Sarcomas of the vagina and uterus: the Intergroup Rhabdomyosarcoma Study.

During a 12-year period (1972-1984), 43 patients with sarcomas of the female genital tract were admitted to the Intergroup Rhabdomyosarcoma Study (IRS), including 31 with primary tumors of the vagina; and 12 with tumors of the uterus, including the cervix. Thirty-four of these can be evaluated on the basis of periods of observation from 18 months to 12 years. Primary tumors of the uterus were a distinct group, distinguished from those arising in the vagina by patient age-range and probably by prognosis, as well as site. Patients with vaginal tumors, with a mean age of 1.8 years, responded to a multimodality approach employing combinations of chemotherapy (vincristine sulfate and actinomycin D, or the aforementioned two drugs with cyclophosphamide +/- doxorubicin hydrochloride), irradiation, and/or surgery, with only one tumor-related death, among 24 evaluable patients. In contrast, among the patients with primary uterine tumors, in which the mean age was greater than 14 years, four of ten evaluable patients died secondary to tumor relapse or progression.