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

Pediatr Neurosurg. 2002 Aug;37(2):64-70. doi: 10.1159/000065107.


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.

MeSH terms

  • Adolescent
  • Central Nervous System Neoplasms / diagnostic imaging*
  • Central Nervous System Neoplasms / pathology*
  • Central Nervous System Neoplasms / therapy
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Staging
  • Retrospective Studies
  • Rhabdoid Tumor / diagnostic imaging*
  • Rhabdoid Tumor / pathology*
  • Rhabdoid Tumor / therapy
  • Teratoma / diagnostic imaging*
  • Teratoma / pathology*
  • Teratoma / therapy
  • Tomography, X-Ray Computed