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A Case of Atypical Teratoid/Rhabdoid Tumor Arising from the Supratentorial Area

Korean Journal of Pediatrics 2005;48(2):228-231.
Published online February 15, 2005.
A Case of Atypical Teratoid/Rhabdoid Tumor Arising from the Supratentorial Area
Kyeong Hun Jung1, Young Se Kwon1, Yong Hun Jun1, Soon Ki Kim1, Young Jin Hong1, Byong Kwan Son1, Eun Young Kim2, In Suh Park3
1Department of Pediatrics, College of Medicine, Inha University, Incheon, Korea
2Department of Neurosurgery, College of Medicine, Inha University, Incheon, Korea
3Department of Pathology, College of Medicine, Inha University, Incheon, Korea
천막상부에 발생한 비정형 기형/횡문근양 종양 1례
정경훈1, 권영세1, 전용훈1, 김순기1, 홍영진1, 손병관1, 김은영2, 박인서3
1인하대학교 의과대학 소아과학교실
2인하대학교 의과대학 신경외과학교실
3인하대학교 의과대학 해부병리학교실
Correspondence: 
Young Se Kwon, Email: ysped@inha.ac.kr
Abstract
Atypical teratoid/rhabdoid tumor may arise at any central nervous system location, but it is most commonly located in the cerebellum(60 percent). The incidence of this tumor remains unclear but it occurs most commonly in children less than 2 years of age. This highly malignant tumor shows a rapid progression and nonspecific radiologic findings. We report a case of primary intracranial atypical teratoid/rhabdoid tumor arising from the supratentorial area in early infancy. The diagnosis was made based on distinctive light microscopy and immunohistochemical findings. Despite aggressive surgical treatment with adjuvant chemotherapy, he died six months after his second operation.
Key Words: Atypical teratoid/rhabdoid tumor , Supratentorium


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