3
(monosomy, deletions. etc). I n these cases, subsequent generations are not affected
and RB is more commonly unilateral. Hereditary forms comprise 40% of all cases
and may result from a sporadic germinal mutation or an autosomal dominant anom
aly involving band 14 of chromosome 13 ( R B I gene). Penetrance of the RB I gene
in familial RB is high, approaching 95% .
1l1e most common genetic anomaly associated with RB is the deletion of band 1 4
of the long arm of chromosome 1 3 , responsible for codification of the enzyme es
terase 0, which has been implicated in the tumorigenesis of several malignancies.
The RB I gene is thought to act as a tumor supressor gene or antioncogene. Ac
cording to Knudson's theory, an individual with a familial predisposition inherits
IORBIT AND VISUAL PATHWAYS

one inactive gene so that only one other mutational event during the susceptible
stage of embryonic development is necessary for retinoblasts to become apparent.
When larger deletions of chromosome 13 occur, systemic dysmorphic features also
may be apparent. These include microcephaly, ear anomalies, mental retardation
and genital anomalies.
Trilateral RB is a rare variant of RB, consisting of bilateral intraocular RB and an
ectopic tumor in the pineal gland, suprasellar or parasellar regions. These tumors
are known to be different primaries with a common embryonic origin in primitive
neuroectodermal cells. It comprises 4% of all cases of RB and 8% of familial cases.
The RB I gene also has been demonstrated to predispose to second nonocular
malignancies, which are unrelated to the primary tumor and may occur in locations
outside standard radiation ports. Most commonly these tumors are bone or soft
tissue sarcomas, including osteo-, chondro- and fibrosarcomas or malignant fibrous
histiocytomas. The prevalence of a second tumor in non irradiated areas is estimated
to be 1 0% at 10 years of age and increases to 70% at 30 years with a cumulative life
risk of 32% in bilateral RB survivors.
Pathologically, RB is characterized by the presence of Winterstein rosettes ( neu
ronal cells linning a central zone of polysaccharides) and fleurettes (flowerlike
groups of cells which are markers of photoreceptor differentiation). Hower-Wright
rosettes. a group of neuronal cells lining a central area of cobweb filaments. are
found in other P ET and are not specific for retinal differentiation. RB is a small
round cell tumor. Prominent foci of calcification are typical of this tumor and result
from the formation of calcium complexes when DNA is released from necrotic cells.
Areas of glial differentiation may also be seen in this tumor.
Retinoblastomas metastasize in approximately 10% of patients. The most fre
quent site is meningeal seeding via the subarachnoid space surrounding the optic
nerve. Hematogeneous spread occurs primarily to the bone marrow and liver. Lym
phatic spread to the preauricular, submandibular and internal jugular chains may
occur.

Clinical Findings
The most frequent presenting sign of retinoblastoma is leukocoria (60% of cases).
Leukocoria, or white pupillary reflex, is a non pecific finding with an extensive dif
ferential diagnosis, which includes, besides the pathologic conditions listed above.
congenital cataracts and coloboma. I n these circum tances all efforts should be
made to reach an accurate diagnosis. The importance is twofold: avoiding unneces
sary enucleations for benign lesions and avoiding delayed diagnosis of RB, leading
to increased mortality and precluding ocular salvage therapies.
Other common presenting signs include strabismus and pain, secondary to closed
angle glaucoma (when the tumor obstructs the irido-corneal angle). Decreased
visual acujty, heterochromia iridis, hyphema and phthisis bulbi may develop with
progression of the disease. Patients with trilateral RB may first present with
headache and other symptoms of increased intracranial pressure, due to noncom
municating hydrocephalus, in the absence of ocular symptoms.
Ophthalmologic examination is the mainstay of the diagnosis, able to detecl
tumor beyond the resolution of imaging, as small as 0.02mm. [t may also detect
metastatic seeding of the vitreous. Limitations of ophthalmoscopy include the pres
ence of hyphema, cataracts or vitreous hemorrhage, precluding visualization of the
retina and evaluation of tumor extent beyond the globe. Ophthalmoscopic exami
nation of the contralateral eye is mandatory. Screening of the progeny of patients
with bilateral RB and genetic counseling should be provided.
RB can be divided into four clinical stages, modified by Prat, et al: limited to the
retina (stage I ) , extraretinal intraocular disease (stage I I), extraocular disease lim
ited to the adnexal structures (stage I l l ) and extraocular disease with distant metas
tasis (stage IV). A metastatic workup should be performed in clinically advanced
ORBIT A N D VISUAL PATHWAYSI

lesions including a complete physical exam, liver function test, lumbar puncture and
bone marrow aspirate.

I maging Findings
In addition to helping in the differential diagnosis, imaging is mandatory in ad
vanced lesions to determine local extent and evaluate for the presence of intracra
nial disease or distant metastasis. Imaging modalities include US, CT and MRI for
local disease, and liver and bone scanning to rule out metastatic involvement in
advanced cases.
The imaging appearance of RB depends on the size of the lesion and pattern of
growth. Endophytic lesions are the most common, manifesting as a soft tissue mass
protruding into the vitreous chamber, and sometimes associated with floating
islands of tumor within the semiliquid vitreous and anterior chamber. The exophytic
form appears as a soft tissue mass protruding into the subretinal space, causing reti
nal detachment and sometimes invasion of the choroid. Finally, the diffuse, or en
plaque, pattern is the hardest to recognize and manifests as diffuse retinal thicken
ing, generally without calcification. The most common location of the tumor is the
posterolateral aspect of the globe. The imaging hallmark of RB is the presence of
calcification.
Ocular US is a quick, inexpensive and readily accessible modality, well-suited for
pediatric patients, because it does not involve ionizing radiation and does not re
quire sedation. It easily separates solid from cystic masses and detects calcification.
On US, RBs are usually depicted as echogenic masses protruding into the ane
chogenic vitreous chamber and showing hyperechogenic foci with acoustic shadow
ing, corresponding to calcification. Retinal detachment with subretinal fluid, and
vitreous and anterior chamber seeding may also be depicted using this modality.
Limitations include: ( 1 ) detection of en plaque and exophytic lesions, which cannot
be separated from the echogenic ocular layers and retrobulbar fat, and (2) deter
mining extraocular extent.
CT is the most specific imaging modality due to its unique ability to detect calci
fication as small as 2mm. On CT, RB manifests as a soft tissue mass, showing gross
nodular or punctate calcification in 90% of cases. Endophytic lesions are usually
lobular in shape and well marginated. Exophytic lesions, which grow subretinally,
may mimic a chronic organized retinal detachment and be confused with other be
nign diseases such as PHPV, ROP, Coat's disease, and larval endophthalmitis. En
plaque lesions rarely calcify.
A dense vitreous may result from seeding with neoplastic cells or hemorrhage.
Extension along the optic nerve manifests as optic nerve enlargement. III-defined
margins of the posterior aspect of the globe indicates extraocular invasion into the
retrobulbar fat. Extraocular muscle enlargement and soft tissue masses within the
orbit may also result from tumor growth. Extension along the optic pathway and
subarachnoid seeding is best appreciated on M R I . A pineal or suprasellar enhanc
ing mass, with or without associated hydrocephalus, makes the diagnosis of trilateral
RE. Macrophthalmia is occasionally seen and results from anterior extension of the
tumor, leading to obstruction of the irido-corneal angle and increased intraocular
pressure.
Due to its multi planar capability and high contrast resolution, M R l is the imag
ing modality of choice to evaluate tumor extent and separate the solid neoplastic
component from any associated subretinal fluid. Although M R I is less specific than
CT, because of its lack of sensitivity for calcification, the signal characteristics of RB
are specific enough to differentiate it from other pathologic conditions. RE is iso- to
hyperintense of vitreous on Tl WI and hypointense on T2WI. It shows moderate to
intense enhancement and may show areas of necrosis and calcification. The soft
tissue mass is easily separated from any subretinal fluid, which is usually hyperin
tense on both Tl WI and T2WI due to high protein content or hemorrhage. Only the
I ORBIT AND VISUAL PATHWAYS

solid neoplastic component enhances and violates anatomic landmarks such as the
ora serrata and optic nerve head.
M R l is the modality of choice for depiction of extraocular extent. I nvasion of the
uveal tract may be depicted, and manifests as diffuse thickening of the ocular lay
ers. Fat-suppressed, enhanced TI WI are especially useful in determining extention
along the optic nerve. Enhanced TI WI are also necessary to depict metastatic seed
ing of the vitreous chamber and subarachnoid space. M R I is also useful in the eval
uation of patients with the noncalcified en plaque form of retinoblastoma.
Imaging should be used in the follow-up of patients undergoing conservative
therapies (ocular salvage ) and in patients with bilateral RB to allow for early
detection of a third pineal or suprasellar tumor. which may occur in 8% of patients.
AU pineal calcifications under 6 years of age should be viewed with suspicion and
prompt a search for an associated ocular tumor. A metastatic imaging workup
should be performed in patients with advanced disease (stages I I I and I V). includ
ing enhanced M R I of the neuroaxis. and bone and liver scans.
10 the near future, immunoscintigraphy using monoclonal antibodies against
membrane antigens. such as neural ceU adhesion molecule ( CAD) marked with
gama emitters, may be used to target neoplastic cells and eventually to direct cyto
toxic agents.
Sometimes a differential diagnosis cannot be made based on clinical and imaging
findings, and ocular FNA under imaging guidance may be required. eoplastic seed
ing along the needle tract is a concern. The reliability of aqueous humor cytology
following anterior chamber paracentesis is controversial, and therefore ocular enu
cleation is still performed in cases were the diagnosis is in doubt.

Management
Therapy and prognosis greatly depend on tumor extent and staging. When the dis
ease extends beyond the globe (stages I I I and I V ) the mortality rate approaches
1 00% , while early disease. limited to the globe has a 5 year survival rate of 92% . Few
cases of spontaneous regression of RB have been described in the literature.
When the tumor is small, ocular salvage can be attained in 75% of cases. Conser
vative techniques include external beam and episclera plaque radiation, cryother
apy and photocoagulation.
Enucleation is the therapy of choice for more advanced disease. Care should be
taken to avoid spillage of the tumor into the orbit. and to remove all neoplastic
tissue. This is because one of the worst prognostic factors in RB is the presence of
tumor in the surgical margin (associated with a greater than 60% mortality rate) .
The extent of optic nerve involvement may change the surgical approach. When ex
tensive, it mandates a lateral canthotomy to facilitate maximal optic nerve removal.
Disseminated disease has a dismal prognosis and responds poorly to systemic
chemotherapy. Clinical and imaging follow-up of patients treated conservatively.
and of patients with a family history, is recommended to detect recurrent disease or
a third neoplasm, respectively.

Suggested Readings
Ainbinder, OJ . Haik. B.G., Frei, D.F., Gupta, K.L.. Mafee. M.E Gadolinium en
.

hancement: improved M R I detection of retinoblastoma extension into the optic


nerve. Nellroradiology 38(8):778-8 1 , 1996 Nov.
Amoaku, W.M., Willshaw, H.E., Parkes. S.E., Shah. KJ . Mann, J. R. Trilateral
.

retinoblastoma. A report of five patients. Callcer 78(4):858-63. 1 996 Aug 1 5.


Beets-Tan. R.G. . Hendriks, M.J., Ramos, L . M .. Tan. K.E. Retinoblastoma: CT and
M R I . Neuroradiology 36( 1 ):59-62. 1 994.
De Potter, 1'.. Flanders, A.E., Shields, J.A., Shields, c.L., Gonzales. C.F.. Rao. Y.M.
The role of fat-suppression technique and gadopentetate dimeglumine in magnetic
ORBIT AND VISUAL PATHWAYSI

resonance imaging evaluation of intraocular tumors and simulating lesions [see


PEARLS/PITFALLS comments]. Archives of Opluhalmology 1 1 2(3):340-8, 1 994 Mar.
RB appears as a calcified retro Karcioglu, Z.A., al-Mesfer, S.A., Abboud, E., Jabak, M.H., Mullaney, P.B. Workup
lental mass, most commonly seen for metastatic retinoblastoma. A review of 261 patients. Ophthalmology
in children. 1 04(2):307- 1 2, 1997 Feb.
Potter, P.O., Shields, CL., Shields, lA., Flanders, A.E. The role of magnetic reso
In RB, the retinal mass appears
nance imaging in children with intraocular tumors and simulating lesions. Ophthal

hyperintense on T1 WI and hy mology 1 03( 1 1 ): 1 774-83, 1 996 Nov.


pointense on TIWI , and is easily
differentiated from subretinal Ramji, EG., Siovis, T.L., Baker, J.D. Orbital sonography in children. Pediatric Radi
fluid. ology 26(4):245-58, 1 996.
Skulski, M., Egelhoff, J.C, Kollias, S.S., Mazewski, C, Ball, WS. Jr. Trilateral
Frequency selective fat suppres retinoblastoma with suprasellar involvement. Neu.roradiology 39( 1 ):41 -3, 1 997 Jan.
sion imaging (FATSAT) en Smirniotopoulos, IG., Bargallo, N., Mafee, M.F. Differential diagnosis of leukokoria:
hanced T1 WI should be per radiologic-pathologic correlation. Radiographies 14(5): 1 059-79, quiz 1081-2, 1 994
formed to determine extraocular Sep.
extent.
Tarlton, J.E, Easty, D.L. Applications of monoclonal antibodies in the investigation,
Follow-up of familial and bilat diagnosis, and treatment of retinoblastoma. British Journal of Ophthalmology
eral cases should be done to look 77( 1 2):805- 1 2 , 1 993 Dec.
for a possible pineal suprasel
OT

lar neoplasm.
Care should be taken to avoid
failing to recognize a bilateral
tumor.
Avoid making a otisdiagnosis for
a benign condition.
Care should be taken to avoid
not recognizing extraocular ex
tent. which mandates more radi
cal surgery.
Take care not to fail to consider
noncalcified en plaque form in
older children with leukokoria.
VI.

Oral Cavity and Oropharynx


o O ROPH A RYNX1

Case 51
Clinical Presentation
An adult female pre ented with a 5-year history of a swelling of the floor of her
mouth combined with pain during swallowing (Fig. 51-A).

233 1
IORAL AV ITY A N D OROPHARYNX

B C

Radiologic Findings
A n axial T I -wcighted image ( Fig. 5 1 -B ) hows a rounded well defined central
2 x 3 em mass in the po terior lOngue. The mass shows a high signal inten ity com
pared w i t h l he normal muscle of the longu . -nlC 'agi llal T I W view ( Fig. 5 1 - )
sh w similar findi ngs. The radiolabclled i dine [ 1 - 1 3 I J l udy ( Fig. 5 1 - D ) show

1 23 4
ORAL CAVITY AND OROPHARYNXI

increased activity within the mass and no normal thyroid activity. (Images courtesy
Dr. Thomas Vogl.)

Differential Diagnosis: Midline Tongue Mass


Lingual thyroid
Dermoid
Thyroglossal duct cyst
Atypical carcinoma
Lingual tonsil
Hemangioma
Schwan noma
Atypical ranula

Diagnosis
Lingual thyroid

Discussion
Pathophysiology
The primordial thyroid forms an epithelial Lined tube known as the thyroglossal
duct (see thyroglossal duct cyst). TIle embryologic thyroid tissue arises within the
foramen cecum (behind the circum vallate papillae of the tongue) and descends
along the hyoid bone to the thyroid isthmus. The thyroid gland normally traverses
the length of the thyroglossal duct to reach its final position anterolateral to the
trachea.
This migration may be arrested at any point. When the thyroid fails to migrate
below the tongue it is known as a lingual thyroid. Even with a normal appear
ing thyroid gland, residual thyroid tissue may appear anywhere along the thy
roglossal duct. The reported incidence of ectopic tissue along the tract varies from
0.5-35.0% .

E F
1 0RAL CAVITY AND OROPHARYNX

Clinical Findings
PEARLSIPITFALLS
A lingual thyroid can be located Patients may present with an asymptomatic posterior reddish tongue base mass. The
anywhere along t hyroglossal incidence of lingula thyroid is slightly higher in women than men.
duct.
I maging Findings
All thyroid pathologies may oc
cur in ectopic tissue, ie. carci On M R l , the lingual thyroid appears as a relatively homogeneous midline mass
noma, etc. below the foramen cecum. On CT scanning, the ectopic thyroid has the same x-ray
increased attenuation features (due to intrinsic iodine) found in normally located
Always perform 1 - 1 3 1 scan prior thyroid tissue. Images in a second patient show typical focal increased attenuation
to surgery to identify if other in the area of ectopic (lingual) thyroid tissue. Figure 5 1 -E shows an axial noncon
normal thyroid tissue is present. trast CT view. The coronal noncontrast CT view can be seen in Figure 5 1-F.
Radiolabelled iodine may be used to define the location of the active thyroid tissue
Always assume that a patient has (Fig. 5 1 -D).
normally located thyroid tissue
after lingual thyroid resection.
Manage ment
If surgery is contemplated, a radiolabeled iodine scan should be performed to con
firm the presence of other normally functioning tissue elsewhere in the neck (Fig.
5 1 -D). It should also be remembered that all of the pathological conditions that may
be found in normally located thyroid tissue, may also occur in ectopic thyroid tissue
(such as carcinoma, etc.).

Suggested Readings
Douglas, P.S., Baker, A.w. Lingual thyroid. Brirish Journal of Oral and Maxillofacial
Surgery 32(2) : 1 23-4, 1 994 Apr.
Giovagnorio, F., Cordier, A., Romeo, R Lingual thyroid: value of integrated imag
ing. European Radiology 6( 1 ) : 1 05-7, 1 996.
Hsu, c.Y., Wang, SJ. Thyroid hemiagenesis accompanying an ectopic sublingual thy
roid. Clinical Nuclear Medicine 1 9(6):546, 1 994 Jun.
Jayaram, G., Kakar, A., Prakash, R Papillary carcinoma arising in sublingual ectopic
thyroid concentrating both Tc-99m pertechnetate and 1-1 3 1 . Diagnosis by fine nee
dle aspiration cytology. Clinical Nuclear Medicine 20(4):381-3, 1995 Apr.
Vogl, T., B rUning, R, G revers, G, et. al. MR Imaging of the oropharynx and tongue:
comparison of plain and Gd-DTPA studies. Journal of Compurer Assisred Tomogra
phy 1 2:427-433, 1 988.
Vogl, T., Mees, K., Muhling, M., Lissner, 1. Magnetic resonance imaging in diagnos
ing diseases of the neck. HospimedicaI 1 7-23, 1 987 Jan/Feb.
ORAL CAVITY AND OROPHARYNXI

Case 52
Clinical Presentation
A 26-year-old female presented with a long term history of swelling in the floor of
her mouth.

A B

Radiologic Findings
A well-defined midline mass in the floor of the mouth, appears hyperintense rela
tive to muscle on Tl -weighted images (Fig. 52-A). The coronal image shows the
mass to be located superior to the geniohyoid muscle and between the genioglossus
muscles, elevating the tongue. The increased signal on T l -weighted images may be
due to a combination of lipid content and/or complex proteinaceous fluid. An en
hanced CT scan also shows the mass (Fig. 52-B). ( Images courtesy of Dr. Edward
Kassel.)

Differential Diagnosis: Cystic FO M Mass


Ranula
Dermoid cyst
Thyroglossal duct cyst
Abscess
I ORAL CAVITY AND OROPHARYNX

Cystic salivary gland neoplasm


Cystic hygroma
Necrotic neoplasm

Diagnosis
Dermoid cyst of the floor of the mouth (sublingual).

D iscussion
Dermoid cysts are epithelial-lined cavities with associated skin appendages, includ
ing hair, hair follicles, and sebaceous glands. The epithelial tissue usually arises from
embryonic rests and occasionally from traumatic or iatrogenic implantation. Of der
moid cysts of the head and neck, 25% occur in the oral cavity, usually in the ante
rior floor of the mouth.

c D

1238
ORAL CAVITY AND OROPHARYNXI

They are invariably midline and may be found either above the mylohyoid in the
PEARLSIPITFALLS sublingual space or below it in the submental space. Sublingual dermoids may raise
A dermoid cyst appears as a well the tongue and simulate a ranula. Submental space dermoids present as a neck mass.
circumscribed, anterior, midline
mass on M R . Pathophysiology
A dermoid cyst may contain fat The term "dermoid cyst" is used to describe three histologically distinct types of
globules, hair, fluid, keratin de cysts in the head and neck. Most are localized to the orbit, oral cavity, and sinonasal
bris and/or calcification. regions. The more common epidermoid cysts are lined with simple squamous cell
epithelium and contain no skin appendage. Rare teratoid cysts contain derivatives
Imaging findings for dermoid of skin appendages, endoderm and mesoderm. All three types of cysts may contain
cysts can be variable. a cheesy keratinaceous material.
The clinician must beware not to
confuse a dermoid cyst with a Clinical Findings
ranula.
Dermoid cysts of the oral cavity usually present in the midline anterior floor of the
The clinician must beware not to mouth in the second or third decades. Such cysts may be either acquired implanta
confuse a dermoid cyst with a tions or congenital enclaves of tissue and are subclassified into sublingual and sub
cystic neoplasm. mental varieties.
Sublingual dermoids are superior to the mylohyoid muscle, splitting the midline
extrinsic tongue muscles (geniohyoid-genioglossus complex) to occupy the floor of
the mouth. Less frequently the sublingual cyst may be laterally located displacing
the geniohyoid complex medially. M R I scans of other patients with sublingual der
moid cysts are seen in Figures 52-C and 52-D. Note the variable appearance. A coro
nal Tl W I shows decreased signal within the mass (Fig. 52-C). An axial TIWI shows
increased signal (Fig. 52-D).
Submental dermoids lie inferior to the mylohyoid muscle, between it and the
platysma, and tend to present as an anterior neck mass. Either type may elevate the
tongue, interfere with deglutition, or grow to a large size and present as a sub
mandibular mass. An axial CT scan of another patient with a multilocular submen
tal dermoid cyst can be seen in Figure 52-E.

