Urogenital rhabdomyosarcoma in children: is a conservative surgical approach justified?
Between 1970 and 1990, 23 patients were treated for embryonal rhabdomyosarcoma of the bladder (8), prostate (7), uterus (4) or vagina (4). One girl underwent exenteration without chemotherapy. Of the patients 22 received chemotherapy as initial treatment. There was 1 treatment-related death. A total of 21 patients completed the first phase: 4 were treated with chemotherapy alone and 17 subsequently underwent an operation. Three boys underwent total cystoprostatectomy and local resection was performed in 14 patients. One boy was lost to followup. Mean followup was 11.2 +/- 6.3 years in 15 survivors. Complete remission was achieved 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 disease 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 rhabdomyosarcoma is justified. Although late relapses do occur, they can usually be salvaged by a combination of chemotherapy, radiotherapy and surgery.
Department of Pediatric Surgery, Academic Medical Centre/Emma Children's Hospital, Amsterdam, The Netherlands.
H A
HA
Heij
A
A
Vos
J
J
de Kraker
P A
PA
Voute
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000601:surgery*; D014565:Urogenital Neoplasms / Q000188:drug therapy / Q000601:surgery*