OUTCOME ANALYSIS OF RHABDOMYOSARCOMA OF THE LOWER URINARY-TRACT

Purpose: Multimodal therapy has resulted in a high cure rate for genit ourinary rhabdomyosarcoma. We propose that the strategy of induction c hemotherapy followed by excision and reconstruction without radiothera py may provide a high cure rate without the late sequelae of pelvic ra diotherapy. Materials and Methods: We reviewed the records of 13 patie nts with a mean age of 3.3 years diagnosed with rhabdomyosarcoma of th e urinary tract from 1986 to 1996. The primary site was the bladder in 4 cases and prostate in 9. After biopsy confirmation of the diagnosis patients were treated with chemotherapy and subsequent surgery, while radiotherapy was reserved for those with residual disease. Results: A fter induction chemotherapy 8 patients underwent tumor excision via an organ sparing approach, which involved radical prostatectomy and part ial cystectomy with or without bladder augmentation, In 5 patients the final pathological analysis showed positive margins. Repeat surgery i n 6 patients included complete cystectomy and urinary diversion in 2 w ho had positive margins and recurrence after radiotherapy, urethrectom y and continent diversion for positive margins in 1, partial cystectom y and continent diversion in 1, and radical prostatectomy, partial cys tectomy and continent diversion in 1 with stage TV disease who had loc al recurrence. In the latter case ileocystoplasty had been performed f or a small noncompliant bladder. Six patients with microscopic residua l or metastatic disease received radiotherapy. At a mean followup of 5 .6 years 11 patients are disease-free. Continence was preserved in all reconstructed cases. Erections were reported in 8 patients, and renal function was maintained in 10. Conclusions: Our results suggest that urinary tract reconstruction may be safely performed at primary excisi on surgery, radiotherapy induced morbidity may be minimized by limitin g radiotherapy to residual and metastatic disease, frozen section resu lts may be false-negative, potency may be achieved and radiological di sappearance of the tumor should be confirmed histologically.