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Journal of the Korean Child Neurology Society 2004;12(2):235-240.
Published online November 30, 2004.
A Case of an Atypical Teratoid/Rhabdoid Tumor of the Cerebellum.
Ki Soo Kim, Cheol Goo Jung, Yoon Mee Kim, Kyong Og Ko, Young Hyuk Lee
1Department of Pediatrics, College of Medicine, Konyang University, Daejon, Korea. y2hyuk@unitel.co.kr
2Department of Neurosurgery, College of Medicine, Konyang University, Daejon, Korea.
3Department of Pathology, College of Medicine, Konyang University, Daejon, Korea.
Abstract
Primary central nervous system atypical teratoid/rhabdoid tumors are rare and extremely aggressive malignancies of early childhood. These tumors are most common in infants less than 2 years of age. Diagnosis is based on distinctive light microscopic and immunohistochemical findings, coupled with a molecular genetic analysis. A histologic features of these tumors are epithelial and/or mesenchymal components in addition to rhabdoid, with or without neuroepithelial fields. The expression of the epithelial membrane antigen, vimentin, and the smooth muscle actin are characteristic of these tumors. Treatment includes surgery, chemotherapy, and radiotherapy. Prognosis is poor despite of an aggressive therapy. We report one case of an atypical teratoid/rhabdoid tumor in a young child. She is an 18 month-old-girl who presented with central type facial palsy. After extensive surgery she improved transiently but relapsed immediately. Her condition was not permitted to receive radiotherapy or chemotherapy. Thereafter, with phrenic nerve palsy, she suffered from recurrent episodes of pneumonia and respiratory difficulties. Finaly, she was expired three months after the diagnosis and treatment.
Key Words: Atypical teratoid/rhabdoid tumor
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