Interdisciplinary Neurosurgery 19 (2020) 100577

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Interdisciplinary Neurosurgery
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Review Article

Presentation and surgical management of a WHO grade II meningioma of T

the cerebellopontine angle: A case report and review of the literature
⁎
Joel Kayea, , Ahmed Meleisb, Shabbar Danisha, Zhenggang Xiongc
a
Department of Neurosurgery, Rutgers-Robert Wood Johnson Medical School, 10 Plum St., New Brunswick, NJ 08901, USA
b
Department of Neurological Surgery, Rutgers-New Jersey Medical School, DOC Suite 8100, Newark, NJ 07103, USA
c
Department of Pathology and Laboratory Medicine, Rutgers-Robert Wood Johnson Medical School and University Hospital, 1 Robert Wood Johnson Place, New
Brunswick, NJ 08901, USA

A R T I C LE I N FO A B S T R A C T

Keywords: Meningiomas are relatively slow-growing, typically benign tumors that arise from the arachnoid cells which
Meningioma form the meninges. They are the most common primary brain tumor and account for nearly one-third of all
Atypical meningioma primary brain and spine tumors. Meningiomas can arise from any dural site, however they are infrequently
WHO grade II found at the cerebellopontine angle (CPA). While WHO grade I meningiomas are benign and the slowest
Stereotactic radiosurgery
growing, WHO grade II meningiomas grow significantly faster, display cellular atypia, and frequently recur.
Cerebellopontine angle
Vestibular schwannoma
Compared with WHO grade I meningiomas, these often present a therapeutic challenge. An 87-year-old woman
presented to the emergency department with a two-month history of dizziness and dysequilibrium. MRI revealed
a large left-sided CPA lesion which was not present on MRI just two years prior. She underwent surgical resection
and post-operative imaging confirmed gross-total resection. Surgical histology identified the tumor as an aty-
pical WHO grade II meningioma. WHO grade II meningiomas of the CPA are exceedingly rare entities, but should
be considered in patients who present with a rapidly growing CPA mass. The literature supports the notion that
complete resection, when possible, is likely the strongest determinant of overall control. Stereotactic radio-
surgery (SRS) has established itself as a suitable adjunct for higher grade lesions of the CPA, particularly those
that were resected subtotally. These tumors can be successfully managed, even in the elderly population.

1. Introduction known as anaplastic or malignant meningiomas, are the most ag-

Meningiomas are relatively slow-growing, typically benign tumors
that arise from the arachnoid cells, which form the meninges. They are
the most common primary brain tumor and account for nearly one-third
of all primary brain and spine tumors [4]. Meningiomas can arise from
the dura at any site; most commonly the skull vault, from the skull base,
and at sites of dural reflections [6]. Less commonly, they arise from the
cerebellopontine angle (CPA). CPA meningiomas account for as low as
1% of all intracranial meningiomas, however some sources cite this
number as high as 10% [1,2].
Meningiomas are further designated into one of three categories
based on their morphologic and histologic features in accordance with
the World Health Organization (WHO) system. WHO grade I me-
ningiomas are benign and the slowest-growing. In contrast, WHO grade
II meningiomas grow significantly faster and display cellular atypia.
These confer a less favorable outcome; 10-year survival is less than 80%
with recurrence rates as high as 50%. WHO grade III meningiomas, also
gressive [3,5,17]. With the adoption of the revised 2007 WHO grading
system, grade I meningiomas account for 65–80% of all newly diag-
nosed meningiomas, grade II account for 20–35%, and grade III account
for < 3%. For decades prior to the 2007 revision of the WHO grading
system, only 5% of meningiomas were identified as WHO grade II. With
this major epidemiological change in the incidence of WHO grade II
meningiomas, a redefining of its natural history and management
strategies is warranted [23].
We present a case report of a patient who was found to have, and
subsequently treated for, a large, rapidly-growing WHO grade II me-
ningioma of the CPA. The literature is limited with respect to the fea-
tures, management, and outcomes of WHO grade II meningiomas of the
CPA. This case report and review will contribute to the existing lit-
erature on WHO grade II meningiomas of the CPA, with special atten-
tion to its natural history, management, and outcomes.

