Cerebellopontine Angle Tumors: Clinic Al Pearls

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Tumors

43 
Cerebellopontine Angle Tumors
ROBERT S. HELLER, LUKE SILVEIRA, CARL B. HEILMAN

CLINICAL PEARLS
• Comprehensive knowledge of the complex anatomy of the and facial nerves, should be identified early; the tumor
cerebellopontine (CP) angle is a prerequisite for achieving should be initially debulked; the dissection from the
good surgical results. The crucial neurovascular structures surrounding structures should be performed only after
should be identified as early as possible during surgery, which sufficient internal decompression is achieved; the dissection
enables their preservation and guides subsequent operative should always be performed in the arachnoid plane; and
steps. Whatever the tumor size and extension, the anatomic bipolar coagulation, especially in the vicinity of a cranial nerve,
relationships of the cranial nerves in the area of the fundus of should be avoided.
the internal auditory canal and in the brainstem exit/entry • Our preferred method is the retrosigmoid approach. It is safe,
zone are constant. relatively simple, and provides a panoramic view of the CP
• Most of the CP angle tumors are benign, and their complete angle and petroclival area. Importantly, it is related to a very
removal leads to excellent long-term outcomes. The only low procedure-related morbidity rate. The additional removal
exception to complete tumor removal is the attempt to of the suprameatal tubercle provides access to tumors with
preserve function, such as in surgery for vestibular extensions into the Meckel cave, the petroclival area, and even
schwannoma in the only hearing ear. the posterior cavernous sinus.
• The major principles of CP angle tumor removal include
the following: important neural structures, such as the cochlear

History of Cerebellopontine Angle Surgery As the 19th century ended, the clinical manifestations of a
CPA mass, at least in its advanced stages, were becoming
The cerebellopontine angle (CPA) is the most common site for increasingly well known and recognized among clinicians. Sir
posterior fossa neoplasms. Tumors occupying this region Charles Ballance is credited with performing the first successful
account for approximately 10% of all intracranial neoplasms, complete surgical removal of a CPA tumor in November 1894.
with vestibular schwannomas accounting for 80% of these Patient symptomatology, which incited suspicion for a CPA
CPA tumors.1 Other tumors involving this region include mass and spurred the operation, included a 1-year history of
meningiomas, dermoid tumors, arachnoid cysts, lipomas, and vertigo, headache, instability, and unilateral loss of vision. Bal-
metastases. The cerebellopontine angle is densely inhabited by lance performed the operation in two stages, set 1 week apart.
vital neurologic tissue. As such, tumor growth in this region In the first stage, a right posterior fossa craniectomy was per-
may give rise to significant neurologic dysfunction and, if left formed. One week later, Ballance removed the tumor by insert-
untreated, can ultimately lead to death. ing an ungloved finger between the pons and petrous bone.
As early as 1777, Dutch physician and anatomist Eduard The patient, 46 at the time of the operation, went on to live
Sandifort provided the first documented postmortem descrip- an additional 18 years, unfortunately suffering from facial
tion of a CPA tumor. However, no clinical correlation with the anesthesia, hemifacial paralysis, and delayed corneal ulceration
mass was made at the time.2 In 1810, Leveque-Lesource cor- requiring removal of her right eye.
related symptoms of a CPA mass with postmortem findings. In 1903 Krause of Berlin set forth the unilateral suboccipital
His clinical description was that of a 38-year-old woman with approach for the removal of vestibular schwannomas. Like
vomiting, headache, decreased vision, numbness of the extrem- Ballance, Krause utilized his finger as the instrument for tumor
ities, dysarthria, and deviation of the tongue. At autopsy, dissection and removal. Krause’s mortality rate was close to
Lesource documented the deceased patient to have a tumor 85%, a result of the uncontrollable bleeding he encountered
adherent to the eighth cranial nerve.3 with this approach. In 1904 Panse attempted the first

