Multidisciplinary management of pediatric soft-tissue sarcoma

The management of pediatric soft-tissue sarcomas has improved drastically t hrough the use of multimodal therapy. These tumors include rhabdomyosarcoma s and nonrhabdomyosarcomas. Both are staged using physical, radiographic, a nd histologic evaluation, and both have intricate staging and grouping syst ems that correlate closely with prognosis. However, approaches to therapy f or the two tumor types remain somewhat different. Rhabdomyosarcomas are tre ated primarily with chemotherapy. Surgical intervention is limited to initi al biopsy, wide local excision when clear margins are feasible, and resecti on of residual disease. Radiation therapy is reserved for patients with per sistant or recurrent disease and may be delivered by external beam or brach ytherapy. Nonrhabdomyosarcomas are best treated primarily by surgical resec tion, although radiation and chemotherapy are now being used with some succ ess. Another major difference concerns evaluation of lymphatics. Nonrhabdom yosarcomas in children frequently behave similarly to adult sarcomas, and l ess commonly involve regional lymph nodes, whereas pediatric patients with rhabdomyosarcomas often have nodal involvement necessitating surgical evalu ation of regional lymph nodes as part of the staging protocol. Multimodal t herapy has led to improved survival as well as better functional and cosmet ic results. With further clinical trials and improved techniques such as br achytherapy and lymphatic mapping with sentinel node biopsy, we expect to c ontinue to optimize therapy for pediatric patients with soft-tissue sarcoma s.