Cerebral Medulloepithelioma: A Report of Five Cases

S ATTAFI, A ZEHANI, I CHELLY, H AZOUZ, B CHELLY, S MEJRI, H JMAL, S HAOUET, N KCHIR

Abstract


Medulloepithelioma, first described by Bailey and Cushing in 1926, is rare, highly malignant primitive neuroectodermal tumors usually occurring in childhood. It corresponds to World Health Organization Grade IV. Histologically, this tumor is characterized by neoplastic neuroepithelium recapitulating the embryonic neural tube. We propose to study the clinicopathological features of this entity and its diagnostic difficulties.
Five Tunisian cases of medulloepithelioma were diagnosed in the Pathology Department of Rabta hospital over 18 years (1995-2013).
There were 2 females and 3 males. The ages at diagnosis ranged from 3 months to 17 years. The tumor was involved the frontal lobe (2 cases), the temporal lobe (1 case), the parietal lobe (1 case), or the right cavernous lodge (1 case). At histological examination the tumor was constituted by papillary, tubular or trabecular arrangements of neoplastic neuroepithelium with an external limiting membrane. Tumor cells had a columnar to cuboidal shape with marked anaplasia. Mitotic figures were prominent. They stained positively to vimentin. GFAP, S-100 protein and neurofilament protein were negative.
Medulloepithelioma is a rare, primitive, fast growing brain tumor which carries a dismal prognosis with a median survival of 5 months. Combined gross total tumor resection and radiotherapy are recommended to obtain the most favorable outcome.

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