622
CHAPTER 43  Cerebellopontine Angle Tumors 623

translabyrinthine approach for removal of a vestibular schwan- overall 5-year mortality rate approaching 54%.4 Cushing’s
noma. With limited instrumentation, the approach did not student protégé, Walter E. Dandy, found this recurrence rate
permit sufficient exposure and was similarly associated with intolerable and pursued complete tumor capsule removal after
high mortality rates. In 1905 Victor Horsley performed a employing Cushing’s bilateral suboccipital craniectomy and
complete removal of a vestibular schwannoma at National thorough intracapsular decompression. Dandy also followed
Hospital in London. The patient survived for only a few years Cushing’s example with respect to careful hemostatic control,
after the operation and suffered from presumed severe brain- clipping all vessels around the tumor to avoid bleeding com-
stem ischemia. In the same year, Borchardt performed the first plications, and became the first surgeon to achieve complete
transsigmoid removal of a vestibular schwannoma, but fatal tumor removal with low operative mortality.5
hemorrhage from the lateral sigmoid sinus led to the rapid In 1934 Dandy abandoned the bilateral approach in favor
abandoning of this approach.3 Owing to the extensive bleeding of the unilateral suboccipital approach similar to that originally
complications experienced by surgeons attempting to operate performed by Krause.6 In addition to his profound surgical
on CPA masses, Harvey Cushing would later refer to the ana- skill, Dandy had the advantage of operating on generally
tomic region as “the bloody angle.”2 smaller tumors than those addressed by previous surgeons.
Early attempts at CPA tumor resection were complicated Cushing’s popularized observance of tinnitus preceding ipsilat-
by a lack of diagnostic capabilities or refined surgical instru- eral hearing loss coupled with Dandy’s invention and utiliza-
ments coupled with unreliable anesthesia, limited possibilities tion of pneumoencephalography made earlier and more
for hemostasis, and an incomplete appreciation of the CPA accurate diagnosis of CPA masses feasible. Dandy also had the
anatomy. As such, early surgeries were fraught with intra- opportunity to take advantage of other technologic advance-
operative difficulty and extremely high mortality rates nearing ments in the surgical realm, including improved cauterization
70% to 85% even among the most practiced surgeons.2 Harvey technology, more reliable anesthesia, and the availability of
Cushing, finding these mortality rates unacceptable, would be blood transfusions. Dandy progressively improved upon his
the next pioneer to significantly advance the field of CPA technique by utilizing ventricular and cisterna magna taps to
surgery. lower CSF pressure and achieved adequate CPA access by
In 1917 Cushing published his famous Tumors of the Nervus resecting the lateral cerebellar hemisphere. In 1941 Dandy
Acusticus and the Syndrome of the Cerebellopontine Angle in reported on 46 vestibular schwannoma complete resections
which he documented his operative and perioperative experi- with an operative mortality of just 10.8%.7
ences with 30 vestibular schwannoma patient cases. Cushing The modern era of CPA surgery was ushered in by otologist
reported markedly lower mortality rates than those of his pre- William House. In 1961 House took full advantage of the
decessors, with an initial rate of 20% that he would later reduce newly minted instrumentation available to him, including the
to as little as 4%.4 Though his predecessors broadly recognized surgical microscope, otologic drill, and irrigation suction.
the symptomatology, it was during that time that Cushing first Alongside neurosurgeon John B. Doyle, House pursued surgi-
coined the persisting diagnostic entity CP angle syndrome and cal resection of small acoustic neuromas via the middle fossa
first accurately professed the early development of tinnitus approach. With advancements in audiometric testing and
followed by ipsilateral hearing loss in patients afflicted with improvements in x-ray techniques of the temporal bone, House
CPA tumors. and Doyle were able to identify acoustic tumors before the
Cushing’s vastly improved outcomes were made possible by masses were large enough to incite hydrocephalus and papille-
a number of key factors, starting with his belief that the com- dema. In cases where the tumor was confined to the internal
plete removal of a vestibular schwannoma was simply unattain- auditory canal, tumor extraction and high rates of facial nerve
able. Instead, he focused primarily on achieving decompression preservation were feasible via the microsurgical middle fossa
of the brainstem and avoiding medullary compression during approach. However, in cases where the tumor extended into
his intracapsular subtotal tumor resections. To control intra- the CPA, the middle fossa approach did not permit adequate
cranial pressure in a patient population that was still univer- exposure for resection. Hence, House went on to partner with
sally plagued by elevated intracranial pressures at the time of neurosurgeon William E. Hitselberger, and the two pioneered
diagnosis, Cushing utilized a ventricular tap. Cushing per- a microsurgical approach to the formerly abandoned translaby-
formed a bilateral suboccipital craniectomy, permitting him rinthine technique.8
to explore both CPAs and achieve an osseous decompression. By 1968 House had documented 200 cases of microsurgical
Early access to the cerebellomedullary cistern allowed Cushing neuroma resection with a markedly low mortality rate of 7%
to drain cerebrospinal fluid (CSF) and decompress the cer- and an astonishing facial nerve preservation of 88%. Neuro-
ebellar tonsils.4 In addition to his new approach, Cushing’s surgeons of the time, including Rand and Kurze, also aimed
meticulous operative technique included close attention to to perfect the microsurgical suboccipital approach. By 1995
patient vital signs and persistence in achieving intraoperative collective efforts at technique improvement and the advent of
hemostasis with the implementation of vessel clipping and new diagnostic imaging, including the computed tomography
electrocautery. (CT) and magnetic resonance imaging (MRI), yielded an
Despite Cushing’s comparatively stellar immediate postop- overall reduction in the mortality associated with acoustic
erative mortality rates, his intracapsular, partial resection neuroma surgery of less than 0.5% and facial nerve preserva-
approach was associated with high recurrence rates and an tion nearing 90%.8
624 PART 6 Tumors

Over time, it became clear that acoustic neuromas are the superior vestibular nerve in the posterosuperior quadrant.
neither tumors of the cochlear nerve nor have the neuron as The nervus intermedius runs in close association with the facial
the cell of origin. Rather, the tumor arises from Schwann cells nerve in the anterosuperior quadrant, and it exits the IAC
of the vestibular nerve. To be more accurate, an acoustic alongside the facial nerve through the facial canal.
neuroma is now referred to as a vestibular schwannoma.

