Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012.

摘要: Atypical teratoid rhabdoid tumors (ATRTs) are recently defined highly aggressive embryonal central nervous system with a poor prognosis and no definitive guidelines for treatment. We report on the importance of an initial correct diagnosis disease-specific therapy outcome in 22 consecutive patients propose new treatment strategy. From 1992 to 2012, nine initially diagnosed correctly as ATRT (cohort A, median age 24 months) were treated according intensive multimodal regimen (MUV-ATRT) consisting three 9-week courses dose-dense including doxorubicin, cyclophosphamide, vincristine, ifosfamide, cisplatin, etoposide, methotrexate augmented intrathecal therapy, followed by high-dose chemotherapy (HDCT) completed local radiotherapy. Thirteen differently B, 30 months) most whom protocols use their respective diagnoses. As July 2013, 5-year overall survival (OS) event-free (EFS) all was 56.3 ± 11.3% 52.9 ± 11.0%, respectively. For MUV-ATRT regimen-treated A) OS 100% EFS 88.9 ± 10.5%. B) 28.8 ± 13.1%. All alive 76 months (range: 16–197), eight first complete remission. Our results compare favorably previously published data. The drug combination sequence used proposed appear be efficacious preventing early relapses also young children M1–M3 stage disease allowing postponement radiotherapy until after HDCT.