Role of surgery in the management of soft tissue tumors

Pediatric soft tissue sarcomas (STSs), of which rhabdomyosarcoma is the most common, constitute approximately 5-6% of all cancers in children. Biopsy, being incisional or rarely excisional, is an essential primary diagnostic step, while in some cases it may be followed by primary re-excision to establish a correct diagnosis in which molecular analysis can be helpful. Most European STS treatment groups favor preoperative chemotherapy, sometimes combined with preoperative irradiation. Local therapy is essential, in which surgery remains an important adjunct. However, it has to be applied in a multidisciplinary, combined fashion in close cooperation with oncologists, pathologists and radiotherapeutists. Current 5-year survival in rhabdomyosarcoma reaches 60-70% in nonmetastatic cases and remains below 20% in metastatic situations. Outcome in pediatric nonrhabdomyosarcoma STS is similar.