Soft Tissue Sarcomas in Children

Abstract

Pediatric soft tissue sarcomas (STS) are a heterogeneous group of malignant tumors constituting about 7% of all cancer cases. Rhabdomyosarcomas (RMS) constitute about half of all soft tissue sarcomas in children, the rest being constituted by non- rhabdomyosarcoma soft tissue sarcomas (NRSTS). Most RMS present in young children <6 y of age while the NRSTS occur in adolescents and young adults. The latter constitute a diverse group of tumors and are rare in children. The STS generally present as painless enlarging mass or with symptoms of compression/infiltration of adjacent organs or structures. Staging, risk stratification and multidisciplinary approach are needed for the treatment of STS and outcome depends on stage, site and histological type. Treatment of RMS has evolved systematically through various clinical trials. Chemotherapy remains the backbone of treatment for RMS and local control is achieved either with surgery or radiotherapy or both. Management of NRSTS is still a challenge as it is generally chemotherapy-resistant and surgery remains the mainstay of treatment. Outcome therefore depends on whether wide local excision with negative margins is possible. Local radiotherapy is reserved for recurrent, residual and large high grade NRST. The prognosis of metastatic as well as recurrent STS remains dismal.