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Andrew Coughlin, MD

Faculty Advisor: Tomoko Makishima, MD, PhD


The University of Texas Medical Branch
Department of Otolaryngology
Grand Rounds Presentation
September 30, 2010

History of CPA tumors


Discuss Relevant Anatomy
Epidemiology and Tumor Biology
Signs/Symptoms of CPA tumors
Brief overview of other CPA tumors
Treatment options
Present a case presentation

Represent 10% of all intracranial tumors


Vestibular Schwannomas/Acoustic Neuroma
represent 78% of these tumors (1)
Differential

Meningiomas
Epidermoids
Other Cranial Nerve Schwannomas
Dermoid Tumors ( Chordomas, Teratomas)
Arachnoid Cysts
Lipomas
Metastatic Tumors
Vascular Tumors (Hemangioma/Glomus Tumor)

Sir Charles Balance (2,3)

1894 First successful removal of Vestibular


Schwannoma
1907 Patient still alive but had CN VII paresis

Harvey Cushing

1917 Monograph described CPA syndrome


Pioneered subtotal resection through bilateral
suboccipital craniectomy

Walter Dandy (5)

Advocated debulking with capsule removal and


decreased operative mortality to 10%

William House (6-8)

Advocated Translabyrinthine approach 1960s


Introduced the microscope and dental drill to
identify the facial nerve
Introduced middle cranial fossa approach

Ipsilateral
Hearing Loss
Facial
Hypesthesia

Hydrocephalus

Respiratory
Failure and
Death

Autopsy series have shown incidence of 1.7 to


2.7% (9,10)
MRI of 10,000 patients seen for non-otologic
reasons showed 7 positive cases or 0.07% (11)
Denmark reports of 1.3 per 100,000 population
(12)

1.

They are not Neuromas

2.

They rarely are found on the acoustic portion of


the nerve

*Involvement of the acoustic portion of the nerve


often leads to erosion of the cochlea.

Arise from Schwann cells within the IAC (1)


Equal frequency between inferior and superior
divisions of the vestibular nerve.
Arise from Scarpa Ganglion instead of
Obersteiner-Redlich zone.

Scarpas ganglion has highest density of schwann


cells

NF 2 Gene found at 22q12

Sporadic Variety (95%) (13)

Tumor suppressor gene preventing schwann cell


proliferation
Hypothesized that there are two hits to the normal
NF gene

Neurofibromatosis type II

Autosomal Dominant
Inherit one abnormal and one normal gene with one
hit to the normal allele.
Blood tests available to screen family members.

1.

Neuregulin
Expressed by schwann cells to control proliferation
and survival of schwann cells

2.

Chemokines

3.

FGF, TGF-, VEGF, PDGF (14-17)

Sex Hormones (18)

Previous studies showed increased growth in


pregnancy
Recent studies have not shown growth modulation
or receptors for sex hormones

History and Physical Exam

Audiologic testing

Vestibular Testing

Imaging

Intracanalicular tumors
1.
2.
3.

CPA extension
1.

Hearing Loss
Tinnitus
Vestibular dysfunction/Vertigo
Disequilibrium/Ataxia

Brainstem Extension
1.
2.

3.

Midface Hypesthesia
Hydrocephalus (vision loss and headache)
Other Cranial Neuropathies

*SNHL>tinnitus>disequilibrium>facial hypesthesia
(13)

Present in >85% of patients (19)


5% of patients with VS have no associated
hearing loss (20)
Speech discrimination out of proportion to HL

Many notice difficulty on the telephone

Occurs in up to 20% of patients (13)


1% of patients with SSNHL have a vestibular
schwannoma (21)
48% of patients with SSNHL will have some
recovery of hearing (21)

Dont rule out VS if they recover

2nd most common presenting sign


Often precedes hearing loss
Can be present without hearing loss
Can be high pitch, hissing, or a roar
Can localize to the opposite ear
Unilateral tinnitus should be evaluated

36-50% of patients describe disequilibrium (1)


Vague, transient lightheadedness
Acute vertigo is presenting symptom in 27% of
patients but is associated with smaller tumors.

Presenting symptom in 4% of patients


Larger tumors >2cm
Maxillary division 1st
Corneal reflex is the first to go
Facial weakness is rare

If present should assume a different type of tumor.

