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Figure 2: Post puncture magnetic resonance imaging (axial and sagittal T1
post gadolinium and axial T2 weighted images) showing a huge right parietal
Figure 1: Preoperative brain computed tomography scan without contrast; tumor, enhancing with contrast and with moderate peritumoral edema.
axial view and sagittal reconstruction, showing a huge right fronto-parietal
cyst and a slightly hyperdense cortical solid lesion with thin calcifications.

Figure 4: Follow-up magnetic resonance imaging at 20 months (axial T1,
sagittal T1 with gadolinium and coronal T2 weighted images) showing
complete removal of the lesion without recurrence.

in adults than children. There are only 49 cases
reported in the literature. [1-5]

Ependymomas are known to arise from ependymal cells.
The pathogenesis of the extraventricular tumor type
Figure 3: Photomicrograph of the tumor specimen showing marked
hypercellularity, nuclear atypia and brisk mitotic activity. (HE, X40). remains uncertain. Pure cortical ependymomas may
protein (GFAP), poly styrene 100, and keratin, but not to arise from embryonic remnants of ependymal tissue
synaptophysin. Kalium iodidum (Ki)-67 was positive in encapsulated in the developing cerebral hemispheres. [2]
35%. These findings are in keeping with an anaplastic CEs are rare in pediatric patients and exceedingly
ependymoma, World Health Organization (WHO)
classification grade III [Figure 3]. Cerebrospinal fluid uncommon in very young children.
studies were negative for malignant cells and no drop Among the 49 cases reported in the literature, 16 involved
metastases were detected on neuroaxis MRI. Intensive children and only 4 occurred in patients under the age of
chemotherapy with BBSFOPP protocol consisting of 3 years. [1,2,4,6]
seven cycles of 3 courses alternating 2 drugs at each
course (carboplatin/procarbazine, cisplatin/etoposide Our patient is the 5th case reported of CE in those under
and vincristine/cyclophosphamide) was given post 3 years of age.
operatively for 16-month period. After 20 months of
follow up, the patient remained neurologically normal The age of these 5 young children ranged from 12-24
without recurrence or metastasis on surveillance MRI months, with a mean age of 19.4 months. There was male
[Figure 4]. preponderance with male to female ratio of 3:2 [Table 1].
DISCUSSION The typical presentation of CE was with seizures and
focal neurological deficits, and rarely with signs of raised
Ependymomas account for 2-9% of all neuroepithelial intracranial pressure. [1,2,7]
tumors, and are graded as grade II (low grade), and grade III
(anaplastic) according to 2007 WHO classification. They Our patient presented with left hemiparesis, a seizure,
involve frequently the spinal cord and ventricular system, and rapid deterioration with signs of intracranial
especially the fourth ventricle, and they commonly occur hypertension, whereas the others four young children
in children and young adults. [1] reported, presented either with seizures or with
hemiparesis [Table 1].
Supratentorial CE is an uncommon ependymoma
located in the superficial cortex and more often found Intracranial ependymomas appear on brain CT scans as

142 Neuroimmunol Neuroinflammation | Volume 3 | June 20, 2016