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

Abstract

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. We retrospectively analyzed oncologic and urological outcomes of 11 consecutive patients treated at our institution between 1998 and 2012. Two patients underwent urethrectomy and placement of a heterotopic catheterizable ileal neobladder. The membranous urethra was preserved in 9patients, 6 underwent primary Padua ileal neobladder at radical cystectomy, 2underwent 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. 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.