Rare
Loss of corneal reflex
Nystagmus (toward affected side)
Diplopia

Involvement of CN VI

Blurry vision

Papilledema and optic atrophy from compression

Hitselberger Sign

Decreased sensation of EAC


Sensory VII more sensitive than Motor VII

Absent corneal reflex, nystagmus


Hypesthesia to pinprick and touch
Weakness of Temporalis/Masseter muscles
Other cranial neuropathys
Gait disturbances or difficulty with finger to
nose testing

Downsloping high frequency SNHL 65% (22)


5% of patients show no hearing loss (23)
Rollover: Speech discrimination worse than
expected and worse with increased sound
intensities

Retrocochlear losses more common than cochlear

AAO-HNS classification
Class

Pure tone average (0.5, 1, Speech discrimination


2, 3 kHz measured in dB score (%)
HL)
A
030
70100
B
3150
50100
C
>50
50100
D
Any
<50
Gardner-Robertson Classification
Class
Pure Tone/Speech
Speech Discrimination
Reception Threshold (dB Score (%)
HL)
1
030
70100
2
3150
5069
3
5190
549
4
>90
14
Word Recognition Scores
Class
Word Recognition Score (%)
I
70100
II
5069
III
150
IV
0%

**Positive test has 85% sensitivity for identifying


retrocochlear problem

Acoustic Reflex Threshold

Increased (compared with cochlear norms) or absent


if retrocochlear process

Sensitivity of 85 to 90% (25)


False Positive rate 10%
False Negative rate 18-30% for intracanalicular
tumors

Number larger than in the past

Five waveforms are produced with the most


common being a latency in wave V compared
to the normal ear of >0.2msec.
Recommended as a screening test for those
with low suspicion of vestibular schwannoma.

70-90% of patients will show some abnormality


(26)
50% of small tumors produce no abnormalities
(27)
Caloric testing is commonly the only
abnormality
Inferior vestibular nerve tumors may be missed

Superior nerve showed decrease in 98%


Inferior nerve shows decrease in only 60% (28)
Therefore not used as a screening test

90% of vestibular schwannomas will enhance


with contrast
Frequently misses tumors that are not
intracanalicular and do not extend >5mm to
the CPA.

63% accuracy at diagnosis (29)

Gold Standard for Vestibular Schwannomas

Gadolinium is preferentially taken up by tumor

Hyperintense on T1 and T2 studies

Non-contrasted studies

Better visualization of small tumors

Gadolinium enhanced studies

Can identify tumors as small as 3mm (30)

Hypointense T1, Isointense on T2

Some recommend T2 fast spin-echo MRI as


screening test but most will likely require at thin
slice MRI if abnormalities are found (31)

VESTIBULAR
SCHWANNOMA

Centered on IAC
Globular appearance
Ice cream cone
appearance in IAC
Bony erosion of IAC
Cystic degeneration or
hemorrhage may be
present

MENINGIOMA

Extend along petrous


ridge
Sessile appearance
Dural Tail at
periphery
Iso/Hypointense on T1
Hypo to Hyperintense
on T2

3% of all CPA tumors (13)


Do not metastasize but do recur due to bony
invasion
Are formed from cap cells around arachnoid
villi near dural sinus and where CNs enter
their foramina
Generally are not intracanalicular so must be
larger to cause hearing concerns

Signs and Symptoms (32)

Usually cause spontaneous nystagmus, facial


hypesthesia, and gait ataxia
If inferior can cause hoarseness, dysphagia, tongue
atrophy
If within the IAC can produce similar symptoms as
Vestibular Schwannoma

Hearing tests will show retrocochlear process if


large enough, and 25% will have normal ABRs
(33)

Treatment

Surgical Excision
Must remove rim of normal dura and possibly bone

Poor hearing
Translabyrinthine approach

Good hearing
Suboccipital or Middle Fossa Approach
Middle Fossa is better for more superiorly based

tumors.