I maging Findings
Dermoids tend to lie anterior to the foramen cecum region, which make a thy
roglossal duct cyst less likely. They appear well circumscribed. Dermoid cysts may
contain various amounts of hair, calcification, fluid, and/or fat.
Dermoid tumors are typically hyperintense on T 1-weighted images and less in
tense on TI-weighted images due to the short Tl and intermediate TI caused by the
high lipid content within the cyst. Other tumors have demonstrated variable degrees
of Tl and T2 lengthening, related to a variety of contained elements, or areas of sig
nal void, if regions of dense focal calcification are present. Tumors rich in keratin de
bris have long T1 and TI signal i ntensities, whereas tumors rich in cholesterin (lipid)
have short T1 signal intensities that fade on TI-weighted images. Some cysts may
show a characteristic fluid level on MRI. If a portion of the fluid within dermoid
cysts is rich in lipids, a chemical shift artifact will be generated in the direction of the
frequency-encoding gradient. On cr imaging, dermoid cysts appear as rounded,
well-circumscribed, low density masses that may appear multi-loculated or contain
fat and calcific densities as well as fluid.

Suggested Reading
Batsakis, 1.G. Tumors of the head and neck: clinical and pathological considerations,
2nd ed. Baltimore: Wiliams and Wilkins, 226-228, 1 979.
Davidson, H . D., Ouchi, T., Steiner, R.E. N M R imaging of congenital i ntracranial
germinal layer neoplasms. Neuroradiology 27:301-303, 1 985.
I ORAL CAVITY AND OROPHARYNX

Dillon, W.P., Miller, E.M. Cervical soft tissues. In: Newton, T.H., Hasso, A.N., Dillon,
W.P., ed. Modern Neuroradiology, vol. 3: Computed tomography of {he head and
neck. New York: Raven Press, 1 1 .3 1- 1 1 .33, 1 988.
Hunter, T.B., Palplanus, S.H., Chernin, M . M . , Coulthara, S.W. Dermoid cyst of the
floor of the mouth: CT appearance. AJR 1 4 1 : 1 239-1 240, 1 983.
ORAL CAVITY AND OROPHARYNXI

Case 53
Clinical Presentation
The patient presented with a painless fullness in the floor of the mouth.

A B

Radiologic Findings
The axial CT scan with contrast shows a cystic mass in the right sublingual space.
There is no associated soft tissue mass or mandibular involvement (Fig. 53-A). The
coronal view confirms these findings and shows that the mass does not involve the
submandibular space (Fig. 53-B).

Differential D iagnosis: Cyst of the Lateral


Floor of the Mouth Region
Simple ranula
Plunging ranula
Epidermoid/dermoid
Cystic hygroma
Atypical thyroglossal duct cyst
Atypical branchial cleft cyst
Abscess

Diagnosis
Simple ranula
I ORAL CAVITY AND OROPHARYNX

D iscussion
A ranula is a mucous retention cyst of the subLingual salivary gland. The name is de
rived from rana, the Latin word for frog, most likely because the lesion may clini
cally resemble a frog's belly. A ranula can occur in two forms. A simple ranula is con
fined to the sublingual space and often presents as intraoral mass lesions (Fig. 53-C,
image left). A diving or plunging ranula is a simple ranula that has herniated either
around or through the mylohyoid muscle to escape the sublingual space (Fig. 53-C,

D
ORAL CAVITY AND OROPHARYNXI

image right). I t usually results from the rupture of a simple ranula. Plunging ranu
PEARLS/PITFALLS lae extend into the submandibular space and present as a submandibular or neck
A simple ranula is a retention mass.
cyst located in the sublingual
space. Pathophysiology
A plunging ranula is a pseudo Ranulae are simple mucous retention cysts of the sublingual glands arising in the
cyst which extends to the sub sublingual space. The simple ranula is a true cyst whereas the plunging ranula is a
mandibular space or neck. pseudocyst.

It's important not to confuse ran


ulae with other lesions. Clinical Findings
A simple ranula typically presents as an i ntraoral mass. The diagnosis is often made
clinically. A plunging ranula presents as a submandibular or neck mass and may
have no clinically apparent oral connection.

I maging Findings
M R I or CT may be used to study these lesions. Ultrasound has also been used by
some experts to evaluate patients with this condition. The contents of the cyst are of
the density of water unless they are studied during an acute episode of infection or
recent hemorrhage. MR images show the lesion with signal intensity varying
according to protein content or the presence of hemorrhage (Fig. 53-0, arrow). On
CT or M R I , ranulae usually show an enhancing lining following contrast injection
because they are frequently associated with inflammation. Ranulae may occasion
ally cross the midline on either side of the frenulum.

Management
These lesions are treated surgically by complete resection. Imaging studies may be
valuable to define the entire extent of disease which may not be apparent based on
c1injcal examination alone.

Suggested Readings
Coit, W.E. et al. Ranulas and their mimics: CT evaluation Radiology 163:21 1-2 1 6,
1 987.
Davison, MJ., Morton, R.P., Mcivor, N.P. Plunging ranula: clinical observations.
Head and Neck 20(1 ):63-8, 1 998 Jan.
Garcaia, c.J., Flores, P.A., Arce, J.D., Chuaqui, B., Schwartz, D.s. Ultrasonography in
the study of salivary gland lesions in children Pediatric Radiology 28(6):41 8-25, 1 998
Jun.
Morton, R.P., Bartley, J.R . Simple sublingual ranulas: pathogenesis and manage
ment. Journal of Otolaryngology 24(4):253-4, 1 995 Aug.
1 0RAL CAVITY AND OROPHARYNX

Case 54
Clinical Presentation
A 45-year-old male was referred for evaluation of a right sided tongue mass.

A B

Radiologic Findings
On T2-weighted, non-contrast images ( Fig. 54-A), the right side of the tongue ap
pears of higher signal intensity than normal tongue muscles. In addition, there is a
posterior bulge extending into the airway on the right. Coronal post contrast images
(Fig. 54-B) show less contrast between the lesion and the healthy tongue. The
process is strictly unilateral without any signs of infiltration or displacement of sur
rounding tissues.

D ifferential Diagnosis: Tongue Assymetry


Post operative changes: post partial glossectomy
Hypoglossal denervation atrophy
Floor of mouthltongue neoplasm

D iagnosis
Hypoglossal denervation atrophy.

D iscussion
H ypoglossal nerve (cranial nerve X I I ) palsy is uncommon. Damage to this nerve
produces characteristic manifestations, of which unilateral atrophy of the tongue
musculature is the most important. Patients with this disorder have characteristic
ORAL CAVITY AND OROPHARY N XI

clinical and imaging findings which should be recognized at the time of presenta
tion.

Pathophysiology
The hypoglossal nerve is the motor nerve of the tongue musculature. The hypoglos
sal nerve leaves the skull base through the hypoglossal canal medially and above the
occipital condyle, close to the anterior rim of the foramen magnum. Beyond the
skull base, the hypoglossal nerve is joined by motor nerves from the CI -C2 roots of
the ansa cervicalis. These cervical branches proceed unmodified to the geniohyoid
muscle and do not exchange fibers with the hypoglossal nerve. Therefore, depend
ing on the site of the lesion, the geniohyoid muscle may or may not be spared. The
mylohyoid muscles and anterior bellies of the digastrics remain unaffected with
hypoglossal palsy.
When such findings of muscular fatty infiltration are present on cr and MRI, a
lesion along the course of the ipsilateral hypoglossal nerve from the tongue to the
brainstern should be excluded. In one large series of patients from a large public
hospital over 26 years, almost half of the patients presenting with hypoglossal palsy
were found to have a malignant tumor as the cause.

D E

245
I ORAL CAVITY AND OROPHARYNX

Clinical Findings
PEARLS/PITFALLS
Unilateral atrophy of extrinsic The clinical appearance of hypoglossal palsy may range from obvious atrophy in
and intrinsic tongue musculature severe cases to slight muscle fasciculations in early or mild involvement, which may
is the most important manifesta only be noticed by close friends or lovers. In the earliest stages, it may present with
tion of hypoglossal denervation muscular edema. Muscular wasting will occur after 2-3 weeks in any peripheral le
atrophy. sion to a motor nerve. Permanent damage leads to muscle atrophy and replacement
of the muscle fibers by fat and fibrous tissue. Unilateral hypoglossal nerve palsy
Flaccidity may create a c1inicaUy results in selective atrophy of the muscles innervated by the twelfth cranial nerve
confusing ipsilateral pseudomass which are the intrinsic and extrinsic tongue muscles. The resulting flaccidity of the
appearance on the lesion. muscles can result in a pseudomass on the side of the lesion.

Fifty percent of the cases pre


Imaging Findings
senting with xn nerve palsy may
be a result of a malignant tumor. Both computed tomography and magnetic resonance imaging are useful in assess
ing dysfunction of the hypoglossal nerve. The choice depends on the status of the
Take care not to mistake xn patient and the preference of the radiologist. I n either case the appearance is char
nerve palsy pseudo mass for a acteristic, with increased fatty infiltrations surrounding the atrophic muscles with
tumor.
sparing of the uninvolved groups (Fig.54-C). Figure 54-0 shows marked atrophy of
the genioglossus muscles on the involved side when compared to the other.
Do not fail to evaluate the
Atrophy of the intrinsic muscles of the tongue caused by paresis of the hypoglos
course of the X I I nerve for the
sal nerve leads to prolonged Tl and T2 relaxation times in a characteristic unilateral
primary cause of atrophy.
pattern. When these features are recognized, the radiologist, armed with knowledge
of the normal anatomy of the area, can focus on each segment of the nerve in search
of a cause. The hypoglossal nerve may be divided into five segments: the medullary,
cisternal, skull base, nasopharyngeal/oropharyngeal carotid space, and sublingual
segments. Because each segment is usually affected by different disorders, localizing
a lesion to a particular segment allows the radiologist to narrow the differential di
agnosis. Figure 54-E shows characteristic denervation atrophy of the right hemiglos
sus due to a large glomus tumor in the nasopharyngeal/oropharyngeal carotid space
segment of the hypoglossal nerve.

Suggested Readings
Keane, J.R. Twelfth-nerve palsy. Analysis of 1 00 cases. A rchives of Neurology
53(6):5 6 1 -6, 1 996 Jun.
Thompson, E.O., Smoker, w.R . Hypoglossal nerve palsy: a segmental approach. Ra
diographies 1 4(5):939-58, 1 994 Sep.
ORAL CAVITY A N D OROPHARYNX1

Case 55
Clinical Presentation
A previously healthy 50-year-old female presented with a 3-month history of pro
gressive soreness of her tongue, dysphagia, and left neck fullness. The patient
admitted to a long history of tobacco use (3-4 cigars/day).

A B

Radiologic Findings
The axial Tl-weighted image shows a mass in the left base of the tongue that ex
tends posteriorly into the airway (Fig. 55-A). Notice that the tumor appears isoill
tense with the normal musculature of the tongue. The TI-weighted axial image
shows increased signal intensity in the mass which now is easily distinguished from
adjacent muscle (Fig. 55-B).

Differential D iagnosis: Solid


Tongue B ase Mass
Squamous ceU carcinoma
Lingual thyroid
Hemangioma
Dermoid
Lymphoma

247 1
IORAL D OROPHARYNX

Diagnosis
Squamous ceU carcinoma involving the base of the left tongue with adenop
athy.

c D

248
ORAL CAVITY AND OROPHARYNXI

Discussion
Epide miology
The most common malignant tumor of the oral cavity and oropharynx is squamous
cell carcinoma, accounting for over 90% of malignant neoplasms. In spite of their
common histology, the behavior of individual tumors depends on their location in
the mouth. In general, a "malignant gradient" and likelihood of metastases increases
with a more posterior location in the oral cavity.
Anterior lesions (carcinoma of the lips, buccal mucosa, gingiva, alveolar mucosa,
and anterior tongue) are generally moderately to well differentiated and metasta
size relatively late to regional nodes (submandibular nodes). They are also relatively
easy to evaluate clinically, and therefore, CT and MR imaging have limited useful
ness in this area.
Carcinomas of the floor of the mouth, on the other hand, are usually moderately
to poorly differentiated and metastasize early to bilateral regional (submandibular,
sublingual, and high jugular) nodes. Figure 55-C shows a more anterior floor of
mouth carcinoma. Tumors of the base of the tongue (posterior 1 /3) tend to be diag
nosed at a more advanced stage than tumors of the oral (anterior 2/3) tongue.
Tongue base cancers tend to be more aggressive than those of the oral tongue. Their
poorer prognosis is also due to overall larger size and increased incidence of nodal
metastasis at the time of diagnosis. Approximately 70% of patients with tongue base
tumors present with advanced disease (stage I I I or I V ) compared with 30% of pa
tients with carcinomas of the oral tongue. Tongue base cancers may spread laterally
to involve the mandible, anteriorly to involve the oral tongue, and inferiorly to
involve the preepiglottic space and supraglottic larynx. Lymphatic spread may oc
cur bilaterally to submandibular, sublingual, and high jugular nodes.
The presence of nodal disease affects the staging of tumors, the patient's overall
prognosis, and whether a nodal dissection will be performed with the primary sur
gical resection. High in the neck and deep to the sternocleidomastoid muscle,
adenopathy can be inaccessible to palpation and is best evaluated with an imaging
study.
When the carotid artery is involved with a tumor, the chance for a primary
surgical cure is greatly reduced, and the overall treatment of the patient must be
reevaluated. MR images can show to what extent the vessel in question is sur
rounded by the tumor. As well, when a tumor extends to the mandible, the like
lihood of a cure by radiation therapy is greatly reduced, and surgery must be
considered. Although the dense cortical bone of the mandible produces no signal on
M R images, M R I can still resolve tumor involvement of this structure. An adjacent
tumor with replacement of the normal low signal of cortical bone is evidence of
tumor invasion.

Pathophysiology
Squamous cell carcinoma account for over 90% of malignant neoplasms of the
oral cavity and oropharynx. Adenocarcinoma, lymphoma, minor salivary gland
malignancies, and other rare neoplasms account for the remaining 1 0% . The three
major risk factors associated with tumors of squamous cell origin in this region
of the head and neck have long been recognized. They are tobacco, alcohol, and
syphillis.

Clinical Findings
It is often difficult to clinically evaluate the deep floor of the mouth and tongue
base, because deep palpation of this area often causes gagging and retching, and is
intolerable to most patients. Therefore, CT and especially M R imaging play an im
portant role in identifying lesions and determining tumor extent.
/ ORAL CAVITY AND OROPHARYNX

Imaging Findings
Carcinoma tends to have a low signal intensity (long T l ) on T l -weighted images,
which makes it difficult to differentiate from normal musculature. On TI-weighted
images, the tumor has varying degrees of high signal intensity, which allows sharp
differentiation of the tumor from the low intensity surrounding muculature. Nor
mal lymphoid tissue also has increased signal on TI-weighted images that may be
confused with the tumor. TIle lack of infiltration helps to separate this benign
process. The excellent tissue differentiation and spatial resolution of MRI allow
the detection of tumor spread along tissue planes, vascular channels, and muscle
bundles.
Since the definite diagnosis of cancer is obtained primarily by biopsy, the role of
CT and MRJ is to provide an accurate staging work-up in planning therapy. It is clin
ically important to determine whether the tumor has spread over the midline of the
lingular septum, to the mandible, and to the pharynx. The determination of sparing
or involvement of the lingual artery and hypoglossal nerve predicts the appropri
ateness of partial or hemiglossectomy.
TIle main bulk of a tongue base cancer is usually of high density and encroaches
to varying degrees on the low density of the intrinsic musculature beneath the base
of the tongue. Tumor masses may ulcerate or form bulky masses that change the
configuration of the tongue. On the other hand, it is surprising bow frequently tbe
tumors may infiltrate widely without producing appreciable changes in the volume
of the tongue base.
As the tumors extend slightly further cephalad and anteriorly beneath the bori
zontal intrinsic muscle plane, they encroach on the two bundles of the genioglossus
muscle. Remembering that the main bulk of the tongue is formed by this paired
genioglossus muscle, most tumors involve the genioglossus muscle to some extent.
The genioglossus is always separated by a midline low-density raphe. If this low
density region is obliterated, it is a reliable sign of infiltration across the midline by
a tumor mass.
The two bundles of the genioglossus should be quite symmetrical and their fan
shaped course from caudad to cephalad is also an exceLlent landmark on coronal
and sagittal scanning. Anteriorly and to either side of the genioglossus muscles are
broad low-density planes between it and the mylohyoid muscles. I n this groove lie
the sublingual salivary glands and the duct of the submandibular gland. Infiltrating
lesions of the floor of the mouth or extension of the tumor into the mobile tongue
will obliterate these low-density planes.
The sagittal and coronal MR images are superb for giving a volume determina
tion of any tumor lying within the tongue and floor of the mouth. Equally impor
tant is the anatomical fact of obliteration of fascial planes and muscle bundles.
The spatial information found on axial images (Figs. 55-C-55-D) is supple
mented by coronal images (Fig. 55-E) of an anterior right sided floor of mouth
carcinoma.

Management
Both surgery and radiation therapy are used for treating lesions in this area. The hy
poglossal nerve and the lingual nerve pass along the surface of the deep lobe of the
submandibular gland to penetrate the base of the tongue. Lying between the inter
digitation of the styloglossus and hyoglossus muscles, are the lingual artery and
veins. The fascial planes surrounding the artery and vein are loose and serve as an
avenue for anterior extension of tumors of the tongue base. 1l1is region should be
carefully scrutinized because tumors are frequently found infiltrating adjacent to
the vessels. I n addition, an important surgical consideration is the preservation of
one hypoglossal nerve and one lingual artery and vein (Lufkin et aI, 1 986). If tumor
infiltration is present about both of these vessels, hemiglossectomy is usually not
possible.
ORAL CAVITY AND OROPHARYNXI

PEARLS/PITFALLS Suggested Readings


There is more malignant behav Arakawa, A., Tsuruta, 1., Nishimura, R., Sakamoto, Y, Korogi, Y , Baba, Y ,
ior with posterior lesions of the Furusawa, M., lshimaru, Y, Uji, Y , Taen, A., et al. Lingual carcinoma. Correlation of
mouth. M R imaging with histopathological findings. Acta Radiologica 37(5):700-7, 1 996
Sep.
The status of midline, carotid Crecco, M., Vidiri, A., Palma, 0., Floris, R., Squillaci, E., Mattioli, M., Marzetti, E
artery, and mandible are impor T stages of tumors of the tongue and floor of the mouth: correlation between M R
tant when determining treat with gadopentetate dimeglumine and pathologic data. American Journal of Neuro
ment. radiology 1 5(9): 1 695-702, 1 994 Oct.

Take care not to overlook sup Lufkin, R.B., Wortham, D.G., Dietrich, R.B., et al. Tongue and oropharynx: findings
traglottic larynx extension. on M R imaging. Radiology 1 6 1 :69-75, 1 986.
Sigal, R., Zagdanski, A.M., Schwaab, G., Bosq, J., Auperin, A., Laplanche, A.,
Francke, J. P , Eschwege, E, Luboinski, B., Vanel, D? and M R imaging of squamous
cell carcinoma of the tongue and floor of the mouth. Radiographics 1 6(4):787-810,
1996 luI.
Yasumoto, M., Shibuya, H., Takeda, M., Korenaga, T. Squamous cell carcinoma of
the oral cavity: M R findings and value of Tl-versus T2-weighted fast spin-echo
images. A merican Journal of Roentgenology 1 64(4):98 1 -7, 1 995 Apr.
VII.

Salivary Glands
SALIVARY G LANDSI

Case 56
Clinical Presentation
A 35-year-old female presented with a painless left-sided cheek mass. On clinical
exam a non tender, nonmobile, soft, 2-cm mass was palpated in the left buccomas
setric region. The left facial nerve was intact.

A B

Radiologic Findings
An M R I of the parotids: (Fig. 56-A) axial T1 WI and (Fig. 56-B) axial TIWI through
the parotid glands shows a 2-cm mass, located laterally to the left masseter muscle
and caudal to Stenson's duct, following the signal characteristics of the parotid
gland parenchyma.

Differential Diagnosis: Unilateral


Cheek Mass
Pseudomass: masseteric hypertrophy, accessory parotid gland, facial process of
the parotid gland
Infection/Inflammatory: abscess of the masticator or buccal space, sialocele
(obstruction of stenson's duct), lymphadenopathy
Benign tumors: hemangioma/ Lymphangioma, lipoma, rhabdomyoma, neural
sheath tumors (neurofibroma/ schwan noma) , nodular fasciitis/ aggressive fibro
matosis, salivary gland tumors (pleomorphic adenoma, Warthin's tumor, oncocy
toma)
Malignant tumors: sarcomas (fibro/chondr% steosarcoma), salivary gland malig
nancies (adenoid cystic and mucoepidermoid carcinomas), rhabdomyosarcoma,
liposarcoma
ISALIVARY G LANDS

Diagnosis
Acces ory parotid gland

Discussion
Epidemiology
Acce ory parotid gland are a frequent finding in the general population. The
reported incidence in autop y tudies vary from 20 to 50% . Thi accessory parotid
ti ue may be un i- or bilateral and ha a similar incidence in male and females. The
size of this acce ory parotid ti ue i variable but does noi generally surpass 3 cm
in maximal diameter.

c D

1 256
SALIVARY GLANDSI

Pathophysiology
Accessory parotid glands are composed of salivary tissue which is completely sepa
rated from the bulk of the parotid g.land proper. It is usually located anterior to the
superficial lobe of the parotid gland, lateral to the masseter muscle and superior to
Stenson's duct. It drains into this duct via a variable number of tributary accessory
ducts.
The accessory tissue appears to originate from embryologic remnants of parotid
gland tissue that are left over when encapsulation of the gland occurs. Although
congenital in origin, this tissue is usually not apparent at birth and grows later in life,
eventually presenting in adulthood as a palpable mass.
Pathologically, this salivary tissue is identical to that of the parotid gland of chil
dren, containing mixed acini, both serous and mucinous, as opposed to the parotid
gland proper in adults, which contains only serous acini.
This accessory salivary tissue is susceptible to the same pathologic processes that
affect the parotid gland. The most frequent benign neoplasm occuring in the acces
sory parotid gland is a pleomorphic adenoma. On an M R I of the parotid glands, the
axial T1 WI ( Fig. 56-C) and (Fig. 56-D) axial T2WI shows a 2.3-cm mass along the
course of the Stenson's duct, anterior to the parotid gland proper, and lateral to the
masseter muscle. Note the different signal characteristics between this mass and
the parotid gland parenchyma. A coronal T I WI (Fig. 56-E) shows accessory parotid
gland tissue in the contralateral side and the mass on the right with slightly differ
ent T l W signal intensity. Diagnosis: B ilateral accessory parotid gland tissue. Pleo
morphic adenoma arising from the right accessory parotid tissue. However, the
incidence of malignant neoplasms is similar to that of minor salivary glands
(approximately 50% ) and there is a preponderance of mucoepidermoid carcinoma
over adenoid cystic carcinoma, as is seen in children. This again is explained by the
histologic composition of the accessory salivary tissue. One to 7% of all parotid
gland tumors occur in accessory parotid glands. I n flammatory pathology such as
sialoceles, resulting from obstruction of the draining accessory ducts or Stenson's
duct, and chronic sialoadenitis may also be seen.

