Current therapy of rhabdomyosarcoma involves the use of several modalities of treatment: surgery, radiotherapy, and chemotherapy. The present controlled study was designed to evaluate the role of chemotherapy in the control of occult residual disease following surgery and radiotherapy. The results of this study support the adjunctive use of combined chemotherapy with actinomycin D and vincristine sulfate for 1 year in preventing growth of metastatic or recurrent disease when children are made grossly tumor free by surgery and radiotherapy. Twenty‐four (85.7%) of 28 children in this category are disease free for periods greater than 2 years after initial treatment and a year of chemotherapy. Initial staging and the degree to which a patient can be made tumor free with primary definitive therapy are the most significant variables in prognosis. Vinblastine was not useful in the treatment of metastatic rhabdomyosarcoma.
From May, 1970 through December, 1972, Children's Cancer Study Group entered 112 patients on an amended treatment program for rhabdomyosarcoma and undifferentiated sarcoma in children. These patients had Group II disease with residual tumor remaining after surgery, or metastatic disease at onset. Another group consisted of patients who previously had treatment with surgery and radiotherapy and had recurrent disease. Cyclophosphamide was added to a previously used drug regimen which consisted of actinomycin D and vincristine. The drugs were given sequentially in repeated cycles for 18 months. Of 97 evaluable patients, there were 24 with microscopic residual disease, 37 with gross residual disease, 22 with metastatic disease at onset, and 14 patients who were treated with chemotherapy for the first time with recurrent or metastatic disease. All patients have been followed for 3 or more years. Survival in each group was 70.8%, 43.2%, 27.2%, and 28.2%, respectively. Although the number of complete remissions was greater than with two-drug therapy, survival with three-drug therapy was not significantly different than that seen in the earlier study.
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