Management and Outcomes of Pediatric, Adolescent and Young Adult Sarcoma Patients: Results from the French Nationwide Database NETSARC

Abstract Background . The initial management of patients with sarcoma is critical. We used the French National Cancer Institute-funded nationwide sarcoma database NETSARC to report the management and oncologic outcomes in children, adolescents and young adults (AYAs) patients with sarcoma at the national level. Patients and methods . Data from patients with sarcoma are collected, regularly monitored and updated using NETSARC. NETSARC was searched for pediatric (<15 years) and AYA (15-30 years) patients with sarcoma, diagnosed from 2010 to 2017. We report the management, locoregional recurrence-free survival (LRFS), progression-free survival (PFS), and overall survival (OS) in children and AYA treated in French reference sarcoma centers (RSC) and outside RSC (non-RSC), and conducted multivariate analyses adjusted for classical prognostic factors to explore survival. Results . We collected data from 3,972 pediatric and AYA patients with sarcoma diagnosed between 2010 and 2017, including 714 children, 1,290 AYAs treated in RSC, and 937 AYAs in non-RSC. Significant differences in compliance to guidelines were observed regarding pre-treatment biopsy (Children: 83.5%; AYA in RSC: 85.9%; AYA in non-RSC 48.1%), pre-treatment imaging (Children: 81.2%; AYA in RSC: 86.8%; AYA in non-RSC: 56.5%) and R0 margins (Children: 52%; AYA in RSC 57.6%; AYA in non-RSC: 20.2%) (p<0.001). 3y-OS rates were 81.6% (95%CI 77.9-84.7) in children, 81.1% (95%CI 78.3-83.6) in AYA in RSC and 82.7% (95%CI 79.4-85.5) in AYA in non-RSC, respectively. LRFS and PFS were improved in AYAs treated in RSC compared to AYAs treated in non-RSC (Hazard Ratios HR: 0.64 and 0.85 with statistical significance at 1% and 10%, respectively). Multivariate regression analysis identified no significant differences in OS in AYAs treated in RSC and in non-RSC (p=0.272). Conclusions . This study highlights the importance for AYA patients with sarcoma to be managed in national sarcoma reference centers involving multidisciplinary medical teams with paediatric and adult oncologists.