Clinical features and outcomes of young patients with epithelioid sarcoma: an analysis from the Children’s Oncology Group and the European paediatric soft tissue Sarcoma Study Group prospective clinical trials

Spunt SL, Francotte N, De Salvo GL, Chi YY, Zanetti I, Hayes-Jordan A, Kao SC, Orbach D, Brennan B, Weiss AR, van Noesel MM, Million L, Alaggio R, Parham DM, Kelsey A, Randall RL, McCarville MB, Bisogno G, Hawkins DS, Ferrari A. Clinical features and outcomes of young patients with epithelioid sarcoma: an analysis from the Children’s Oncology Group and the European paediatric soft tissue Sarcoma Study Group prospective clinical trials. Eur J Cancer. 2019 May;112:98-106. doi: 10.1016/j.ejca.2019.02.001. Epub 2019 Apr 5. PMID: 30954717; PMCID: PMC6944741.

Data on the clinical features, optimal treatment, and outcomes of pediatric patients with epithelioid sarcoma (ES) are limited and mostly retrospective. A subset analysis of ES patients < 30 years of age enrolled on two international prospective clinical trials conducted between 7/2005 and 11/2015 was performed. Risk-adapted therapy was based on tumor diameter, histologic grade, extent of surgery, and presence/absence of metastases, and included surgery +/− radiotherapy for all patients with the addition of ifosfamide/doxorubicin chemotherapy for intermediate-/high-risk patients. Response to therapy, event-free and overall survival, and pattern and predictors of treatment failure were evaluated.