Cerebellopontine Angle Anatomy Surgical Approaches to the


The CPA in the posterior fossa is the region adjoining the Cerebellopontine Angle
cerebellum, brainstem, and adjacent skull base. The neural Retrosigmoid
elements of the CPA exit the brainstem and pass through the
cerebellopontine cistern as they course toward the skull base. Perhaps the most versatile of the available approaches, the
The trigeminal, facial, and vestibulocochlear nerves arise retrosigmoid approach provides excellent visualization of the
between the superior and inferior limbs of the cerebellopontine CP angle, brainstem, and IAC. After the induction of general
fissure, the sulcus between the pons, middle cerebellar pedun- anesthesia, the patient is positioned supine with the head
cle, and the cerebellum.9 The trigeminal nerve courses anterior turned to the contralateral side and fixated using 3-point May-
toward Meckel cave, where it divides into its three branches. field pins. Use of a blanket or gel roll can aid in turning the
The sensory rootlets of the trigeminal nerve, termed portia body to allow greater lateral rotation of the head if needed.
major, are lateral and inferior to the motor rootlets, termed Although under-rotation of the head can limit visualization of
portia minor. The facial and vestibulocochlear nerves exit the the CP angle, over-rotation carries the risk of venous occlusion
brainstem adjacent to one another in the pontomedullary at the level of the internal jugular vein in the neck.
fissure with the facial nerve anterior and medial to the vestibu- Neuromonitoring during surgery in the CP angle can
locochlear nerve. These two then pass anterolaterally toward provide vital information. Facial nerve motor function is moni-
the internal auditory canal. The glossopharyngeal and vagal tored through electrodes inserted into the orbicularis oculi and
nerves are found in the caudal region of the CPA as they course orbicularis oris muscles. Brainstem auditory evoked response
toward the jugular foramen. (BAER) monitoring can follow hearing function during the
The vascular elements of the cerebellopontine (CP) angle operation (Fig. 43.1). The normal BAER waveforms consist of
originate from the basilar artery, which runs along the ventral seven waves: I, cochlear nerve; II, cochlear nucleus; III, supe-
aspect of the brainstem. The anterior inferior cerebellar artery rior olivary nucleus; IV, lateral lemniscus; V, inferior colliculus;
(AICA) is the main artery of the CP angle, though occasionally VI, medial geniculate body of the thalamus; and VII, auditory
the caudal trunk of the superior cerebellar artery (SCA) can radiations and cortex. It remains unknown whether monitor-
descend into the CP angle. The AICA bifurcates in the cerebel- ing of the auditory brainstem response (ABR) during surgery
lopontine cistern to form a rostral and caudal trunk; the rostral for a vestibular schwannoma improves hearing preservation
trunk supplies the middle cerebellar peduncle and superior rates. Routine monitoring of the ABR in all cases where hearing
surface of the cerebellopontine fissure, whereas the caudal preservation is attempted may inform the surgeon over time
trunk supplies the inferior ventral surface of the cerebellum.9 of the surgical maneuvers that result in hearing loss, thus
The superior petrosal vein, also known as the Dandy vein, is improving outcomes.
the largest vein in the CP angle and is located rostrally inferior The incision is planned approximately 2 cm posterior to the
to the tentorium. This vein drains into the superior petrosal mastoid, extending from the level of the superior aspect of the
sinus, which courses along the petrous ridge. pinna to 2 cm below the occiput. Typically, this leads to an
The internal auditory canal, located in the temporal bone, incision 1 cm behind the hairline at the level of the midpinna.
is the conduit from the cerebellopontine cistern to the tempo- The incision can be made in either a straight line or slightly
ral bone. The porus acousticus is the opening to the internal
auditory canal (IAC) from the CP angle cistern. There are five
6.03
nerves in the IAC: the facial nerve, the cochlear nerve, the +

superior vestibular nerve, the inferior vestibular nerve, and the 5


+
nervus intermedius. The vascular portion of the IAC consists 2.84 6.84 (0.21)
of the labyrinthine artery, a branch of the AICA, which sup- + 3.81 6.5 +
1.5 + +
+
plies the cochlea and inner ear. Occasionally, AICA itself can
5.4
loop into the IAC. +
2.93
The location of the nerves within the IAC is constant and 1.56
+
4.15 7.28 (0.26)
+ +
organized by quadrants. The facial nerve is located anterosu- +

periorly in the IAC with the cochlear nerve in the anteroinfe-


rior quadrant. Posteriorly, the superior vestibular nerve is
• Figure 43.1  Intraoperative snapshot of BAER recording during resec-
rostral to the inferior vestibular nerve. The transverse crest tion of a left vestibular schwannoma. Waves I through VI are identified, VII
delineates the superior and inferior IAC, whereas Bill’s bar not shown. See text for correlation of BAER wave and neuroanatomic
separates the facial nerve in the anterosuperior quadrant from structure.
CHAPTER 43  Cerebellopontine Angle Tumors 625

