CONSERVATIVE SURGERY IN MULTIMODAL THERAPY FOR PELVIC RHABDOMYOSARCOMA IN CHILDREN

Twenty-six previously untreated children, median age 3.4 years, with p elvic rhabdomyosarcoma (RMS) were seen between 1983 and 1988. Fourteen were girls. The planned strategy was to conserve pelvic organs, espec ially the bladder, by using primary chemotherapy, conservative surgery and, in most cases, radiotherapy. With a median follow-up of 71 month s (range 34-103 months) overall survival was 73%, with no treatment-re lated death. The bladder salvage rate of 88% in survivors with bladder base/prostate primaries was much higher than that reported by the Uni ted States Intergoup Rhabdomyosarcoma Studies (IRS), though many of th e 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 predic tion of tumour recurrence was only 81%, and endoscopic biopsies proved misleading in four of the ten bladder base/prostate patients monitore d by serial cystoscopy. We conclude that a higher cure rate can be ach ieved by using intensive chemotherapy/radiotherapy and conservative su rgery 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 monitor ed and operated on all these patients and (b) our increasing awareness , during the study, that post-chemo therapy/radiotherapy histopatholog y and pelvic CT scan appearances may be misleading. Referral to paedia tric centres with special experience of pelvic RMS may help raise the rate of bladder salvage in these children.