Identical to cholesteatoma
Develop from epithelial rest cells
Slow growing

Commonly dont present until 20-30s

Arise adjacent to brainstem and infiltrate areas


of least resistance

Can have irregular shape and infiltrate widely

Signs/Symptoms

Facial twitching is commonly described


Can become quite large before causing symptoms
Progressive facial paralysis more common than
schwannomas

Audio/ABR/Speech discrimination consistent


with other retrocochlear losses
Treatment is surgical excision

T1 post
Gadolinium

T1 post
Gadolinium

T2

T1
Flair

T1

T2

T1

T1

Histologically identical to Vestibular


Schwannomas
Characteristics
Rarely restricted to IAC
Commonly have skip lesions
Generally involve part of geniculate ganglion

Signs/Symptoms

Unilateral hearing loss


Tinnitus
Vertigo
Aural fullness (if distal to geniculate)
Facial weakness is rare unless very large

Hearing tests show retrocochlear process but


impedance testing can show ipsilateral absent
acoustic reflex

Treatment is observation
If growth or facial nerve dysfunction

Resection of nerve with cable grafting


Translabyrinthine approach most commonly used

Facial nerve decompression can be used if


paresis is developing

Glomus Tumors

Hemangiomas

Bright on T1 and T2, Drained via infralabyrinthine approach

Embryonic tumors (Dermoids, teratomas, chordomas)

Treatment is drainage

Cholesterol Granulomas

Centered on geniculate, slow progressive facial weakness,


surgical excision with facial nerve grafting

Arachnoid Cysts

Jugular Foramen Syndrome (IX,X,XI), Surgical excision

Excision with dysfunction

Primary Axial Tumors of CNS (glioma,


hemangioblastoma, medulloblastoma)

Surgical excision +/- radiation therapy

Observation
Surgery
Stereotactic Radiosurgery
Radiation Therapy

**Generally tumors <3cm can be treated with


stereotactic radiosurgery but microsurgery is the
treatment of choice

Growth rates vary from 0 to 2cm per year with an


average of 2mm (34)
38.9-82% have some growth at 38 months (24)
14 to 24% of patients watched will go on to have some
treatment (12)
Age should not be determining factor
1.
2.
3.

Single MRI is not adequate

Young patient with small tumor


Older patient with large tumor
Older patient with small tumor

Repeat at 6 months and then again yearly

Longstanding hearing loss may represent a slow


growing tumor (35)

First Introduced Leskell in 1969


Gamma Knife

LINAC

Uses multiple beams from a linear accelerator


One session

Fractionated Radiotherapy

Uses 201 ionizing beams of gamma rays from cobalt 60 source


One session

Newer therapy to eradicate cells in different cell cycle stages


Multiple sessions

Goal is to stop tumor growth, not shrink or remove


tumor

Local control = not requiring salvage therapy

87 to 100% local control rates all three combined

23% of cases have transient increase tumor


volume due to central necrosis

6mo to 5yr to disappear


Therefore some patients receive unnecessary salvage
therapy

Hasegawa et al. 2005 (36)

317 patients
Median follow up 7.8 years
10 yr local control >92%
Partial or complete radiographic response 62%
Progression free survival
96% if <15cm3 vs 57% if >15cm3 p<0.001
Better with no brainstem compression/4th ventricle

obstruction p<0.001

Most tumor progression within 3 years

Freidman et al. 2006 (37)

390 patients
Median follow up 40 months
Median dose of 12.5 Gy
5- and 10-year local control 90%
Only 1% of patients required surgery for treatment
failure

Many different regimens studied


Dose 15-57.6 Gy/3-32fx
Median Follow ups of 48 months
5- year local control of >90%

**Relatively new idea so no long term outcomes


available

Tumor Control
73.8 to 100% for Radiosurgery
91.4 to 100% for FSRT

Tumor Shrinkage
38 to 76.2% for Radiosurgery with single dose
34 to 76% for FSRT

Tumor Growth
0-26.2% for Radiosurgery
0-12.5% for FSRT

Hearing Preservation
47-71% for Radiosurgery
57-100%for FSRT

Defined as G-R class I/II


Early studies 16Gy = 46%
Later Studies 12-13Gy = 68-78% with no change
in control (24)
Prasad et al. (39)
153 patients
Median follow up of 4.2 years
Useful hearing preservation rate of 58%
Size matters