semilunar fashion to aid in scalp retraction, depending on the retrosigmoid approach. An incision is made approximately
surgeon’s preference, and dissection using electrocautery is 1 cm anterior to the tragus, extending superiorly as needed.
made down through the periosteum. The mastoid emissary Dissection is carried through the temporalis fascia and under-
vein is controlled with bone wax. This hole in the outer table lying muscle and down through the periosteum. To ensure that
of the skull can be a useful landmark, as it usually exits the the craniotomy is flush with the floor of the middle fossa,
skull about 1 cm below the transverse sinus and 1 cm behind dissection should be carried inferiorly to the root of the
the sigmoid sinus. zygoma. The craniotomy is then made approximately 4 to
Using a high-speed drill, a trough is drilled through the 5 cm in diameter. The dura is lifted from the middle fossa floor
skull along the inferior edge of the transverse sinus and along in a posterior to anterior direction to avoid injury to or eleva-
the posterior edge of the sigmoid sinus. The mastoid emissary tion of the greater superficial petrosal nerve. Once the middle
vein typically enters in the posterior aspect of the sigmoid sinus fossa floor has been exposed, the roof of the internal auditory
at its junction with the posterior fossa dura. If the emissary canal is removed with a high-speed drill. Selecting the mid-
vein is large, the bone around it can be carefully removed with point of the angle between the greater superficial petrosal nerve
the drill and then the vein ligated with a suture; smaller veins and the arcuate eminence at the level of the petrous ridge helps
can be controlled with bipolar coagulation. Once the mastoid guide the surgeon to the location of the IAC. Once the IAC
emissary vein is ligated and the edges of the transverse and has been opened, tumor removal can proceed as before with
sigmoid sinus are exposed, the dura is dissected off the inner careful attention paid to preserving neural function.
aspect of the occipital bone.
The craniotomy is made extending from the transverse sinus Translabyrinthine
on the superior border, the sigmoid sinus on the lateral border,
and 3 to 5 cm medially. The dura can then be opened along The translabyrinthine approach, often preferred by neuro-
the sigmoid and transverse sinuses, leaving a small cuff for otologists, is favored for its early identification of the facial
suturing on closure. Once the dura has been opened, the cer- nerve and excellent visualization of the IAC. Removal of the
ebellum is gently elevated with a brain ribbon, exposing the contents of the inner ear makes it unsuitable for patients with
arachnoid at the foramen magnum. Opening the arachnoid serviceable hearing.
and patiently draining CSF from the cisterna magna provides The patient is positioned in a supine position with head
significant cerebellar relaxation. This reduces the need for turned toward the contralateral side. Mayfield pin fixation is
excess retraction and subsequent edema. Telfa or Biocol is then not necessary, though many surgeons will employ it. A post-
placed over the cerebellum for protection, and then a retractor auricular C-shaped incision is made, with the apex of the C
is placed along the cerebellar hemisphere if needed in order to approximately 2 cm behind the postauricular crease for small
expose the CP angle. tumors and farther back for large tumors. The dissection is
Careful identification of the cranial nerves early in the carried to the periosteum and the limit of the external auditory
operation is crucial to preserve function. A nerve stimulator canal, following which a mastoidectomy is performed. Once
can be used to locate the motor fibers of the facial nerve, which the lateral semicircular canal and the mastoid segment of the
are usually located anterior to vestibular schwannomas but can facial nerve are identified, the labyrinth can be removed and
rarely be found within or posterior to a tumor. Early tumor the IAC exposed.
debulking can also aid in exposure and visualization of normal The facial nerve is most often identified at its genu and
anatomy, especially in cases of large tumors. The posterior wall followed proximally to the labyrinthine segment. The jugular
of the IAC is drilled when visualization of the IAC contents is bulb is then skeletonized so that it can be mobilized to afford
necessary. The tumor is then removed with a combination of greater visibility into the IAC. Extension of the bony opening
bipolar cautery, tumor forceps, ultrasonic aspiration, and should be carried until the surgeon can identify Bill’s bar and
suction. thus the facial nerve in the anterosuperior quadrant of the IAC.
Following tumor removal, the dura is closed in watertight The dura to the posterior fossa is then opened, and tumor
fashion. Mastoid air cells exposed during drilling should be resection carried out per routine.
sealed with either bone wax or bone cement to reduce the risk Closure of the translabyrinthine approach requires packing
of CSF leak. The bone flap is then replaced, and the fascia and of the inner ear defect, usually with a combination of fascia,
subcutaneous layers are closed according to surgeon’s preferred muscle, and fat. The eustachian tube can be occluded with
method. muscle or fascia, followed by insertion of the incus, which is
inserted as a plug. The remainder of the closure is then per-
Middle Fossa formed according to the surgeon’s preferred method.

The middle fossa approach to tumors of the CP angle is most Mass Lesions of the Cerebellopontine Angle
useful when tumors isolated to the IAC, as the approach poorly
visualizes the cistern of the CP angle and the brainstem. The differential diagnosis of mass lesions in the CP angle is
The patient is positioned in a manner similar to that used vast: vestibular schwannoma, meningioma, epidermoid cyst,
for the retrosigmoid approach and again fixed in the Mayfield arachnoid cyst, metastasis, vascular malformations such as a
3-point system. Neuromonitoring should be used as in the thrombosed, saccular aneurysm, exophytic brainstem gliomas,
626 PART 6 Tumors

