British Journal of Neurosurgery

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Mature cystic teratoma of the right

cerebellopontine angle: a rare case report

Reshma Sattar, Vishwaraj Ratha, Suresh Bapu R. Kandallu, Sunil Kapilavayi,

Nishanth Sampath, Vijay Sankaran & Partheeban Balasundaram

To cite this article: Reshma Sattar, Vishwaraj Ratha, Suresh Bapu R. Kandallu, Sunil Kapilavayi,
Nishanth Sampath, Vijay Sankaran & Partheeban Balasundaram (19 Aug 2021): Mature
cystic teratoma of the right cerebellopontine angle: a rare case report, British Journal of
Neurosurgery, DOI: 10.1080/02688697.2021.1967287

To link to this article: https://doi.org/10.1080/02688697.2021.1967287

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BRITISH JOURNAL OF NEUROSURGERY
https://doi.org/10.1080/02688697.2021.1967287

ORIGINAL ARTICLE

Mature cystic teratoma of the right cerebellopontine angle: a rare case report
Reshma Sattar , Vishwaraj Ratha, Suresh Bapu R. Kandallu , Sunil Kapilavayi, Nishanth Sampath, Vijay Sankaran
and Partheeban Balasundaram
Department of Neurosurgery, Institute of Neurosciences, SRM Institute for Medical Science, Chennai, India

ABSTRACT ARTICLE HISTORY

Background and importance: Intracranial mature cystic teratomas are benign neoplasms that commonly Received 29 December 2020
occur at the midline. Mature cystic teratomas at the cerebellopontine (CP) angle are very rare. They are Revised 17 May 2021
unique germ cell tumours curable by safe total surgical resection and have good prognosis. This case Accepted 9 August 2021
report documents the clinical, radiological, histological features and operative findings of mature cystic
KEYWORDS
teratoma at CP angle. Mature cystic teratoma;
Clinical presentation: We present a rare case of a mature cystic teratoma at the CP angle in a 24-year-old cerebellopontine angle;
woman who presented with brainstem compression and cranial nerve deficits. Brain MRI showed atypical intracranial tumour
findings like hyperintense areas in both T1 and T2 weighted images, calcification and diffusion restriction
in part of the lesion. She underwent near total resection of the tumour via right retrosigmoid approach.
Intraoperatively, the lesion was intra-arachnoidal unlike schwannomas and the cyst contained sebum-like
material, fibrous areas with calcification which are unusual features of common CP angle tumours.
Histopathological examination showed well differentiated mature tissues from all three germinal layers
and confirmed the diagnosis of a mature cystic teratoma arising from the right CP angle. Patient had
good outcome with neurologic recovery.
Conclusions: Mature cystic teratoma is a rare clinical entity and should be considered in patients with CP
angle tumours when there are atypical findings in brain MRI imaging. Cysts with sebum-like material,
fibrous areas with calcification and poor tumour-arachnoid plane intraoperatively strongly suggest the
possibility of mature cystic teratoma.

Introduction
Cerebellopontine (CP) angle tumours account for 5–10% of all
intracranial tumours.1 Most common tumours are vestibular
schwannomas, meningiomas and epidermoids. Mature cystic ter-
atomas of the central nervous system are rare and represent
approximately 0.5% of all intracranial tumours.2 They may occur
at any age, but a higher incidence is seen in young patients.3
They are cystic or occasionally solid tumours consisting of well
differentiated tissues arising from all three germinal layers: endo-
derm, mesoderm, and ectoderm.4 Being developmental inclusion
tumour, intracranial teratomas are most often located at the mid-
line – in the pineal region (50%) and suprasellar region (30%).5
Mature cystic teratomas at the CP angle are very uncommon.6
Clinical presentation is similar to other common tumours at CP
angle. Though radiological features often give clue to pathological
diagnosis preoperatively, histopathological examination is
required in many cases. We report a rare case of a mature cystic
teratoma arising primarily within the right CP angle in a young
adult female.
Case report
A 24-year-old female presented with a 1-month history of grad-
ually progressive hearing loss in the right ear and imbalance on
walking. She also gave 10-day history of early morning headache
associated with vomiting, giddiness, and regurgitation of food.
A neurological examination revealed horizontal gaze nystag-
mus, impaired corneal reflex, reduced sensations in the region of
V2–V3 distribution, sensorineural hearing loss, decreased palatal
movements – all on the right side and cerebellar ataxia. Brain
magnetic resonance imaging (MRI) revealed a well-defined,
extra-axial mass measuring 4.7  3.7  3.6 cm at the right CP
angle. Lesion crossed the midline; extended to the left prepontine
cistern with significant compression of the pons, right cerebellar
hemisphere, and fourth ventricle; displaced the right middle cere-
bellar peduncle towards left; and encased the basilar artery and
its branches. It was hyperintense on T1- and T2-weighted imag-
ing suggestive of subacute bleed or white epidermoid (Figure
1(A)). Central hypointense area suggested the rare possibility of
partly thrombosed aneurysm (Figure 1(B)). Calcification was seen
on susceptibility weighted imaging (Figure 1(C)). Restricted dif-
fusion was observed in a part of the lesion with correlative fea-
tures on apparent diffusion coefficient imaging (Figure 1(D,E)).
A small nodular enhancement pointed against a diagnosis of epi-
dermoid on post contrast subtraction image (Figure 1(F)). In
view of these atypical findings, digital subtraction angiography
(DSA) was done to exclude vascular lesions. DSA showed no evi-
dence of vascular lesion and the tumour was relatively avascular.