<1cm3 = 75% hearing preservation


>1cm3 = 57% hearing preservation

GK vs FSRT
Andrews et al. 2001 (40)

109 patients
63 GK at 12Gy
46 FSRT at 50Gy/25fx

Follow up was 3 years


33% vs 81% hearing preservation (GK/FSRT)
98% vs 97% local control (GK/FSRT)

Thought is that lower dose in multiple fractions


leads to decreased late normal tissue damage

GK dose decrease from 16Gy to 12-13Gy


decreased rates from 15% to 0% (41)
LINAC dose decrease of 16Gy to 12.5Gy
showed drop in neuropathy from 4.4% to 0.7%
(37)

RS vs FSRT ranges of 0-52% vs 0-4%(38)

GK dose decrease from 16Gy to 12-13Gy


decreased rates from 16% to 4.4% (41)
LINAC dose decrease of 16Gy to 12.5Gy
showed drop in neuropathy from 3.7% to 0.7%
(37)

RS vs FSRT ranges of 2.4-29% vs 0-16% (38)

Hydrocepahlus

Tinnitus

3.6%

Malignant transformation

1.4 to 1.7%

Peritumoral cyst formation

1.4 to 3.6%

Vertigo

0.2 to 5%

Ataxia

0-11% (24), with highest rates in FSRT

0 to 0.3%
3 cases in all studies reviewed with time to presentation of 5, 7.5, and 18
yrs post therapy

Disequilibrium

33% likely due to weaker central compensation (42)

1.

2.
3.

Translabyrinthine Approach
Middle Cranial Fossa Approach
Retrosigmoid-Suboccipital Approach

**All will require discussion/collaboration with


Neurosurgery

ADVANTAGES

Least cerebellar retraction


Wide exposure of
posterior fossa
No size limit for resection
Facial nerve easily
identified throughout
Ease of facial nerve repair
if damaged/resected
during removal
Low recurrence
Low headaches

DISADVANTAGES

Residual hearing is
sacrificed
Requires abdominal fat
graft

ADVANTAGES

Best hearing
preservation

<30db PTA
>70% speech
discrimination

Good exposure of
lateral IAC, CPA, and
clivus
Drilling is extradural
decreasing morbidity

DISADVANTAGES

Limited to tumors <2cm


in greatest dimension
Temporal lobe
retraction
Must dissect around
facial nerve due to its
superior position
Limited posterior fossa
exposure

ADVANTAGES

Can attack any size tumor


Hearing preservation
possible
Wide exposure of
brainstem and lower
cranial nerves
Neurosurgeon familiarity
Consistent facial nerve
identification

DISADVANTAGES

Must be medially located


with <2cm CPA extension
Lateral tumors risk injury
to endolymphatic sac and
vestibular labyrinth
Cerebellar retraction
occulomotor
abnormalities and postop
disequilibrium
Increased air embolism
risk (Sitting vs Park Bench
position)

Translabyrinthine

Total Resection 99.5 to 99.7% (44-45)


Near total resection (<25mm2 or 2mm thick)
Visible on MRI in 50% of patients with 3% risk of

recurrence (46-47)

Middle Cranial Fossa

Total Resection 98% (48)

Retrosigmoid

Total resection 95% (49)

Serviceable hearing defined as class A/B or

1/2 (24)

Middle cranial fossa 51%


Retrosigmoid 31%

Meyer et al. 2006 (50)


162 consecutive patients via MCF approach
Class A/B hearing preserved in 41%
56/113 (50%) with WR >70% preoperatively maintained
that level.

Tumor 0.2-1.0cm = 59%


Tumor 1.1-1.4cm = 39%
Tumor 1.5-2.5cm = 33%

Reporting function at 6-12 month point is gold


standard

Preserved = Grade I/II (24)


Retrosigmoid 91%
Middle Cranial Fossa 88%
Translabyrinthine 77%

Delayed paralysis (>72hrs after surgery)


Described by Grant et al. 2002

Incidence 5% (51)
79% regain postoperative function by 1 yr

Pooled data from 19 studies 360/4297 (8%) rate


(52)

MCF approach 6%
TL approach 8%
RS approach 11%

Occurs at two different time points

POD 2-3 (Early)


POD 10-14 (Late)

Occurs roughly 10% of patients >3 months


postop (52)

RS approach 21%
MCF approach 8%
TL approach 3%

Mechanisms
1.
2.
3.
4.

