PRIMARY MALIGNANT RHABDOID TUMOR OF THE CEREBELLUM

Malignant rhabdoid tumor (MRT) is a recently described variety of chil dhood renal neoplasm. MRT arising primarily in the central nervous sys tem (CNS) is still a rather unfamiliar pathological entity and is freq uently misdiagnosed as medulloblastoma or primitive neuroectodermal tu mor (PNET). We describe a 7-month-old boy who harbored a CNS-MRT that originated within the IV ventricle and invaded the brain stem and the cerebellar hemispheres, After an initial documented good response to c hemotherapy the turner recurred locally 9 months after operation. We d iscuss clinical and histopathological features distinguishing between PNET/medulloblastoma and MRTs. Current literature on MRT of the CNS is briefly reviewed.