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Case Report

Huge supratentorial cortical ependymoma in a

young child: case report and literature review

Mehdi Darmoul, Mohamed Kilani, Atef Ben Nsir, Mohamed Nejib Hattab

Neurosurgery Department, Fattouma Bourguiba University Hospital, 5000 Monastir, Tunisia.
A B S T R AC T
Supratentorial cortical ependymomas are uncommon in the pediatric population and extremely rare in very young children. Histologically,
tumors of the anaplastic type are also less common in children. The authors report one case of anaplastic cortical ependymoma in a
16-month-old girl who presented with a 7-day history of left side weakness and rapid neurological deterioration. Brain imaging with computed
tomography and magnetic resonance imaging scanning showed a huge right fronto-parietal cystic and solid lesion compressing the brain
parenchyma. The young child was operated via a transparietal approach with gross total resection of the lesion. The tumor’s histology was
anaplastic ependymoma. Intensive chemotherapy was given post operatively and the patient remained well without recurrence after 20
months of follow-up.

Key words: Cortical ependymoma; young child; surgery; chemotherapy; prognosis

INTRODUCTION scanning revealed a huge right fronto-parietal cystic-
solid lesion mesearing 76 mm × 70 mm × 70 mm. It
Ependymomas are rare neuroectodermal tumors arising was slightly hyperdense in its solid component, with
from ependymal cells of the ventricular system, choroid thin calcifications and was well-demarcated from
plexus, filum terminale or the central canal of the brain parenchyma [Figure 1]. During hospitalization,
spinal cord. They account for 1.2-7.8% of all intracranial the patient had a focal seizure followed by rapid
neoplasms. [1-3] Pure supratentorial cortical ependymomas deterioration in consciousness and right pupillary
(CE) are uncommon. To our knowledge, 49 cases of CE have dilatation. The patient was transferred immediately to
been reported, of which 16 involved pediatric patients, the operating theatre and underwent in emergency cyst
with only 4 occurring in very young children (less than 3 puncture with solid component biopsy. The outcome
years). [1-5] Here we report the 5th case of CE in very young was good, with improvement in her clinical state and
child, treated surgically and with chemotherapy with a resolution of the hemiparesis.
good outcome at 20 months of follow-up.
The brain magnetic resonance imaging (MRI) performed
CASE REPORT after the first surgery revealed a large cortical fronto-
parietal lesion, hypointense on T1, hyperintense on
A 16-month-old female, with normal development T2, markedly enhanced with contrast, with moderate
milestones and without other past medical history, surrounding edema [Figure 2]. The tumor was totally
was admitted to our department of neurosurgery after resected in an elective procedure using a right
presenting with 7 days of left-sided weakness. At transparietal approach. The postoperative course
initial neurological examination, the young child was was uneventful, without complications. Histological
conscious, had a left hemiparesis mainly affecting the examination of the resected tumor showed perivascular
upper limb, a normal head circumference for age and pseudo rosettes of small round cells with mitosis (5
no papilledema. Brain computed tomography (CT)
mitosis/10 fields) and necrosis. Immunohistochemical
Corresponding author: Prof. Mehdi Darmoul, Neurosurgery studies showed positivity to glial fibrillary acidic
Department of Fattouma Bourguiba. University Hospital Avenue
1er Juin, 5000 Monastir, Tunisia.
Email: mehdi.darmoul@laposte.net This is an open access article distributed under the terms of the Creative
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Cite this article as: Darmoul M, Kilani M, Nsir AB, Hattab MN. Huge
supratentorial cortical ependymoma in a young child: Case report and
DOI: 10.20517/2347-8659.2015.53 literature review. Neuroimmunol Neuroinflammation 2016;3;141-4.
Received: 11-12-2015; Accepted: 25-02-2016.

© 2016 Neuroimmunology and Neuroinflammation | Published by OAE Publishing Inc. 141