SmminaryTwenty-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 Intergoup 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.
An 18-year-old man presented in October 1986 with a 2day history of pain in the right testis. He had spina bifida with paraplegia. He had practised self-catheterisation for many years but began to suffer from incontinence between catheterisations due to decreasing bladder size and in January 1985 he underwent a caecocystoplasty elsewhere which cured this problem.On examination his right testis was shrunken and tethered to the scrotal skin by a sinus tract which was discharging purulent material. Abdominal X-ray showed bladder calculi containing surgical staples and other staples in the region of the vault of the bladder (Fig.). Urine culture grew Escherichiu coli sensitive to cephalosporins. He was treated with cephradine and advised to have his right testis and bladder calculi removed. At operation his right testis was excised via a scrotal incision and lithotripsy was carried out using a Storz lithotrite. Fragments of triple phosphate calculi were confirmed to contain staples. Histology of the testis showed an abscess cavity and little remaining testicular tissue.
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