Atypical teratoid/rhabdoid tumors of the central nervous system: Clinical, radiographic and pathologic features

Nicholas C. Bambakidis, Shenandoah Robinson, Mark Cohen, Alan R. Cohen

Research output: Contribution to journalArticle

Abstract

Introduction: Atypical teratoid/rhabdoid tumors (ATT/RT) of the central nervous system (CNS) are uncommon malignancies of childhood with an aggressive course and a uniformly fatal outcome. Methods: The medical records, radiographic images and pathologic files at the Rainbow Babies and Childrens Hospital over the previous 6 years were retrospectively reviewed. Results: Eight children underwent surgery for CNS ATT/RT at our institution since 1996. There were 6 boys and 2 girls. Median age at presentation was 21 months. Four tumors had multifocal disease at the time of diagnosis. Six patients received multiagent chemotherapy including 3 patients with autologous bone marrow transplantation, and 6 patients received radiation therapy. Median survival was 9 months from the time of diagnosis. Conclusions: In spite of aggressive therapy, the prognosis for ATT/RT remains dismal. The search for effective treatment strategies will require a better understanding of the biology and molecular genetics of this tumor.

Original languageEnglish (US)
Pages (from-to)64-70
Number of pages7
JournalPediatric Neurosurgery
Volume37
Issue number2
DOIs
StatePublished - Sep 14 2002
Externally publishedYes

Keywords

  • Brain tumors, pediatric
  • Teratoid/rhabdoid tumor, atypical

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Atypical teratoid/rhabdoid tumors of the central nervous system: Clinical, radiographic and pathologic features'. Together they form a unique fingerprint.

  • Cite this