Outcome analysis of rhabdomyosarcoma of the lower urinary tract

Multimodal therapy has resulted in a high cure rate for genitourinary rhabdomyosarcoma. We propose that the strategy of induction chemotherapy followed by excision and reconstruction without radiotherapy may provide a high cure rate without the late sequelae of pelvic radiotherapy.

We reviewed the records of 13 patients with a mean age of 3.3 years diagnosed with rhabdomyosarcoma of the 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.

After induction chemotherapy 8 patients underwent tumor excision via an organ sparing approach, which involved radical prostatectomy and partial cystectomy with or without bladder augmentation. In 5 patients the final pathological analysis showed positive margins. Repeat surgery in 6 patients included complete cystectomy and urinary diversion in 2 who had positive margins and recurrence after radiotherapy, urethrectomy and continent diversion for positive margins in 1, partial cystectomy and continent diversion in 1, and radical prostatectomy, partial cystectomy and continent diversion in 1 with stage IV disease who had local recurrence. In the latter case ileocystoplasty had been performed for a small noncompliant bladder. Six patients with microscopic residual 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.

Our results suggest that urinary tract reconstruction may be safely performed at primary excision surgery, radiotherapy induced morbidity may be minimized by limiting radiotherapy to residual and metastatic disease, frozen section results may be false-negative, potency may be achieved and radiological disappearance of the tumor should be confirmed histologically.