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Cerebello-Pontine Angle Tumors

Shikher Shrestha
History..

Challenge despite benign – complexity of the anatomy

Sir Charles Balance (1894) – first successful complete removal


(Right posterior fossa craniectomy and removal through finger insertion) – patient lived
for 18 years post surgery

Krause – faradic stimulation to differentiate facial from audiovestibular nerve

Cushing – reduced morbidity and mortality by intracapsular removal; however with high
recurrence rate
History

Dandy – introduced currently widely accepted concept

“benign tumors should be removed completely to prevent future recurrence even


at the expense of a somewhat higher perioperative mor tality rate”

Now – much lower morbidity and mortality

advent of newer imaging modality, intraoperative electrophysiological monitoring,


more reliable and safe operative and anesthetic technique
CP angle Anatomy

CP angle: Triangular space bounded by –

pyramid anteriorly

tent superiorly

pons medially and

cerebellum dorsally
CP angle Anatomy

CP angle cistern is located between

anterolateral surface of pons &


cerebellum and posterior surface of the petrous bone

Contains:
CN V, VI, VII and VIII
SCA and AICA
variable number of draining veins
flocculus of the cerebellum
choroid plexus that protrudes through the foramen of Luschka
VII Nerve exits from the lateral part
of the pontomedullary sulcus;
1-2 mm
anterior to the entry zone of VIII nerve.

IX, X and XI CNs are located inferiorly


5 nerves pass through the internal auditory canal
superior

Facial
Vestibular (superior and inferior) vii sv
Cochlear and
Nervus intermedius Ant
(accompanied by labyrinthine artery and
occasionally by branches of AICA or coch iv
a loop of the AICA itself)
Histopathology of Vestibular Schwannoma

Antoni A fibers – narrow elongated bipolar cells

Antoni B fibers – loose reticulated fibers

Verocay bodies – acellular areas surrounded by parallel arrangement of spindle shaped


schwann cells
Tumors of the Cerebello Pontine Angle

5-10% of all intracranial tumors


Mnemonic:
S – schwannomas
VS – the commonest (80-95%)
A - aneurysm, arachnoid cysts
Followed by M – meningiomas, mets
E - epidermoids
Meningioma (3-10%)
Epidermoids (2-4%)

Others: schwannomas of other CNs; paragangliomas (glomus jugulare/glomus


tympanicum) , chordomas, chondrosarcomas, arachnoid or neuroenteric cysts, dermoid
and metastasis.
Tumors of the Cerebello Pontine Angle

CP angle secondarily involved by:

tumors from brainstem or fourth ventricle: gliomas, ependymomas, choroid plexus


papillomas, medulloblastomas or lymphomas

Bilateral CP angle tumors: characteristics of NF2: typical histology: VS


Imaging

Things to consider:

site of origin

location

size, shape and margins

density and signal intensity

contrast enhancement characteristics


Imaging

Non enhancing extra axial CP angle lesions:

Cystic
Epidermoid cysts, arachnoid cysts, Neurenteric cyst

Containing Fat
Dermoid cyst, Lipoma

Intrinsic brain tumor with a significant exophytic extension into the CP angle may be
difficult to differentiate from an extra axial lesion based on imaging
Bone window thin slice CT: bony changes of the pyramid and IAC – essential in surgical
planning

Erosion or dilatation of IAC seen in 70-90% of VS

Nevertheless, the Diagnostic tool of choice: MRI


MRI Characteristics of VS:

T1- weighted sequences:


isointense to slightly hypointense

T2- weighted sequences:


hyperintense

Gadolinium contrast:
intense and homogenous enhancement with exception of cystic portion

Intrameatal VSs are best visualized with gadolinium enhancement.


