Atypical teratoid rhabdoid tumors of the posterior fossa in children

Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
Arthur J DiPatriTadanori Tomita

Abstract

Atypical teratoid rhabdoid tumors (AT/RT) are rare, aggressive, central nervous system neoplasms that typically affect children under 3 years of age and have a very poor prognosis. Early case series consistently demonstrated rapid recurrence with progression to death, but more recent experience has shown significant improvements in progression free and overall survival. A retrospective analysis of the clinical data of children diagnosed with AT/RT at the Ann & Robert H. Lurie Children's Hospital of Chicago (formerly Children's Memorial Hospital) between 2000 and 2014 was performed. Overall survival (OS) was used to describe outcome. Our small sample size and the utilization of different adjuvant regimens over the study period precluded a detailed statistical analysis. Eight children with AT/RT of the posterior fossa were included in our report. Gross total resection (GTR) was achieved in five children (63 %), two children underwent subtotal resection (25 %), and there was one who underwent biopsy. Patients were treated with various combinations of chemotherapy with or without conformal radiation therapy (RT). Median overall survival was 5 months (range 1 to 107 months) with two patients achieving sustained responses to 45 and 1...Continue Reading

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Citations

Apr 15, 2019·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Wan-Chin YangYi-Wei Chen

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