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Atypical teratoid/rhabdoid tumors of the central nervous system: clinical, radiographic and pathologic features
Nicholas C Bambakidis1, Shenandoah Robinson, Mark Cohen
1Division of Pediatric Neurological Surgery, Rainbow Babies and Childrens Hospital, Cleveland, Ohio 44106, USA. nbamba@juno.com
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.