MRI characteristics of Meningiomas:

T1-weighted images:
isointense to slightly hypointense to brain parenchyma

T2-weighted images:
higher intensity than VS and with homogenous contrast enhancement
Criteria for radiological differential between VS and CPA
meningioma
Meningiomas are

centered usually away from the IAC

broad contact with petrous bone or the tent

obtuse angle between tumor and the pyramid

IAC not widened and tumor very rarely extends into the IAC (secondary invasion of
the IAC might be observed in 10-20%)
Criteria for radiological differential between VS and CPA
meningioma

Calcification and cystic changes are frequent findings

Tail of enhancement along the dura (the dural ‘tail’ sign) – 60-70% meningiomas

Calcifications are extremely rare in VS


Imaging of Epidermoids

Hypodense on CT

Rim calcification observed in 25% cases

T1 + T2 weighted MRI: isointense to cerebrospinal fluid

Signal intensity related to cyst content. Eg. If cholesterol predominates, the cysts are
hyperintense on T1 weighted images and hypointense on T2

Because of similar characteristics, it might be difficult to differentiate it with arachnoid


cyst.
Epidermoid vs Arachnoid cyst

Two major differences

smaller mass effect by arachnoid cyst

arachnoid cysts have more homogenous signal intensity on T2 weighted images

DWI restriction in epidermoids


Evaluation of patient with suspected CPA mass

MRI with and without contrast; FIESTA MRI if available (Fast Imaging Employing Steady
State Acquisition): uses CSF as the contrast agent and NOT gadolinium

CT scan if MRI contraindicated

Audiometric evaluation

Pure tone Audiogram


Speech Discrimination Evaluation
Patients with small VSs (<15 mm diameter) gets ENG, VEMP and ABR
ENG (Electronystagmography)

tests horizontal semicircular canal

hence, assesses the superior vestibular nerve which innervates it

normally, each ear contributes an equal portion of the response

abnormal if >20% difference between the two sides


Vestibular Evoked Myogenic Potential (VEMP):

assesses inferior vestibular nerve by testing the saccule.

independent of hearing (can be done even with severe SNHL)


Auditory brainstem responses (ABR):

aka BAER

prognosticates chance of hearing preservation

most common findings – prolonged I-III and I-V interpeak latencies

poor wave morphology correlates with lower chance of preserving hearing (even
with good hearing).
Pure Tone Audiogram (PTA)

First step screening test

Air conduction assesses the entire system

Bone conduction assesses from the cochlea and proximally

PTA assesses the functionality of hearing (to help in treatment decision making) +
baseline for future comparison

The single numerical score is an average of the thresholds for frequencies across the audio
spectrum
Pure Tone Audiogram (PTA)

Xs denote the left ear (AS) and Os denote the right ear (AD)

Progressive unilateral or asymmetric SNHL of high tones occurs in >95% of VS

DDx: age related and noise related hearing loss (usually symmetrical)

Other differentials of asymmetric hearing loss:


meningioma
inner ear lesions
intraaxial lesions leading to infarctions
multiple sclerosis

PTA difference of >10-15 dB between 2 ears should be investigated


Speech Discrimination Evaluation

SD is maintained in conductive hearing loss


Class WRS %
Moderately impaired in cochlear lesion I 70-100%
II 50-69%
Worst with retrocochlear lesion
III 1-49%

A score of 4% suggests retrocochlear lesion IV 0

Open set word recognition score (WRS) is a more sensitive measure of communication
ability than PTA.
Gardener and Robertson modified hearing classification
(modification of Silverstein and Norrell system)

Class Description Pure tone Speech


audiogram (dB) discrimination
I Good-excellent 0-30 70-100%
II Serviceable 31-50 50-69%
III Non serviceable 51-90 5-49%
IV Poor 91-max 1-4%
V None Not testable 0

NB: class > or = III is generally considered non serviceable hearing


American Academy of Otolaryngology- Head and Neck
Surgery Foundation hearing classification system

Class Pure tone threshold (dB) Speech Discrimination


(%)
A < or = 30 > Or = 70
B >30 and < or = 50 > Or = 50
C >50 > Or = 50
D Any level <50

Class A & B are considered “useful”, Class C is “aidable”, & Class D is “non functional”
Definition of serviceable hearing

Even non serviceable hearing can offer some benefit

If WRS is good (> or = 70) but PTA is poor, a hearing aid may provide significant benefit.
Definition of serviceable hearing

1. AAO-HNS class A or B

2. “50/50 rule”: Gardner-Robertson class I or II (pure tone audiogram threshold < or =


50 dB and speech discrimination score > or = 50%)

3. Some prefer a 70/30 rule (70% WRS, 30 dB PTA)

4. In a patient with good hearing in the contralateral ear, a speech discrimination score
(SDS) of <70% in the affected ear is not considered good hearing; whereas if the
contralateral ear is totally deaf, a SDS of > or = 50% can be useful.
Addendum to CT scan imaging…

Small lesions may be visualized by introducing 3-4 ml of subarachnoid air via lumbar
puncture

Scanning is done with the affected side up (to trap air in region of IAC)

Non filling of IAC is indicative of an intracanalicular mass.

