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Table 1: The summary of previous reported young children supratentorial cortical ependymoma with our case.
References Age (months) Location Clinical Neuroimaging WHO grade Treatment Follow-up
/gender presentation finding
Lehman et al, [4] 12/F Rt frontal Seizures Solid lesion not III GTR No recurrence in 48
enhanced [clear cells] months
Lee et al, [2] 21/M Rt fronto-parietal Seizures Diffuse enhancing II GTR No recurrence in 12
mass, some focal months
calcifications
Liu et al, [1] 24/M Lt frontal Right side Large mass, III GTR Recur at 3 years after
weakness irregular peripheral surgery alive at 4 years
enhancement no
edema
Kambe et al, [6] 24/M Rt parietal Seizures Solid mass, II [tanycytic] GTR No recurrence in 20
calcification months
homogeneous
enhancement
Our case 16/F Rt fronto-parietal Left side Large solid cystic III GTR No recurrence in 20
weakness, lesion heterogeneous months
seizures, enhancement
ICH signs
F: female; M: male; Rt: right; Lt: left; WHO: world health organization; GTR: gross total resection; ICH: intracranial hypertension
isodense or mildly hyperdense soft tissue lesions with or ring-like positivity for epithelial membrane antigen
frequent large cyst, calcifications in 50%, hemorrhage and/or membranous with or without perinuclear dot-like
in approximately 10% and, often, heterogeneous staining pattern for cluster of differentiation 99. [1,4]
enhancement. [8]
Immunohistochemical cell proliferation markers, that
MRI is the brain imaging modality of choice for have some specificity for ependymomas are available.
ependymoma. CE generally appears as large, MIB-1 L1 and Ki-67 are associated with high-grade
well-demarcated lesions, T1 hypointense and ependymomas, as in our case. Recently, further markers
T2 hyperintense, showing cyst formation. After have been found, notably topo-II-α and p53 and murine
gadolinium administration, T1 weighted images double min 2 protein expression which are correlated
usually show heterogeneous enhancement of the solid with high-grade tumors and a poor prognosis. [12]
component. Frontal and parietal region are the most
[8]
common locations. [8] Although nearly 70% of all ependymomas diagnosed in
the pediatric population are histologically benign, CEs
These radiologic findings are, however, non-specific are more frequently anaplastic in young children (3/5)
and glioblastomas, pleomorphic xanthoastrocytomas, [Table 1].
oligodendrogliomas, primitive neuroectodermal tumors,
astroblastomas and angiocentric gliomas should be Given the superficial location, radical resection is the
considered in the differential diagnosis of a large treatment of choice for CE.
supratentorial cortical lesion. [1,8-11] There has been much debate about adjuvant radiotherapy
and chemotherapy.
Histologically, the classic cellular features of
ependymomas include round to oval nuclei with Radiotherapy was thought to increase the length of
evenly dispersed stippled chromatin, perivascular survival and reduce or postpone tumor recurrence. It
pseudo rosettes or true ependymal rosettes. Unusual is used in patients with anaplastic ependymomas in
morphological features like clear cells, spindle cells cases of partial resection of either benign or malignant
and giant cells can also be seen. [1,4] tumor. [13,14] Whole brain irradiation with additional
local fractions is recommended by many authors in
Anaplastic ependymomas are characterized by malignant ependymoma. [14]
increased cellularity, cytological atypia, increased
mitotic activity, microvascular proliferation and/or In very young children, delayed radiotherapy and a
pseudopalissading tumor necrosis. There is normally period of close observation after gross total resection
a clear interface between tumor and adjacent brain may be chosen for some cases to defer the side-effects of
tissue and relative uniformity of tumor cell nuclei. [1,4] radiotherapy. [4,6,13,15]
However, some authors have described ependymomas,
that infiltrate at their peripheries. [4] Chemotherapy has mainly been studied in children
with incomplete tumor resections and multiple agents
Immunohistochemical studies revealed frequent have been given in an effort to delay or avoid irradiation.
immunoreactivity for GFAP, S100, a perinuclear dot-like These studies suggest that chemotherapy offers limited

Neuroimmunol Neuroinflammation | Volume 3 | June 20, 2016 143