Children’s Oncology Group’s 2023 blueprint for research: Soft tissue sarcomas

Oberoi S, Crane JN, Haduong JH, Rudzinski ER, Wolden SL, Dasgupta R, Linardic CM, Weiss AR, Venkatramani R. Children’s Oncology Group’s 2023 blueprint for research: Soft tissue sarcomas. Pediatr Blood Cancer. 2023 Sep;70 Suppl 6(Suppl 6):e30556. doi: 10.1002/pbc.30556. Epub 2023 Jul 10. PubMed PMID: 37430436; PubMed Central PMCID: PMC10519430.

In the United States, approximately 850–900 children and adolescents each year are diagnosed with soft tissue sarcomas (STS). STS are divided into rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma STS (NRSTS). RMS and NRSTS are risk stratified into low-, intermediate-, and high-risk categories with 5-year survival rates of approximately 90%, 50–70% and 20%, respectively. Recent key achievements from the Children’s Oncology Group (COG) STS Committee include the identification of new molecular prognostic factors for RMS, development and validation of a novel risk stratification system for NRSTS, successful completion of a collaborative NRSTS clinical trial with adult oncology consortia, and collaborative development of the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT). Current COG trials for RMS are prospectively evaluating a new risk stratification system that incorporates molecular findings, de-intensification of therapy for a very-low-risk subgroup, and augmented therapy approaches for intermediate- and high-risk RMS. Trials for NRSTS exploring novel targets and local control modalities are in development.