F
I SALIVARY GLANDS

Clinical Findings:
PEARLS/PITFALLS
An accessory parotid gland pre Patients usually present with a palpable, painless mass in the cheek which is fre
sents as a non tender soft tissue quently felt after some form of facial trauma tbat cells their attention to that region.
mass in the buccomasseteric re On clinical exam a soft, non tender and nonmobile mass is palpated in the bucco
gion. masseteric region, witbin the middle third of a line drawn from the tragus to the
inferior border of the nasal ala. The absence of mobility is due to the medial at
An accessory parotid gLand is a tachment of the accessory parotid tissue to the masseteric fascia.
soft tissue mass, Located Lateral to Clinically, the diagnosis is not readily apparent, as the findings mimic any other
the masseter, anterior and clearLy mass lesion in the masticator or buccal space.
separate from the parotid
proper, with the same tissue ar Imaging Findings
chitecture as the parotid gLand.
Imaging is crucial in the diagnosis of pseudomasses of the buccomasseteric region.
Care should be taken not to mis Sialography, US, cr and M R I can all establish the diagnosis. Sialography usually
take the facial process of the demonstrates accessory ducts draining into the superior aspect of Stenson's duct,
parotid gLand for accessory but may not clearly separate an facial process of the parotid gland proper from true
parotid tissue. accesory parotid tissue. US, cr and MRl aLI demonstrate a soft tissue mass lateral
to the masseter muscle, anterior and clearly separate from the superficial lobe of the
Do not mistake accessory parotid parotid gland, with the same tissue architecture as the parotid gland. On an
tissue for a pathologic process. enhanced CT (Fig. 56-F) of the parotid glands, the axial section through the parotid
glands shows bilateral anterior extensions of normal parotid gland tissue overlying
the lateral aspect of the masseter muscle. Note that this tissue is contiguous with the
parotid gland proper. M R l , due to its higher contrast resolution and multi planar
capability, is the method of choice to diagnose this situation. The normal accessory
gland tissue shows the same signal characteristics as the parotid proper on both
T 1 W and T2W images. Correlation of both axial and coronal sections, free of den
tal artifacts, shows the separation from the parotid proper to advantage. An acces
sory parotid gland is frequently an incidental finding on studies performed for
unrelated reasons and has no clinical significance other than the potential confusion
with pathologic processes.

Management
o therapy is required if there are no pathologic abnormalities in the accessory sali
vary tissue. Resection may be required for cosmesis. When performing surgery, a
preauricular approach is preferred i n order to protect Stenson's duct and the buc
cal branch of the facial nerve from i njury.

Suggested Readings
Horii, A., Honjo, Y, Nose, M., Ozaki, M., Yoshida, 1. Accessory parotid gland tumor:
a case report. Auris, Nasus, Larynx 24( 1 ): 1 05-10, 1 997.
Nemecek, 1.R., Marzek, P.A., Young, Y.L. Diagnosis and treatment of accessory
parotid gland masses. Annals of Plastic Surgery 33( 1 ):75-9, 1 994 Jul.
Tart, RP, Kotzur, I.M., Mancuso, A.A., Glantz, M .S., Mukherji, S.K. CT and MR
imaging of the buccal space and buccal space masses. Radiographies \ 5(3):53 \ -50,
1 995 May.
SALIVARY G LANDSI

C ase 57
Clinical Presentation
The patient presented with swelling and tenderness in the left cheek region that was
exacerbated with eating.

Radiologic Findings
Figure 57-A is an axial CT scan which shows increased density of the left parotid
gland compared to the assymptomatic side. I n addition, a small area of calcification
is present on the right, near the entry point of Stensen's duct into the gland.

Differential Diagnosis: Painful Swelling of


Single Salivary Gland with Eating
Sialolithiasis
Small carcinoma causing obstruction .
Other causes of functional stricture of salivary gland duct.

D iagnosis
Sialolithiasis of the left parotid gland
ISALIVARY GLANDS

c D
SALIVARY GLANDSI

Discussion
Epidemiology
Over 80% of salivary gland stones are found i n the submandibular gland. Ten to
fifteen percent occur in the parotid gland and the remainder occur in the sublingual
glands. Most are solitary with less than 25% being m u ltiple. The three common
locations of submandibular sialolithiasis are the ductal opening into the mouth
(30% ) , the posterior edge of the myohyoid muscle (35% ), and the mid portion of the
duct (25% ) . A stone within the gland itself or the hilum is rare.

Pathophysiology
TIle higher incidence of submandibular stones is believed to be due to several fac
tors: ( 1 ) a more alkaline p H of the submandibular saliva, which tends to precipitate
salts; (2) thicker, more mucousy submandibular saliva; (3) a higher concentration of
hydroxy apatite and phosphatase in the submandibular saliva; (4) a narrower Whar
ton's duct orifice compared to the main lumen; (5) a slight uphill direction of sali
vary flow in Wharton's duct when the patient is in the upright position.

Clinical Findings
Patients with sialolithiasis often present with pain and swelling of the gland, associ
ated with eating. A lthough when a patient presents with these obstructing symp
toms, the other possibility to consider is floor of mouth squamous cell carcinoma.
Small stones may be entirely asymptomatic.

I maging Findings
Approximately 80% of salivary gland stones are radiopaque . The radiological study
should start with either plain films (AP, Lateral, submental view) or limited CT. I f
n o stone i s found o n plain films, sialography o r thin section CT may be useful t o rule
out radiolucent stones. Figure 57-B shows an axial CT scan of a patient with a right
salivary stone along the course of Wharton's ducl.
Sialographic findings of sialolithiasis are contrast fi lling defect and ductal dilata
tion, if the duct is obstructed. Since CT is much more sensitive in detecting a tiny
focus of calcium compared to plain radiograph, a thin section CT scan has been used
for patients with suspected salivary gland stones. CT usually reveals bigh density
stones with or without proxin1a1 ductal dilatation. A mimic of submandibular gland
stones on CT is unilateral calcification of the stylohyoid ligament.
When intravenous contrast is given, the affected gland may show strong and per
sistent enhancement. This is similar to the persistent nephrogram in patients with
obstructing renal calculi.
Because of the lower sensitivity of M R I compared to x-ray for the detection of
calcification, M R I is less well suited for the study of patients with this disease.
Larger stones may be seen 011 M R I as discrete areas of signal void, as in this patient
with a submandibular duct calculus (Fig. 57-C & 57-D ) However, M R sialography,
using beavily TIW FSE sequences allows for detection of ductile declaration and
filling defects within the duct i n a noninvasive fashion and with good correlation
with conventional sialography.
Ultrasound has been receiving growing attention as a technique for the evalua
tion of sialolithiasis. Endoscopy of the salivary ducts has also been reported.

Management
The approach depends on the location and the size of stones. A small stone may
spontaneously pass with the flow of saliva. Some stones may be removed with an
I SALIVARY G LANDS

endoluminal approach. Larger ones close to the duct orifice can often be removed
P EARLS/PITFALLS by intraoral approach. Stones within the central Wharton's duct may need total sub
Approximately 80% of stones mandibulectomy. An interesting new approach that has been described is to use
occur in the submandibular shock wave lithotripsy to destroy the stones.
glands.

Approximately 80% are radio Suggested Readings


opaque.
Fischbach, R., Kugel, H . , Ernst, S., Schroder, u., Brochhagen, H . G., Jungehulsing, M.,
X-ray is superior to M R in evalu Heindel, W. M R sialography: initial experience using a T2-weighted fast SE se
ating patients with this disease. quence. Journal of Computer Assisted Tomography 2 1 (5 ):826-30, 1 997 Sep-Oct.
Nahlieli, 0., Baruchin, A.M. Sialoendoscopy: three years' experience as a diagnostic
Make sure not to miss small floor and treatment modality. Journal of Oral and Maxillofacial Surgery 55(9):912-8, dis
of mouth squamous cell carci cussion, 1 997 Sep. 9 19-20.
noma.
Ottaviani, F., Capaccio, P , Rivolta, R., Cosmacini, P , Pignataro, L., Castagnone, D.
Do not mistake calcification in Salivary gland stones: U S evaluation i n shock wave lithotripsy. Radiology
stylohoid ligament on cr for a 204(2):437-41 , 1 997 Aug.
stone.
SALIVA RY G LANDSI

Case 58
Clinical Presentation
A 35-year-old female presented with parotid fullness. The patient's husband is a
human immunodeficiency virus positive ( H I V + ) intravenous drug abuser.

Radiologic Findings
An axial cr with contrast (Fig. 58-A) shows bilateral thin walled cysts within the
parotid gland.

Differential Diagnosis: Bilateral


Parotid Cysts
Benign lympboepithelial cysts
Bilateral Warthin's tumor
Cystic pleomorphic adenoma
Sjogren disease
Branchial cleft cyst

Diagnosis
Benign lympboepitbelial cysts related to H I V infection

Discussion
Epidemiology
Isolated benign lymphoepithelial cysts ( B LC) are a rare disorder of the parotid
gland with a reported incidence of 0.6% in the general population. In HIV seropos-
I SALIVARY GLA DS

Illve patient the incidence of multiple and bilateral parotid Iymphoepithelial cysts
i 3-10%. Parotid Iymphoepithelial cy t may manife t prior to the HIV scrocon
ver ion.

Pathophysiology
The cy t are typically located in the superficial portion of the parotid gland. Most
are bilateral and epithelial lined. The etiology of the e cyst remains unclear. The
hypothe i of the development of H I V-a ociated Iymphoepithelial cy"t" from pre
exi ting salivary lymph node inclu ion i one pos ibility.
Mo t expert now believe that the development of tbe cy ts i actually secondary
to the mechanical obstruction of alivary duct caused by lymphoid hyperplasia and

264
SALIVARY GLANDSI

not due to true de novo cyst formation. The enormous cystic dilatation of the ducts
PEARLSIPITFALLS presumably is a consequence of ductal obstruction through basal cell hyperplasia of
BLC presents as bilateral parotid striated ducts and intense intraglandular Iymphofollicular hyperplasia.
cysts. BLC occur almost exlusively in the parotid glands with little or no involvement
of the other salivary glands. This may be due to the fact that the parotid gland is the
mv seropositivity can be sug only salivary gland to contain significant lymphoid tissue within it.
gested by associated adenopathy
and lymphoid hyperplasia.
Imaging Findings
BLC may occur prior to H I V The lesions appear on imaging studies as thin walled cysts of the parotid gland.
seropositivity. Either CT or M R I scanning can suggest the diagnosis. A search for other findings
characteristically associated with H I V infection may be useful in establishing these
Do not mistake for bilateral
as H I V-associated lesions. The diagnosis of H I V seropositivity can be suggested
Warthin tumors.
by the presense of lymphoid hyperplasia as suggested by three findings in the
extracranial head and neck: 1 ) bilateral benign lymphoepithelial cysts of the parotid,
2) extensive bilateral cervical adenopathy, and 3) hypertrophy of the tissues of
Waldeyer's ring which include the adenoids, lingual tonsils, and palatine tonsils.
Figure 58-B shows CT scans of another 44 year old H I V + patient presenting with
characteristic findings. Small right sided parotid masses are present, and there is ex
tensive lymphoid hyperplasia of the nasopharyngeal tissues. On this higher section
(Fig. 58 -C) additional lesions are seen on the left parotid coronal TIW M R I in an
other patient with BLP (Fig. 58-D) shows bilateral intraparotid cysts.
In the absence of H I V infection, parotid cysts are rare. A Warthin tumor should
be considered in the differential diagnosis because this lesion may appear bilater
ally in 1 0% of cases. There is often a small mass associated with the cysts.

Management
The lesions are usually self-limited and therapy is performed for cosmesis or physi
cal discomfort. Some authors advocate enucleation as a safe and effective procedure
that provides the patient with complete removal of tbe cyst and a low recurrence
rate.

Suggested Readings
Beitler, J.J., Vikram, B., Silver, CE., Rubin, J.S., Bello, J.A., Mitnick, R.J., Gejerman,
G., Davis, L.w. Low-dose radiotherapy for multicystic benign Iymphoepithelial
lesions of the parotid gland in H I V-positive patients: long-term results. Head and
Neck 1 7 ( 1 ):31-5, 1 995 Jan-Feb.
Cohen, M . N., Rao, U, Shedd, D.P. Benign cysts of the parotid gland. Journal of Sur
gical Oncology 27: 1 1 56-1 1 79, 1 984.
Ferraro, FJ. Jr., Rush, B.F Jr., Ruark, D., Oleske, J. Enucleation of parotid Iym
phoepithelial cyst in patients who are human immunodeficiency virus positive.
Surgery, Gynecology and Obstetrics 1 77(5):524-6, 1 993 Nov.
Finfer, M.D., Scinella, RA., Rothstein, S.G., et al. Cystic parotid lesions in patients
at risk for the acquired immunodeficiency syndrome. A rchives of Otolaryngology
and Head and Neck Surgery 1 1 4 : 1 290-1 294, 1 988.
Holliday, RA., Coben, W.A., Schinella, RA., et al. Benign Iymphoepithelial parotid
cysts and hyperplastic cervical adenopathy in A IDS-risk patients: a new CT ap
pearance. Radiology 1 68:439-441 , 1 988.
Martinoli, C, Pretolesi, F, Del Bono, v., Derchi, L.E., Mecca, D., Chiaramondia, M .
Benign Iympboepitbelial parotid lesions i n H I V-positive patients: spectrum o f find
ings at gray-scale and Doppler sonography. A merican Journal of Roentgenology
1 65(4):975-9, 1 995 Oct.
Mayer, M . , Haddad, 1. Jr. H uman immunodeficiency virus infection presenting witb
I SALIVARY G LANDS

Iymphoepitbelial cysts i n a six-year-old cbild. A nnals of Otology, Rhinology and


Laryngology 105(3):242-4, 1 996 Mar.
Schrot, R.J., Adelman, H.M., Linden, C.N., WaUach, P.M. Cystic parotid gland en
largement in H I V disease. The diffuse infiltrative lymphocytosis syndrome. lama
278(2 ) : 166-7, 1 997 Jul 9.
Seddon, B.M., Padley, S.P., Gazzard, B.G. Differential diagnosis of parotid masses in
HIV positive men: report of five cases and review. Internalional lournal of SId and
A ids 7(3):224-7, 1996 May-Jun.
Som, P.M., Brandwein, M.S., Silvers, A. Nodal inclusion cysts of the parotid gland
and parapharyngeal space: a discussion of lympboepithelial, A IDS-related parotid,
and branchial cysts, cystic Warthin's tumors, and cysts in Sjogren's syndrome. Laryn
goscope 1 05 ( 1 0) : 1 1 22-8, 1 995 Oct.
SALIVA RY GLANDSI

Case 59
Clinical Presentation
The patient is a middle aged male wbo complains of asymmetry of his cbeek and
excessive sweating over the area at meal time.

A B
I SALIVARY G LA N DS

Radiologic Findings
The right parotid gland is small in size compared to the left side on the axial T I
weighted image (Fig. 59-A). The 1'2 weighted axial ( Fig. 59-B) and T 1 weighted
coronal (Fig. 59-C) show similar findings. The remainder of the surrounding struc
tures are unremarkable.

Differential Diagnosis: Small Parotid Gland


Normal variation
Chronic post obstructive atrophy
Postradiation
Postoperative

Diagnosis
Normal postoperative findings (total parotidectomy)

Discussion
Assymetry of the parotid gland can be due to a number of conditions. Postobstruc
tive and postradiation atrophy can result in a small gland. However. complete ab
sence with these conditions is uncommon. Total parotidectomy leaves little normal
glandular tissue, while a superficial parotidectomy leaves the portion of the gland
deep to the plane of the facial nerve (deep lobe of the gland). Gustatory sweating is
a well-known sequela of parotid surgery.

Pathophysiology
G ustatory sweating is also known as Frey syndrome which affects the auriculotem
poral nerve. I n this interesting condition, the secretory parasympathetic fibers of the
parotid gland are thought to communicate with the sympathetic nerve fibers of
sweat glands and blood vessels of the skin following parotidectomy. Miscommuni
cation results in subjective gustatory sweating and facial flushing, which appear
early with mastication in the postoperative period.

Clinical Findings
The diagnosis of post operative changes can be easily made by the patient history.
Patients with Frey Syndrome have facial flushing, sweating, or both, localized to the
distribution of the auriculotemporal nerve that occurs in response to gustatory stim
uli (i.e., gustatory sweating). Up to 40% of patients in some series report this con
dition following parotid surgery or trauma to this area. I t has also been reported as
a rare complication of diabetes.

Imaging Findings
Either M R I or CT can show the soft tissue anatomy of this area well. I f there is a
question of the presense of calculi, CT is the preferred study. Post obstructive or
post radiation atrophy may show alteration of signal intensity as well as volume loss
of the gland. I f the patient's surgical history is unclear or absent, the radiologist
should be able to suggest the correct diagnosis.

Management
Parotid resection may be performed in one of two general forms. In a superficial
parotidectomy, the gland deep to the facial nerve is spared. Alternatively, a total
parotidectomy results in the removal of all parotid tissue on that side.
SALIVARY GLANDSI

PEARLSIPITFALLS Suggested Readings


A parotidectomy may be either Yamashita, T. , Tomoda, K., Kumazawa, T. The usefulness of partial parotidec
superficial or total. tomy for benign parotid gland tumors.A retrospective study of 306 cases. Acta Oto
Laryngologica. Supplement 500: 1 1 3-6, 1993.
Gustatory sweating may occur in
up to 40% of patients following
parotidectomy.
A clinician should not mistake
post surgical changes for
pathology of the uninvolved side.
I SALI VARY G LANDS

Case 60
Clinical Presentation
A 40-year-old female presented with recurrent episodes of bilateral parotid swelling
and xerostomia.

A B

Radiologic Findings
A CT scan of the parotid glands ( Fig. 60- A ) showed enlargement and heterogene
ity of the glandular parenchyma due to the presence of multiple dense micronod
ules. Another CT scan performed 2 years later ( Fig. 60-B) shows progression of
disease with atrophy and fatty replacement of the parenchyma (Courtesy of Dr.
Andrew Berger).

Differential Diagnosis: Bilateral


Parotid Enlargement with
Parenchymal Heterogeneity
Sialoadenosis: chronic alcoholism. diabetes. malnutrition ( nutritional mumps), hy
perlipidemia, drugs, post-radiation therapy
Sialadenitis: infectious (viral parotitis, Iymphoepithelial cysts), inliammatory (sar-
coidosis, graft versus host disease. Mikulicz's syndrome)
Bilateral parotid neoplasms: Warthin's tumor. oncocytoma. pleomorphic adenoma
Multiple intraparotid lymphadenopathy: reactive. metastatic, lymphoma
Others: amyloidosis. polycystic (dysgenetic) disease. clear cell oncocytosis

D iagnosis
Sjogren syndrome or autoimmune sialadenitis

1270
SALIVARY GLANDSI

D iscussion
Epidemiology
Sjogren syndrome is a systemic autoimmune disease of the exocrine glands which
may occur alone (primary Sjogren syndrome), or in association with connective tis
sue disorders such as rheumatoid arthritis or lupus (secondary Sjogren syndrome).
The real incidence of the disease is probably underestimated because symptoms
may be nonspecific, and serologic studies may be negative in the quiescent stages.
Autopsy studies show an incidence of 0.4% , placing Sjogren syndrome as the sec
ond most common autoimmune disease following rheumatoid arthritis.
There are childhood and adult forms of the disease. The former, also known as
recurrent parotitis, is rare and predominantly affects children between 3 and 6 years
of age. Most cases regress spontaneously at puberty and there is a low incidence of
disease progression to the adult form. The latter is a chronic progressive disease
more common in females (90 to 95% of cases) with a peak incidence between 40
and 60 years of age.

P athophysiology
Sjogren syndrome is a chronic inflammatory exocrinopathy affecting, primarily, the
salivary and lacrimal glands. The precise mechanism triggering this autoimune
process is unknown, although Epstein-Barr Virus ( EBV) and retroviruses have
been postulated as possible candidates. Among the salivary glands the parotid
glands are the most commonly affected.
Sjogren syndrome (SS) is characterized by a diffuse lymphocytic infiltration form
ing small collections around the intralobular ducts and causing atrophy and re
placement of the acinar structures. Concurrently, the ductal epithelium proliferates,
obliterating the ductal lumina and producing epithelial islands which may undergo
granular metaplasia and hyalinization. The presence of these islands is necessary to
establish the histologic diagnosis of benign lymphoepithelial lesion or Godwin
tumor, but is not specific for SS. In the later stages, fatty infiltration of the glands
becomes a prominent feature. Both the epithelial and lymphoid component of these
lesions may undergo malignant transformation accounting for the increased inci
dence of lymphoma (44 times higher than in the general popUlation) and epithelial
malignancies in patients with SS.

Clinical Findings
SS is defined by a triad which includes xerostomia, keratoconjuntivitis sicca and
connective tissue disorder. The presence of two or more of these features makes the
diagnosis of SS. Most patients present with oral and ocular symptoms including oral
dryness and soreness, difficulty swallowing, oral ulcers, multiple recurrent dental
caries and eye dryness and grittiness. Symptoms reflecting involvement of other
exocrine glands may also be present, such as skin itching, dyspareunia and nonpro
ductive cough. Xerostomia is a subjective and nonspecific symptom common to
other conditions. Definitive diagnosis of SS requires documentation of decreased
salivary flow and the presence of at least one of the following: ( 1 ) Keratocon
junctivitis sicca, (2) Characteristic findings on salivary or lacrimal gland biopsy,
(3) Specific findings on sialogram (diffuse nodular or globular pooling of contrast
material > 1 mm in diameter).
Symptoms and signs of an associated connective tissue disorder may be present
in secondary SS.
The serologic markers of the disease are the SS-A and SS-B antibodies. However,
these may only be detected in the active phase of the disease and there are sero
logically negative forms, particularly in males. The presence of persistent or rapid
I SALIVARY G LANDS

parotid enlargement along with cervical lymphadenopathy suggests malignant de


generation, most often lymphoma.