Although many VSs enlarge the ostium of the IAC (called trumpeting) (normal diameter
of the IAC is 5-8 mm), 3-5% of VSs do not enlarge the IAC on CT

Advantage over MRI: bony anatomy delineation and thus, useful for planning
translabyrinthine approach.
Vestibular Schwannomas

Histopathologically benign typically slow growing neoplasms

Originates from the transition zone between central and peripheral myelin that usually is
located in the medial part of the IAC – Obersteiner Reidlich zone

2 different forms:

Sporadic unilateral (95%)


Bilateral associated with NF2 (5%)
Vestibular Schwannomas

SPORADIC NF 2
Presents at 5th or 6th decade Usually younger at presentation
Predisposition to multiple tumors like
meningiomas, ependymomas,
neurofibromas
Autosomal dominant inheritance
Mutation in chromosome 22q12: affects
gene encoding schwannomin/merlin:
implicated in cellular remodeling and
growth regulation
Tumors just displaces VIIIth nerve without NF2 tumors form grape like clusters that
infiltrating may infiltrate the nerve fibers
VS progression – Four Stage Concept of Growth

Hydrocephalic
stage with
Compresses the obstruction of
brainstem and IV th ventricle
the cerebellum and its outlets
Occupy the CP
angle cistern,
Gradually fills the displacing VII
IAC first and VIII cranial
nerves and AICA

Symptomatology of patient differs according to the stage of progression


Symptoms

Intrameatal:
vestibulocochlear dysfunction: hearing loss, tinnitus or vestibular dysfunction
Hearing loss is insidious and high frequency sensorineural type

Cisternal stage:
progressive hearing loss
sense of dysequilibrium gradually replaces vertigo

Later stage:
trigeminal symptoms, headache, ataxia and obstructive hydrocephalus
Symptomatolgy and signs
Sign %

With further brainstem compression: Abnormal corneal reflex 33

Nystagmus 26
contralateral long tract signs

severe gait disturbance Facial hypoesthesia 26

lower cranial nerve palsies Facial weakness 12

signs of intracranial hypertension Abnormal eye movement 11

Papilledema 10

Babinski sign 5
Classification of VS based on size or degree of extension into
CP angle
According to size:

Intrameatal

up to 1 cm

1 to 2.5 cm

from 2.5 to 4 cm

larger than 4 cm
INI (International Neuroscience Institute) or Samii
Classification of Vestibular Schwannoma extension:

Tumor Grade Tumor Extension


T1 Purely intrameatal
T2 Intra-, extrameatal
T3a Filling the CPA cistern
T3b Reaching the brainstem
T4a Compressing the brainstem
T4b Severe compression and distortion of brainstem and fourth ventricle
T5 Giant tumors (maximal diameter >4 cm), extension over the midline
House and Brackmann
Facial Nerve Grading System
Management

Expectant management

Radiation therapy

Surgery

Chemotherapy
Expectant management

Follow symptoms, hearing (audiometrics) and tumor growth on serial imaging

Intervention is performed for progression

Growth patterns observed:

a. little or no growth: applies to most (83%) VSs confined within the IAC and 30%
extending into CPA
b. Slow growth ~2 mm/yr
c. Rapid growth > or = 10 mm/yr
d. A few actually shrink
Intracanalicular or CPA tumors < or = 20 mm diameter that are non cystic and non NF2:
observation with serial imaging and hearing tests

Presence of cysts: cystic tumors may display sudden and dramatic growth; high recurrence