⁎
Corresponding author.
E-mail address: kayej1@rwjms.rutgers.edu (J. Kaye).

https://doi.org/10.1016/j.inat.2019.100577
Received 24 March 2019; Received in revised form 22 July 2019; Accepted 26 August 2019
2214-7519/ © 2019 Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/BY-NC-ND/4.0/).

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J. Kaye, et al.
Fig. 1. Pre-operative axial MRI. T1 (Left) and T2 (Right) showing the left CPA
lesion compressing the brainstem and cerebellum with effacement of the 4th
ventricle.
2. Case presentation
An 87-year-old female with a past medical history of hypertension,
type II diabetes mellitus, hyperlipidemia, and hypothyroidism pre-
sented to the emergency department with a 2-month history of dizzi-
ness and unsteadiness. Over this time period, she had the sensation that
the room was spinning, and that the ground was constantly moving
underneath her legs. She had multiple falls over this time period. At
baseline, she is independent and can perform all activities of daily
living. She denied headaches, changes in vision, hearing loss, or facial
weakness. Initial workup included an MRI of the brain which showed a
large (4.3 cm × 3.4 cm × 3 cm) left CPA lesion (Fig. 1) that was com-
pressing the brainstem and cerebellum. The mass was not present on an
MRI obtained 2 years prior. Subsequent comprehensive medical workup
failed to reveal a primary source. Management options were discussed
with the patient, and the decision was made to proceed with surgery.
3. Surgical approach
The patient underwent a left retrosigmoid craniotomy for resection
of the tumor. The tumor was located along the left CPA and was pressed
up against the tentorium. It was purple in color, soft, and notably
vascular. The tumor capsule was in contact with one nerve at its inferior
anterior portion and another nerve at its superior anterior portion. We
stimulated and identified these as the facial and trigeminal nerves, re-
spectively. The facial nerve fibers were slightly splayed by the tumor,
but otherwise the nerve was in continuity in its course from the
brainstem to the internal auditory canal. There were no nerves coursing
within the tumor. The resection itself was fairly bloody. There were no
intra-operative complications and a post-operative MRI confirmed
gross-total resection (Fig. 2). Post-operatively the patient had no facial
weakness, numbness, or other lower cranial nerve palsies. She did
complain of some residual vertigo and remained unstable with gait. She
was discharged to a rehabilitation facility on post-op day #4. Upon
histopathological analysis, the tumor exhibited increased cellularity,
spindle shapes cells with high N/C ratio, sheeting growth, and focal
hemorrhage with ischemic necrosis (Fig. 3). This confirmed the diag-
nosis of an atypical WHO grade II meningioma with an angiomatous
component.
4. Discussion
Meningiomas are the most common primary intracranial tumor.
Incidence is highest after the fifth decade of life and they are at least
twice as common in females as they are in males [6]. Although many
meningiomas are asymptomatic and found incidentally, symptoms,
when present, vary and depend on both the location of the mass and the
time course over which the tumor grows. Overall, the most common
presenting symptoms are headache and seizures [7]. Characteristic
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Interdisciplinary Neurosurgery 19 (2020) 100577
focal impairments depend on the specific location of the tumor.
Treatment modalities include observation, surgery, radiotherapy, or a
combination; individualized treatment plans depend on the sympto-
matology and goals of treatment [2].
Meningiomas can be further classified based on their location and
histological grade. They can arise from the dura at any site, most
commonly from the skull base and at sites of dural reflections.
Meningiomas of the cerebellopontine angle (CPA) are relatively rare,
comprising between 1 and 10% of all meningioma cases [1,2]. Despite
their low incidence, meningiomas are the second most common tumor
of the CPA behind schwannomas, which account for 70–90% of all CPA
tumors [2]. Histologically, meningiomas are classified in accordance
with the World Health Organization (WHO) system as WHO I, which
are benign (65–80%), WHO II, which are atypical (20–35%), and WHO
III, which are anaplastic or malignant (< 3%). Growth rates of me-
ningiomas correspond with their WHO grade [8–10], with WHO grade
II and III meningiomas displaying higher volumetric growth rates
(VGR) and shorter doubling times than those seen in grade I me-
ningiomas [8]. Average VGRs of WHO grade II meningiomas (6.40 cm3/
year—23.3 cm3/year) were significantly higher than those of WHO
grade I meningiomas (1.34 cm3/year—1.51 cm3/year) in both retro-
spective and prospective studies [9,10].
We reported a case of a large, rapidly-growing, WHO grade II me-
ningioma of the CPA. The combination of features seen in this case
make it rather unique. Although there is incidence data on both CPA
meningiomas and WHO grade II meningiomas, we could find no men-
tion of incidence data on WHO grade II meningiomas of the CPA in the
literature. To try and better quantify this, we pooled data from three
studies [19–21] that each utilized a cohort of consecutive meningioma
cases over a given time period at that respective institution, and each
with information about the location and histology of the tumors. Out of
the 955 total cases, only 2 (0.2%) were confirmed WHO grade II me-
ningiomas of the CPA. An additional 5 cases were WHO grade II me-
ningiomas of the posterior fossa, but their specific locations were not
revealed. Assuming these patient sets are representative of the general
population, the incidence of WHO grade II meningiomas of the CPA
appears to be safely less than 1% of all meningioma cases. Furthermore,
upon its discovery on MRI, the tumor volume in the present case was
approximately 44 cm3, yielding a volumetric growth rate of, at the very
least, 22 cm3/year. This conservative estimate falls along the upper
limit of reported growth rates of grade II meningiomas [9,10].
Meningiomas of the CPA can present similarly to vestibular
schwannomas (VS), with a few notable distinguishing features. Facial
pain, facial spasm, and dysesthesias, which are rarely seen with VS,
occur in up to 30% of patients with CPA meningiomas. Cerebellar signs
are common with CPA meningiomas, but are rarely seen with VS.
Lastly, audio-vestibular symptoms (hearing loss, tinnitus, and dis-
equilibrium), while relatively common with both conditions, are less
frequent and of shorter duration with CPA meningiomas than they are
in VS [1,2].
An asymptomatic CPA meningioma can be followed with serial MRI
scans to assess for growth. This approach may be used in patients with
advanced age or comorbidities that preclude surgery; radiosurgery may
also be considered in this population. If surgical resection is planned,
the goal is maximum removal of the tumor with wide excision of the
dural attachment, avoiding injury to involved cranial nerves, and re-
section of hyperostotic bone which can contain infiltrative tumor.
Incomplete resection is associated with higher recurrence rates, parti-
cularly among grade II and III meningiomas [11]. The most common
surgical approach is the retrosigmoid craniotomy, which offers access
to the CPA when hearing preservation is the goal. Other surgical ap-
proaches include the translabyrinthine and middle fossa craniotomies;
combined approaches may also be utilized depending on the tumor
location2.
Outcomes from surgical resection of CPA meningiomas vary de-
pending on the tumor’s specific location within the CPA, size, and
J. Kaye, et al. Interdisciplinary Neurosurgery 19 (2020) 100577

