title: Conservative surgery in multimodal therapy for pelvic rhabdomyosarcoma in children.
abstract: Twenty-six previously untreated children, median age 3.4 years, with pelvic rhabdomyosarcoma (RMS) were seen between 1983 and 1988. Fourteen were girls. The planned strategy was to conserve pelvic organs, especially the bladder, by using primary chemotherapy, conservative surgery and, in most cases, radiotherapy. With a median follow-up of 71 months (range 34-103 months) overall survival was 73%, with no treatment-related death. The bladder salvage rate of 88% in survivors with bladder base/prostate primaries was much higher than that reported by the United States Intergroup Rhabdomyosarcoma Studies (IRS), though many of the preserved bladders did not function normally. We identified problems with both radiological and histological off-treatment monitoring. The overall accuracy of computerised tomographic (CT) scanning for prediction of tumour recurrence was only 81%, and endoscopic biopsies proved misleading in four of the ten bladder base/prostate patients monitored by serial cystoscopy. We conclude that a higher cure rate can be achieved by using intensive chemotherapy/radiotherapy and conservative surgery to treat children with pelvic RMS. Factors that might contribute to our favourable bladder salvage results, compared with those of the IRS, include (a) the fact that one of two specialist surgeons monitored and operated on all these patients and (b) our increasing awareness, during the study, that post-chemotherapy/radiotherapy histopathology and pelvic CT scan appearances may be misleading. Referral to paediatric centres with special experience of pelvic RMS may help raise the rate of bladder salvage in these children.
authors.affiliation: Department of Haematology and Oncology, Hospitals for Sick Children, London, UK.
authors.forename: A
authors.initials: A
authors.lastname: Atra
authors.forename: H C
authors.initials: HC
authors.lastname: Ward
authors.forename: K
authors.initials: K
authors.lastname: Aitken
authors.forename: M
authors.initials: M
authors.lastname: Boyle
authors.forename: C
authors.initials: C
authors.lastname: Dicks-Mireaux
authors.forename: P G
authors.initials: PG
authors.lastname: Duffy
authors.forename: C D
authors.initials: CD
authors.lastname: Mitchell
authors.forename: P N
authors.initials: PN
authors.lastname: Plowman
authors.forename: P G
authors.initials: PG
authors.lastname: Ransley
authors.forename: J
authors.initials: J
authors.lastname: Pritchard
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010386:Pelvic Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D011877:Radionuclide Imaging; D012208:Rhabdomyosarcoma / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D001743:Urinary Bladder / Q000502:physiology / Q000601:surgery*