Bladder management in children with genitourinary sarcoma.

Between 1977 and 1991, 14 patients were treated for genitourinary sarcoma (mean age, 7.4 years). The primary site was bladder in six patients, prostate in five, and vagina in three. Histological study showed embryonal rhabdomyosarcoma in 12 and leiomyosarcoma in 2 children. Initial therapy included biopsy followed by chemotherapy in all patients. Subsequently, five patients had anterior exenteration, four underwent partial cystectomy, and one patient had a radical prostatectomy; four patients were treated with chemotherapy and external beam pelvic irradiation (4,000 to 6,000 rads) alone. Overall survival for the group is 100% (follow-up 6 to 168 months). Two of four patients undergoing partial cystectomy had bladder augmentation at the time of surgery. All patients having partial cystectomy had negative surgical margins, are tumor free, and have volitional voiding. Two of four patients (50%) initially treated with chemotherapy and radiotherapy alone have had significant bladder deterioration requiring bladder reconstruction. There has been an evolution toward less radical, initial surgical intervention in pediatric genitourinary sarcoma; however, surgical resection continues to be the primary curative modality. Partial cystectomy with or without primary reconstruction may be preferable to exenteration for selected patients. Primary reconstruction at the time of partial cystectomy leaves a functional bladder and excellent long-term results. Children treated with chemotherapy and radiotherapy protocols alone must be monitored for late bladder deterioration.