title: Primary intracranial atypical teratoid/rhabdoid tumor in a child: a case report.
abstract: Rhabdoid tumors of the central nervous system are rare malignancies. Primary central nervous system atypical teratoid/rhabdoid tumors (ATT/RhTs) mostly occur during early childhood and are almost invariably fatal. These tumors show similar histological and radiological features to primitive neuroectodermal tumormedulloblastoma (PNET-MB) but have different biological behaviors. We report a case of primary intracranial ATT/RhT in the posterior cranial fossa of a child. Preoperative radiological diagnosis was PNET-MB, but pathological diagnosis is ATT/ RhT. The case involved a 16-month-old baby boy who presented with severe headache, vomiting, and gait disturbance. He was treated by surgical resection, chemotherapy, and radiotherapy. Despite aggressive therapy, he died 19 months after diagnosis. Clinical, radiological, and histopathological features of primary intracranial ATT/RhT are discussed with a special emphasis on the differential diagnosis from PNET-MB.
authors.affiliation: Department of Neurosurgery, Seoul National University College of Medicine & Neurological Research Institute, SNUMRC, Seoul, Korea.
authors.forename: You-Nam
authors.initials: YN
authors.lastname: Chung
authors.forename: Kyu-Chang
authors.initials: KC
authors.lastname: Wang
authors.forename: Sang-Hoon
authors.initials: SH
authors.lastname: Shin
authors.forename: Narae
authors.initials: N
authors.lastname: Kim
authors.forename: Je G
authors.initials: JG
authors.lastname: Chi
authors.forename: Kyung-Soo
authors.initials: KS
authors.lastname: Min
authors.forename: Byung-Kyu
authors.initials: BK
authors.lastname: Cho
mesh_terms: D001932:Brain Neoplasms / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008527:Medulloblastoma / Q000175:diagnosis; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis; D018335:Rhabdoid Tumor / Q000175:diagnosis* / Q000473:pathology; D013724:Teratoma / Q000175:diagnosis* / Q000473:pathology; D014057:Tomography, X-Ray Computed