Intensive Multimodality Treatment for Children With Newly Diagnosed CNS Atypical Teratoid Rhabdoid Tumor

摘要: Purpose Atypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primarily affecting young children, with historic median survival ranging from 6 to 11 months. Based on previous pilot series, prospective multi-institutional trial was conducted for patients newly diagnosed ATRT. Patients and Methods Treatment divided into five phases: preirradiation, chemoradiation, consolidation, maintenance, continuation therapy. Intrathecal chemotherapy administered, alternating intralumbar intraventricular routes. Radiation therapy (RT) prescribed, either focal (54 Gy) or craniospinal (36 Gy, plus primary boost), depending age extent disease at diagnosis. Results Between 2004 2006, 25 were enrolled; 20 eligible evaluation. Median diagnosis 26 months (range, 2.4 19.5 years). Gross total resection achieved in patients. Fourteen had M0 diagnosis, one patient M...