title: Atypical teratoid/rhabdoid tumors of the central nervous system: clinical, radiographic and pathologic features.
abstract: BACKGROUND
  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.
authors.affiliation: Division of Pediatric Neurological Surgery, Rainbow Babies and Childrens Hospital, Cleveland, Ohio 44106, USA. nbamba@juno.com
authors.forename: Nicholas C
authors.initials: NC
authors.lastname: Bambakidis
authors.forename: Shenandoah
authors.initials: S
authors.lastname: Robinson
authors.forename: Mark
authors.initials: M
authors.lastname: Cohen
authors.forename: Alan R
authors.initials: AR
authors.lastname: Cohen
mesh_terms: D000293:Adolescent; D016543:Central Nervous System Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000628:therapy; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009367:Neoplasm Staging; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000628:therapy; D013724:Teratoma / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed