The role of chemotherapy in the management of soft tissue sarcomas.

Prior to the use of chemotherapy, survival for rhabdomyosarcoma which had been completely resected was 50-60%. A controled study done by Children's Cancer Study Group showed the effectiveness of combined chemotherapy used as part of primary therapy in similar patients. Children who received actinomycin D (Act D) and vincristine (Vcr) for 1 year after surgery and radiotherapy had a lower metastatic rate than those who received none. This combination resulted in an 89% survival in patients with localized disease which was surgically resectable. Patients with microscopic residual disease had a 91% survival. With more aggressive use of combined chemotherapy, experience has accumulated demonstrating improved response and survival in both localized and gross residual disease. Combinations of Act D, Vcr, and cyclophosphamide can reduce initial tumor size, making large tumor masses more amenable to surgery and radiotherapy. Currently, an intergroup study is testing the response to four different drug combinations and the duration of therapy needed for various stages of the disease.