Imaging repeated if shows >2mm growth then intervention

Q6mo for 2 yrs; if stable then annually until year 5 after diagnosis

If stable then at years 7, 9 and 14 after diagnosis


Radiation therapy (alone or in conjunction with surgery):

a. External Beam Radiation Therapy (EBRT)

b. Stereotactic radiation

a. Stereotactic radiosurgery (SRS): single dose


b. Stereotactic radiotherapy (SRT): fractionated

Recommended current dose of radiation 12-14 Gy to reduce cranial n. morbidity


Tumor control achieved in 93-98%
Facial neuropathy in 1 to 20% and trigeminal dysfunction in 2-30% cases
Hearing preservation in 40-75% cases
2-7% - tumor enlargement occurs even after radiotherapy
Post radiation changes  obliteration of dissection plane  difficulty in functional
preservation of cranial nerves if microsurgical removal required in later dates

Larger VS: staged procedure: microsurgical debulking and brainstem decompression


followed by radiosurgery of the remnant as second stage
Surgery: Approaches…

Retrosigmoid (aka suboccipital): may be able to spare hearing

Translabyrinthine (and its several variations): sacrifices hearing, may be slightly better for
sparing VII

Middle fossa approach (extradural subtemporal): only for small lateral VSs

Complete tumor removal achieved in 80-99% cases


Recurrence rate: 0.5 to 5%
Facial nerve preservation in >90% cases
Chemotherapy:

Early promise for NF2 related VS

Bevacizumab (Avastin; it is an anti VEGF vascular endothelial growth factor monoclonal


antibody)

In 6 patients, 4 had radiographically significant tumor shrinkage and 4 had improvement in


auditory word recognition score
Surgical approaches…

MIDDLE FOSSA APPROACH/ Kawase approach

Hearing preserving technique


Direct access to lateral end of IAC
Most lateral part of the tumor can be safely resected
Low risk of CSF leaks
Applicable to small tumors

Necessitates temporal lobe retraction


Endangers vein of Labbe
Restricted access to the CP angle
TRANSLABYRINTHINE APPROACH: Rudolf Panse, 1904

Avoidance of cerebellar retraction


Shorter distance to the tumor
Early identification of the facial nerve at the lateral end of IAC

Sacrifice hearing
Restricted access to the CP angle
Difficult dissection and hemostasis close to the brainstem
Poor visualization and access to the caudal cranial nerves
RETROSIGMOID APPROACH: Fedor Krause, 1903

Allows hearing preservation even in large VSs


Excellent visualization of whole CP angle
Increased safety during dissection from brainstem and lower cranial nerves
VII/VIII n complex can be identified both proximally towards brainstem and distally
towards IAC

Needs cerebellar retraction


Difficulty visualizing the most lateral part of the IAC without endangering inner ear
integrity
Higher rate of postoperative headache
Addit ional r emoval of t he suprameat al t uber c le ( SAMII Tec hnique)
1 9 8 2 ; p r o v i d e s a c c e s s t o t u m o r e x t e n s i o n s i n t o M e c k e l ’s c a v e , t h e
petroclival area and even the posterior caver nous sinus.
Booking the case – retrosigmoid craniectomy

Position: supine with shoulder roll, Mayfield 3 pin head rest

Equipment:
microscope
ultrasonic aspirator
image guided navigation system

ENT to assist IAC drilling

Neuromonitoring: facial EMG, direct cochlear nerve monitoring and SSEPs

Post op: ICU


Consent:
procedure in lay term

alternatives: non surgical management with follow up MRIs, other surgical


approaches, radiation (stereotactic radiosurgery)

complications: CSF leak with possible meningitis, loss of hearing in ipsilateral ear (if
not already lost), paralysis of facial muscles on the side of surgery with possible need
for surgical procedures to help correct (correction is often far from perfect), post-op
balance difficulties/vertigo, brainstem injury with stroke.
Technique Summary..

Position: 30 degree elevation of head

Percutaneous lumbar drain (optional)

Incision is shaped like the pinna of the ear, 3 finger breadths behind the external auditory
canal

The craniotomy has to be lateral enough to expose part of the sigmoid and part of the
transverse sinuses.