I maging Findings
Several imaging modalities including sialography, scintigraphy, US, cr and MRI
may be used and give complimentary information. Among these, sialography is con
sidered the gold standard in the diagnosis and staging of this disease. An x-ray sialo
gram of anotber patient ( lateral view) (Fig. 60-C) shows pooling of contrast mater
ial in a globular pattern with multiple small collections uniformy distributed
thoughout the periphery of the gland resembling a mulberry tree. Imaging findings

1272
SALIVARY GLANDSI

depend on the stage of the disease and are more striking in the later stages when
cystic degeneration and fatty infiltration predominate. X-ray and MR sialograms
may show three different patterns which reflect progressive destructive changes.
The earliest stage shows a punctate pattern of numerous peripheral punctate col
lections of contrast material, less then 1mm in diameter, uniformly distributed
throughout the gland. I n the second stage, larger globular collections of contrast ma
terial measuring between 1 and 2 mm and resembling a mulberry tree are seen. Fi
nally, the destructive or cavitary pattern shows large, irregular collections of contrast
up to 1 cm in diameter, combined with central changes of sialadenitis resulting from
ascending infection. I n the earlier stages, the uniformity in the size and distribution
of the lesions allows differentiation from chronic sialadenitis. In the end of the ex
amination a secretagogue may be given showing retention of the contrast material
in the ductal collections.
Salivary gland scintigraphy with technetium-99m allows for assessment of glan
dular function. Resting and stimulated tinle-activity curves reflect the production
and accumulation of saliva and are a valuable tool in the evaluation of disease pro
gression. Typically, patients with SS show decreased accumulation, decreased secre
tion and decreased response to secretory stinlUlation. However, these findings are
not specific for SS and may be seen in other chronic diseases of the salivary glands.
Ultrasound is a noninvasive and relatively inexpensive imaging modality which is
advocated by some experts as the primary screening imaging modality in patients
suspected of SS. The most important sonographic finding is bilateral parenchymal
inhomogeneity, ranging from multiple discrete hypoechoic foci separated by hyper
echoic septa to cystic changes in the gland parenchyma. I n the early stages of acute
inflammation the gland may appear enlarged and hypoechoic, an appearance indis
tinguishible from that seen in other inflammatory conditions and with sialoadeno
sis. I n advanced cases, the findings are those of end stage parotid disease of any eti
ology; the gland becomes atrophic and may show large cystic cavities. US may also
exclude sialolithiasis as the cause of symptoms.
On CT scans the parotid glands are enlarged and denser than normal (iso- to
hyperdense of the masseter muscle). These early findings are nonspecific and are
also found in chronic sialadenitis and sialosis. Later, the glands become heteroge
neous with an appearance ranging from a micronodular to a honeycombed pattern.
A multicystic appearance may be seen similar to that seen with multiple lymphoep
ithelial cysts, cystic intraparotid lymphadenopathy, multiple abscesses and multicen
tric Warthin tumors. However, in most circumstances, the history and associated
clinical findings allow for the differentiation of these conditions. CT may also be
performed after the injection of sialographic contrast material and allow for optimal
visualization of the ductal system.
MRI findings are similar to those seen on CT, manifest as parenchymal hetero
geneity, cystic degeneration and fatty replacement. On an MRI of the parotid
glands, axial TI W (Fig. 60-D), and axial TIW (Fig. 60-E) images show bilateral en
largement of the parotid glands and parenchymal hetrogeneity with a granular ap
pearance. There are small micronodules hypointense on T l WI and high on signal in
tensity on TIWI corresponding to mildly dilated peripheral ducts. This MRI
appearance is the counterpart of the punctate pattern seen in X-ray sialography in
the earliest stages of the disease. MR-sialogram using 3D fast spin echo (SE) heav
ily TIW sequences has been shown to have good correlation with X-ray sialography
and may replace thjs invasive modality.

Management
Most cases of SS are managed with medical therapy including steroids and, eventu
ally, other immunosupressors. Parotidectomy is seldom necessary to relieve symp
toms of recurrent infection but may be necessary in cases of malignant degenera
tion.
ISALIVARY GLANDS

PEARLS/PITFALLS Suggested Readings


Sicca syndrome (xerostomia and Bohuslavizki, K.H., Brenner, w., Wolf, H., Sippel, C, Tbnshoff, G., Tinnemeyer, S.,
xerophthalmia) can be major Clausen, M., Henze, E. Value of quantitative salivary gland scintigraphy in the early
symptoms of SS. stage of Sjogren's syndrome. Nuclear Medicine Communications 16( 1 1 ):917-22,
1 995 Nov.
A cr scan should show bilateral Izumi, M., Eguchi, K., Nakamura, H., Nagataki, S., Nakamura, T. Premature fat de
enlargement of the parotid position in the salivary glands associated with Sjogren syndrome: MR and cr evi
glands in a patient with SS. dence. American Journal of Neuroradiology 18(5):95 1-8, 1997 May.
SS is characterized by diffuse Izumi, M., Eguchi, K., Ohki, M., Uetani, M., Hayashi, K., Kita, M., Nagataki, S.,
heterogeneity of the glandular Nakamura, T. MR imaging of the parotid gland in Sjogren's syndrome: a proposal
parenchyma progressing to fat for new diagnostic criteria. American Journal of Roentgenology 166(6):1483-7, 1 996
replacement and cystic changes. Jun.
Izumi, M., Eguchi, K., Uetani, M., Nakamura, H., Takagi, Y , Hayashi, K., Nakamura,
The clinician should avoid mis T. M R features of the lacrimal gland in Sjogren's syndrome. American Journal of
taking SS for sialadenitis (viral Roentgenology 1 70(6):1661-6, 1 998 Jun.
parotitis). Makula, E., Pokorny, G., Rajt<ir, M., Kiss, 1., Kovacs, A., Kovacs, L., Parotid gland ul
The clinician should avoid failing trasonography as a diagnostic tool in primary Sjogren's syndrome. British Journal of
Rheumatology 35( 10):972-7, 1 996 Oct.
to recognize malignant trans
formation. Nagahiro, S., Mantani, A., Yamada, K., Ushio, Y. Multiple cerebral arterial occlusions
in a young patient with Sjogren's syndrome: case report. Neurosurgery 38(3):592-5,
discussion 595, 1996 Mar.
Saito, T. , Fukuda, H., Horikawa, M., Ohmori, K., Shindoh, M., Amemiya, A. Salivary
gland scintigraphy with 99mTc-pertechnetate in Sjogren's syndrome: relationship to
clinicopathologic features of salivary and lacrimal glands. Journal of Oral Pathology
and Medicine 26( 1):46-50, 1 997 Jan.
Som, P.M., Brandwein, M.S., Silvers, A. Nodal inclusion cysts of the parotid gland
and parapharyngeal space: a discussion of Iymphoepithelial, AIDS-related parotid,
and branchial cysts, cystic Warthin's tumors, and cysts in Sjogren's syndrome. Laryn
goscope 105( 10): 1 122-8, 1995 Oct.
Yoshiura, K., Yuasa, K., Tabata, 0., Araki, K., Yonetsu, K., Nakayama, E., Kanda, S.,
Shinohara, M., Higuchi, Y Reliability of ultrasonography and sialography in the di
agnosis of Sjogren's syndrome. Oral Surgery, Oral Medicine, Oral Pmhology, Oral
Radiology and Endodontics 83(3):400-7, 1 997 Mar.
S A L I VA RY G LA Dsl

Case 61
Clinical Presentation
A 25-year-old female presented with a painless left cheek mass.

A B
ISALIVARY GLANDS

Radiologic Findings
Using M RI a relatively homogeneous 3 cm mass in the left parotid was found that
involves both the deep and superficial lobes (Fig. 6 1 -A). The lesion demonstrates
hyperintensity on the T2-weighted images (Fig. 6 1 -8 ) . On tbe coronal postcontrast
T I weighted image, there is heterogeneous enhancement (Fig. 6 I -C).

Differential D iagnosis: Parotid Mass


Pleomorphic adenoma
Warthin's tumor
Mucoepidermoid carcinoma
Adenoid cystic carcinoma
Intraparotid lymph node
Metastasis
Lymphoepithelial cyst
Monomorphic adenoma
Oncocytoma

Diagnosis
Pleomorphic adenoma (benign mixed tumor)

Discussion
The pleomorphic adenoma (or benign mixed tumor) is the most common benign
salivary gland tumor and the most common tumor of the parotid gland.

Epidemiology
Salivary gland tumors affect 1 -3 individuals per 1 00,000 population. 111 i s number is
increased 5 - 1 0 times in people of I nuit (Eskimo) ancestry. Approximately 70% of
parotid. 50% of submandibular, 30% of sublingual, and 1 0-40% of accessory sali-

D E
SALIVARY GLANDSI

vary gland tumors are benign. Therefore, the smaller the salivary gland of origin, the
smaller the likelihood that the tumor is benign.
Pleomorphic adenomas occur in all ages, although 60% of cases occur between
the third to fifth decades of life. The female/male ratio exceeds 2 : 1. They tend to be
solitary lesions, with true multicentric origin being rare.

Pathophysiology
The pleomorphic adenoma is composed of a combination of myoepithelial cells and
a variety of epithelial and mesenchymal tissue surrounded by a fibrous capsule. Al
though th e y are well encapsulated, small areas of n e oplas t i c cells will often extend
beyond the capsule, known as satellitosis. If simple enucleation is performed, these
areas may be left behind and lead to residual disease. Figures 61-D and 61-E show
the appearance of such recurrent disease following incomplete resection. Note the
rounded, nodular appearance. Larger lesions may show areas of calcification, hem
orrhage, and necrosis.
Malignant transformation may take place in the form of carcinoma expleomor
phic adenoma. This should be suspected in patients with long standing tumors that
show a sudden increase in size or develop new symptoms. Estimates for the likeli
hood of this occurring, range from 1 -25% of untreated pleomorphic adenomas in
various series.

Imaging Findings
On MRl, normal parotid tissue has an intermediate intensity between muscle and
fat. Because of its high fat content, parotid tissue has a relatively short Tl relaxation,
allowing excellent visualization of these glands. On T l -, spin density, and
T2-weighted images, the parotid gland is hyperintense relative to adjacent muscula
ture, but less intense than fat, with parotid tissue very similar in intensity to fat on
T2-weighted images.
Pleomorphic adenomata have bright signal intensities on T2-weighted images.
Tumor-muscle or tumor-normal gland interfaces are better seen on T2- than
T l-weighted images where both tumor and parotid gland may have intermediate in
tensity signals. Pleomorphic adenoma tend to be homogeneous and smoothly mar
ginated. By contrast Warthin's tumors are more heterogeneous. Multiplicity of
lesions also suggests a Warthin's tumor.
MRI characteristics that may suggest malignancy include irregular margination,
heterogeneous signal, lymphadenopathy, adjacent soft tissue (or bony) invasion, or
facial perineural spread. A specific tissue diagnosis of salivary gland tumors is best
obtained from aspiration cytology, as the MRI or CT appearance is ultimately non
specific. Dystrophic calcification does suggest the diagnosis of pleomorphic ade
noma because tbis finding is rare in other parotid tumors.
The deep lobe of the parotid gland extends between the mandibular ramus and
styloid process into the parapharyngeal space. For ideal surgical planning, masses
arising within the deep lobe ( transparotid approach) must be differentiated from
other parapharyngeal space masses (transcervical approach). In true deep lobe
tumors, there will be absence of the fatty tissue plane on some or all axial slices
between the parapharyngeal deep lobe mass and the normal parotid gland. Normal
parotid tissue partially wrapped around the mass or extension of the mass laterally
into the stylomandibular tunnel further suggests a deep parotid lobe origin. Deep
lobe tumors may extend to the prestyloid compartment of the parapharyngeal
space, displacing the internal carotid artery posteriorly.
Extraparotid parapharyngeal masses should display a fatty tissue plane between
the mass and the deep lobe. Neuromas, paragangliomas, and metastatic nodal
masses arise within the poststyloid compartment displacing the internal carotid
artery forward.
Recurrences foLlowing parotidectomy for pleomorphic adenoma often appear as
I SALIVARY G LANDS

multifocal nodular densities (Fig. 6 1 E). This reflects the common histologic occur
-

PEARLSIP ITFALLS rence of extracapsular tumor pseudopods which may evade primary resection.
The pleomorphic adenoma is the
most common benign sa livary Management
gland tumor.
Surgery (superficial or total parotidectomy) is the treatment of choice. Simple exci
The pleomorphic adenoma is sion is generally not done because of the risk of residual disease.
composed of a fibrous capsule,
but extracapsular neoplasstic tis
sue is common. Suggested Readings
Malignant transformation may Batsakis, J.G. Tumors of the head and neck: clinical and pathological considerations.
take place, but the percentage is 2nd ed. Baltimore: Williams and Wilkins. 1 979;21-26.
controversial ranging from Curtin, H .D. Assessment of salivary gland pathology. Otolaryngologic Clinics of
1 -25% of untreated pleomorphic North A merica 2 1 :547-573, 1988.
adenomas. Som, P.M., Biller, H . E High grade malignancies of the parotid gland: identification
with MR imaging. Radiology 1 73:823-826, 1989.
Calcification in parotid mass sug
gests the diagnosis of pleomor Som, P.M . , Braun, I.E, Shapiro, M.D., Reede, D.L., Curtin, H . D., Zimmerman, R.A.
phic adenoma. Tumors of the parapharyngeal space and upper neck: MR imaging characteristics.
Radiology 1 64:823-829, 1 987.
Do not fail to perform adequate Som, P.M., Shugar, J.M., Sacher, M., Stollman, A.L., B iller, H.E Benign and malig
surgical removal, leaving disease nant parotid pleormorphic adenomas: CT and MR studies. jou.rnal of Computer As
behind. sisted Tomography 25:65-69, 1 988.
Swartz, J.D., Rothman, M .l . , Marlowe, EI., Berger, A.s. MR imaging of parotid mass
lesions: attempts at histopathologic differentiation. jou.rnal of Computer Assisted
Tomography 1 3:789-796, 1 989.
Tabor, E . K., Curtin, H.D. M R of the salivary glands. Radiologic Clinics of North
America 27:379-392, 1 989.
Teresi, L.M., Lufkin, R.B., Wortham, D.G., et al. Parotid masses: M R imaging. Radi
ology 163:405-409, 1 987.

1278
SALIVA RY G LANDSI

Case 62
Clinical Presentation
An adult male presented with a painless fullness over the angle of the right
mandible.

B c

Radiologic Findings
TI weighted images in the axial coronal, and sagittal plane ( Figs. 62-A, 62-B, and
62-C) show a well defined combined deep and superficial lobe mass. It has areas of

279 1
I SALIVARY G LANDS

high signal intensity on both Tl and T2 weighted images that suggest a multicystic
nature.

Differential Diagnosis: Cystic Parotid


Gland Mass
Warthin's tumor
Multiple Iymphoepithelial cysts
Intraparotid lymph node metastases
Pleomorphic adenoma
Sjogren syndrome
Intra parotid 1st branchial cleft cystlretention cyst (mucocele)
Dermoid cyst
Abscesslsialocele
Lymphangioma

Diagnosis
Warthin's tumor

Discussion
Epidemiology
Although Warthin's tumors have been reported to occur in a wide age span (2.5 to
92 years), 82% are found in patients between 40 and 70 years of age (average 55
years). There is a decided (5 : 1 ) male predominance, although some recent studies
suggest that the incidence in women may be actually closer to that of men. Warthin's
tumors represent 6%-10% of all parotid gland tumors, of benign tumors second in
frequency only to the pleomorphic adenoma.

Pathophysiology
Warthin's tumors are peculiar tumors that contain both salivary and lymphoid com
ponents. They consist of heterotopic salivary gland ductal elements withi.n lymphoid
tissue within or external to the parotid gland. This is why some older textbooks
refer to them as cystadenolymphoma or papillary cystadenoma Iymphomatosum.
With their limited growth potential, most pathologists consider them to be a
hamartoma arising from epithelial inclusions in intraparotid lymph nodes rather
than a true neoplasm. Malignant transformation and facial nerve involvement are
rare. Because of the pathogenic relationship with lymph nodes, Warthin's tumor
may also arise in lymph nodes superficial or medial to the parotid gland. Because
the parotid is the only salivary gland to contain appreciable lymphoid tissue, this
tumor is rare in the submandibular, sublingual or minor salivary glands.
The tumor is encapsulated often with cystic areas. Multiple lesions are common
and they are bilateral in 10% of cases.

Clinical Findings
This tumor usually manifests as a painless movable mass with only 10% of patients
presenting with pain and pressure sensations. They are most often located over the
angle of the mandible in the tail of the parotid.

Imaging Findings
The typical Warthin's tumor is a well-defined, lobulated, intermediate signal inten
sity mass with cystic areas. However, any other benign or low-grade malignant
tumor could have a similar appearance. I n the majority of cases, M R l is not any
SALIVARY GLANDSI

more accurate than CT in predicting the histologic nature of the parotid gland
PEARLS/PITFALLS because of the unusual histology of salivary gland tumors.
Most pathologists consider Technitium 99m-pertechnetate accumulates in a Warthin's tumor. Some experts
Warthin's tumor to be a hamar suggest that this effect is due to the large number of mitochondria present in the
toma involving salivary ductal oncocytes within the tumor. Not surprisingly, the only other parotid tumor to show
elements and lymphoid tissue. this Technitium uptake is the oncocytoma.
The role of ultrasound in the evaluation of the parotid gland has major limita
Warth in's tumor is found almost tions. These include the failure to visualize the entire parotid gland, relationship of
exclusively in the parotid gland. the mass to the facial nerve, and evaluating the spread of large tumors.

This tumor is a cystic encapsu


lated parotid mass. Management
Simple enucleation is adequate for control of this lesion. However, because of the
Warthin's tumor is found bilater
possibility of multicentricity, most surgeons recommend a superficial or total
ally in 1 0% of cases.
parotidectomy (depending on the size and location of the lesion) to avoid leaving
Technicium 99m accumulates in a behind residual disease.
Warthin's tumor.

Avoid confusing with other cystic Suggested Readings


parotid lesions. Bruneton, 1.N., Mourou, M . Y., Ultrasound in salivary gland disease. Journal of
Oto-Rhino-laryngology and Its Related Specialties 55(5):284-9, 1 993 Sep-Oct.
It's important not to overlook
bilateral disease. Joe, Y.Q., Westesson, P.L. Tumors of the parotid gland: M R imaging characteristics
of various histologic types. American Journal of Roentgenology 1 63(2):433-8, 1994
Aug.
Minami, M., Tanioka, H . , Oyama, K., l tai, Y., Eguchi, M., Yoshikawa, K., Murakami,
T., Sasaki, Y. Warthin
tumor of the parotid gland: M R-pathologic correlation. Amer
ican Journal of Neuroradiology 1 4 ( 1 ) :209-14, 1 993 Jan-Feb.
Som, P.M., Brandwein, M.S., Silvers, A. Nodal inclusion cysts of the parotid gland
and parapharyngeal space: a discussion of \ymphoepithelial, AIDS-related parotid,
and branchial cysts, cystic Warthin's tumors, and cysts in Sjogren's syndrome. Laryn
goscope 1 05( 1 0 ) : 1 1 22-8, 1 995 Oct.
Teresi, L.M., Lufkin, R.E., Warthan, D.G., et al. Parotid masses: MR imaging. Radi
ology 1 63:405-409, 1987.

281
ISALIVARY GLANDS

Case 63
Clinical Presentation
A 74-year-old male presented with a painless mass in the left parotid gland, which
had grown slowly over several years.

A B
SALIVARY GLANDSI

Radiologic Findings
On an MRI of the parotid glands the axial T l W (Fig. 63-A), coronal T1 W (Fig.
63-B), and axial TIW (Fig. 63-C) images show a large well circumscribed mass in the
left parotid gland replacing most of the superficial lobe and extending into the deep
lobe of the gland. The small areas of T l W and TIW hyperintensity may represent
hemorrhage or small cystic spaces with high protein content.

Differential Diagnosis: Multiple Discrete


Intraparotid Lesions
inflammatory lymphadenopathy (locallregional infection, sarcoidosis, Kimura's
disease)
Metastatic lymphadenopathy (lymphoma, melanoma, squamous cell carcinoma
of the external auditory canal, midface or scalp)
Multiple benign Iymphoepithelial lesions
Multiple intraparotid cysts
Multicentric Warthin's tumor
Multicentric oncocytmas
Multiple pleomorphic adenomas

Diagnosis
Oncocytoma

Discussion
Epidemiology
Oncocytoma is a rare neoplasm accounting for less than 1 % of all salivary gland tu
mors. Oxyphilic adenoma, oncocytic adenoma and oxyphilic granular cell adenoma
are synonyms used to refer to this lesion. It is a neoplasm of the elderly with a peak
incidence during the sixth and seventh decades of life. There is no gender predomi
nance.
The parotid gland is the most common site of oncocytomas in the head and neck
region. The submandibular gland and minor salivary glands are rarely involved but
when they are, they are more likely to harbor aggressive tumors. Also, oncocytic
metaplasia of minor salivary glands is common among the elderly.
Multiple or bilateral tumors are seen in 7% of cases making oncocytomas the sec
ond most common cause of multicentric parotid neoplasms following Warthin's tu
mor. The malignant counterpart of this neoplasm is even more uncommon account
ing for only 0.005% of all epithelial salivary tumors.

Pathophysiology
The hallmark of this neoplasm is the presence of large cells containing abundant
eosinophylic and granular cytoplasm called oncocytes, which is a term derived from
"onkos", the greek word for swelling. Ultrastructural and histochemical studies
show that tbe granular appearance of the cytoplasm results from mitochondrial
hyperplasia with bizarre forms and loss of typical architectural features. Oncocytes
are common in the salivary glands, but can also occur in endocrine organs (thyroid,
parathyroid and pituitary glands) and the kidney. These cells, once thought to arise
from a degenerative process are now thought to result from redifferentiation of
epithelial cells which develop an increased but unbalanced metabolism. This is due
to an acquired enzymatic defect in the mitochondrial oxydative process. As such,
oncocytes are the result of a mitochondriopathy. [n the salivary glands, oncocytes
are not exclusively seen in oncocytomas. They are also a component of other neo-
283 1
I SALIVARY GLANDS

plasms, such as Warthin's tumor and oncocytic papillary cystadenoma which is a


transitional lesion.
Histologically, it is difficult to clearly separate oncocytic hyperplasia from a true
neoplasm. This distinction is based on cytologic features which include the nuclear
cytoplasmic ratio and cell polarity.
True oncocytomas are primarily composed of oncocytes and should not contain
lymphoid tissue. Oncocytomas are usually benign, well-encapsulated neoplasms.
Malignant criteria include the presence of mitoses and cellular pleomorphism,
extracapsular extent with invasion of adjacent structures, perivascular, lymphatic or
perineural invasion, and regional or distant metastasis.

