Abstract
Soft-tissue sarcoma (STS) are a group of rare tumors diagnosed in children and adults that require multi-modality specialty care. The etiology of STS in children is largely unknown although they are among the tumor types associated with cancer predisposition syndromes and included in the differential of radiation-induced malignancies. STS include a broad variety of histologic subtypes, diverse presentations, and wide-ranging lethality. Evaluation and management of STS in pediatric patients has evolved based on knowledge derived from clinical trials performed in adults and more recently prospective studies performed exclusively with children. Surgery, irradiation modalities, and conventional chemotherapy have identified roles in the treatment of pediatric patients with current studies focused on toxicity reduction in low-risk patients, optimal sequencing of therapy and new agent testing in intermediate and high-risk patients, and systematic salvage strategies for patients that do not respond to front-line therapy.
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Tinkle, C.L., Lucas, J.T. (2018). The Non-rhabdomyosarcoma Soft Tissue Sarcomas, Desmoid Tumor and Osteosarcoma. In: Merchant, T., Kortmann, RD. (eds) Pediatric Radiation Oncology. Pediatric Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-43545-9_4
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