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

J Neurooncol. 1998 Dec;40(3):265-75. doi: 10.1023/a:1006125120866.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Brain Neoplasms / diagnosis*
  • Brain Neoplasms / pathology
  • Brain Neoplasms / therapy*
  • Child, Preschool
  • Combined Modality Therapy
  • Fatal Outcome
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Rhabdoid Tumor / diagnosis*
  • Rhabdoid Tumor / pathology
  • Rhabdoid Tumor / therapy*
  • Survival Analysis
  • Teratoma / diagnosis*
  • Teratoma / pathology
  • Teratoma / therapy*