title: Primary malignant rhabdoid tumor of the cerebellum.
abstract: Malignant rhabdoid tumor (MRT) is a recently described variety of childhood renal neoplasm. MRT arising primarily in the central nervous system (CNS) is still a rather unfamiliar pathological entity and is frequently misdiagnosed as medulloblastoma or primitive neuroectodermal tumor (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 chemotherapy the tumor recurred locally 9 months after operation. We discuss clinical and histopathological features distinguishing between PNET/medulloblastoma and MRTs. Current literature on MRT of the CNS is briefly reviewed.
authors.affiliation: Regional Service of Neurosurgery, Virgen de la Arrixaca, University Hospital, National Health Service, Murcia, Spain.
authors.forename: J F
authors.initials: JF
authors.lastname: Martínez-Lage
authors.forename: A
authors.initials: A
authors.lastname: Nieto
authors.forename: J
authors.initials: J
authors.lastname: Sola
authors.forename: R
authors.initials: R
authors.lastname: Domingo
authors.forename: T R
authors.initials: TR
authors.lastname: Costa
authors.forename: M
authors.initials: M
authors.lastname: Poza
mesh_terms: D002528:Cerebellar Neoplasms / Q000473:pathology* / Q000601:surgery; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local; D018335:Rhabdoid Tumor / Q000473:pathology* / Q000601:surgery