Renal sarcomas in children and adolescents: a retrospective, multicenter cohort study

Background: Renal sarcomas arise rarely in children and adolescents and represent a histologically and biologically diverse disease category. Consequently, standardizing optimal therapies for pediatric renal sarcomas remains challenging. Leveraging a large North American research collaborative, the purposes of this study were to evaluate the current state of patient, disease, and survival characteristics among pediatric renal sarcomas and to expose knowledge gaps that will inform future discovery. Methods: Patients 21 years or younger and treated for a primary renal sarcoma between January 1st, 2000 and November 30th, 2022 were identified through the Pediatric Surgical Oncology Research Collaborative. Patient (e.g., demographics) and disease (e.g., histology, stage, molecular alterations) characteristics were abstracted from contributing institutions. Descriptive statistics, Pearson-Chi square (categorical variables), Kruskal-Wallis (continuous variables), Cox regression (Hazard ratios), and Kaplan-Meier 4-year event-free and overall survival (OS) analyses were completed. Findings: Among 158 patients, clear cell sarcoma of the kidney (CCSK; n = 94), Ewing sarcoma (EWS; n = 33), and undifferentiated sarcoma (n = 8) predominated. Sarcoma type correlated significantly with age at diagnosis (p < 0.0001), with infantile fibrosarcoma (IFS) and CCSK occurring in the youngest patients, whereas EWS and synovial sarcoma presented in the oldest. Predisposition syndromes were identified