Fig. 2. Post-operative T1 axial MRI with gadolinium showing an absence of residual enhancement in the surgical cavity. Compression on the brainstem and
cerebellum is significantly improved.

relationship to critical anatomy. Reported rates of gross total resection
(GTR) range from 45% to 86%. Post-operatively, patients experience
permanent facial weakness (6%–11%), swallowing problems
(2%–12%), and hearing decline (9%–17%). Mortality ranges from 0%
to 5% [2]. In a 2013 series, outcomes from surgical resection of CPA
meningiomas in 34 patients were reviewed at a single institution. Three
tumors (8.8%) were WHO grade II. GTR was achieved in 19/34
(55.9%). New cranial nerve (CN) deficits occurred in 12/34 (35.3%),
two-thirds of which were permanent. The most common CN deficit was
that of CN X. Tumor progression occurred in three patients (8.8%), two
of whom had WHO grade II tumors. In all three cases of tumor pro-
gression, resection during the primary procedure was subtotal [22]. In
another series from 2017, He et al. looked at treatment outcomes in 53
patients with CPA meningiomas. Of the 53 patients, 49 were treated via
retrosigmoid approach and 4 with gamma-knife stereotactic radio-
surgery. Three tumors (5.7%) were WHO grade II. GTR was achieved in
41/49 (83.7%). The most common post-operative disturbances were
facial numbness, facial palsy, and hearing loss. Tumor recurrence oc-
curred in three patients (6.1%), two of whom had WHO grade II tumors
[13]. In the two aforementioned series’, 6/87 (6.9%) patients

Fig. 3. This tumor exhibits increased cellularity

with high N/C ratio and sheeting growth pattern (A,
H + E 400x), focal ischemic necrosis (B, H + E
400x), hemorrhage (C, H + E 400x), and angioma-
tous component (D, H + E 200x). The tumor cells
are immunologically positive for EMA (E, 400x), PR
(F, 400x), and with relatively high proliferative
index (G, Ki67 400x).

