A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children’s Oncology Group prospective study

Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS. A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children’s Oncology Group prospective study. Lancet Oncol. 2020 Jan;21(1):145-161. doi: 10.1016/S1470-2045(19)30672-2. Epub 2019 Nov 27. PMID: 31786124; PMCID: PMC6946838.

Tumor grade, size, resection potential, and extent of disease influence outcome in pediatric non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) but no risk stratification systems exist and the standard of care is poorly defined. This trial evaluated a risk stratification system developed from known prognostic factors in the context of risk-adapted therapy for young NRSTS patients. Treatment goals were to limit radiotherapy use in low-risk disease, decrease radiotherapy doses in those requiring it, and evaluate the feasibility/efficacy of a neoadjuvant chemoradiotherapy approach for higher-risk disease.