Controversies and challenges in the management of paediatric non-rhabdomyosarcoma soft tissue sarcomas

Non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) is a heterogeneous group of mesenchymal tumours distinct from rhabdomyosarcoma that affects children and adolescents, with clinical presentation varying from relatively benign to highly malignant. 1 Spunt SL Million L Chi Y-Y et al. 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; 21: 145-161 Summary Full Text Full Text PDF PubMed Scopus (43) Google Scholar , 2 Ferrari A van Noesel MM Brennan B et al. Paediatric non-rhabdomyosarcoma soft tissue sarcomas: the prospective NRSTS 2005 study by the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG). Lancet Child Adolesc Health. 2021; 5: 546-558 Summary Full Text Full Text PDF PubMed Scopus (10) Google Scholar To develop a consensus approach to NRSTS, the International Soft-Tissue Sarcoma Consortium (INSTRuCT) 3 Hawkins DS Bisogno G Koscielniak E Introducing INSTRuCT: an international effort to promote cooperation and data sharing. Pediatr Blood Cancer. 2020; (published online Sept 12.)https://doi.org/10.1002/pbc.28701 Crossref Scopus (10) Google Scholar was founded by the leadership of three large cooperative groups—the Children's Oncology Group (COG), the Cooperative Weichteilsarkom Studiengruppe (CWS), and the European paediatric Soft tissue sarcoma Study Group (EpSSG)—to pool expertise and resources to improve knowledge of these tumours in children, adolescents, and young adults, and to improve patient outcomes.