Korean J Pediatr Hematol Oncol.
2000 Oct;7(2):293-298.
Two Cases of Central Nervous System Atypical Teratoid/Rhabdoid Tumors
- Affiliations
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- 1Department of Pediatrics, Hanyang University College of Medicine, Seoul, Korea, Inchon, Korea. Hahnglee@email.hanyang.ac.kr
- 2Department of Pathology, Hanyang University College of Medicine, Seoul, Korea, Inchon, Korea.
- 3Department of Pediatrics, Gachon Medical School, Gil Medical Center, Inchon, Korea.
Abstract
- Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) of infancy and childhood, a new entity among malignant pediatric brain tumors, is unique histologic entity with an extremely aggressive natural history. Histologically AT/RT is defined as a polymorphous neoplasm often featuring rhabdoid, primitive neuroectodermal tumor, epithelial, and mesenchymal components. AT/RT occurs mainly in the posterior fossa, so mimics medulloblastoma. AT/RT is characterized by the cytogenetic finding of monosomy 22 rather than i (17q). Standard chemotherapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective. Most children survive less than 12 months (mean survival: 8.5 months) after diagnosis. We report two cases of central nervous system AT/RT in young children, one of them is alive so far with multimodal treatment.
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