Prognostic significance of reduced-dose and rduced-volume radiotherapy in children with DE NOVO medulloblastoma: results of intercenter pilot study (Like-SJMB03тезисы доклада
Аннотация:Background and Aims: The aim of this study was to estimate event free survival (EFS) depending on the R/M group and therapeutic approaches.Methods: From 2008 till 2016 forty eight patients were included in trial. Children underwent adjuvant therapy: for R0M0 – CSI 23.4Gy, for R1M0 and R0/1M+ – CSI 36.0Gy (instead of 39.6Gy and additional irradiation to the metastatic sites up to 50.4–55.8Gy for M2/M3) + local RT to the tumor bed up to 54Gy (instead of 55.8Gy and primary site up to 59.4Gy for R1) followed by 4 cyclesvincristine/cyclophosphamide/cisplatin with auto stem cell transplantation. R0M0: n=25, 3–7 years of age/older – 13/12, vermis and 4-th ventricle/cerebellar hemisphere (primary site) – 21/4; tumor samples – classic/LCA/desmoplastic histology in 22/2/1, MYC-N amplification in 1, Iso17q in 2, WNT/SHH/Group3/Group4/no data in 3/1/3/8 and 10. R1M0: n=8, 3–7 years of age/older – 5/3, vermis and 4-th ventricle/cerebellar hemisphere – 8/0; tumor samples – classic/LCAhistology in 7/1, Iso17q in 2, WNT/SHH/Group3/Group4/no data in 1/0/1/2 and 4. R0/1M+: n=15, 3–7 years of age/older - 10/5, vermisand 4-th ventricle/cerebellar hemisphere – 12/3; M1/M2/M3 – 4/2/9; tumor samples – classic/LCA/desmoplastic histology in 13/1/1,MYC amplification in 1, MYC-N amplification in 2, Iso17q in 4, WNT/SHH/Group3/Group4/no data in 1/2/3/6 and 3.Results: 5-year EFS was 82.0% in R0M0 group (6 relapses – 1 WNT, 1 SHH, 3 Group4, 1 secondary osteosarcoma in Group3, 2 events after5 years), 3–7 years of age/older 88.9% and 58.3% (p=0.044); 62.5% in R1M0 group (progression – 1 Group3, relapse – 1 Group 4, 1 case with fatal septic complication); 33.3% in R0/1M+ group (8 relapses – of them 1 SHH, 1 Group3, 3 Group4; progression – 1 Group3, 1 case with fatal septic complication).Conclusions: Reduced-dose and reduced-volume radiotherapy led to a significant decrease in the EFS rate in childrenwith R1M0and R0/1M+status.