Children’s Oncology Group’s 2023 blueprint for research: Bone tumors

Reed DR, Grohar P, Rubin E, Binitie O, Krailo M, Davis J, DuBois SG, Janeway KA. Children’s Oncology Group’s 2023 blueprint for research: Bone tumors. Pediatr Blood Cancer. 2023 Sep;70 Suppl 6(Suppl 6):e30583. doi: 10.1002/pbc.30583. Epub 2023 Jul 27. PubMed PMID: 37501549; PubMed Central PMCID: PMC10499366.

The Children’s Oncology Group (COG) Bone Tumor Committee is responsible for clinical trials and biologic research on localized, metastatic and recurrent osteosarcoma and Ewing sarcoma (EWS). Results of clinical trials in localized disease completed and published in the past 10 years have led to international standard of care chemotherapy for osteosarcoma and EWS. A recent focus on identifying disease subgroups has led to the identification of biologic features associated with poor outcome including the presence of circulating tumor DNA (ctDNA) at diagnosis, and specific genomic alterations — MYC amplification for osteosarcoma and STAG2 and TP53 mutation for EWS. Studies validating these potential biomarkers are underway. Clinical trials evaluating the addition of multi-targeted kinase inhibitors, which are active in relapsed bone sarcomas, to standard chemotherapy are underway in osteosarcoma and planned in EWS. In addition, the committee has data analyses and a clinical trial underway to evaluate approaches to local management of the primary tumor and metastatic sites. Given the rarity of bone sarcomas, we have prioritized international interactions and are in the process of forming an international data sharing consortium to facilitate refinement of risk stratification and study of rare disease subtypes.