作者: You Nam Chung , Kyu Chang Wang , Sang Hoon Shin , Na Rae Kim , Je G Chi
DOI: 10.3346/JKMS.2002.17.5.723
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摘要: Rhabdoid tumors of the central nervous system are rare malignancies. Primary atypical teratoid/rhabdoid (ATT/RhTs) mostly occur during early childhood and almost invariably fatal. These show similar histological radiological features to primitive neuroectodermal tumormedulloblastoma (PNET-MB) but have different biological behaviors. We report a case primary intracranial ATT/RhT in posterior cranial fossa child. Preoperative diagnosis was PNET-MB, pathological is ATT/ RhT. The involved 16-month-old baby boy who presented with severe headache, vomiting, gait disturbance. He treated by surgical resection, chemotherapy, radiotherapy. Despite aggressive therapy, he died 19 months after diagnosis. Clinical, radiological, histopathological discussed special emphasis on differential from PNET-MB.