A randomized study of 264 children and adults with previously untreated 
localized Ewing's sarcoma of bone was undertaken between 1973 and 1978 by 83 
institutions of three national study groups: Children's Cancer Study Group, 
Southwest Oncology Group, and Cancer and Leukemia Group B. The Intergroup Study 
was designed to determine if the addition of adriamycin (ADR) or bilateral 
pulmonary radiotherapy (RT) to vincristine, dactinomycin, and cyclophosphamide 
(VAC therapy) would improve survival and reduce local recurrences and 
metastases. All patients received RT to the primary lesion, and the survival 
rate after 3 years was 65%. The most effective treatment regimen was VAC plus 
ADR; 74% of the patients were free of disease at 2 years. The lengths of 
disease-free status and survival of patients treated with VAC plus ADR or VAC 
plus RT did not differ. However, both regimens were significantly superior to 
treatment with VAC alone. The addition of ADR or bilateral pulmonary RT to VAC 
was highly advantageous to patients with nonpelvic primaries. Bone and lung were 
the major sites of distant relapse, but the addition of bilateral pulmonary RT 
showed no advantage over that of ADR in reducing the occurrence of lung 
metastases. These recent results should eliminate some of the pessimism that has 
accompanied a diagnosis of Ewing's sarcoma, although distant metastases 
continued to be a major reason for failure in the control of this tumor. 
Survival of these patients can be improved through well-controlled clinical 
trials designed to determine optimal adjuvant chemotherapy and treatment of the 
primary lesion.