Central nervous system atypical teratoid tumor rhabdoid tumor: Response tointensive 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 n atural history. Standard therapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective; most childre n 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 resectio ns and localized disease at diagnosis. One child treated prior to bone marr ow transplant availability died of progressive disease 9 months after diagn osis. Another child, treated with high-dose chemotherapy and radiotherapy i n preparation for bone marrow transplant, had a recurrence and died 20 mont hs after diagnosis, without undergoing the transplant. Two children receive d high-dose chemotherapy and autologous bone-marrow transplant and had a go od response to treatment; one survived 19 months, the other child is free o f disease 46 months from diagnosis. Intensified therapy has altered the nat ural history of central nervous system ATT/RT.