A rare primitive neuroectodermal tumor: the medulloepithelioma

Primitive neuroectodermal tumors (PNET) is a name used to describe rare and highly malignant tumors composed of undifferentiated cells resembling germ inal or matrix cells of the embryonic neural tube. These tumors occur most commonly in the first decade of life, and have a particular radiological, h istological and evolution features. We report a case of a PNET in an 8-year-old boy who presented intracranial hypertension studied with computed tomography scan, which demonstrated a sh arply circumscribed expansive mass in the frontoparietal deep white matter, with a large cystic component, which was considered at first as a glial tu mor. It was treated by total excision, and the histological study demonstra ted a medulloepithelioma. We studied the concept of PNET and their pathological, radiological and pro gnostic features.