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

Sapna Oberoi, Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Manitoba, Canada.
Jacquelyn N. Crane, Department of Pediatrics, Stanford University, Stanford, California, USA.
Josephine H. Haduong, Division of Oncology, Hyundai Cancer Institute, Children's Hospital Orange County, Orange, California, USA.
Erin R. Rudzinski, Division of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Suzanne L. Wolden, Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, New York, USA.
Roshni Dasgupta, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Corinne M. Linardic, Department of Pediatrics, Duke University School of Medicine, Durham, North Carolina, USA.
Aaron R. Weiss, Department of Pediatrics, Maine Medical Center, Portland, Maine, USA.
Rajkumar Venkatramani, Division of Hematology/Oncology, Department of Pediatrics, Texas Children's Cancer Center, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA.

Abstract

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.