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

J Urol. 2014 Jun;191(6):1850-5. doi: 10.1016/j.juro.2013.12.040. Epub 2013 Dec 30.

Abstract

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.

Keywords: cystectomy; prostate; rhabdomyosarcoma; urinary bladder; urinary diversion.

MeSH terms

  • Biopsy
  • Child
  • Child, Preschool
  • Cystectomy / methods*
  • Cystoscopy
  • Female
  • Forecasting*
  • Humans
  • Male
  • Prostatic Neoplasms / diagnosis
  • Prostatic Neoplasms / physiopathology
  • Prostatic Neoplasms / surgery*
  • Retrospective Studies
  • Rhabdomyosarcoma / diagnosis
  • Rhabdomyosarcoma / physiopathology
  • Rhabdomyosarcoma / surgery*
  • Treatment Outcome
  • Urinary Bladder / physiopathology
  • Urinary Bladder / surgery
  • Urinary Bladder Neoplasms / diagnosis
  • Urinary Bladder Neoplasms / physiopathology
  • Urinary Bladder Neoplasms / surgery*
  • Urinary Reservoirs, Continent*
  • Urination / physiology*