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J. Kaye, et al.
experienced tumor recurrence. Of the six total instances of recurrence,
four (66.7%) were WHO grade II, and at least three (50%) underwent
subtotal resection during the primary procedure. This reinforces a) the
critical importance of GTR in WHO grade II meningiomas of the CPA,
and b) the need for continued investigation into the utility of adjuvant
treatment modalities, such as stereotactic radiosurgery.
While attempts at GTR remain the mainstay of treatment for CPA
meningiomas, stereotactic radiosurgery (SRS) is another option which
has demonstrated excellent long-term control and a low side-effect
profile2. Although the literature clearly supports the utility and effec-
tiveness of SRS for WHO grade I CPA meningiomas [14,24], there have
been no studies on SRS for WHO grade II CPA meningiomas, pre-
sumably due to the few number of cases reported. In general, the lit-
erature is somewhat conflicting regarding the role of adjuvant SRS in
the management of WHO grade II meningiomas. In 2013, Hasan et al.
found that adjuvant SRS did not significantly affect tumor recurrence
rates or progression-free survival in patients with WHO grade II me-
ningiomas. Instead, only GTR was predictive of progression-free sur-
vival [15]. However, more recent studies suggest a more promising role
for adjuvant radiotherapy. A 2017 series by Dohm et al. looked at
outcomes after early or late radiotherapy following surgical resection of
WHO grade II meningiomas. They found that adjuvant radiotherapy
improved overall survival in patients with WHO grade II meningiomas
compared to surgery alone, and that early adjuvant radiotherapy im-
proved progression-free survival [16]. More recently, Budohoski et al.
found that adjuvant radiotherapy after surgical resection of WHO grade
II meningiomas was independently associated with a reduced rate of
early recurrence. Interestingly, their subgroup analysis revealed that
the benefits of adjuvant radiotherapy were only seen after subtotal
resection, and not after GTR, suggesting a less useful role of prophy-
lactic adjuvant radiotherapy [17]. In fact, the risks of radiotherapy may
outweigh the benefits in certain settings, as radiotherapy-induced ma-
lignant transformation, though rare, has happened. A 2017 review
found that 7/316 (2.2%) of meningiomas treated with SRS underwent
malignant transformation at a median of 4.9 years [18]. Therefore,
watchful waiting may be appropriate for patients after GTR of WHO
grade II meningiomas without evidence of re-growth. Clearly, the role
of adjuvant radiotherapy for WHO grade II meningiomas is not fully
established and is currently the focus of a major prospective trial.
In our case, an 87-year-old woman with 2 months of dizziness was
found to have a large left CPA lesion which was not present on MRI
2 years earlier. Histology confirmed a grade II (atypical) meningioma.
Due to its location, we initially speculated this to be a schwannoma;
however, the tumor’s rapid rate of growth (at least 22 cm3/year) and
patient’s advanced age would have been highly atypical for schwan-
noma (median age 50, VGR 0.19 cm3/year) [12]. In an older patient
with a rapidly growing tumor of the CPA, meningioma should be con-
sidered on the differential diagnosis when formulating a treatment
paradigm. The aggressive nature of atypical meningiomas underscores
the importance of complete resection and delineates a clear goal for the
neurosurgical team.
5. Conclusion
WHO grade II meningiomas of the CPA are exceedingly rare entities.
In cases where patients present with a rapidly growing CPA mass, this
should be considered in the differential diagnosis. The literature sup-
ports the notion that complete resection, when possible, is the strongest
determinant of overall control. SRS has established itself as a suitable
adjunct for higher grade lesions of the CPA, particularly those that were
resected subtotally. These tumors can be successfully managed, even in
the elderly population.
Declaration of Competing Interest
The authors declare that they have no known competing financial
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Interdisciplinary Neurosurgery 19 (2020) 100577
interests or personal relationships that could have appeared to influ-
ence the work reported in this paper.
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