Bone dust contacting CSF/Meninges Aseptic


Meningitis
Occipital nerve entrapment
Scarred neck muscles and dura
Migraine

Mortality

1% due to neurovascular injury

Meningitis

1-8%
Aseptic more common than bacterial (bone
dust/inflammation)
S. aureus most common pathogen

Tinnitus

Balance abnormalities

50% with preop tinnitus will have resolution postop

Occurs in most patients but gone by 6-9 months

Seizure/Hydrocephalus/Stroke (24)

Rare and <2% of cases

Myrseth et al 2005 (53)

Retrospective review 189 patients tumors 3cm


86 by microsurgery vs. 103 by GK
5.9 year mean follow up
Local control rates of 89.2% Surgery vs 94.2% GK
HB 1-2 in 79.8% Surgery vs 94.8% GK p=0.0026
Quality of life significantly lower in surgery group

compared to gamma knife group

*MCF approach was not utilized

Pollock et al. 2006 (54)

Prospective cohort of 82 patients unilateral VS <3cm


36 Surgery vs 46 GK

Average follow up of 42 months


Local control 96% Surgery vs 100% GK p= 0.50
HB 1/2 in 75% Surgery vs 96% GK p<0.01
Hearing Preserved 5% Surgery vs 63% GK p<0.001
Quality of life all statistically better for GK

Physical functioning
Bodily pain scores
Dizziness Handicap Inventory

Vestibular schwannomas are the most common


CPA tumor
Unilateral Tinnitus or SNHL must be evaluated
Tumors <3cm in size treated with radiosurgery

GK or LINAC have comparable results


FSRT may prove more beneficial but requires more than 1
day of treatment

For patients with no serviceable preoperative


hearing, translabyrinthine approach is best choice
For Hearing Preservation options the middle
cranial fossa approach appears to be slightly better
than the retrosigmoid approach due to decreased
rates of complications.

55 y/o female with 2 year history of falls and


disequilibrium
Falls are now progressive and daily
Denies true vertigo just imbalanced
Also has tinnitus, neck spasms, and migraines
She denies any hearing loss but family feels
otherwise

PMHx:

PSHx:

HCTZ, metoprolol, sydol prn, soma, and ambien prn

Allergies:

45pack year history of smoking, -EtOH

Medications:

Noncontributory

SHx:

Partial hysterectomy and R thyroid lobectomy

FHx:

HTN, Hypothyroidism

Sulfa

ROS:

Otherwise negative. No vision or constitutional problems

General: NAD oriented x3


Head: NCAT
Eyes: No nystagmus, PERRLA, EOMI
ENT: TMs intact, Tuning forks normal
Neck: No masses or lymphadenopathy
Neuro: CN II-XII intact, unsteady Romberg,
tandem gait falling to the left, and left finger to
nose ataxia

Audiogram

Normal hearing with mild high frequency SNHL at


4000Hz bilaterally

ENG
Decreased accuracy on horizontal smooth pursuit
54% weakness on left air caloric testing

MRI

1.5 to 2cm lesion closely associated with CN


VII/VIII. It is intracanalicular with extension into
CPA. No evidence of brainstem compression or
ventricular obstruction

1.
2.
3.

4.

Finger to nose and gait ataxia


Essentially normal hearing
ENG positive for left lateral canal
weakness
2cm tumor, no hydrocephalus

Patient had previous MRI 2005 showing no


tumor
Therefore it is decided that we perform
suboccipital craniotomy for excision of tumor

Tumor

Tentorium
Cerebelli

Retracted
Cerebellum

CN
VII/VIII
Complex

Tumor

Tentorium
Cerebelli

Retracted
Cerebellum

CN
VII/VIII
Complex

Tumor

Tentorium
Cerebelli

AICA
CN V
Retracted
Cerebellum

CN
VII/VIII
Complex

Tumor

CN VI

Tentorium
Cerebelli

AICA
CN V
Retracted
Cerebellum

Final Pathology: Meningioma


No complications at this point
Tuning forks 256hz and 512hz normal
HB I immediately and 72 hours postoperatively

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