Oncologic Outcome and Urinary Function after Radical Cystectomy for Rhabdomyosarcoma in Children: Role of the Orthotopic Ileal Neobladder Based on 15-Year Experience at a Single Center

PURPOSE: We determined the oncologic and urological outcomes in patients with bladder/prostate rhabdomyosarcoma according to the type and timing of urinary tract surgery, with emphasis on the role of the Padua orthotopic ileal neobladder. MATERIALS AND METHODS: We retrospectively analyzed oncologic and urological outcomes of 11 consecutive patients treated at our institution between 1998 and 2012. RESULTS: Two patients underwent urethrectomy and placement of a heterotopic catheterizable ileal neobladder. The membranous urethra was preserved in 9 patients, 6 underwent primary Padua ileal neobladder at radical cystectomy, 2 underwent delayed Padua ileal neobladder and 1 underwent bilateral cutaneous ureterostomy. Four of these 9 patients experienced disease recurrence, including local recurrence in 2 despite negative intraoperative biopsies. Survivors undergoing heterotopic catheterizable ileal neobladder or primary Padua ileal neobladder learned to empty the bladder to completion without long-term upper tract deterioration. Both cases managed by delayed Padua ileal neobladder required clean intermittent catheterization eventually. Erections were reported in 5 of 6 surviving males. CONCLUSIONS: The Padua ileal neobladder allowed preservation of volitional urethral voiding in all survivors in whom it was placed at radical cystectomy. Nevertheless, local recurrence was noted in 2 of the 9 cases where the membranous urethra was preserved. By comparison, patients undergoing delayed Padua ileal neobladder after attaining disease-free status never achieved voiding per urethra. Therefore, a heterotopic reservoir might be a more reliable choice under these circumstances. Erectile function is preserved in the majority of cases