ependymoma, trigeminal schwannoma, facial schwannoma, structures due to direct compression by the tumor. Most com-
lipoma, neurosarcoidosis, endolymphatic sac tumor, choroid monly patients present with unilateral hearing loss, though
plexus papilloma, hemangioblastoma, chordoma with dural symptoms of tinnitus, vertigo, and gait disturbances are fre-
erosion, chondrosarcoma with dural erosion, and cholesterol quent as well.21–23 Atypical symptoms are more frequent when
granuloma. Distinguishing between these can be difficult. The vestibular schwannomas reach a size greater than 3 cm and can
following section details the most common mass lesions of the include facial numbness, diplopia, and headache.24 Audiomet-
CP angle. ric evaluation of patients with vestibular schwannomas show
characteristic findings of sensorineural hearing loss in the ear
Vestibular Schwannomas ipsilateral to the tumor, with a descending pattern and loss of
high-frequency hearing first.25
Vestibular schwannomas (VSs) are benign, slow-growing neo- Radiology has greatly improved the ability to accurately
plasms arising from Schwann cells that account for approxi- diagnosis vestibular schwannomas, and MRI has become the
mately 10% of all primary brain neoplasms.10 The majority of gold standard imaging test (Fig. 43.2). VSs are isointense to
vestibular schwannomas arise from the inferior vestibular the adjacent pons and brainstem on T1-weighted MR images
nerve.11 The incidence of symptomatic VSs is approximately and slightly hyperintense on T2-weighted MR images. These
1.2 per 100,000 population, evenly divided among males and tumors enhance avidly with the administration of gadolinium
females.12,13 Routine magnetic resonance studies obtained for contrast and may contain cystic components.26 They may have
purposes other than audiovestibular symptoms or evaluation a characteristic “ice cream cone” appearance due to contain-
for vestibular schwannoma detected incidental VSs with a two- ment within the IAC and expansion in the extracanalicular
to fourfold higher incidence, indicating that a significant space. VSs tend to expand and dilate the IAC compared to a
portion of vestibular schwannomas remain asymptomatic.14,15 contralateral normal IAC. Advanced MRI techniques such as
The incidence of vestibular schwannomas in patients with diffusion tensor imaging have been shown to accurately predict
neurofibromatosis type 2 (NF2) is considerably higher than the spatial relationship between the facial nerve and tumor,
that of the general population. Up to 95% of NF2 patients thus assisting in surgical planning.27
will develop vestibular schwannomas, 90% being bilateral and Vestibular schwannomas are histopathologically char-
5% being unilateral.16 acterized by the presence of Antoni A regions and Antoni
Reviews have shown wide ranges with regard to the percent- B regions.28,29 Antoni A regions consist of uniform, densely
age of VSs that grow from 7% to 85%.17,18 This heterogeneity packed cells arranged in fascicles, whereas Antoni B regions
was decreased in a review of prospective studies where the consist of small, hyperchromatic cells with large extracellu-
estimated tumor growth frequency was 29% over a 41-month lar matrices. Verocay bodies are formed by the palisading of
period.17 The annual tumor growth rate of vestibular schwan- nuclei in Antoni A regions (Fig. 43.3). VSs from patients
nomas averages 1 to 2 mm per year across several studies; with NF2 are histologically more active with greater cellular-
however, this increases to 2 to 4 mm per year when the growth ity and an increased number of growth patterns and Verocay
rate calculation is restricted to only those tumors that grow.18–20 bodies.30
Presenting symptoms for patients with vestibular schwan- Management and decision making for vestibular schwan-
nomas are related to loss of function of adjacent neural nomas have become increasingly complex as smaller tumors

• Figure 43.2  Magnetic resonance imaging of a right-sided vestibular schwannoma. The tumor is avidly
enhancing with cystic areas and expansile of the internal auditory canal; it extends into the CPA cistern.
Postgadolinium axial T1-weighted image in the left panel, axial T2-weighted image in the center panel,
and postgadolinium axial T1-weighted image in the right panel.
CHAPTER 43  Cerebellopontine Angle Tumors 627

TABLE
43.1  House-Brackmann Facial Nerve Grading System
*
Grade Description
1 Normal facial muscle function
2 Slight asymmetry, usually unnoticeable at rest,
most identifiable in the forehead and mouth

** 3 Moderate asymmetry with preservation of eye


closure; only slight movement of the forehead
4 Moderate asymmetry, noticeable at rest, with
incomplete eye closure; forehead without
movement
5 Severe asymmetry with only slight movement of the
eyelid and mouth
6 Absent facial muscle function
• Figure 43.3  Hematoxylin and eosin preparation of a vestibular schwan-
Adapted from House JW, Brackmann DE. Facial nerve grading system.
noma specimen with Antoni A (*) and Antoni B (**) regions. Adjacent
Otolaryngol Head Neck Surg. 1985;93:146–147.
Verocay bodies are illustrated in the inset. The authors thank Knarik Arkun,
M.D., for her assistance with pathologic slides.

