Childhood rhabdomyosarcoma of the extremity: Results of combined modality therapy

Abstract

Fifteen children with rhabdomyosarcoma of an extremity were treated with one of several combinations of surgery, radiotherapy, and chemotherapy, depending on stage of disease at diagnosis. All patients received combination chemotherapy administered as a 4‐ to 6‐week induction course, followed by 12 to 18 months of continuation chemotherapy. When feasible, the primary tumor was resected en bloc before chemotherapy was begun. Following the induction regimen, patients with either unresectable regional tumor, or disseminated tumor and an initial complete response to chemotherapy, received megavoltage radiotherapy to the primary site and regional lymphatics. Eleven of 15 patients (0.73) developed or maintained a complete tumor response to induction therapy alone. Radiotherapy maintained control of local disease in six of six patients treated. Overall, six patients, including one with disseminated disease at diagnosis, are surviving 15 to 45 months from diagnosis; four have been clinically free of tumor for more than 36 months. The presence or absence of dissemination at diagnosis and location of primary tumor on the extremity were significant determinants of survival (p < .01). Antecedent trauma to the primary site resulted in delay in diagnosis in five patients but did not directly affect survival. Documented bone marrow infiltration during the clinical course was associated with either cardiac or intracranial tumor or both in five of six evaluable patients. Primary involvement of an extremity by rhabdomyosarcoma has been reported to have an unfavorable influence upon survival compared with involvement of nonextremity sites. The combined results of the current study and more recent prospective controlled series fail to support these earlier studies and demonstrate that any prognostic significance of anatomic site is lost, when analyzed by stage. Cancer 40:2810‐2816, 1977. Copyright © 1977 American Cancer Society