Two Cases of Central Nervous System Atypical Teratoid/Rhabdoid Tumors.
- Author:
Young Dai KIM
1
;
Ki Joong KIM
;
Ho Joon IM
;
Eun Kyung HONG
;
Hahng LEE
Author Information
1. Department of Pediatrics, Hanyang University College of Medicine, Seoul, Korea, Inchon, Korea. Hahnglee@email.hanyang.ac.kr
- Publication Type:Case Report
- Keywords:
Atypical teratoid/rhabdoid tumor
- MeSH:
Brain Neoplasms;
Central Nervous System*;
Child;
Combined Modality Therapy;
Cytogenetics;
Diagnosis;
Drug Therapy;
Humans;
Infant;
Medulloblastoma;
Monosomy;
Natural History;
Neuroectodermal Tumors, Primitive
- From:Korean Journal of Pediatric Hematology-Oncology
2000;7(2):293-298
- CountryRepublic of Korea
- Language:Korean
-
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.