CONTACT Suresh Bapu R. Kandallu krsureshbapu@gmail.com Department of Neurosurgery, Institute of Neurosciences, SRM Institute for Medical Science, No.
1, Jawaharlal Nehru Road, Vadapalani, Chennai 600026, Tamil Nadu, India
ß 2021 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group
This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License (http://creativecommons.org/licenses/by-nc-nd/4.0/),
which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited, and is not altered, transformed, or built upon in any way.
2 R. SATTAR ET AL.

Figure 1. (A–F) Brain magnetic resonance image showing an axial. (A) T1 pre contrast – hyperintense with areas of hypointensity and coronal. (B) T2-hyperintense
with central hypointense extra-axial lesion at the right cerebellopontine angle with significant compression of the pons and right cerebellar hemisphere. Axial (C) sus-
ceptibility weighted image showing calcification and (D) shows diffusion restriction in part of the lesion with (E) apparent diffusion coefficient image showing correla-
tive features. Axial (F) post contrast subtraction image – shows enhancing nodule.

Considering the large size of the tumour and significant mass
effect, microsurgical excision was performed using total intraven-
ous anaesthesia. Intraoperative neuromonitoring for 5th, 7th and
10th cranial nerves was done with triggered electromyography
(EMG) and corticobulbar motor evoked potentials for 7th cranial
nerve were recorded intraoperatively. A right retrosigmoid suboc-
cipital craniotomy was performed using microsurgical techniques,
and a cystic mass with a thin, translucent wall was found at the
CP angle cistern. Cysts containing thick sebum like content were
evacuated, maximum excision of cyst wall was done and sent for
histopathological examination. Though sebum like material
(Figure 2(A)) was suggestive of epidermoid, there were fibrous
solid areas with calcification (Figure 2(B)). There was no evi-
dence of hemorrhage at surgery, though it was suspected in brain
MRI preoperatively. The 5th nerve, facial nerve and the lower
cranial nerves were preserved during the excision of cystic mass
and this was verified by triggered EMG using direct nerve stimu-
lation at root entry zones. The basilar and right vertebral arteries
were seen and preserved. Unlike vestibular schwannoma, the
tumour was located within the subarachnoid space along with
vessels and nerves (Figure 2(C)), tumour-arachnoid plane was ill
defined and it required sharp dissection. Small portions of cap-
sule adherent to anterior inferior cerebellar artery and cranial
nerves were left in situ. However, the tumour could be easily sep-
arated from the brainstem.
Histopathological analysis of the excised mass revealed well
differentiated tissues from all three germinal layers: a fibrocollag-
enous cyst wall lined with hyperkeratotic stratified squamous epi-
thelium, fragments of the cyst wall lined by ciliated columnar
epithelium (Figure 3(A,B)), along with the foam cell reaction,
cholesterol clefts and inflammatory granulation tissue. The
patient’s postoperative course was uneventful and she gradually
improved. The 1-year follow-up MRI revealed no evidence of a
residual or recurrent lesion (Figure 4(A,B)). She had no neuro-
logical deficits during her follow-up visit.
Discussion
Mature cystic teratomas of the CP angle are very rare with only
two cases reported in the literature. The first case of CP angle
mature teratoma was reported in a 6 and half year-old child at
pre-MRI era.7 Second case of mature teratoma at CP angle has
been reported in a 70-year-old female in whom the imaging fea-
tures suggest the epicentre of the tumour was behind the CP
angle proper.8 The author found that, part of the capsule was
 BRITISH JOURNAL OF NEUROSURGERY 3

Figure 2. (A–C) Intraoperative image showing (A) cysts containing thick sebum like content (black arrow). (B) Calcification (black arrow). (C) Tumour and vessels at
same subarachnoidal plane.

