Central nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature.
Central nervous system atypical teratoid/rhabdoid tumor (ATT/RT) of infancy and childhood is a unique histologic entity with an extremely aggressive natural history. Standard therapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective; most children survive less than 12 months after diagnosis. Intensified therapy has been recently used for children with this disease, with promising results [1,2]. We report four cases of ATT/RT in young children; all had subtotal resections and localized disease at diagnosis. One child treated prior to bone marrow transplant availability died of progressive disease 9 months after diagnosis. Another child, treated with high-dose chemotherapy and radiotherapy in preparation for bone marrow transplant, had a recurrence and died 20 months after diagnosis, without undergoing the transplant. Two children received high-dose chemotherapy and autologous bone-marrow transplant and had a good response to treatment; one survived 19 months, the other child is free of disease 46 months from diagnosis. Intensified therapy has altered the natural history of central nervous system ATT/RT.
Duke Scholars
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- Teratoma
- Survival Analysis
- Rhabdoid Tumor
- Oncology & Carcinogenesis
- Male
- Magnetic Resonance Imaging
- Infant
- Humans
- Female
- Fatal Outcome
Citation
Published In
DOI
ISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Teratoma
- Survival Analysis
- Rhabdoid Tumor
- Oncology & Carcinogenesis
- Male
- Magnetic Resonance Imaging
- Infant
- Humans
- Female
- Fatal Outcome