are more prevalent due to earlier diagnosis.31 The majority of likely to retain hearing after surgery.38,41 Tumors growing from
patients with VSs are treated in a multidisciplinary fashion the superior vestibular nerve are also less likely to cause intra-
with a combination of neurosurgeons, neuro-otologists, and operative hearing loss than those growing from the inferior
radiation oncologists.32 vestibular nerve.41
Conservative management for vestibular schwannomas has Loss of facial nerve function during vestibular schwannoma
been advocated for a larger percentage of patients with these surgery is a significant source of morbidity. Facial nerve func-
tumors. Ideal patients for whom conservative therapy is pre- tion is assessed by the House-Brackmann (HB) scale (Table
ferred are older patients with higher surgical risks, small tumor 43.1).42 The immediate postoperative HB grade is predictive
size, minimal symptom severity, and patient preference.33 of long-term function with poor initial grades being unlikely
Tumors that are not treated at the time of diagnosis can be to recover facial nerve function in the follow-up period.43 In
expected to grow in 35% to 50% of patients, remain static in patients with near-total and gross-total resections, facial nerve
size in 40% to 50% of patients, and decrease in size in the preservation is estimated in 74% of cases of large vestibular
remaining 10% to 20% of patients.19,20,34 An estimated 20% schwannomas.44 As with hearing preservation, larger tumors
to 40% of patients for whom conservative therapy is initially predispose patients to postoperative facial palsy.45 Attempts to
recommended will require microsurgery or radiosurgery in mitigate this effect have led to the option of planned subtotal
their future.20,33,35,36 Patients with worsening vertigo were more surgical resection with postoperative radiosurgery to the resid-
likely to undergo treatment due to decreased quality of life.37 ual tumor. This strategy has preserved facial nerves in greater
Some authors propose that vestibular schwannomas 2 cm or than 90% of cases without compromising tumor control rates
greater in size should be treated, as these tumors are likely to in cases of small tumors.46–48 Debate remains regarding the
continue growing.34 efficacy of subtotal resections with moderate to large residual
Surgical management of vestibular schwannomas has been tumor volumes, as concerns exist regarding the durability of
the mainstay of treatment for these tumors. Preservation of tumor control (Video 43.2).49
neurologic function is of utmost importance and includes Selection of surgical approach can be difficult and often is
hearing in those patients with serviceable hearing and facial a matter of the surgeon’s preference. Nonetheless, certain VSs
muscle function in all patients (Video 43.1). lend themselves to a particular approach. In patients with small
Hearing preservation during microsurgery for vestibular tumors isolated to the IAC, the middle fossa approach is safe
schwannomas is dependent on several factors, including pre- with a small risk of intraoperative hearing loss. The inability
operative hearing status, tumor size, and nerve of origin. to adequately visualize the CP angle cistern makes it less favor-
Approximately 40% to 55% of patients with serviceable able in large tumors. Patients with preoperative hearing loss
hearing prior to surgery will retain the same level of auditory can be treated with the translabyrinthine approach, which
function after surgery.38,39 Intuitively, surgical resection of affords excellent tumor visualization and ability to protect
smaller tumors results in less hearing loss than larger tumors.39 facial nerve function but is not suitable for patients with ser-
Hearing preservation surgery is most often successful in viceable hearing. For tumors of all sizes, the retrosigmoid
patients with small VSs (<1 cm), with rates exceeding 90% in approach is versatile in its ability to preserve both hearing and
these cases.40 Patients with better preoperative hearing are more facial nerve function.44,50–52
628 PART 6 Tumors

Meningiomas structures; and the need to resect adjacent dura all increase the
complexity of meningioma surgery in the posterior fossa.57
Meningiomas constitute the second most common tumor type Despite these obstacles, surgical resection of meningiomas has
in the CP angle, accounting for approximately 10% of CP been shown to result in fewer postoperative cranial nerve defi-
angle lesions in total. In addition to hearing loss, patients with cits than vestibular schwannoma surgery, with facial function
CP angle meningiomas also frequently present with headache preservation in upward of 90% of cases and hearing preserva-
and ataxia from cerebellar compression.53 Due to their large tion in upward of 70% of patients.58,59 Cases requiring explora-
size at the time of presentation, meningiomas frequently tion of the IAC for tumor removal carry a higher risk of cranial
present with symptoms of cranial nerve V and X dysfunction nerve deficit, a finding attributable to tumor invasion of the
(trigeminal neuralgia, facial dysesthesias, facial numbness, dif- involved nerves rather than surgical opening of the IAC.56
ficulty swallowing). Meningiomas of the CP angle are classified
by the location of their dural tail with regard to the internal Epidermoid Cysts
auditory canal. Tumors with tails anterior to the IAC are clas-
sified as premeatal, and those with tails posterior to the IAC Epidermoid cysts constitute the third most common cerebel-
are postmeatal.54 CP angle meningiomas extend into the IAC lopontine mass, accounting for 1% of all intracranial neo-
in 17% of cases, but arise from the IAC itself in a significantly plasms with 50% of these lesions located in the cerebellopontine
smaller percentage of cases (2%).55,56 angle. Usually presenting between the third and fifth decades
Meningiomas are important to differentiate from vestibular of life, epidermoid cysts are benign congenital lesions and
schwannomas on radiology studies (Fig. 43.4). Unlike vestibu- thought to be the result of abnormally migrated ectodermal
lar schwannomas, the slow-growing trait of meningiomas often cells during neural tube formation and separation. Clinically,
leads to intratumoral calcifications best seen on CT. The patients with epidermoid cysts present similar to those with
administration of gadolinium contrast agents leads to homo­ vestibular schwannomas and meningiomas, with symptoms
geneous enhancement of meningiomas with identification of related to cranial nerve dysfunction, especially trigeminal neu-
a dural tail. Hyperostosis is a common radiographic finding ralgia and hemifacial spasm, and brainstem and cerebellar
associated with meningiomas, manifested in the CP angle by compression.60,61
either hyperostosis of the temporal bone or narrowing of the Detection of epidermoid cysts is radiographically challeng-
IAC in cases of intrameatal meningiomas.26,55 ing. CT images will reveal well-circumscribed hypodense cysts
Surgical resection of CP angle meningiomas is considered that are often indistinguishable from normal cerebrospinal
more challenging than that of vestibular schwannomas. fluid.61 Epidermoid cysts are best visualized on MRI; although
Increased tumor vascularity; tumor invasion into the IAC, they have a propensity to match cerebrospinal fluid signal
jugular foramen, and other areas; adherence to neurovascular on nearly all sequences, they appear in stark contrast to

