Current Approaches to the Treatment of Pediatric Soft Tissue Sarcomas: Rhabdomyosarcoma and Nonrhabdomyosarcoma Soft Tissue Sarcomas

Aaron R. Weiss, MaineHealth
Andrea Ferrari, Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian, 1 20133 Milano, MI, Italy; Department of Oncology and Hematology-oncology, University of Milan, Milan, Italy.
Leo Mascarenhas, Departments of Pediatric and Medicine, Guerin Children's and Cedars-Sinai Cancer, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, California 90048, USA.
Gianni Bisogno, Division of Pediatric Hematology Oncology, Department of Women's and Children's Health, University Hospital of Padua, Via Giustiniani, 3 -35121 Padua, Italy.

Abstract

Soft tissue sarcomas (STS) are rare mesenchymal tumors representing up to 7% of all cancers in children. In the pediatric population, rhabdomyosarcoma (RMS) is the most common histology while the remainder is composed of several distinct histotypes collectively known as nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). RMS and NRSTS have individualized staging and treatment paradigms. Through cooperative group clinical trials, our understanding of STS and outcomes have improved. However, patients with distant metastases and who relapse continue to fare poorly. Global collaborations are necessary to make greater progress for this highest risk population.