To prevent CSF leak, seal all bone edges with bone wax
Dural opening Y shaped making base to sinuses

Exposure is enhanced by opening the CP angle cistern and the cisterna magna under the
microscope and draining CSF (20-40 ml of CSF can also be drained via a lumbar
subarachnoid catheter)

The petrosal vein is often sacrificed at the beginning of the procedure to allow the
cerebellum to relax and fall back and to avoid tearing off the transverse sinus. Be careful
not to coagulate the SCA that often runs with the petrosal vein.
Using the facial nerve stimulator, the posterior aspect of the tumor is inspected to make
sure the facial nerve has not been pushed posteriorly

The thin layer of arachnoid that covers most tumors is identified. Vessels within the
arachnoid may contribute to cochlear function and may be preserved by keeping them
with the arachnoid.

The plane between tumor and cerebellum may be followed to the brainstem, and
occasionally to the VII nerve (this plane is harder to follow once bleeding from tumor
debulking occurs)
Posterolateral tumor capsule is opened, and internal decompression is performed. The
tumor is collapsed inward and the capsule is kept intact and is rolled laterally off of VII
and is eventually removed.

The most difficult area to separate VII from tumor is just proximal to the entrance to the
porus acousticus.

Large tumors: capsule adherent to brainstem  portion must be left; recurrence rate: 10-
20%  may involve V superiorly and IX, X, XI inferiorly; these cranial nerves are spared
by protecting with cottonoids
After extracanalicular portion of tumor is removed, the dura over the IAC incised

IAC drilled open and tumor removed from this portion

Bony labyrinth must not be violated to preserve hearing

The most vulnerable structure is posterior semicircular canal

Preop CT help determine maximum amount of temporal bone drilling

Any opening of labyrinth should be plugged with bone-wax or muscle


CP angle Meningiomas

Meningiomas located lateral to trigeminal nerve regardless of the site of dural attachment
are termed CP angle meningiomas

If located medial to trigeminal n.  petroclival meningioma

Slow growing, benign

Women in 5th to 6th decade

Arise from groups of arachnoid cells located in high concentration around the IAC, lower
border of superior petrosal sinus, lateral border of inferior petrosal sinus, around cranial n.
foramina and in the region of the geniculate ganglion.
Classified as premeatal and retromeatal in relation to the IAC

The more medially located the tumor, the greater is the surgical challenge and the poorer is
the outcome

Further subclassified as premeatal, postmeatal, suprameatal, inframeatal and those


centered at the IAC.
Signs:

Hearing loss 30-73%

Trigeminal nerve signs 13-49%

Cerebellar signs 25-52%

Tinnitus 10-12%

Signs of increased ICP 16-29%

Hydrocephalus 20-31%
Three percent of the cases of trigeminal neuralgia are due to tumor compression at the
root entry zone

Premeatal tumors are diagnosed earlier and consequently are smaller. Their clinical
presentation is with trigeminal signs and facial and cochlear nerve signs

Retromeatal meningiomas present with cerebellar signs


Total surgical removal is the optimal treatment option but should not be achieved at the
expense of new neurological dysfunctions or worsening quality of life.

Different surgical approaches: retrosigmoid, translabyrinthine, transpetrosal

Principles:
adequate exposure
interruption of the blood supply along the dural attachment
internal decompression
cautious dissection of the tumor capsule from the brainstem and cranial nerves at the
arachnoid plane
With the retrosigmoid approach hearing is preserved in 82-90.8% of the patients

Compared to VS, CP angle meningiomas have higher tendency toward recurrence, with a
recurrence rate between 0-9.5%

Radiotherapy or radiosurgery proposed for small meningiomas

Surgery may still be required in case of treatment failure or secondary trigeminal neuralgia
after irradiation
Epidermoids

Might expand through the tentorial incisura into the middle cranial fossa, grow toward the
contralateral CP angle, or extend toward the foramen magnum

Are sometimes densely adherent to the neurovascular structures

Usually present between the third and fifth decades of life with long standing history of
tinnitus and hearing loss.

Cause relatively more frequently trigeminal neuralgia or hemifacial spasm when compared
to VS and meningiomas; lead to facial nerve signs much earlier than VS.
Treatment of choice is surgical and preferred approach is retrosigmoid

Endoscopic assisted microsurgical technique

Small remnants of capsule firmly attached to important structures should not be removed
for risk of additional neurological deficit

Subtotal resected epidermoid have late recurrence up to 20-30 years and thus mandates
close follow up on a long term basis.
Thank you!!!