• Figure 43.4  Left-sided CPA meningioma extending from the internal auditory canal to the CPA cistern.
Postgadolinium axial T1-weighted image on the left shows a homogeneously enhancing mass. Magnified
bone-window axial CT image on the right shows intratumoral calcification and hyperostosis of the internal
auditory canal and petrous face.
CHAPTER 43  Cerebellopontine Angle Tumors 629

• Figure 43.5  Epidermoid cyst of the right CPA with compression of the brainstem and displacement
of the basilar artery (left panel; axial T2-weighted image). The tumor matches the signal of cerebrospinal
fluid on all sequences except diffusion-weighted imaging, where it is markedly hyperintense compared to
normal cerebrospinal fluid (right panel.)

cerebrospinal fluid on diffusion-weighted imaging (Fig. 43.5).


The hyperintense signal of epidermoid cysts on diffusion-
weighted MRI is the result of T2 shine-through rather than
true diffusion restriction.62
Due to recurrence rates of over 90% following subtotal
resection, surgical strategy is directed at total removal of all cyst
contents and cyst wall material without sacrificing neurologic
function.63 As epidermoid cysts have a tendency to expand the
cerebellopontine angle and grow around neurovascular struc-
tures rather than displace them, achieving complete resection
is difficult. Emerging and increasingly prevalent use of the
endoscope has enabled greater resections than were possible
with microsurgical technique alone (Video 43.3).64,65 Some
authors have proposed isolated use of the endoscope in order
to reduce brain retraction and decrease associated complica-
tions.66,67 In cases of epidermoid cysts that expand multiple
intracranial locations such as the cerebellopontine angle in the
posterior fossa, middle fossa, and basal cisterns, surgeons are
encouraged to pursue multistage procedures to obtain com-
plete resection.68

• Figure 43.6  Axial T2-weighted image of a left CPA arachnoid cyst.


Arachnoid Cysts
Arachnoid cysts are benign, developmental collections of cere-
brospinal fluid. Though they are common intracranial masses,
they rarely occur in the CP angle and are most frequently headache, nausea, ataxia, trigeminal neuralgia, facial numb-
asymptomatic (Fig. 43.6). CP angle arachnoid cysts may ness, hemifacial spasm, and hearing loss.69–71 Surgical resection
become symptomatic when they exert sufficient pressure on of the cyst and its wall have been reported to afford excellent
the brainstem, cerebellum, or cranial nerves, and obstruct clinical outcomes, with many cases of complete and near-
the normal circulation of CSF to result in compromise of complete resolution of symptoms.72 Options for fenestration
neurologic function. Common presenting symptoms include include microsurgical or endoscopic release of the arachnoid
630 PART 6 Tumors

