Atypical teratoid/rhabdoid tumors of the central nervous system: clinical, radiographic and pathologic features.
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.
Bambakidis
Nicholas C
NC
Division of Pediatric Neurological Surgery, Rainbow Babies and Childrens Hospital, Cleveland, Ohio 44106, USA. nbamba@juno.com
Robinson
Shenandoah
S
Cohen
Mark
M
Cohen
Alan R
AR
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