Metastatic Rhabdomyosarcoma: Results of a single center in Russia

Introduction: Metastatic Rhabdomyosarcoma (RMS) has a poor prognosis despite multimodal therapy.The aim of the study -analyze the results of therapy of metastatic RMS in children treated in the national center in RF.Methodology: 181 included (<18 years) prospectively enrolled (02.2012 -12.2019).159 received program therapy according to the CWS Group guidance, version 2009.47 (30%) had metastatic disease at presentation and were included.Results: Median age at diagnosis -46.93 months (1.5 -196.2).The age distribution: <1 (n=1; 2%), 1-9 (n=35; 75%), >10 (n=11; 23%).Male to female -1:0.7.Histopathologic types: embryonal (n=18; 38%), alveolar (n=29; 62%).FP-alveolar RMS by FISH -19/29 (66%).43/47 had T2.Initial surgery: biopsy in 34/47 (72%) (primary tumor-27, metastases-7), surgery 13/47 (28%) (R1-4, R2-9).hemotherapy: CEVAIE (n=28; 60%), CEVAIE/other (n=17; 36%), other (n=2; 4%).Local control of the primary tumor -46/47: only surgery 11/46 (24%), only radiotherapy 12/46 (26%), surgery and RT 23/46 (50%).The best second-look surgery of the primary tumor: R0 (n=8), R1 (n=15), R2 (n=1).Regional lymph node dissection -8/27 (30%).Local control of distant metastases -11/47 (23%).Maintenance treatment -37/47 (79%): O-TI/E (n=12), CYC/VNL (n=25).Median follow-up time: 53.93 months (1.10-139.9).23 alive, 24 died.5-year EFS and OS: 42,1% (7,2%; 95% Cl), 51% (7,2%; 95% Cl).