Journal of the Korean Pediatric Society 2001;44(12):1475-1479.
Published online December 15, 2001.
A Case of Central Nervous System Atypical Teratoid/Rhabdoid Tumor of The 4th Ventricle : A Highly Malignant Tumor in Infancy and Childhood Frequently Mistaken for Medulloblastoma
So Hee Eun1, Sung Hee Jang1, Myoung Hee Han1, Myoung Ja Chung2, Pyoung Han Hwang1
1Department of Pediatrics, College of Medicine, Chonbuk National University, Chonju, Korea
2Department of Pathology, College of Medicine, Chonbuk National University, Chonju, Korea
4 뇌실에 발생한 중추신경계 비전형 기형/횡문근양 종양 1례 : 수모세포종으로 오인되는 소아기 악성종양
은소희1, 장성희1, 한명희1, 정명자2, 황평한1
1전북대학교 의과대학 소아과학교실
2전북대학교 의과대학 병리학교실
Correspondence: 
Pyoung Han Hwang, Email: hwaph@moak.chonbuk.ac.kr
Abstract
Central nervous system atypical teratoid/rhabdoid tumor(CNS rhabdoid tumor) is a rare malignancy of uncertain origin. It typically occurs in infants and young children and comprises only a small fraction of pediatrics CNS malignancies. The tumor contains a large spindled cell component as classical rhabdoid morphology and focal areas resembling primitive neuroectodermal tumor. The tumor is defined histopathologically by the presence of rhabdoid cells, but contains considerable heterogeneity of the cell type, including the frequent presence of primitive neuroectodermal tumor. The prognosis for children with CNS rhabdoid tumor is dismal. We experienced a case of a three-year-old female who had been transferred to our hospital for seizure and vomiting. She was diagnosed as CNS atypical teratoid/rhabdoid tumor by biopsy. In spite of surgery and intensive postoperative multi-agents chemotherapy, she developed a local recurrence around the operation site at six months after surgery. We present this case with a brief review of related literatures.
Key Words: CNS atypical teratoid/rhabdoid tumor


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