UROGENITAL RHABDOMYOSARCOMA IN CHILDREN - IS A CONSERVATIVE SURGICAL APPROACH JUSTIFIED

Between 1970 and 1990, 23 patients were treated for embryonal rhabdomy osarcoma of the bladder (8), prostate (7), uterus (4) or vagina (4). O ne girl underwent exenteration without chemotherapy. Of the patients 2 2 received chemotherapy as initial treatment. There was 1 treatment-re lated death. A total of 21 patients completed the first phase: 4 were treated with chemotherapy alone and 17 subsequently underwent an opera tion. Three boys underwent total cystoprostatectomy and local resectio n was performed in 14 patients. One boy was lost to followup. Mean fol lowup was 11.2 +/- 6.3 years in 15 survivors. Complete remission was a chieved in 19 patients, while 11 (53%) had relapse at an interval of 3 to 102 months. Of 13 patients with bladder and prostate tumors 8 had relapse and 7 survived. Of 8 patients with uterus and vagina tumors 3 had relapse and all survived. Six patients died of recurrent disease ( overall mortality rate 32%). The interval from initial diagnosis until death was always less than 5 years. The mortality rate was greater in boys (5 of 12) than in girls (1 of 9). Preservation of pelvic organs in girls was 75%, while of the boys 44% retained the bladder. Since di sease relapsed in patients in whom no tumor was found in the resection specimen after chemotherapy, we conclude that a conservative surgical approach combined with chemotherapy towards urogenital rhabdomyosarco ma is justified. Although late relapses do occur, they can usually be salvaged by a combination of chemotherapy, radiotherapy and surgery.