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. 2023 Sep;70 Suppl 6(Suppl 6):e30556.
doi: 10.1002/pbc.30556. Epub 2023 Jul 10.

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

Sapna Oberoi  1   2 , Jacquelyn N Crane  3 , Josephine H Haduong  4 , Erin R Rudzinski  5   6 , Suzanne L Wolden  7 , Roshni Dasgupta  8 , Corinne M Linardic  9   10 , Aaron R Weiss  11 , Rajkumar Venkatramani  12 ; the Children's Oncology Group Soft Tissue Sarcoma Committee
Affiliations

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

Sapna Oberoi et al. Pediatr Blood Cancer. 2023 Sep.

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.

Keywords: cancer; children; desmoid tumor; non-rhabdomyosarcoma soft tissue sarcoma; rhabdomyosarcoma.

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Conflict of interest statement

Conflict of Interests: C.M.L. receives grant research support from Ryvu Therapeutics; this does represent a conflict of interest with respect to the work described in this manuscript.

References

    1. Siegel DA, King J, Tai E, Buchanan N, Ajani UA, Li J. Cancer incidence rates and trends among children and adolescents in the United States, 2001–2009. Pediatrics. Oct 2014;134(4):e945–55. doi:10.1542/peds.2013-3926 - DOI - PMC - PubMed
    1. Gurney JG, Young JLJ, Roffers SD, Smith MA, Bunin GR. Soft tissue sarcomas. In: Ries LAG, Smith MA, Gurney JG, et al., eds. Cancer Incidence and Survival among Children and Adolescents: United States SEER Program 1975–1995 Pub No 99–4649. National Cancer Institute SEER Program; 1999.
    1. Institute NC. NCCR*Explorer: An interactive website for NCCR cancer statistics [Internet]. Accessed March 7, 2023. https://NCCRExplorer.ccdi.cancer.gov/
    1. Li H, Sisoudiya SD, Martin-Giacalone BA, et al. Germline Cancer Predisposition Variants in Pediatric Rhabdomyosarcoma: A Report From the Children’s Oncology Group. J Natl Cancer Inst. Jul 01 2021;113(7):875–883. doi:10.1093/jnci/djaa204 - DOI - PMC - PubMed
    1. Haduong JH, Heske CM, Allen-Rhoades W, et al. An update on rhabdomyosarcoma risk stratification and the rationale for current and future Children’s Oncology Group clinical trials. Pediatr Blood Cancer. Apr 2022;69(4):e29511. doi:10.1002/pbc.29511 - DOI - PMC - PubMed

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