Clinical Findings
Oncocytomas typically present in an elderly patient as a painless slow growing
parotid mass. Bilateral enlargement of the parotid glands is common and indicates
multicentricity. Facial nerve dysfunction and peri parotid or cervical lymphadenopa
thy are both suggestive of malignancy.

Imaging Findings
Like other benign appearing parotid neoplasms, the imaging findings of onco
cytomas are nonspecific. On cross-sectional imaging, they appear as well
circumscribed, homogeneous solid masses that tend to be hypoechoic on US and hy
perdense to the normal parenchyma on CT. On M R I the signal characteristics are
also nonspecific showing low signal intensity on T1 and high signal intensity on
TIWI. The diagnosis should be considered when multicentric or bilateral neoplasms
are seen in the appropriate clinical setting. The presence of aggressive features (ill
defined margins, perineural spread and regional lymphadenopathy) suggest malig
nancy. These lesions are indistinguishible from other more common parotid malig
nancies.
The most specific imaging study is sialoscintigraphy using technetium 99m.
Unlike other salivary cell types, oncocytes are able to concentrate pertecnetate ions
so that tumors containing oncocytes show intense uptake and retention of activity
after stimulation. Therefore, sialoscintigraphy allows differentiation of Warthin's
tumor and oncocytoma from other benign and malignant salivary gland tumors. I t
may also localize regional and distant metastasis when a malignant oncocytoma is
present. Other salivary gland conditions, particularly infection and inflammatory
processes, also show increased radionuclide uptake either due to hyperemia or
retention of the radiotracer in cystic spaces. However, in these circumstances the
uptake is usually diffuse and the clinical history is revealing. US and M R I may also
be used to guide fine needle aspirate of indeterminate lesions.

Management
Benign oncocytomas have a good prognosis and recurrence after complete removal
is unusual. Some experts disagree regarding the necessity of resecting asymptomatic
lesions in elderly patients, due to their very slow growth and the rarity of malignant
degeneration. Malignant oncocytomas require wide resection and radical neck
dissection when regional lymph node metastases are present. The role of adjuvant
radiotherapy in the treatment of this rare tumor is undetermined. Prognosis is sim
ilar to that of other epithelial parotid malignancies.

Suggested Readings
Davy, c.L., Dardick, I.. Hammond, E., Thomas, M.l. Relationship of clear cell onco
cytoma to mitochondrial-rich (typical) oncocytomas of parotid salivary gland. An
SALIVARY G LANDSI

ultrastructural study. Oral Surgery, Oral Medicine, and Oral Pathology 77(5):469-79,
PEARLS/PITFALLS 1 994 May.
Oncocytomas are painless slow Kandiloros, D., Segas, 1., Papadimitriou, K . , Koutsomanis, P.. Adamopoulos, G. Ma
growing parotid mass(es) in an lignant oncocytoma of the parotid with oncocytic change of the contralateral gland.
elderly patient. American loumal of Otolaryngology 1 6(3):200-4, 1 995 May-1un.
Laforga, 1.B., Aranda, F.I . Oncocytic carcinoma of parotid gland: fine-needle aspira
The most specific imaging study
tion and histologic findings. Diagnostic Cyropathology 1 1 (4):376-9, 1 994 Dec.
is sialoscintigraphy with tech
netium pertecnetate. Mahnke, CG., Hinig, u., Werner, 1.A. Metastasizing malignant oncocytoma of the
submandibular gland. loumal of Laryngology and Otology 1 1 2( 1 ): 1 06-9, 1 998 1an.
Oncocytomas are the second Roden, D.M., Levy, F.E. Oncocytoma of the parotid gland presenting with nerve
most common multicentric pri paralysis. Otolaryngology and Head and Neck Surgery 1 1 0(6):587-90, 1 994 1un.
mary tumor of the parotid gland.
San Pedro, E.C, Lorberboym, M., Machac, 1., Som, P, Shugar, 1. Imaging of multiple
Do not mistake multiple tumors bilateral parotid gland oncocytomas. Clinical Nuclear Medicine 20(6):51 5-8, 1 995
for intra parotid lymphadenop Jun.
athy. Shintaku, M., Honda, T. Identification of oncocytic lesions of salivary glands by anti
mitochondrial immunohistochemistry. Histopathology 3 1 (5):408- 1 1 , 1 997 Nov.
Do not mistake oncocytoma for
malignancy.

285
ISALIVARY GLA DS

Case 64
Clinical Presentation
A previously healthy 50-year-old female developed gradual right facial nerve weak
nes .

A IIIII B

D c
1286
SALIVARY G LANDSI

Radiologic Findings
CT scans through the right temporal bone show fusiform widening of the descend
ing portion of the facial nerve canal ( Figs. 64-A and 64-8). Axial MR images
through the parotid gland on that side show an ill defined parotid mass involving
both the superficial and deep lobe of the gland ( Figs. 64-C and 64-D).

Differential D iagnosis: Facial Nerve


Widening and Parotid Mass
Parotid mass with seventh nerve invasion
Adenoid cystic carcinoma
Squamous carcinoma
Mucoepidermoid carcinoma

Diagnosis
Adenoid cystic carcinoma of the parotid gland with perineural extension along the
facial nerve into the temporal bone

Discussion
Epidemiology
Adenoid cystic carcinoma is a malignant salivary gland tumor that may occur in
patients of any age, although it has a peak incidence in the sixth decade. There is a
slight female predominance, and it is rare in patients under 20 years of age. Perineural
invasion is a defining feature of this tumor. Thus, the short-term (5 year) survival fig
ures of 70% are misleading. Unlike most other malignancies, recurrences at 1 0 and 1 5
years postoperative are common with this tumor and the actual cure rate is low.
Perineural extension is a pathway in which the malignant tumor spreads along
perineural or endoneural spaces. In this method, tumors can gain access to deep
regions that may not be contiguous with the location of the primary tumor. Recog
nition of this form of extension may alter the form of treatment and prognosis.
Many forms of head and neck neoplasms may spread via neural pathways, but a few
cell types have a greater propensity than others. Adenoid cystic carcinoma, although
uncommon, has the highest association with this type of spread.

Pathophysiology
Adenoid cystic carcinoma is a malignant salivary gland tumor composed of cuboidal
cells with a predilection for invading perinew'al lymphatic spaces. Because of its
microscopic appearance resulting in cross-sections of tubular cylinders, it was previ
ously referred to as a "cylindroma." The majority arise from minor salivary glands
but can also occur in parotid and submandibular glands.

Clinical Findings
The tumor presents as a superficial parotid mass, and despite its malignant nature,
its growth rate is quite slow. I t may initially be mobile, but with time becomes in
durated and fixed to surrounding tissues. Because of its propensity for perineural
spread, facial nerve involvement is not uncommon.

Imaging Findings
The imaging findings of this tumor may be nonspecific and are similar to other
parotid neoplasms. Ill-defined margins suggest a more aggressive lesion. Signs of
perineural extension should suggest this histology.
I SALIVARY G LA N DS

Fine needle aspiration cytology is well accepted for the evaluation of head and
PEARLS/PITFALLS neck masses. Specific diagnoses are routinely obtained in greater than 90% of sam
Perineural extension is the hall pled lymph nodes, salivary glands, thyroid, and other soft tissue masses. This proce
mark of this tumor. dure has limited complications, is relatively inexpensive, and, if positive, allows the
surgeon to proceed immediately with appropriate therapy. In deep lesions the use
There is good short term progno of CT and M R I guided aspiration biopsies has become a valuable tool in the evalu
sis for patients with adenoid cys ation of these masses.
tic carcinoma. Typical imaging features of perineural spread include smooth thickening of the
nerve by an isointense mass, obliteration of adjacent fat, and concentric enlarge
Slow growth with recurrences at ment of the nerve. Atrophy of muscle groups supplied by the involved cranial nerve
1 0- 1 5 years post diagnosis is not is indirect evidence of neural involvement, but may be the sole imaging finding.
uncommon.

FNA cytology is well accepted Manageme nt


for evaluation of head and neck
Parotidectomy is the treatment of choice, with examination of frozen sections for
masses.
evidence of neural involvement at the time of surgery. Distant spread to lungs or
Avoid failing to identify per bone is more common than nodal disease. Cervical metastases are uncommon at the
ineural extensions. time of initial presentation, however if present. a neck dissection should be per
formed. The most problematic aspect of the management of adenoid cystic carci
Do not mistake an adenoid cystic noma is its persistence and tendency to recur locally. Because of radiosensitivity and
carcinoma for a benign tumor. problems with local control, postoperative radiation is given in many cases.

Suggested Readings
Huang, M., Ma, D., Sun, K., Yu, G., Guo, c., Gao, F. Factors influencing survival rate
in adenoid cystic carcinoma of the salivary glands. Inlernalional Jol.lrnai of Oral and
Maxillofacial Surgery 26 6:435-9. 1 997 Dec.
Spiro, R.H. Distant metastasis in adenoid cystic carcinoma of salivary origin. Amer
ican Journal of Surgery 1 74 5:495-8, 1 997 Nov.

1 288
SALIVARY GLANDSI

Case 65
Clinical Presentation
A 37-year-old female presented with a left-sided facial mass.

A B

289 1
I SALIVARY GLANDS

Radiologic Findings
A 2 em mass in the left parotid gland can be seen arising in the superficial lobe and
extending for a moderate distance into the deep lobe (Fig. 65-A). The lesion is het
erogeneous on TZ-weighted images (Fig. 65-8). The sagittal image shows the lesion
displacing the adjacent parotid tissue superiorly. The stylomastoid foramen is clear.
Small lymph nodes are visible on this section (Fig. 65-C). (Figures courtesy Dr. Joel
Swartz).

Differential D iagnosis: Solitary Parotid Mass


Pleomorphic adenoma
Lymph node
Warthin's tumor
Oncocytoma
Mucoepidermoid carcinoma
Adenoid cystic carcinoma

D iagnosis
Mucoepidermoid carcinoma

Discussion
Most lesions of the parotid gland arise in the superficial lobe. The most common be
nign lesions are the pleomorphic adenoma (benign mixed-cell tumor) and papillary
cystadenoma (Warthin's tumor). The most common malignant tumor of the parotid
gland and second most common in the submandibular gland is the mucoepidermoid
carcinoma.

Pathophysiology
Mucoepidermoid carcinomas have 3 predominant cell types, which are mucous. epi
dermoid, and intermediate. The behavior of the tumor varies greatly depending on
its grade and based on its histopathological features. TIle low grade lesions havc a
preponderance of mucous cells and cystic areas. Although they are unencapsulated
or only partially encapsulated, they are generally well circumscribed. TIley have lim
ited metastatic potential.
The high grade lesions have a high proportion of stratified squamous epilithelial
(epidermoid) cells and may be confused with squamous cell carcinoma of the
parotid gland. TIley are aggressive. poorly circumscribed lesions that metastasize to
lymph nodes, bone. and lung and have a propensity for perineural spread. The
intermediate grade tumors fall between these two extremes.

Clinical Findings
They typically appear as a focal, well defined. often movable mass in the superficial
portion of the parotid gland. High grade lesions tend to be more indurated and
fixed. Facial nerve weakness is uncommon, but when present. indicates a higher
grade lesion.

Imaging Findings
TIle low grade tumors are generally well-circumscribed. Higher grade lesions may
be well circumscribed or may be more indistinct with infiltrative margins. In a recent
report an attempt was made to histopathologically distinguish various parotid le-

1 290
SALIVARY GLANDSI

sions based on their M R I signal characteristics. I t was found that, compared to nor
PEARLS/P ITFALLS mal parotid gland tissue, the majority of pleomorphic adenomas were homoge
The behavior of the tumor is neously iso- to hyperintense on T l -weighted images and became homogeneously
based on histologic grade of the bright on T2-weighted images. Warthin's tumors and the malignancies were much
mucous and epidermoid cells. more heterogeneous with the latter associated with cervical lymphadenopathy.
There are many tumors that do not show any reliable growth pattern. Therefore,
Low grade lesions are well cir aspiration cytology is required for all parotid lesions. Whenever malignancy is a pos
cumscribed. sibility, the course of the facial nerve should be included in the study to avoid miss
ing perineural spread of the tumor.
High grade lesions are iU defined
with metastases.
Management
Whenever malignancy is a possi
Surgery is the treatment of choice for mucoepidermoid carcinoma, employing wide
bility, the course of the facial
local excision. Neck dissections are generally not performed for low or intermedi
nerve should be included in the
ate grade tumors unless cervical metastases are palpated. For high grade tumors.
study to avoid missing perineural
some experts advocate elective neck dissection as well as post operative radiother
spread.
apy. The 5-year survival rate for low grade lesions is over 90% in most studies. For
Take care not to confuse these high grade tumors, this drops to 50-70% .
tumors with squamous carci
noma histologically.
Suggested Readings
Curtin, H .D. Assessment of salivary gland pathology. Otolaryngolgic Clinics of
North America 2 1 :547-573, 1 988.
Goode, R.K., Auclair, P.L., E llis, G.L. Mucoepidermoid carcinoma of the major sali
vary glands: clinical and histopathologic analysis of 234 cases with evaluation of
grading criteria. Cancer 82(7): 12 1 7-24, 1 998 Apr J .
Swartz, J.D., Rothman, M.L, Marlowe, F. l . M R imaging of parotid mass lesions: at
tempts at histopathologic differentiation. Journal of Compwer Assisted Tomogra
phy 1 3:789-796, 1 989.
Teresi, L.M., Lu[kin, R.B., Wortham, D.G., Harafee W. Parotid masses: M R imaging.
Radiology 1 63:405-409, 1987.

29 1 1
VIII.

Sinonasal
SINONASAd

Case 66
Clinical Presentation
A 9-month-old infant came in with a nasal mass that has been present since birth.

Radiologic Findings
CT axial views (soft tissue window, Figs. 66-A and 66-B) show a discrete benign ap
pearing mass with fat density (-43.5 H U ) in the nasal ridge. Note the absence of
bony changes.

Differential Diagnosis: Nasal Ridge Mass on


a Child
Congenitalldevelopmental lesions: dermal sinus, nasal dermoid/epidermoid, nasal
glioma, nasal encephalocele
Inflammatory/infectious: cellulitis/Phlegmon or abscess, Pott's puffy tumor, in-
flammatory polyp, congenital syphilis
Trauma to the nasal bones
Fibrous displasia involving the nasal bones and forehead
Benign and malignant neoplasms: angioma, neurofibroma, teratoma, eosinophilic
granuloma, rhabdomyosarcoma, olfactory neuroblastoma, lymphoma, metastases

Diagnosis
Dermoid cyst of the nasal ridge

D iscussion
Dermoid cysts of the nasal ridge are part of a spectrum of embryological defects re
lated to the closure of the anterior neuropore. These include sinus tracts, dermoid
and epidermoid cysts, nasal gliomas and cephaloceles.
ISINONASAL

Brain

(Tissue or
Brain Porenchyma)
(Intranasal
Encephalocele)
Pre-Nasal
Space
Dura Nasal Bones
Dermoid Cyst

(Adapted from English OM (cd):


Otolarynyology, New York,
c Harper and Row, Revised Edition, 1979)

D E

Epidemiology
Congenital abnormalities involving the anterior neuropore are most often detected
in the pediatric age group, although they can go unrecognized until adulthood.
Most diagnoses are made before age 5 years (85% ) and some series report a slight
male predominance. A familial tendency has also been consistently reported in the
literature.

Pathophysiology
The mechanism in the origin of these congenital defects is related to closure of the
anterior neuropore. It includes entrapment of ectodermal tissue in the prenasal or
the prefrontal space (fronticulus frontalis) and failure of mesodermic ingrowth with
subsequent bony defects in the nasofrontal, ethmoidal or sphenoidal regions. This
leads to herniation of meningeal and/or neural tissue. Figure 66-C is a schematic
representation of the developmental abnormalities in the closure of the anterior
neuropore. Compare the dermoid cyst and intranasal encephalocele.
S INONASAU

Clinical Findings
Clinical presentation is variable and includes detection of a mass, dimple or punc
tum, recurrent inflammatory process in the fronto-nasal region, discharge of pus or
cheesy material through a sinus tract, or even recurrent bouts of meningitis.

Imaging Findings
I maging has a very important role in the diagnosis and management of these lesions.
CT and M R I are often able to distinguish between dermoids, nasal gliomas and
cephaloceles because these lesions usually have different densities and signal char
acteristics. They also detect the full extent of the lesion either by direct or indirect
findings. Direct signs of intracranial extension include visualization of a soft tissue
tract containing neural tissue, dura or fibrous tissue. The presence of this tract is not
always depicted by imaging so that it cannot always be definitely excluded. I ndirect
signs consist of bony anomalies such as enlargement of the foramen caecum, broad
ened nasal bones, "biphid" crista galli process and other frontal bone defects.
Dermal sinuses are sinus tracts lined by epithelium that can appear isolated or
associated with dermoids or epidermoid cysts. They generally open into the nasal
region. Their extent varies from a short blind tip, to a fistulous tract that can extend
all the way into the intracranial compartment. They are usually apparent clinically,
but imaging studies can be helpful i n delineating their extention and ruling out
associated lesions.
Dermoids and epidermoids are benign appearing cystic lesions. Epidermoids con
tain only stratified keratinizing epithelium. Dermoids also have skin appendages
and secrete sebaceous material containing keratin and cholesterol. M R I can usually
distinguish between these lesions. The former is hypointense on Tl W and hyperin
tense on TIW images, the latter is hyperintense on Tl W and lower signal on TIW
images. On CT the presence of fat density makes dermoid cyst the most likely diag
nosis.
These lesions can be connected to the i ntracranial compartment by a cord of
fibrous tissue through the foramen caecum, but if this stalk is free of epithelial
lining, it can be left in place without risk of recurrence.
The most frequent complication is infection. I n fection is more common in epi
dermoids than in dermoids. When infection occurs the mass will have the same clin
ical and imaging characteristics as cellulitis or abscess, and the correct diagnosis is
possible only if these entities are considered.
The term glioma is a misnomer because this lesion does not represent a true neo
plasm. It is, rather, an embryologic remnant of glial tissue occasionally connected to
the intracranial compartment through a fibrous stalk.
As opposed to encephaloceles this lesion does not communicate with the sub
arachnoid space. Other distinguishing features are an absence of pulsations, no
Ftirstenburg sign (enlargement of the mass with compression of the internal jugular
vein) and no increase in size with Valsalva. CT and M R I are both nonspecific and
show a mass with features identical to gray matter.
Encephaloceles consist of herniation of the meninges or meninges plus brain tis
sue through a bony defect. Those that contain only meninges are called meningo
celes. Those containing both meninges and brain tissue are called encephalome
ningoceles. By definition these lesions communicate with the subarachnoid space,
contain CSF, and are better imaged with M R I . CT is helpful in defining the under
lying bony defect(s). Imaging has an important role in the detection of basal en
cephaloceles that are often not clinically apparent. MRI Tl W images of another
patient diagnosed with nasoethmoidal encephalocele (Fig. 66-D, coronal view,
Fig. 66-E, saggital view) show herniation of brain tissue through the cribiform plate
protuding into the right nare. ( I mages courtesy Wendy Smoker, MD.)
The most common anterior encephaloceles are the nasofrontal and nasoeth
moidal forms. Anterior encephaloceles, depending on their size, are often accompa-
I S I NONASAL

nied by physical stigmata such as enlargement of the nasal dorsum, hypertelorism


PEARLS/PITFALLS or mass overlying the nasofrontal region or protruding into the nasal cavity.
A dermoid cyst of the nasal ridge
presen ts as a well defined mass. M anagement
On cr the presence of fat den When a nasal ridge mass is detected clinically, a congenital cause should be consid
sity makes a dermoid cyst the ered, particularly if the lesion has been present since birth. Imaging studies, either
most ukely diagnosis. CT or MRI, are essential before any procedures such as biopsies or exploration of
sinus tracts are attempted. Imaging studies allow for the determination of the full
Indirect signs of a dermoid cyst extent of the lesion and can demonstrate intracranial communication when present.
are associated bony abnormali This information permits safe and appropriate surgical treatment.
ties. When promptly and correctly diagnosed dermoid cysts are managed surgically
with good cosmetic results when no previous complications as prolonged infection
This condition is most common
and inflammation or presence of complex sinus tracts are present. One important
among the pediatric group (often
surgical point is to remove all the epithelial lining tissue and skin appendages in
present since birth).
order to avoid recurrence. The main goals of therapy are cosmesis and prevention
Never biopsy a nasofrontal mass of complications.
before imaging studies have been
performed and intracranial com
munication has been excluded. Suggested Readings
Albery, S.M . , Chaljub, G., Cho, N.L. et al. MR imaging of nasal masses. Radiograph
ies 15(6) : 1 3 1 1-27, 1 995 Nov.
Cauchois, R., Laccourreye, 0., B remond, D. et al. Nasal dermal sinus cyst. Annals of
Otology, Rhynology and Laryngology 1 03(8 pt 1 ):615-8, 1 994 Aug.
Fitzpatrick, E., Miller, R . H . Congenital midline masses: dermoids, gliomas and en
cephaloceles fou rnal of the Louisiana StaTe Medical Society 148(3):93-6, 1 996 Mar.
.

