Rhabdomyosarcoma of the female urogenital tract

During the initial 6 yr (1972-1978) of the Intergroup Rhabdomyosarcoma Study (IRS), 27 patients with rhadomyosarcoma of the female urogenital tract were treated, and the results of therapy were evaluated on the basis of 3-7 yr of observation. By primary site these tumors were: bladder, 14; vagina, 9; vulva, 2; and cervix uterus, 2. Twenty-three patients presented with localized tumors; the four remaining patients (1 bladder, 1 vagina, and 2 cervix) had distant metastases at diagnosis. Two patients with bladder tumors underwent pelvic exenteration, local irradiation, and chemotherapy (2 yr), and were without relapse. Ten patients were treated by partial cystectomy and postoperative chemotherapy (with or without local irradiation) and fatal recurrence occurred in three. The remaining seven patients are disease-free for 36-74 mo (mean, 51.8 mo). Six of the eight patients with nondisseminated vaginal tumors were treated by initial pelvic exenteration (2), hysterectomy-vaginectomy (2), or hemivaginectomy (2). All received postoperative chemotherapy and three received local irradiation. Delayed hysterectomy-vaginectomy after primary chemotherapy was performed in the two additional patients with vaginal tumors. Relapse has occurred in one of these eight patients with localized vaginal disease. The remaining seven have been observed for 3-5.5 yr (mean, <4 yr). Two patients with vulval lesions treated by a combined therapy regimen are without recurrence. Our conclusion is that chemotherapy has significantly increased survival in females with urogenital tract rhabdomyosarcomas.