Childhood rhabdomyosarcoma of the trunk and extremities

Since 1979, 15 children with rhabdomyosarcoma have been treated at our institution. Included in this group are six children who presented with rhabdomyosarcoma of an extremity or trunk, requiring the use of combined multimodality therapy. The patients were clinically grouped and treated in accordance with the Intergroup Rhabdomyosarcoma Study protocol. All patients received combination chemotherapy based on their respective stage of disease at diagnosis. When feasible, the primary tumor was resected en bloc before chemotherapy was begun. After surgery, patients with unclear postoperative surgical margins and an initial good response to chemotherapy received radiotherapy to the primary site and at the regional lymphatics. Three of six patients developed or maintained a complete tumor response to induction chemotherapy. Radiotherapy maintained control of local disease in both groups. Overall, four patients, including one with disseminated disease at diagnosis, are alive, with a median survival time from diagnosis of 39 months. In children, treatment must be individualized, but complete local excision of the tumor with a tumor-free margin should be the goal. Major ablative amputation surgery was not performed.