Hladky, J.P., Le Jeune, J.P , Pertuson, B. et al. Nasofrontal dermoid fistulae and cysts:
19 cases. Neuro-Chirurgie 4 1 (5)337-42, 1 995.
McGregor, F.B., Geddes, N.K. Nasal dermoids: The significance of a midline puctum.
A rchives of Disease in Childhood 68(3):41 8-9, 1993 Mar.
Nocini, P.F., Barbaglio, A., Dolci, M., Salgarelli, A. Dermoid cyst of the nose: A case
report and review of the literature. Journal of Oral and Maxillo-facial Surgery 54(3):
54(3):357-62, 1 996 Mar.
Posnick, J.e., Bortoluzzi, P, Amstrong, D.e., Drake, J.M . I n tracranial nasal dermoid
sinus cysts: computed tomographic scan findings and surgical results. Plastic and Re
constructive Surgery 93(4):745-54, discussion 755-6, 1 994 Apr.
Reilly, J.R., Koopman, e.F., Cotton, R. Nasal mass in a pediatric patient. (clinical
conference). Head and Neck 14(5):4 1 5-8, 1 992 Sep-Oct.
Sweet, R.M. Lesions of the nasal radix in pediatric patients: Diagnosis and manage
ment. Southern Medical Journal 85(2 ) : 1 64-9, 1 992 Feb.
SINONASAL!

C ase 67
Clinical Presentation
A 65-year-old female presented with a slow-growing palatal mass and recent onset
of facial deformity, and discomfort on chewing. On clinjcal exam a firm, non-tender,
expansile, palatal mass was noted with no overlying mucosal lesions.

A B
I SI NONASAL

Radiologic Findings
A cr of the paranasal sinuses (Fig. 67-A, axial section (bone window), Fig. 67-8. ax
ial section (soft tissue window), and Fig. 67-C, coronal section (bone window show
a 2.8 X 2 cm midline, well-circumscribed, expansile lesion in the anterior aspect of
the hard palate. The lesion is surrounded by a thin rim of cortical bone, except on its
most anterior and inferior aspects, where cortical disruption is noted. The lesion ex
erts mass effect 011 the collumela, which is displaced anteriorly. The maxilla is eden
tulous and the bone of the maxillary alveolar ridge is thinned and expanded inferi
orly by this mass. There is no associated soft tissue mass, and no calcification or bony
matrix is seen.

Differential Diagnosis: Cystic Lesion


in the Maxilla
Odontogenic cyst: radicular/periapical cyst, residual cyst, primordial/dentigerous
cyst, odontogenic keratocyst, cystic ameloblastoma
Fissural cyst: midline (nasopalatine duct cyst or incisive canal cyst; median palatal
cyst), lateral (globullo-maxillary cyst; nasolabial or nasoalveolar cyst )
Bone cysts: simple, unicystic, hemorrhagic cyst; aneurysmal bone cyst
Antral lesions extending into the maxillary alveolar ridge: mucocele

D iagnosis
Nasopalatine duct or incisive canal cyst

Discussion
Epidemiology
The nasopalatine duct cyst was first reported in 1 9 1 4, by Meyer. An incidence of 1 %
was estimated in the general population, making this the most common nonodon
togenic cystic lesion of the oral region. This cyst can occur at any age, but usually
presents clinically between the fourth and sixth decades of life. A slight male pre
dominance and predilection for caucasians have been noted.

Pathophysiology
Nasopalatine duct cysts are developmental in origin, arising from epithelial rem
nants of the incisive canal. Some experts have postulated that the proliferation of
epithelial remnants is triggered by irritants such as trauma, infection, mucous re
tention or spontaneous cystic degeneration.
The nasopalatine duct cyst belongs to the category of fissural cysts. These are de
fined as cysts that are lined by squamous or respiratory epithelium entrapped along
the fissure lines of the facial bones, during embryologic development. Fissural cysts
are named after their anatomic location and are all histologically similar.
Embryologically, the nasopalatine duct results from fusion of the paired palatine
bones (primary palate) with the palatine processes of the maxilla (secondary
palate). This fusion line is " Y " shaped, the midpoint of the . 'Y" representing the in
cisive foramen and the two arms representing the two separate incisive canals, open
ing in either side of the base of the nasal septum.

Clinical Findings
Incisive canal cysts may be asymptomatic and often present as an incidental imag
ing finding. The most common clinical findings include palatal swelling, pain and, oc
casionally, discharge of mucoid material, with a salty taste.
SINONASAd

On clinical exam, a submucosal bulge is noted in the midline at the level of the in
cisive foramen and displacement of the superior incisors may be apparent with large
lesions.
When infected, pain and a purulent discharge ensue. Electric pulp testing may be
used to evaluate sensitivity of the incisors and may differentiate developmental
cysts from periapical or radicular cysts, the latter associated with reduced sensi
tivity.

Imaging Findings
Small nasopalatine duct cysts are found incidentally on dental radiographic surveys
or on cr studies done for unrelated reasons. They present as a round, ovoid or
heart-shaped radiolucency located at or near the midline. The lesion is well-defined,
with a peripheral rim of cortical bone. Usually, these cysts extend inferiorly into the
alveolar ridge, displacing the incisor roots. They usually do not extend toward the
maxillary sinus, although large cysts may cause superior bulging of the inferior wall
of the antrum. cr, using both axial and coronal planes, is a valuable tool in separat
ing intra- from extra-antral lesions. The demonstration of a bony septum between
the cystic lesion and the maxillary antra, classifies a lesion as extra-antral, except in
the rare case where a mucocele develops in the inferior compartment of a septated
maxillary sinus. Coronal CT of the paranasal sinuses of another patient (Fig. 67-D)
shows an expansile cystic lesion involving the left lateral aspect of the maxillary
alveolar ridge and left maxillary sinus. The superior aspect of this lesion is covered
by a convex cortical margin, which may correspond to the inferior wall of the max
illary sinus displaced superiorly or an expanded maxillary septum. There is marked
thinning of the cortical margin of the alveolar ridge. This patient was diagnosed with
mucocele within a septated maxillary sinus.
New generation panoramic machines, which are capable of tomographic cross
sectional views, may yield the same information as a CT scan with the advantage of
less radiation exposure. M R I is nonspecific and less accurate in the evaluation of
cortical bone. Small nasopalatine duct cysts may be impossible to differentiate from
an enlarged incisive fossa.
lS I 0 ASAL

Differential diagnosis of an incisor canal cyst from other odontogenic and non
PEARLSIPITFALLS odontogenic cystic lesions in the maxilla is based on the typical location of this le
This fissural cyst is a well sion. Absence of abnormal widening of the periodontal ligament in the adjacent in
defined, midline cystic lesion cisors excludes the diagnosis of a periapical or radicular cyst. I n addition.
centered in the nasopallatine or nasopalatine duct cysts tend to be located posterior to the roots of the central in
incisor canal. cisors and displace them forward. In edentulous patients, such as the case presented
here, it may be impossible to differentiate this lesion from a residual cyst.
With an i ncisive canal cyst there The globullomaxillary cyst, another fissural cyst, is located off midline, between
is an absence of periodontal dis the lateral incisor and the canine teeth along the suture line between the lateral
ease (no evidence of enlarge nasal and maxillary processes.
ment of the periodontal space). A soLitary or simple bone cyst is another diagnostic consideration. Although the
most common location of this lesion is the mandible, they may occur in the maxil
Avoid confusing tbis develop lary incisor area and mimic a nasopallatine duct cyst. Distinguishing features in
mental cyst with odontogenic le clude the larger dimensions of the solitary bone cyst when first noticed, and the typ
sions. ical scalloped margins near the alveolar ridge, where it burrows between tbe roots
of the adjacent teeth.
Other odontogenic lesions, such as primordial and dentigerous cysts, are easily
distinguisbed due to their particular relationship to teeth. Odontogenic keratocyst
and cystic ameloblastoma tend to be multilocular and have a more aggressive imag
ing appearance and clinical behavior. Unlike nasopalatine duct cysts, these lesions.
when located in the maxilla, tend to grow superiorly toward t he maxillary antrum.

Management
Nasopallatine duct cysts are benign lesions tbat only require therapy when sympto
matic. Surgery is the treatment of choice and consists of enucleation of the cyst
through a palatal approach. The resulting bony defect heals by new bone formation,
usually with no need for reconstructive intervention.

Suggested Readings
Daley, T.E.D., Wysocki, G.P. New developments in selected cysts of the jaws. Journal
of the Canadian Dental Association. Journal de L Association De/1Iaire Canadienne
63(7):526-7, 530-2, 1 997, lui-Aug.
Han, M.H., Chang, K.H., Lee, e.H., Na, D.G., Yeon, K.M., Han, M.e. Cy tic expan
sile masses of the maxilla: differential diagnosis with CT and M R I . American Jour
nal of Neuroradiology 1 6(2):333-8, 1 995 Feb.
Harris, I . R. , Brown, J.E. Application of cross-sectional imaging in the differential di
agnosis of apical radiolucency. International Endodontic JoumaI 30(4):288-90, 1997
Jul.
SINONASAd

Case 68
Clinical Presentation
A 52 year-old male presented with headaches and nasal congestion.

A B

Radiologic Findings
Coronal CT scans of the paranasal sinuses ( PNS) (Fig. 68-A, bone window and Fig.
68-B, soft tissue window) show a 2 X 1 .2 em pedunculated bony lesion originating
from the left lateral aspect of the interfrontal septum. The density of this lesion is
similar to that of cortical bone, except for a more hypodense region in the center of
the mass. The walls of the frontal sinus are intact. There is no periosteal reaction or
bony erosion.

Differential Diagnosis: Radiopaque


Lesion in the Paranasal Sinus
Focal and well-defined: osteoma, ossifying fibroma, osteochondroma, osteoid os
teoma/osteoblastoma, radiopaque foreign body, sinolith (calcified secretions)
Generalized and ill-defined: fibrous dysplasia, Paget's disease, osteosarcoma

Diagnosis
Frontal sinus osteoma
I SJNONASAL

Discussion
Epidemiology
Osseous and fibroosseous lesions comprise 25% of nonepithelial tumors involving
the paranasal sinuses. The true incidence of sinonasal osteomas is not known be
cause the majority of these tumors are asymptomatic and remain undetected. Inci
dences varying from 0.43% and 3% have been reported in several series reviewing
imaging studies of the paranasal sinuses. Most series report a male predominance of
about 2 : 1 . The age at presentation is broad, usually between the second and fifth
decades of life. TIle sinus most commomly involved is the frontal (75 % ), followed,
in decreasing order of frequency, by the ethmoids and maxillary sinus. Osteomas of
the sphenoid sinus are exceedingly rare.

Pathophysiology
The etiology of osteomas is unknown. 111re e theories have been proposed to explain
their origin: developmental, traumatic and infectious. The embryological theory pos
tulates that osteomas form at junctions of developing membranous and endochon
dral bone, whjch would account for the higher incidence of this tumor in the fronto
ethmoidal region. Although 20 to 30% of patients with osteomas of the paranasal
sinuses have a history of trauma, a causative effect has never been proven. The as
sociation with infection, which is seen in 30% of cases, is controversial because it is
difficult to assess whether infection is a primary or secondary process. Due to the
high prevalence of rhinosinusitis in the general population, this association may be
coincidental. In addition, in 50% of cases, the tumor obstructs the drainage pathway
of the sinus of origin, leading to post-obstructive inflammatory changes.
Pathologically, osteomas are benign fibroosseous lesions. Depending on their os
seous and fibrous content, osteomas are classified into three groups: ( 1 ) ivory or ma
ture osteoma, composed of dense, compact bone with only small amounts of fibrous
tissue, (2) cancellous osteoma, composed of mature cancellous bone with sparse in
tertrabecullar spaces filled with fibrous tissue, fat or hematopoietic elements and
(3) fibrous or "inunature" osteoma, composed of lamellar bone interspersed through
a large amount of fibrous tissue.
I t has been observed that the amount of compact bone is proportional to the pa
tient's age and tbat it tends to progress with time. Osteomas grow slowly but inex
orably. The mean growth rate has been estimated at around 1 .6 mm per year, rang
ing from 0.44 to 6 mm per year.

Clinical Findings
Most osteomas are asympomatic and found incidentally on imaging studies per
formed for unrelated reasons. The most frequent symptoms include headaches and
sinonasal pain. However, some patients have signs and symptoms of rhinosinusitis,
or from complications secondary to sinus obstruction by the tumor. I n addition, pa
tients presenting with headaches, in whom successful resection of the tumor is
achieved, may have persistence of this symptom.
Complications are relatively infrequent and are associated either with obstruc
tion of the sinus drainage pathway or growth of a tumor beyond the boundaries of
the sinus of origin. The most common is mucocele formation due to obstruction of
the naso-frontal duct. Growth of a tumor, breaching through the posterior wall of
the frontal sinus into the anterior cranial fossa, can result in CSF leakage, aerocele
or pneumocephalus, recurrent meningitis and brain abscess.
Large tumors may present with deformation of the forehead due to anterior tu
mor growth or orbital symptoms such as proptosis, diplopia or amaurosis fugax,
when the tumor grows inferiorly or involves the ethmoidal region. Tension pneu
mocephalus is a surgical emergency, requiring prompt diagnosis and treatment.
S INONASALI

C D

I maging Findings
The best imaging method to evaluate fibroosseous lesions of the sinonasal region is
CT. Plain films detect these tumors when they are composed predominantly of com
pact, dense, lamellar bone, but may mistake fibrous osteomas for polyps or retention
cysts. Plain films also do not allow correct identification of the site of origin within
the sinus. This is important information for surgical planning. M R I may be used as
an adjunct to detect intracranial and/or intraorbital complications.
On CT, osteomas present as bone or mixed density lesions arising from the wall
of a paranasal sinus. A coronal CT section of the PNS in another patient (Fig. 68-C)
shows a small pedunculated bony lesion originating from the posterior wall of the
left frontal sinus (ivory osteoma). They may be broad-based, pedunculated or fill in
one or more sinuses. Frontal osteomas may extend to the ethmoidal region, and in
these cases it may be difficult to determine the exact site of origin. CT also demon-
I SI NONASAL

strates the location of the osteoma in regard to the sinus ostium, and postobstruc
tive changes, such as mucocele formation. Although most osteomas remain confined
to the sinus of origin, conforming to the contour of the sinus, they may continue to
enlarge and extend into adjacent structures. A coronal CT section of the paranasal
sinuses was done for a patient with frontoethmoidal fibrous osteoma. Figure 68-D
shows a large mixed density lesion centered in the right fron to-ethmoidal region, ex
tending into and deforming the medial orbital wall. TIle lesion is heterogeneous with
areas of compact cortical bone and more hypodense components with a ground
glass appearance.
Coronal CT section of the paranasal sinuses in a patient with recurrent ivory os
teoma (Fig. 68-E) shows a bony lesion in the right anterior ethmoidal region that is
causing slight deformity of the ipsilateral medial orbital wall. I ntraorbital and in
tracranial extension may be seen with large frontal and/or ethmoidal osteomas. TIle
presence of air in the intracranial compartment implies communication between the
sinus cavity and the subarachnoid space. The intraorbital contents, specifically, the
optic nerve, should be carefully evaluated for signs of compression.
I maging studies are mandatory in the follow-up of patients with asymptomatic
tumors that do not have an indication for surgery or residual tumor after incomplete
resection, to check for growth and possible obstruction of the sinus drainage path
way. On the other hand successfully treated osteomas do not recur and do not need
radiographic follow-up.
When multiple osteomas are detected on radiographs, the diagnosis of Gardner"s
syndrome should be considered, as this may be the first sign of the disease. Gard
ner's syndrome includes multiple osteomas of the facial bones, intestinal polyposis
and skin tumors.
On M R I , osteomas are heterogeneous lesions, usually low to intermediate signal
intensity on all sequences. " Ivory" osteomas may present as signal void and be mis
taken for a normally aerated sinus. Enhanced MRI is the best method to detect in
tracranial and intraorbital complications. It also may clearly delineate the tumor
from any associated postobstructive changes, which are bright on T2W images. CSF
leaks from tumor growth or surgical complication may be detected on CT scans with
intrathecal contrast administration.
Differential diagnosis between fibrous osteoma, ossifying fibroma and monostotic
fibrous dysplasia may be difficult. The hallmark of ossifying fibromas is their ten
dency to behave aggressively and to calcify from the periphery to the center. Fibrous
dysplasia, even when localized to a single bone, is more ill-defined, merging imper
ceptibly with the normal surrounding bone and tends to affect primarily the maxil
lary bone. Monostotic fibrous dysplasia of the frontal bone is rare.
Another condition that mimics osteoma is a foreign body. When present for a
long period foreign bodies act as a nidus for deposition of mineral salts, resulting in
calcified masses called sinoliths. These masses, unlike osteomas, may be separated
from the sinus walls and may move freely within the sinus upon changing the pa
tient's position. Calcified secretions may also minlic osteomas. They usually result
from desiccation of secretions in patients with chronic sinusitis or from fungal in
fections.

Management
Management of osteomas is controversial. Most experts agree that asympto
matic osteomas should be left alone and closely followed radiographically to
assess for possible growth or interval appearance of obstructive changes. I ndica
tions for surgery in asymptomatic osteomas include extension beyond the sinus
boundaries, radiographic demonstration of growth and location near the [ronto
nasal duct.
The surgical technique depends on the location and size of the tumor. Most small
osteomas of the [ron tal sinus may be managed by simple trephination and endo
scopic resection. The osteoplastic approach, with or without secondary obliteration
SI NONASALI

of the frontal sinus, is the prefered method for larger tumors and those that arise
PEARLS/PITFALLS from the posterior wall of the sinus.
A sinsus osteoma presents as a
weU-defined bony lesion in the Suggested Readings
PNS (most commonly in the
frontal sinus). AI-Sebeih, K., Desrosiers, M. Bifrontal endoscopic resection of frontal sinus os
teoma. Laryngoscope 1 08(2):295-8, 1 998 Feb.
Look for the site of attachment Atallah, N., Jay, M.M. Osteomas of the paranasal sinuses. Journal of Laryngology
to the sinus wall and relationship and Otology 95(3):291-304, 1 98 1 Mar.
to the sinus ostium.
Brunori, A., de San tis, S., Bruni, P., Delitala, A., G iuffre, R., Chiappetta, F. Life
Look for post-obstructive threatening intracranial complications of frontal sinus osteomas: report of two
changes (mucocele). cases. AC/a Neurochirurgica 1 38( 1 2) : 1 426-30, 1996.
Hehar, S.S., Jones, N.S. Fronto-ethmoid osteoma: the place of surgery. Journal of
When multiple osteomas are Laryngology and Otology 1 1 1 (4):372-5, 1 997 Apr.
seen, consider the diagnosis of Holness, R.O., Attia, E. Osteoma of the frontoethmoidal sinus with secondary brain
Gardner's syndrome. abscess and intracranial mucocele: case report [letter) [see comments) Neu.rosurgery
35(4):796-7, 1 994 Oct.
The clinician should avoid mis
taking an osteoma for a sinolith Koivunen, P., Lopponen, H . , Fors, A.P., Jokinen, K. The growth rate of osteomas of
or foreign body. the paranasal sinuses. Clinical Otolaryngology 22(2):1 1 1 -4, 1 997 Apr.
Rappaport, J.M . , Attia, E.L. Pneumocephalus in frontal sinus osteoma: a case re
Do not fail to recognize obstruc port. Journal of Otolaryngology 23(6):43Q...6, 1994 Dec.
tion of the sinus ostium.

On MRI an "ivory" osteoma may


mimic a normally aerated sinus.
I SINONASAL

Case 69
Clinical Presentation
A 57-year-old female presented with frontal headaches.

A B

Radiologic Findings
Axial (Fig. 69-A) and coronal ( Fig. 69-B) CT images of tbe paranasal sinuses (P S)
show complete opacification of the right frontal sinus by a soft tissue mass which is
isodense to the brain parenchyma. There is bony remodelling and expansion (note
bowing of the posterior wall of the frontal sinus). Minimal mucosal thickening is
present in the left frontal sinus which is consistent with chronic inflammation. No
evidence of bony erosion or expansion of the mass beyond the confines of the sinus
walls is present.

Differential Diagnosis: Soft Tissue Mass


Filling Paranasal Sinus
I nflammatory sinus disease
Mucocele
eoplasm

Diagnosis
Frontal sinus mucocele

Discussion
Mucoceles of the paranasal sinuses generally have characteristic imaging features,
which make this diagnosis straightforward (see Pearls). I f a lesion meets these cri-
SINONASAd

c D
I SI NONASAL

teria other possibilities become much less likely. However, other benign lesions, ag
gressive infections or even malignant slow growing processes can, of course, not be
excluded on the basis of cr findings alone.
Mucoceles result from obstruction of the ostium of a sinus cavity leading to pro
gressive accumulation of the secretions trapped within the sinus. Retention cysts,
which result from obstruction of the duct of a single mucous gland, can be poten
tially indistinguishible from a mucocele if large enough to fill the sinus. However, re
tention cysts do not usually grow large enough to cause expansion of a sinus cavity.
Retention cysts are lined by the epithelium of the obstructed gland and are a fre
quent finding in patients referred for radiologic studies of the paranasal sinus
( 10% ).

Pathophysiology
The most common mechanism of obstruction preventing sinus drainage is chronic
inflammation resulting in thickening of the m ucoses of the paranasal ostium or from
chronic nasal polyposis. Other causes that can lead to obstruction include congeni
tal anomalies, trauma, prior sinus surgery, fibrosseous lesions, neoplasia and diseases
associated with viscid inspissated secretions such as cystic fibrosis. Mucoceles are
rare in the pediatric population. When present in this age group, cystic fibrosis
should be considered. The presence of complete bony septations within sinus cavity
can isolate a compartment within the sinus and is associated with an increased risk
of mucocele formation.
Several hypotheses bave been proposed to explain the expansile nature of this le
sion. A combination of pressure necrosis, bone devascularization and release of os
teolytic enzymes appear to be the most important factors in the pathogenesis of mu
coceles. Osteolysis of the inner table of the sinus cavity is accompanied by periosteal
new bone formation of the outer table leading to remodeling and subsequent ex
pansion. When bone destruction overcomes bone formation there is destruction of
the bony sinus wall and the mucocele is bounded only by its own mucosal linning.

