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

J M Hilden, J Watterson, D C Longee, C L Moertel, Mary E. Dunn, J Kurtzberg, B W Scheithauer

Research output: Contribution to journalArticlepeer-review

80 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)265-75
Number of pages11
JournalJournal of neuro-oncology
Issue number3
StatePublished - Dec 1998


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

PubMed: MeSH publication types

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


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