membranes into the prepontine cistern and, in cases of recal- Radiosurgery, or stereotactic radiosurgery (SRS), can be
citrant cysts, shunting of the cyst. performed using linear accelerator (LINAC)-based systems,
Gamma Knife, or proton beam–based systems. Though the
Metastatic Tumors principle of administering high-dose radiation to an intracranial
target is the same across the three techniques, the specific
Metastatic involvement of the CP angle from primary tumors protocols differ with regard to patient immobilization, image
distant from the central nervous system, though rare, can lead planning, and radiation dose delivery. Proton beams provide
to significant neurologic compromise. Clinical and radio- the best control of penetration depth compared to either gamma
graphic evaluation of patients with metastatic lesions in the CP or photon beams. With Gamma Knife therapy, 201 collimated
angle can be misleading, with initial diagnostic studies being beams of gamma radiation derived from Co-60 are focused
consistent with tumors such as schwannomas.73,74 Due to the precisely on the lesion of interest. This creates a localized radia-
invasiveness of metastases leading to early neural compression, tion field with relative sparing of healthy surrounding tissue.
symptom onset in these patients is more rapid than that of The LINAC system uses a linear accelerator as its source of
more common lesions in the CP angle.75,76 The aggressive electrons, but it has fewer beams than the Gamma Knife.
nature of metastatic lesions in addition to the morbidity caused Since Leksell first utilized Gamma Knife radiation therapy
by the primary tumor portends the poor prognosis in the for the treatment of an acoustic neuroma in 1969, Gamma
majority of these patients.77 Knife radiosurgery has emerged and evolved into an important
treatment option for patients with small to moderate-sized
acoustic neuromas owing to its excellent outcomes and prefer-
Radiosurgery for Cerebellopontine ence among patients over microsurgery. Gamma Knife radio-
Angle Masses surgery is presently the most commonly utilized treatment
method for acoustic neuromas up to 3 cm.
The history of cerebellopontine angle surgery is rich with Although interstudy variability with regard to definition of
advances in diagnostic and surgical technology. These efforts tumor control makes concrete generalization somewhat diffi-
have culminated in the current approach to treatment, of cult, Gamma Knife therapy consistently demonstrates equal to
which stereotactic radiosurgery is a mainstay. Radiosurgery was or greater than 94% effectiveness in achieving “tumor control”
first utilized for the treatment of CPA masses in 1969 when that is defined as no need for further radiation treatment or
Lars Leksell employed radiosurgery for the treatment of an surgical resection.79 Furthermore, Gamma Knife therapy has
acoustic neuroma. The first patient to undergo Gamma Knife consistently demonstrated excellent results with regard to the
radiosurgery was a young, female patient with NF2. Tumor preservation of facial and trigeminal nerve function. In a 2009
control was achieved for the following 12 years, at which point meta-analysis of 2204 patients undergoing Gamma Knife
CT revealed medial growth and the tumor was surgically radiosurgery for acoustic neuromas, significant facial nerve
resected.78 Advancements in imaging techniques following the dysfunction was exhibited in 3.8% of cases, with multiple
first attempted radiosurgical treatment of a CPA mass have studies reporting rates between 0 and 3%.80
allowed for increasingly accurate localization in addition to The goals of Gamma Knife treatment of acoustic neuro-
improved delivery of radiation dose. These have led to improved mas are fourfold: (1) arrest tumor growth, (2) preserve facial
tumor growth control and reduced unwanted radiation- nerve function, (3) preserve trigeminal nerve function, and (4)
associated tissue damage. Further amendments to dosing mea- preserve hearing. Hearing preservation remains the sobering
surements coupled with the advent of high-resolution MRI outcome with respect to the otherwise high rates of success
have minimized complications associated with radiosurgery for Gamma Knife treatment. Among 4234 patients included
and given rise to excellent long-term arrest of CPA tumor in a systematic review with an average recorded follow-up time
growth. of 44.4 months, overall hearing preservation neared 51%.
Stereotactic radiosurgery involves the precise delivery of a However, when patients were further characterized based on
single, high dose of radiation to a target within a closed-skull, radiation dose, hearing preservation rate climbed to 60.5%
single-treatment session. The therapy can be used to treat intra- for those receiving less than 13 Gy.81 Additional studies have
cranial tumors that are otherwise surgically inaccessible and supported this finding, namely that preservation of hearing
may be either the primary treatment method or an adjunctive is significantly tied to preprocedure hearing status and the
therapy to surgical resection. The timeline for treatment effi- radiation dose delivered. Radiation doses of less than 13 Gy
cacy for radiosurgery depends on tumor type; slow-growing to treat patients with better preprocedure Gardner-Robertson
pathologies such as vascular malformations and schwannomas hearing class is associated with the highest rates of hearing
respond more slowly, whereas fast-growing pathologies such as preservation.82
metastatic lesions respond more quickly. Hence, stereotactic Though somewhat controversial still, the available evidence
radiosurgery may be an inappropriate treatment option for to date suggests that radiosurgery is the best available treatment
patients presenting with profound clinical symptoms or dis- for solitary vestibular schwannomas up to 30 mm in cisternal
comfort owing to mass effect from their intracranial mass. In diameter.83 Although microsurgery achieves similar success
such cases, surgical resection is often the necessary first-line rates with regard to tumor control and cranial nerve preserva-
treatment. tion, Gamma Knife radiosurgery evades some of the inherent
CHAPTER 43  Cerebellopontine Angle Tumors 631

dangers associated microsurgery including the possibility for Breivik CN, Varughese JK, Wentzel-Larsen T, et al. Conservative man-
surgical hemorrhage or anesthetic complications. The question agement of vestibular schwannoma–a prospective cohort study: treat-
as to whether Gamma Knife radiotherapy will become the ment, symptoms, and quality of life. Neurosurgery. 2012;70:1072-1080,
optimal first-line treatment for larger acoustic neuromas con- discussion 1080.
House JW, Brackmann DE. Facial nerve grading system. Otolaryngol
tinues to be explored.
Head Neck Surg. 1985;93:146-147.
Matsushima K, Yagmurlu K, Kohno M, et al. Anatomy and approaches
Selected Key References along the cerebellar-brainstem fissures. J Neurosurg. 2016;124:248-263.
Safavi-Abbasi S, Di Rocco F, Bambakidis N, et al. Has management of
Abolfotoh M, Bi WL, Hong CK, et al. The combined microscopic- epidermoid tumors of the cerebellopontine angle improved? A surgi-
endoscopic technique for radical resection of cerebellopontine angle cal synopsis of the past and present. Skull Base. 2008;18:85-98.
tumors. J Neurosurg. 2015;123:1301-1311. Schmidt RF, Boghani Z, Choudhry OJ, et al. Incidental vestibular
Boari N, Bailo M, Gagliardi F, et al. Gamma Knife radiosurgery for schwannomas: a review of prevalence, growth rate, and management
vestibular schwannoma: clinical results at long-term follow-up in a challenges. Neurosurg Focus. 2012;33:E4.
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