Clinical Findings
Mucoceles are far more frequent in the frontal sinus (60% ) due to the small caliber,
tortuosity and long course of the nasofrontal duct. Ethmoid (30% ), maxillary ( 1 0% )
and sphenoid 1 % ) follow in order of decreasing frequency.
Symptoms depend on the location of the mucocele and result either from me
chanical effects or superinfection. Mass effects include bossing of the forehead,
nasal vault or cheek, nasal obstruction, proptosis, headaches, orbital apex syndrome
and cranial nerve palsies. When infected, mucoceles are known as mucopyoceles,
and may cause pain and systemic symptoms. Even when asymptomatic, mucoceles
must always be treated due to their aggressive behavior.
Axial (Fig. 69-C) and coronal ( Fig. 69-0) cr scans of the paranasal sinus on an
other patient show extensive soft tissue masses involving the ethmoid, frontal, sphe
noid and maxillary sinuses. The axial view shows marked expansion of the ethmoid
air cells bilaterally with demineralization and bowing of the medial wall of both or
bits. There is orbital invasion causing proptosis. I mpingement on the optic nerves is
also noted. Posteriorly, this mass extends intracranially and invades the left cav
ernous sinus. On the coronal view the intracranial extension to the left frontal re
gion and intraorbital extension are better appreciated. Note also slight expansion of
the left maxillary sinus.
Axial TIW M R images of the paranasal sinus on the same patient ( Figs. 69-E
through 69-G) show mixed signal intensities within the Ilasal vault and sinus cavities
due to the different components present in this lesion. The nasal cavity and left max
illary sinus are filled with hyperintense tissue consistent with watery mucous secre
tions (Fig. 69-E). The ethmoid air cells and frontal sinuses show a predominantly hy
pointense component with signal voids consistent with dissecated secretions. These
SI ONASALi

signal void may simulate aerated sinu es (Fig . 69-F and 69-0). M R I beller delin
eates the orbital and intracranial exten ion of this proces. The e cr and M R I pat
tern , although showing some aggres ive features that could sugge t a malignant
neoplastic condition, are quite characteri tic of a benign process. In particular, note
the integrity of the bony trabecula in the ethmoid sinus and the presence of a hy
podense rim separating the central hyperden e soft tissue rna s from the sinus walls
on the cr images and the mixed ignal intensitie een on the M R l tudy.
llle patient in Figures 69-C through 69-0 was diagnosed with chronic nasal pol
yposi with mucocele formation in the ethmoid, frontal, sphenoid and left maxillary
sinuses. (Case courle y of Dr. Hugh Curtin.)
Figure 69-H depicts an axial cr of an ethmoid mucocele in another patient.
The most frequent complications of mucoceles are orbital and intracranial inva-
ion and infection. Intraorbital inva ion with optic nerve impingement can result in
irreversible visual 10 For this reason mucoceles should always be considered in the
s.

differential diagnosis in a patient with sudden onset of visual loss. Other cranial
nerve palsies can occur especially in sphenoid sinu mucoceles extending laterally
into the cavernous sinu . Chronic dural inflammation may be re ponsible for some
cranial nerve palsies when direct mechanical compression of the cranial nerves is

311 /
I S I NONASAL

Table 69-1. M R Signal Voids Within the Paranasal Sinuses


Aerated sinus
Desiccated secretions
Calcifications
Fungal concretions (mycetoma)
Foreign body
Ectopic (undescended) tooth
Dentigerous cyst
Fresh blood

not documented. Meningitis is another possible complication when infection of the


mucocele occurs (mucopyocele).

Imaging Findings
Mucoceles can be diagnosed on plain films of the paranasal sinus but cr and M R I
are superior in defining the extension and potential complications o f this condition
and are essential for therapy planning.
cr is the ideal method to evaluate the bony changes and integrity of the bony
margins of the sinus walls. CT discloses an expansile nonenhancing mass filling a si
nus cavity. The density of this mass depends on the chronicity of the lesion and com
position of the retained secretions. I n early stages mucoceles appear bypodense and
tend to become progressively more byperdense as dessication and inspissation of
secretions occur. Ocasionally dystrophic calcifications are seen. The bony walls of
the sinus appear remodeled and bowed and may appear sclerotic, demineralized or
focally absent. When infected, a thin rim of enhancement is noted around the soft
tissue mass.
M R imaging is the best tool to depict intraorbital and intracranial extension and
is also helpful in the differential diagnosis from malignancy when contrast material
is used. The signal characteristics depend on the protein and water content of the se
cretions and several patterns can be seen. In the early stages watery mucous secre
tions show hypointensity on T1 W and hyperintensity on T2W images. Desiccated
stagnant secretions may show low signal on both TI W and T2W images. With para
magnetic contrast administration mucoceles, enhance peripherally even when not
infected but never centrally as do most neoplasms.
The presence of signal void within a sinus on both Tl W and T2W images can rep
resent a variety of conditions ranging from a normal aerated sinus to fresh blood
(Table 69- 1 ) . Care must be taken not to misdiagnose a mucocele filled with dessic
cated secretions for a normal aerated sinus (Fig. 69-F). Always look for signs of ex
pansion or perform CT in doubtful cases.
When infection is present and complicated by meningitis, dural enhancement can
also be depicted with both CT and MRT.

Management
M ucoceles are generally ways surgical lesions because they behave like slow grow
ing neoplasms and are potentially life threatening. Two different approaches can be
used: intranasal endoscopic surgery or open obliterative surgery.
I ntranasal endoscopic treatment is established as the modality of choice for eth
moid and sphenoid sinus mucoceles, especially when intracranial or intraorbital ex
tension is present. Frontal sinus mucoceles have been treated using a limited frontal
craniotomy, removal of the mucocele and sinus mucosa, followed by packing the si
nus with fat tissue ( Lynch procedure or osteoplastic flap).
S INONASALI

Postsurgical CT images of the patient presented in this case ( Fig. 69-1, coronal;
PEARLSIPITFALLS Fig. 69-J, axial) show the typical appearance of an osteoplastic flap. Bony defects are
The imaging features of a muco seen in the anterior wall of the frontal sinus, and the sinus cavity has been packed
cele in the PNS include a mass with fatty tissue. Recent work has shown some advantage to using endoscopic
made of soft tissue that fills the surgery in frontal sinus mucoceles whether or not intracranial or intraorbital exten
sinus, and sinus bony expansion. sion is present.
The main goal of therapy is to achieve decompression of the mucocele in order to
Mucoceles result from obstruc relieve the symptoms associated with mass effect. Several advantages of the endo
tion of the sinus ostium. scopic technique have been documented. This technique can be performed in an
outpatient basis, sometimes under local anesthesia. No external incisions are re
In pediatric patients consider quired, and lower rate of complications such as CSF leaks and meningitis in com
cystic fibrosis as a differential di parison with the open obliterative procedure.
agnosis. Imaging follow up is recommended in every case to check for recurrence.

Early findings can be easily


missed. Suggested Readings
Benninger, M.S., Marks, S. The endoscopic management of sphenoid and ethmoid
Bony expansion may be masked mucoceles with orbital and intranasal extension. Rhinology 33(3): 1 57-{) 1 , 1 995 Sep.
by the normal sinus wall contour.
Chua, R., Shapiro, S. A mucopyocele of the clivus: case report. Neurosurgery
39(3):589-90, discussion 590- 1 , 1996 Sep.
Delfini, R., Missori, P., lannetti, G., Ciappetta, P., Cantore, G. Mucoceles of the
paranasal sinuses with intracranial and intraorbital extension: report of 28 cases.
Neurosurgery 32(6):901-{) discussion 906, 1993 Jun.
Fligny, 1., Lamas, G., Aidan, P., Fougeront, 8., Soudant, 1. Frontal mucocele. Clinical
symptoms, treatment and results apropos of 17 cases. Acta Oto-Rhino-laryngologica
Belgica 47(4):429-34, 1 993.
Gentile, VG., Isaacson, G. Patterns of sinusitis in cystic fibrosis. Laryngoscope
1 06(8): 1 005-9, 1 996 Aug.
Har-el, G. Telescopic extracranial approach to frontal mucoceles with intracranial
extension. fournal of Otolaryngology 24(2):98- 1 0 1 , 1 995 Apr.
Kpemissi, E., Balo, K., Kpodzro, K. Sinus mucoceles. Apropos of 5 cases. Annales D
Oto-Laryngologie ef de Chirurgie Cervico-Faciale 1 1 3(3):17 9-82, 1 996.
Krishnan, G., Kumar, G. Frontoethmoid mucocele: one-year follow-up after endo
scopic frontoethmoidectomy. fournal of Otolaryngology 25( 1 ):37-40, 1 996 Feb.
Selvapandian, S., Rajshekhar, V, Chandy, MJ. Mucoceles: a neurosurgical perspec
tive. British fournal of Neurosurgery 8( 1 ):57-6 1 , 1994.
Sterling, K.M., Stollman, A., Sacher, M., Som, P.M. Ossifying fibroma of sphenoid
bone with coexistent mucocele: CT and MRT. fournal of Computer Assisted Tomog
raphy 1 7(3):492-4, 1 993 May-Jun.
ISINONASAL

Case 70
Clinical Presentation
A 37-year-old H I V positive male presented with chronic sinusitis and blurred vision.

A B

c D

Radiologic Findings
A cr axial view through the maxillary sinus (soft tissue window, Fig. 70-A) shows
nodular soft tissue thickening in the right maxillary sinus with bone erosion and dis-

1 31 4
SI NONASAd

ruption of the anterior wall of the sinus and soft tissue extension to the cheek. A cr
axial view (soft tissue window, Fig. 70-B) shows opacification of the inferior portion
of the right maxillary sinus with high density soft tissue and associated soft tissue ex
tending to the cheek and bony sclerosis of the sinus walls (compare bony thickness
on both sides). A cr coronal view (bone window) 6 months later ( Fig. 70-C) shows
thinning and erosion of the inferior orbital rim and transosseous soft tissue exten
sion into the extraconal compartment of the right orbit. cr axial view (soft tissue
window, Fig. 70-0) shows interval growth of the soft tissue component as well as en
largement of the bony wall defect in the anterior wall of the right maxillary sinus.

Differential D iagnosis: Sinus Disease with


Bone Destruction
Aggressive infection: nongranulomatous (aspergillosis, mucormycosis, candidia
sis, histoplasmosis, cryptococosis), granulomatous (actinomycosis, tuberculosis,
rhinoscleroma)
Noninfectious granulomatous processes: Wegener's granulomatosis, idiopathic
midline granuloma, sarcoidosis, cocaine nose
Neoplasm: squamous cell carcinoma, lymphoma, adenocarcinoma

Note: [n an H I V positive patient aggressive infection either bacterial (Nocardia) or


fungal (aspergillosis, mucormycosis, candidiasis, cryptococosis) are likely, as is an ag
gressive lymphoma.

Diagnosis
Fungal sinusitis (aspergillosis)

Discussion
Epidemiology
Fungal sinus infections are relatively common involving a large number of organ
isms. Predisposing factors include immunocompromise, diabetes mellitus, and pro
longed antibiotic and steroid therapy. Fungal sinus infections can also be seen in
otherwise healthy individuals, especially those with poor sinus aeration due to

E F

31 5
ISINONASAL

chronic inflammatory disease. There is no definite age or gender predilection. A ge


ography with warm climates is known to favor fungal growth.

Pathophysiology
As in other anatomic regions fungal infections of the sinus can be classified as acute
( fulminant), chronic, allergic and mycetomatous.
Fulminant fungal infection typically occurs in an immunocompromised patient
and can be life threatening when intraorbital or intracranial extension is seen
(rhinocerebral form), with potential complications including vascular invasion and
venous or arterial thrombosis. The organisms most commonly implicated are As
pergillus fumigatus and Mucor species. A cr axial view through the maxillary si-
S INONASALI

nuses (soft tissue window) in a second patient ( Figs. 70-E and 70-F) shows bilateral
PEARLSIPITFALLS opacification of the maxillary sinuses with thinning and erosion of the anterior wall
Predisposing factors include dia and disruption of the aspect of the lateral waLl of the left maxillary sinus. There is
betes mellitus, prolonged antibi associated extension into the pterygopalatine fossa and masticatory space (compare
otic or steroid therapy or an im the density of the fat on both infratemporal fossa). Note bony destruction of the
munocompromised patient. pterygoid plates on this side. TIlis patient was on chronic steroid therapy.
The chronic form usuaLly occurs in an immunocompetent host and can be indis
If the sphenoid sinus alone is in tinguishable both clinically and radiologically from bacterial infection.
volved, consider aspergiJIosis. The allergic form develops in atopic individuals and results from hypersensitivity
reactions. The mechanisms of injury are similar to those of the bronchopulmonary
Chronic characteristics include form of aspergillosis. Associated nasal polyposis and/or aspirin allergy can be pres
nodular mucosal thickening, and ent. Aspergillus fumigatus is the most frequent agent.
bony sclerosis, thinning and re The mycetoma form usually occurs within a single sinus cavity forming a fungus
modeling. ball and inciting minimal inftammatory reaction. On CT imaging, it appears as
opacification of a sinus cavity with a central area of hyperdensity corresponding to
Aggressive features include bony the fungal hyphae, surrounded by peripheral hypodensity and consisting of retained
erosion and destruction, and soft secretions. On M R imaging, hypointensity or a signal void is seen on both T l W and
tissue extension beyond the si T2W images, representing desiccated mycelia. Aspergillosis mycetoma sphenoid of
nuses. tbe sinus in a third patient is seen in Figure 70-0 tbrough 70-1. The left sphenoid
sinus sbows intermediate signal on tbe Tl W image ( Fig. 70-0) and T2W image ( Fig.
Air-ftuid levels rarely occur.
70-H). The right sphenoid sinus shows hypointensity within the sinus on any pulse
In mycetoma, calcified secretions sequence (Tl , spin density or 1'2). There is linear enbancement of the wall of the si
and fungal concretions have hy nus on the postgadolinium image (Fig. 70-1). The CT scan clearly shows tbe findings
pointensity on T1 W and T2W (Fig. 70-J). ( I mages are courtesy of Dr. Peter Som.)
M R images (possibly due to iron, The maxillary and ethmoid air cells are most commonly involved by fungal infec
manganese and calcium phos tion. This involvement is frequently unilateral, but can be bilateral when more ag
phate content of the fungal hy gressive forms are seen, as in immunocompromised patients. When the sphenoid si
phae) . nus is the only sinus involved, aspergillosis sbould be considered.

Early infection can be indistin


guishable from bacterial i nfec Imaging Findings
tion/ chronic inftammatory There are no specific imaging charateristics that allow distinction between the most
changes. frequent bacterial infections and the different fungal agents. When an aggressive,
bone destroying lesion involving only one sinus is present, malignancy cannot be ex
Desiccated sinus secretions can cluded on the basis of imaging alone. Other aggressive noninfectious granulomatous
have the same M R I appearance processes that should be considered are Wegener's granulomatosis, sarcoidosis, id
as fungal mycetoma concretions. iopathic midline destructive disease and exposure to cocaine or other toxic agents
such as chromate salts.
Aggressive features from infec
tion are indistinguishable from
neoplasm particularly when lo Management
calized to a single paranasal cav
ity in an immunocompetent pa The treatment of choice for fungal infections is wide surgical debridement and spe
tient. cific antifungal agents. Recurrence is common and the prognosis can be dismal, par
ticularly for immunocompromised patients who are not properly diagnosed and
promptly treated.

Suggested Readings
Blitzer, A., Lawson, W., Fungal infections of the nose and paranasal sinuses, Part I .
Otolaryngologic Clinics of North America 26(6): 1 007-35, 1 993 Dec.
De Shazo, R.D., Sweain. R.E. Diagnostic criteria for allergic fungal sinusites. Jour
nal ofAllergy and Clinical Immunology 96(1 ):24-35. 1995 Jul.
Johnson, CD., Brandes. W. I nvasive aspergillosis of the sphenoethmoid sinuses in an
immunocompetent host. lourn.al of the A merican Osteopathic Association 92(8):
1047-51 , 1 992 Aug.
I S INONASAL

Lawson, W., Blitzer, A. Fungal infections of the nose and paranasal sinuses, Part I I .
Qrolaryngologic Clinics of North America 26(6): 1 037--68, 1993 Dec.
Som, P.M., Curtin, H.D. Chronic inflammatory sinonasal diseases including fungal
infections-The role of imaging. Radiologic Clinics of North America 3 1 ( 1 ):33-34,
1 993 Jan.
Terk, M.R., Underwood, D.Z., Zee, C.S., Colletti, P. M . MR imaging in rhinocerebral
and intracranial mucormycosis with CT and pathologic correlation. Magnelic
Resonance Imaging 1 0 ( 1 ):8 1 -7, 1 992.
Sf ONASALI

Case 71
Clinical Presentation
A 67-year-old male presented with progressive onset of left nasal obstruction, post
nasal drip and pain in the left cheek.

A B

319
I SINONASAL

Radiologic Findings
On nonenhanced CT images of the paranasal sinuses, a soft tissue lobulated mass
can be seen centered in the lateral wall of the left nare extending to the ipsilateral
maxillary sinus (axial view, Fig. 7 1 -A ) . There is marked bony resorption of the me
dial wall of this sinus and enlargement of the nasoantral window. The mass extends
superiorly into the left ethmoidal air cells (Fig. 7 1 - 8 ) and there is slight expansion
of the bony walls, particularly of the lamina papyracea that is bowed laterally (com
pare to the right side). Mucosal thickening is present in some right sided ethmoidal
air cells. Coronal T2W M R I of the paranasal sinuses (Fig. 7 1 -C) shows a homoge
neous intermediate intensity mass obliterating the left nares and extending to tbe
maxillary and ethmoidal air cells. No signs of secondary obstructive sinusitis is seen.
There is no evidence of orbital involvement.

Differential Diagnosis: Unilateral Nasal


Cavity Mass
Polyp (nasoantral polyp)
Granulomatous process (infectious or noninfectious)
Papilloma
Squamous cell carcinoma (SCCa) of the maxillary sinus
Nasopharyngeal angiofibroma
Angiomatous polyp

Diagnosis
Inverted papilloma

Discussion
The differential diagnosis of this lesion should include nasoantral polyp, granulo
matous processes (both infectious and noninfectious) and papilloma. A lthough the
radiologic features of this case appear benign, a SCCa of the nasal cavity cannot be
excluded on the basis of imaging alone. Sinonasal polyposis is typically a bilateral
process presenting most frequently in children and young adults and can be ex
cluded in this case. Juvenile angiofibroma is not a likely differential in this age
group. Mucoceles, the most frequent expansile legion of the paranasal sinus, should
be included in the differential whenever there is bony expansion of a sinus. How
ever, in this case, such a diagnosis is not likely as the mass is centered in the nasal
cavity.

Epidemiology
Papillomas of the nasal cavity and paranasal sinuses are infrequent lesions, consti
tuting between 0.4% and 4.7% of all sinonasal tumors. Inverted papillomas occur
most commonly in males (4 : 1 ) between the fourth and sixth decades. They are lo
cally invasive, spread readily along epithelial surfaces and may be multifocal. Re
currence is a common problem, varying between 25% and 50% .
The importance of early recognition and treatment is underscored by this le
sion's potential for malignant transformation, most frequently into a squamous
cell carcinoma. The incidence of malignant degeneration or coexistence of in
vetted papilloma with squamous cell carcinoma is around 5% to 1 0% . Some series
reported numbers as high as 56% or more with long follow-up times. For this
reason, although they are benign lesions, they are managed with aggressive surgical
excision.
SINONASAd

Pathophysiology
The pathophysiology of this lesion is not known. Some experts reported an associa
tion with viral infections (notably human papilloma virus) and various environ
mental exposures. A history of atopy and chronic inflammation also have been im
plicated.

Clinical Findings
I nitial symptoms usually include unilateral nasal obstruction, postnasal drip, epi
staxis and headaches. Later symptoms relate to tumor extension into the sinus or or
bits and can include proptosis or diplopia. The lesion almost always originates in the
lateral wall of the nasal cavity and typically extends into the maxillary sinus. Un
usual presentations include septal lesions and isolated lesions inside a sinus cavity.
Bilaterality is rare.

Imaging Findings
Imaging plays an important role in determining the extent of the lesion, and in the
post therapeutic follow-up of this pathologic entity. Usually the imaging findings are
nonspecific but when correlated with the clinical history, they can suggest the diag
nosis.
Whenever papilloma is in question, CT studies should be performed with and
without intravenous contrast. On the precontrast studies these lesions are seen as
soft tissue masses originating from the lateral wall of the nasal cavity. Papillomas be
have as heterogeneously enhancing masses. Heterogeneous enhancement permits
distinction from mucoceles (nonenhancing lesions), and is useful in separating the
real bulk of the tumor from secondary obstructive sinusitis. Bony abnormalities are
not unusual especially when the papilloma becomes large. These abnormalities are
clearly depicted on CT studies and may include remodeling, thinning, bowing, ero
sion or sclerosis of the bony walls. Calcifications can be seen inside the tumor, and
generally represents rests of bony walls of the sinuses rather than dystrophic calci
fications.
Depiction of extension into the maxillary and ethmoid sinuses or into the na
sopharynx is very important for treatment planning, and determines whether the le
sion is amenable to endoscopic intranasal surgery. Regular follow up is necessary for
early detection of recurrence, and aggressive features that may suggest malignant
transformation. These features include aggressive bony destruction, extensive, and
soft tissue infiltration.
MRI is particularly useful in the evaluation of orbital and intracranial extension
and distinction of papillomas from inflammatory processes. It provides similar in
formation as CT scanning and both studies are only required in complex cases.
Papillomas show intermediate signal intensity on Tl W and T2W images and en
hance heterogeneously after administration of gadolinium. Inflammatory processes,
depending on their chronicity, can be hypo- or hyperintense on Tl W images and
hyper- or hypointense on T2W images (see discussion on mucoceles). After contrast
administration, inflammatory lesions usually show peripheral enhancement, except
for highly vascularized polyps that also can enhance centrally.

Pathology
Histopathologically, they are classified as either fungiform, inverted or cylindric. TIle
first two forms are the most commonly seen. TIlese lesions consist on hyperplasia of
the ciliated epithelium lining the nasal cavity and sinuses, also called the Schneider
ian epithelium. Other commonly used designations for sinonasal papillomas include
Schneiderian papillomas, hyperplastic papillomas and transitional cell papilloma.
The inverted papilloma type results from the ingrowth of the epithelium beneath
JSINONASAL

the seromucinous glands with formation of an endophytic mass. Figure


T I -D depicts a histologic section of inverting papilloma from another patient. Note
the characteristic inversion of the epithelium for which the neoplasm is named.

Management
Except for small lesions limited to the nasal cavity, inverted papillomas are typically
managed by open surgery. Despite their benign histology, the high incidence of ma
lignant degeneration results in aggressive surgical management.
The surgical approach, depending on the extent of the lesion, is usually a lateral
rhinectomy with en bloc resection of the lateral wall of the nasal cavity or an eth
moidectomy with medial maxillectomy. Endoscopic intranasal surgical treatment
yields better cosmetic results, but should be reserved for very focal lesions and for
the management of recurrences. Biopsies of any suspicious areas detected on imag
ing studies should be performed before endoscopic surgery in order to exclude ex
tension to those areas, or possible malignant transformation.
The role of radiotherapy in these lesions is poorly defined and should be limited
to unresectable lesions, multiple reccurences or as an adjunct following incomplete
surgery.

