The Intergroup Rhabdomyosarcoma Study: update, november 1978

Between 1972 and 1978, 780 patients were entered on the Intergroup Rhabdomyosarcoma Study. The results indicated that postoperative irradiation of the tumor bed was unnecessary when vincristine, dactinomycin, and cyclophosphamide (VAC) were given in combination for 2 years after excision of a localized tumor (group I). This drug combination was no better than vincristine combined with dactinomycin given to patients with grossly resected tumors (group II) who also received postoperative irradiation. Intensive chemotherapy regimens, "pulse" VAC alone or VAC combined with adriamycin followed by irradiation, were equally effective in advanced stages and induced tumor regression in 85% of the patients who had gross residual disease after surgery (group III) and in 68--74% of the patients with metastatic disease at diagnosis (group IV). Two-year relapse-free survival rates were projected to be 83% for group I, 72% for group II, 65% for group III, and 28% for group IV; the overall survival rates were higher for each group. Patients with local and distant recurrences had equally poor prognoses.