Central nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature.

Published

Journal Article (Review)

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.

Full Text

Duke Authors

Cited Authors

  • Hilden, JM; Watterson, J; Longee, DC; Moertel, CL; Dunn, ME; Kurtzberg, J; Scheithauer, BW

Published Date

  • December 1998

Published In

Volume / Issue

  • 40 / 3

Start / End Page

  • 265 - 275

PubMed ID

  • 10066100

Pubmed Central ID

  • 10066100

International Standard Serial Number (ISSN)

  • 0167-594X

Digital Object Identifier (DOI)

  • 10.1023/a:1006125120866

Language

  • eng

Conference Location

  • United States