Suggested Readings
Deitmer. T., Wiener, e. Is there an occupational etiology of inverted papilloma of
the nose and sinuses? A cta Oto-Laryn.gologica 1 1 6(5):762-5, 1 996 Sep.
Hug, E.B., Wang, e.e., Montgomery, W.W., Goodman, M.L. Management of inverted
papilloma of the nasal cavity and paranasal sinuses: importance of radiation ther
apy. lnternational Journal of Radiacion Oncology, Biology, Physics 26( 1 ) :67-72, 1 993
Apr 30.
Lawson, W., Ho, BT., Shaari, e.M., Biller, H .F. inverted papilloma: a report of 1 1 2
cases. Laryngoscope 1 05(3 Pt 1 ):282-8, 1 995 Mar.
Michaels, L. Benign mucosal tumors of the nose and paranasal sinuses. Seminars in.
Diagnostic Pathology 1 3(2): 1 1 3-7, 1 996 May.
Michaels, L., Young, M. Histogenesis of papillomas of the paranasal sinuses. Archives
of Pathology and Laboratory Medicine vol. 1 1 9, 82 1-26, 1 995 Sept.
S INONASALI

Outzen, K.E., Grontveld, A., Jorgensen, K., Clausen, P.P. , Ladefoged, C. Inverted
PEARLSIPITFALLS papilloma: incidence and late results of surgical treatment. Rhinology 34(2) : 1 1 4-8,
Adult males are most commonly 1 996 Jun.
affected by this condition. Roobottom, C.A., Jewell, F.M., Kabala, 1. Primary and recurrent inverting papil
loma: appearances with magnetic resonance imaging. Clinical Radiology 50(7):
A unilateral nasal mass is one of 472-5, 1 995 Jul.
the primary symptoms found
Stankiewicz, lA., Girgis, S.l Endoscopic surgical treatment of nasal and paranasal
with inverted papilloma.
sinus inverted papilloma. Otolaryngology and Head and Neck Surgery 1 09(6):
An inverted papilloma may con 988-95, 1 993 Dec.
tain rests of bony trabeculae. Vrabec, D.P. The inverted Schneiderian papilloma: a 25-year study. Laryngoscope
1 04(5 Pt 1 ):582-605, 1 994 May.
A n inverted papilloma may show Woodruff, W.W., Vrabec, D.P. Inverted papilloma of the nasal vault and paranasal si
aggressive bone changes. nuses: spectrum of CT findings. A merican Journal of Roentgenology 1 62(2):41 9-23,
1 994 Feb.
Inverted papillomas have a high
malignant transformation rate.

Do not mistake for inflammatory


sinus disease.

Avoid missing aggressive regions


of tumor, which may represent
malignant transformation.
ISINONASAL

Case 72
Clinical Presentation
A 44-year-old male presented with nasal obstruction and chronic purulent rhinor
rhea.

Radiologic Findings
TIle coronal CT scan (Fig. 72-A) shows mucosal thickening of the ethmoid and max
illary sinuses bilaterally. Ln addition, there is a small nasal septal perforation.

Differential Diagnosis: Nasal Lymphoma


Cocaine abuse
Sarcoid
Syphilis
Wegener's granulomatosis
Nasal lymphoma
Squamous cell carcinoma
Trauma
SINONASALI

Diagnosis
Nasal lymphoma

Discussion
Epidemiology
I n the United States and other developed countries, since the early 1 970s, non
Hodgkin's lymphoma has had one of the largest increases in incidence of any can
cer. Between 1 973 and 1 989, there has been a 60% increase in incidence with cur
rent annual increases of 3.3%/year. Although increases in AIDS is a major factor, it
is estimated that only 1 0 to 1 5% of all new cases are A I D S related.
Nasal lymphoma is one of the rarest forms of extra nodal lymphoma in the west
ern population representing less than 0.5% of the group. This is in contrast to Asia
where sinonasal lymphoma is the second most common type of extra nodal lym
phoma, exceeded only by those in the gastrointestinal system. I nterestingly, over
90% of Asian nasal lymphomas have T-cell markers and no B-cell markers. I n all pa
tients, regardless of geographic origin, Epstein-Barr virus has been demonstrated in
all nasal T-cell lymphomas.

Pathophysiology
I n 1 939, Wegener described a "rhinogenic granulomatosis" in patients with necro
tizing granulomatous vasculitis of the upper and lower respiratory tract and kidneys.
The lungs are involved in nearly all cases.
After exclusion of the Wegener's granulomatosis patients, the remaining group of
patients with idiopathic aggressive destructive nasal lesions were known in the
1 950s and 1 960s as having "lethal midline granuloma." This was a particularly poor
choice of terms because it became clear that although the disease was almost always
midline, it was not always lethal or granulomatous.
I n the 1970s and 1 980s, patients with this disorder were subclassified into the fol
lowing 3 groups based on variations in histology and clinical behavior:

( 1 ) Idiopathic midline destructive disease, which was characterized by localized


destructive lesion of the nasal cavity with nonspecific inflammation and necro
sis;
(2) Polymorphic reticulosis or lymphomatoid granulomatosis, which involved a
pseudolymphomatous tissue reaction;
(3) Extranodal lymphoma usually nonHodgkin's type.

Finally, in the 1 990s, advances in immunocytochemical phenotyping have allowed


a great simplification of our understanding of this disease. The vast majority of pa
tients from the previous groups are now known to have lymphoma of the nasal
tract. ll1e use of the previous nomenclature to describe these sinonasal lymphomas
should be discontinued.

Clinical Findings
The median age of patients at diagnosis is 50 years. Nasal obstruction and/or puru
lent nasal discharge are frequent presenting symptoms. Epistaxis and facial swelling
are also sometimes seen. Eventually, septal perforation, sinonasal-oral fistulas, and
even "autorhinectomy" may develop.

325 1
l SI 0 ASAL

B c

I maging Findings
cr or M R I studies will, in early patient , show nonspecific inonasal finding of mu
cosal thickening uggesting chronic sinona al inflammation. Eventually, destructive
lesions appear with septal perforation and occasionally inonasal oral fistulae. An
axial cr can in another nasal lymphoma patient ( Fig. 72-8) hows more complete
destruction of the nasal eptum. The destructive nasal imaging findings may be iden
tical to tho e seen in Wegener's granulomatosi . A n axial cr in a patient with
Wegener's granulomatosis ( Fig. 72-C) shows milder soft tissue changes in the nasal
cavity. Identical findings may be presen t with nasal lymphoma. With advanced dis
ea e, complete na al destruction (autorhinectomy) may occur in either thi disease
or with Wegener's patients ( Fig. 72-D, axial, Fig. 72-E, coronal).

Management
Radiation therapy is the treatment of choice although some experts use chemother
apy alone. or in combination with radiation therapy.

326
SINONASAd

PEARLSIPITFALLS Suggested Readings


A nasal lymphoma is typified by Borisch, 8., Hennig, I . , Laeng, R.H., Waelti, E . R., Kraft, R., Laissue, 1. Association of
a destructive midline nasal le the subtype 2 of tbe Epstein-Barr virus with T-cell nonHodgkin's lymphoma of the
sion. midline granuloma type. Blood 82(3):858-64, 1 993 Aug l .
Chen, H . H . , Fong, L . , Su, 1.1., Ting, L.L., Hong, R.L., Leung, H.W., Lui, L.T. Experi
Patients with nasal lymphoma of ence of radiotherapy in lethal midline granuloma witb special emphasis on centro
ten have T-cell markers. facial T-cell lympboma: a retrospective analysis covering a 34 year period. Radio
therapy and Oncology 38(1 ): 1 -6, 1996 Jan.
Before diagnosing nasal lym
phoma, the clinician must rule Dictor, M., Cervin, A., Kalm, 0., Rambech, E. Sinonasal T-cell lymphoma in tbe dif
out Wegener's granulomatosis, as ferential diagnosis of lethal midline granuloma using in situ hybridization for
well as other causes. Epstein-Barr virus RNA. Modern Pathology 9 ( 1):7-14, 1 996 Jan.
Hartig, G., Montone, K . , Wasik, M . , Chalian, A., Hayden, R. Nasal T-cell lympboma
Another name for nasal lym and the lethal midline granuloma syndrome. Otolaryngology and Head and Neck
phomas is "lethal midline granu Surgery 1 14(4):653-6, 1 996 Apr.
loma," which is a misnomer be Ramsay, A.D., Rooney, N. Lymphomas of the head and neck. 1 : Nasofacial T-cell
cause these lesions are not lymphoma. European fournal of Cancer. Part B, Oral Oncology 298(2):99- 1 02, 1 993
always lethal or granulomatous. Apr.
No longer use these terms: lethal Sevinsky, L.D., Woscoff, A., Jaimovich, L., Terzian, A. Nasal cocaine abuse mimick
midline granuloma, idiopathic. ing midline granuloma. fournal of the American Academy of Dermatology 32(2 Pt
midline destructive disease, poly 1 ):286-7, 1 995 Feb.
morphic reticulosis, lymphoma
toid granulomatosis, or
pseudolymphoma.

Do not mistake for Wegener's or


other causes of midine nasal de
struction.
ISINONASAL

Case 73
Clinical Presentation
A 59-year-old male with a longstanding history of chronic sinusitis presented with
left facial swelling.

A B
SINONASALI

Radiologic Findings
Enhanced axial cr of the paransal sinus (PNS) (Fig. 73-A) shows an enhancing soft
tissue mass in the posterior aspect of the right maxillary sinus, destroying the pos
terior wall of this sinus and invading the right pterygopalatine fossa and masticator
space. The ipsilateral pterygoid plates are eroded. Coronal T1 W M R I (Fig. 73-B)
shows the cranio-caudal extent of the mass and medial extension into the left nasal
cavity. Inferiorly, the mass invades the right superior alveolar ridge and extends into
the oral cavity. Axial T2W M R I (Fig. 73-C) separates the neoplastic tissue from the
postobstructive inflammatory changes in the right maxillary sinus, which show
higher signal intensity.

Differential Diagnosis: Destructive Maxillary


Sinus Process
Neoplasm: squamous cell carcinoma, adenocarcinoma, minor salivary gland
neoplasms, lymphoma, melanoma, esthesioneuroblastoma, sarcomas (osteo-,
chondro-, fibrosarcomas), malignant fibrous histiocytoma, plasmocytoma, metas
tasis. rhabdomyosarcoma eosinophilic granuloma
*, *

Aggressive infection: bacterial osteomyelitis, fungal infection (aspergillosis, mu


comycosis), syphyllis, rhinoseleroma
Others: Wegener's granulomatosis, cocaine abuse, radiation osteitis, rhinotilexo
mania

Note: Those diagnoses that are more likely in the pediatric age group are indicated
by a *.

Diagnosis
Destructive maxillary sinus process

Discussion
Epidemiology
Sinonasal carcinomas are rare, comprising only 3% of all head and neck tumors.
Among these, approximately 50% are of epithelial origin. The maxillary antrum is
most commonly involved (80% of cases) followed in decreasing order of frequency
by the nasal cavity (20% ), ethmoid complex ( 10% ) and sphenoid and frontal sinuses
1 % ) . There is a male predisposition with a male-to-female ratio of 2 : 1 and a peak
incidence in the sixth decade. Ninety percent of cases are seen in patients over age
40 years. In the pediatric population, most destructive sinonasal masses are either
rhabdomyosarcomas or eosinophilic granulomas.

Pathophysiology
Several predisposing factors have been implicated in the pathogenesis of sinonasal
neoplasms. Occupational exposure to irritants such as nickel, chromium, wood fur
niture, mustard gas and radium are associated with an increased incidence of carci
nomas, mostly squamous cell and adenocarcinoma. An increased incidence of
sinonasal carcinoma and sarcoma is also seen in patients previously exposed to ra
diation and thorotrast. Fifteen to twenty five percent of patients with inverting pa
pilloma have synchronous or metachronous squamous cell carcinoma. Other less es
tablished causative factors include chronic sinusitis, sinonasal polyposis and chronic
naso-antral fistulas.
The association with airborne agents explains the increased incidence of sinonasal
carcinomas in the nasal cavity and maxillary antra, the first barrier to inhaled irritants.
IS INONASAL

D E

Because the site of origin and pattern of tumor spread have prognostic and ther
apeutic impact, several systems bave been used to classify squamous cell carcinoma
of the paranasal sinuses, most regarding antral tumors, the most prevalent location.
Axial T l W image in another patient (Fig. 73-C) shows a soft tissue mass filling the
left maxillary sinus, breaching tbe anterior wall of this sinus and infiltrating the
premaxillary soft tissues. Coronal frequency selective fat suppression imaging
(FATS AT) postcontrast T l WI in that same patient (Fig. 73-D) shows medial exten
sion of the lesion into the left nasal cavity with destruction of the middle turbinate
and medial wall of the maxillary antrum, and superior extent into the extraconal
compartment of the left orbit. There has been breaching of tbe medial and inferior
walls of the left orbit and the mass is seen to displace the inferior rectus superiorly
and laterally. The mass enhances homogeneously. This patient was diagnosed witb
squamous cell carcinoma of tbe left maxillary sinus.
In 1 933, Ohngren subdivided the maxillary antrum into a supra- and infrastruc
ture using an imaginary line joining the medial canthus with the angle of the
mandible. A lateral view (Fig. 73-E) demonstrates the division of the maxillary si
nus into a supra- and infrastructure by Ohngren's line (from the medial canthus to
the angle of tbe mandible). Neoplasms located posterosuperior to this line are ei
tber unresectable or require wide craniofacial resections, usually total maxillectomy
with orbital exenteration, and have a grave prognosis ( 1 0% 5 year survival rate). Tu
mors located anteroinferior to this line can be managed with partial or total maxil
lectomy and have a better prognosis (58% five year survival).
The tumor, node, metastasis (TNM ) classification is based in Ohngren's line. Max
illary neoplasms are classified as listed in Table 1 .
Because the lymphatic drainage of the paranasal sinuses is poor, nodal metasta
sis are rare ( 1 5 % ) and usually indicate spread of the tumor beyond the boundaries
of the maxillary sinus, most commonly into the alveolar sulcus, pterygopallatine
fossa and/or premaxillary soft tissues. The primary lymphatic drainage of the
paranasal sinuses is to the retropharyngeal lymph nodes. However. because this
lymphatic pathway is often obliterated due to repeated childhood infections, the
secondary lymph nodes, (high internal jugular chain and submandibular nodes) are
the most commonly involved.
SINONASAU

Table 1. TNM Classification for Grading Maxillary Neoplasms


T l - Tumor limited to the antral mucosa
1'2- bony erosion with no extension beyond the bone
T3- Extension into the orbit, ethmoidal region or premaxillary soft tissues
T4- Extension into the nasopharynx, sphenoid sinus, cribiform plate or
pterygopalatine fossa

Clinical Findings
Sinonasal carcinomas are detected late in the course of the disease because symp
toms (nasal stuffiness, sinonasal fullness and nasal quality of voice), are nonspecific
and frequently mimic common benign diseases such as chronic sinusitis or sinonasal
polyposis. These inflammatory/infectious processes usually arise secondary to an
underlying neoplasm, overshadowing and delaying the diagnosis. Unilateral nasal
obsruction and epistaxis in an adult patient strongly suggest the diagnosis. Unfortu
nately, most cases present with symptoms reflecting invasion of adjacent structures
such as facial swelling, diplopia, orbital pain, proptosis, decreasing vision and
headache. Spread of the tumor to the masticator space and nasolacrimal duct is not
rare and may present with trismus and epiphora, respectively.
On physical exam, the most common finding is a nasal mass. Visual field defects,
decreased visual acuity and cranial nerves deficits (usually cranial nerve V) can oc
cur. Sinonasal e ndoscopy is not reliable for evaluation of tumor extent.

I maging Findings
Cross-sectional imaging is the only reliable method to evaluate tumor extent, al
lowing more precise treatment planning. The radiologist's role is to provide accurate
tumor mapping and detect critical areas of involvement which may alter either the
treatment modality or the surgical approach. In the case of a maxillary tumor, these
critical areas include the orbits, pterygopalatine fossa, anterior and middle cranial
fossa, maxillary alveolar ridge and palate.
M R I and CT are complimentary and, together, can provide adequate mapping of
both soft tissue and bony involvement. Antral neoplasms present as poorly enhanc
ing soft tissue masses, usually extending beyond the boundaries of the sinus of ori
gin. On CT, it may be difficult to differentiate neoplasm from postobstructive in
flammatory changes on the basis of differential attenuation values. 1'2W MRI is of
great advantage because it's able to separate the intermediate signal intensity of
neoplasm from the bright signal intensity of inflammatory material. Squamous cell
carcinoma tends to be fairly homogeneous, although large tumors may contain ar
eas of necrosis or hemorrhage. Small tumors often escape both clinical and radio
logic detection. The only imaging criteria that suggest malignancy are the presence
of aggressive bony changes or enlarged lymph nodes, both signaling advanced dis
ease.
The pattern of bony involvement gives some diagnostic clues in terms of tumor
histology. Rapidly growing aggressive tumors tend to present with bony erosion, the
area of bony destruction being disproportionate relatively to the size of the soft tis
sue mass. On the other end, low growing neoplasms tend cause bony remodeling
and thinning. Sclerotic, thickened .b one usually reflects chronic inflammation. How
ever, some aggressive inflammatory/infectious conditions may present with aggres
sive bony destruction, indistinguishible from a malignant neoplasm.
The presence of calcification/ossification are usually asociated with osseous, chon
droid or fibro-osseous lesions, and are uncommon in sinonasal carcinomas. It may
be difficult to differentiate ossification/ calcification from residual bone due to de
struction.
I SINONASAL

Three dimensional reconstructions of sinonasal neoplasms may be useful in sur


PEARLSIPITFALLS gical or radiation therapy planning. Imaging follow-up is mandatory for detection of
Unilateral nasal obstruction or recurrence or residual tumor and a baseline, posttherapy scan should be obtained
unilateral epistaxis in an adult for future comparison.
patient strongly suggest destruc
tive maxillary sinus process. Management
On cross-sectional imaging some Treatment of sinonasal carcinoma depends on the stage of the disease. For most T3
aggressive inflammatory/infec and T4 tumors the treatment options are radiation therapy or wide craniofacial re
tious conditions show up as irreg sections. While there is more morbidity associated with surgery, especially in terms
ular margins and bony destruc of facial disfigurement, most studies have shown a better survival rate in patients
tion. who have undergone surgery. Tl and T2 tumors are managed surgically with total or
partial maxillectomy.
Perform cr and M R I to evalu
ate bony and soft tissue extent.
Suggested Readings
Avoid confusing a neoplasm with
postobstructive inflammatory Alvarez, I . , Suarez, e., Rodrigo, lP, Nunez, E, Caminero, M.J. Prognostic factors in
changes in the adjacent sinuses. paranasal sinus cancer. A merican Journal of Otolaryngology 16(2) : 1 09-]4, 1995
Mar-Apr.
Avoid failing to recognize the Carinci, E, Curioni, e., Padula, E., Calearo, e. Cancer of the nasal cavity and
full extent of tumor. paranasal sinuses: a new staging system. International Jou.rnal of Oral and Maxillo
facial Surgery 25( 1 ):34--9, 1 996 Feb.
Avoid considering all aggressive
Harbo, G., Grau, e., Bundgaard, T., Overgaard, M . , Elbntmd, 0., S0gaard, H., Over
bony changes as malignant neo
gaard, l Cancer of the nasal cavity and paranasal sinuses. A clinico-pathological
plasms.
study of 277 patients. Acta Oncologica 36( 1 ):45-50, 1 997.
Houston, G.D. Sinonasal undifferentiated carcinoma: report of two cases and review
of the literature. Oral Su.rgery, Oral Medicine, Oral Pathology, Oral Radiology and
Endodontics 85(2) : 1 85-8, 1 998 Feb.
Jakobsen, M.H., Larsen, S.K., Kirkegaard, l, Hansen, H .S. Cancer of the nasal cav
ity and paranasal sinuses. Prognosis and outcome of treatment. Acta Oncologica
36( 1 ):27-3 1 , 1 997.
Lesperance, M.M., Esclamado, RM. Squamous cell carcinoma arising in inverted
papilloma. Laryngoscope 1 05(2):1 78-83, 1 995 Feb.
Parsons, IT., Kimsey, Ee., Mendenhall, W.M., Million, R R , Cassisi, N.J., Stringer,
S.P. Radiation therapy for sinus malignancies. Otolaryngologic Clinics of North
A merica 28(6):1259-68, 1 995 Dec.
Som, P.M., Silvers, A . R., Catalano, P.l, Brandwein, M., Khorsandi, A.S. Adenosqua
mous carcinoma of the facial bones, skull base, and calvaria: CT and MR manifes
tations. A merican Journal of Neuroradiology 1 8( 1 ) : 1 73-5, 1997 Jan.
Wennerberg, l Pre versus postoperative radiotherapy of resectable squamous cell
carcinoma of the head and neck. Acta Oto-Laryngologica 1 15(4):465-74, 1995 luI.

1332
SINONASAU

Case 74
Clinical Presentation
A 67-year-old male presented with a 3-month history of anosmia and epistaxis.

A B

Radiologic Findings
A large, solid mass is demonstrated in the ethmoid sinus extending into the nasal si
nus and the floor of the frontal lobe (Fig. 74-A). Coronal TI WI (Fig. 74-8) show sig
nificant contrast enhancement of the mass. (Case courtesy of Dr William Yuh.)

Differential Diagnosis: Destructive


Nasal Mass
Esthesioneuroblastoma
Carcinoma
Lymphoma
Extramedullary plasmacytoma
Melanoma
Metastasis
Rhabdomyosarcoma
Neurogenic tumors
Inverted papilloma
Nasal polyps

Diagnosis
Esthesioneuroblastoma
I S INONASAL

Table 1. The Kadish System for Grading Esthesioneuroblastoma


Kadish A: tumor confined to the nasal cavity.
Kadish B: tumor confined to nasal cavity and paranasal sinuses
Kadish C: tumor extends beyond these regions.

D iscussion
Epidemio logy