Soft-tissue sarcomas in the young: medical treatment advances in perspective.

One hundred and sixty-two patients with all types of soft tissue sarcoma, less than 21 years old and diagnosed at a single institution during the period 1958 through 1976, were studied. The major histologic subset comprised 115 patients with either embryonal rhabdomyosarcoma or undifferentiated sarcoma. In this group, responses of patients with stage I-II disease to local treatment alone indicated that at least half had no occult metastatic disease. Responses to irradiation alone in stage III disease indicated that only about a quarter of these patients had no occult disease. Sustained local control at the primary site by irradiation was observed in 68% of the patients given doses of 4,500 rad or greater compared with 22% at lower doses. The effectiveness of adjuvant chemotherapy was best seen in patients with stage III disease as a decrease in relapse rate and a lengthening of the time to first relapse. In these patients it appeared that occult metastatic disease could be eradicated in approximately half of the patients affected. In all the other varieties of soft tissue sarcoma in young people, recurrence at the primary site assumed relatively greater importance. This complication was markedly reduced in frequency when local postoperative irradiation was given in doses of 4,500 rad or greater. In this very heterogeneous group of malignancies, the value of adjuvant chemotherapy remained uncertain.