Figure 3. (A) 40 magnification view of a haematoxylin- and eosin-stained section of the resected lesion. The arrow head shows fibrocollagenous cyst wall focally
lined by ciliated columnar epithelium, and the long black arrow displaying cholesterol clefts. (B) 40 magnification view of a haematoxylin- and eosin-stained section
of the resected lesion. The short arrow showing a cyst wall lined by stratified squamous epithelium with luminal keratinous material.

Figure 4. (A,B) Follow-up brain magnetic resonance imaging showing no evidence of a residual or recurrent lesion.

tightly attached to and compressed the occipital bone intraopera-
tively. In our case, mature cystic teratoma was located primarily
at CP angle cistern and this is the first case report in young adult
female to our knowledge.
At our hospital, records of CP angle tumours operated
between the year 2014 and 2020 were analysed; a total of 167 CP
angle tumours were operated, of which 138 were schwannomas,
15 were meningiomas, nine cases of epidermoids, five miscellan-
eous and only one patient had mature cystic teratoma. Malignant
teratomas arising primarily from CP angle have been reported.6,9
A 11-year long period study of 43 cranio-spinal teratomas
reported eight intracranial mature teratomas but none was in CP
4 R. SATTAR ET AL.
angle.10 Teratomas arising primarily from petrous bone extending
to CP angle region have been reported.11,12
Most teratomas are located at the midline and develop along
the pineal-suprasellar axis.13,14 Occasionally, non-midline loca-
tions are also reported in the cerebral hemispheres, lateral ven-
tricle, basal ganglia, temporal bone, orbit, Sylvian fissure,
cavernous sinus, thalamus, and medulla oblongata.15 Only a few
mature cystic teratomas occur exclusively at extra-axial locations
such as the CP angle.16 Our patient had no intra-axial involve-
ment, as the pia-arachnoid over brainstem and cerebellum was
intact. This suggests that our patient had an extra-axial mature
cystic teratoma arising primarily from the right CP angle cistern.
Mature cystic teratomas are unique germ cell tumours that
are curable by surgical excision and have a good prognosis.
Clinical symptoms appear when the tumour expands causing
mass effect. Our patient’s lesion compressed the brainstem, right
cerebellar hemisphere, fourth ventricle and right 5th, 8th and
10th cranial nerves.
In our patient, brain MRI showed the tumour was mostly
hyperintense in both T1- and T2-weighted images. We also
observed area of calcification, nodular enhancement and diffu-
sion restriction within part of the lesion. Pre-operatively, the pos-
sibility of partly thrombosed aneurysm was considered; however,
DSA excluded it. As there was no tumour extension to the
internal auditory meatus, it was presumed preoperatively as
non-acoustic tumour of CP angle. The diagnosis of teratoma was
considered intraoperatively by finding of cyst with sebum-like
material and fibrous areas with calcification and it was confirmed
by histopathological examination. Near-total excision was
achieved and confirmed by postoperative brain MRI imaging.
She has been advised periodic follow up MRI to ensure there is
no recurrence.
Mature teratomas are chemo- and radio-resistant,17 and
malignant transformation is very rare. Treatment of choice is
safe total surgical resection18 at the first surgery. Revision sur-
geries for residual tumours are likely to be more challenging
compared to schwannomas in view of ill-defined tumour-arach-
noid plane. In our case, near total resection was achieved with
good outcome.
Conclusions
Mature cystic teratoma is a rare clinical entity and should be
considered in patients with CP angle tumours when there are
atypical findings in brain MRI imaging. Cysts with sebum-like
material, fibrous areas with calcification and poor tumour-arach-
noid plane intraoperatively strongly suggest the possibility of
mature cystic teratoma.
Acknowledgements
The authors thank Mr. Haribabu Arumugam for helping us acquire the full-
text articles referenced in this manuscript.
Informed consent
Written informed consent was obtained from the patient for this publication.
Disclosure statement
The authors report no conflict of interest.
ORCID
Reshma Sattar http://orcid.org/0000-0002-2055-5068
Suresh Bapu R. Kandallu http://orcid.org/0000-0002-7204-4921
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