title: Radical surgery and different types of urinary diversion in patients with rhabdomyosarcoma of bladder or prostate--a single institution experience.
abstract: OBJECTIVE
  In a retrospective study we analyzed the outcome of patients treated for rhabdomyosarcoma (RMS) of the bladder/prostate with special attention to radical surgery.
  
  
  METHODS
  In 25 patients with genitourinary RMS (15 bladder/10 prostate) the median age at diagnosis was 4 years [1-18], and 8 patients had a stage II RMS, 12 stage III and 5 stage IV. In 19/25 (12 bladder/7 prostate), radical surgery and urinary diversion were performed. Urinary diversion comprised 2 continent anal diversions, 11 continent cutaneous diversions, 4 colon conduits and 2 urethral diversions (2 + 3 years of age). In the younger child with urethral diversion, a cutaneous appendix stoma was additionally constructed in case of inability to void spontaneously.
  
  
  RESULTS
  4/19 patients who underwent radical surgery died of metastatic RMS; 1 patient with neurofibromatosis died of a secondary tumor. After median follow-up of 132 months (14-420), 14 patients currently have no evidence of disease. 8/14 patients who survived developed 17 complications requiring operative revision. All patients with a continent diversion are continent. The patients with orthotopic bladder substitution are continent day & night and void spontaneously.
  
  
  CONCLUSIONS
  For RMS confined to the bladder or bladder neck, radical cystoprostatectomy and orthotopic bladder substitution are an option. Urethral diversion using the ileocecal segment (Mainz-pouch I) offers the advantage of utilizing the appendix as an additional continent cutaneous stoma, which enables parents to evacuate residual urine in young boys, until able to empty the pouch completely themselves. For all other patients with vital tumor after primary chemotherapy, cutaneous urinary diversion is an option. Long-term complication rates in this complex group of patients are acceptable.
authors: Division of Pediatric Urology, Department of Urology, University of Mainz, Medical School, Mainz, Germany. Electronic address: Raimund.Stein@unimedizin-mainz.de.
authors: Raimund
authors: R
authors: Stein
authors: Sebastian
authors: S
authors: Frees
authors: Annette
authors: A
authors: Schröder
authors: Alexandra
authors: A
authors: Russo
authors: Peter
authors: P
authors: Gutjahr
authors: Jörg
authors: J
authors: Faber
authors: Joachim W
authors: JW
authors: Thüroff
mesh_terms: D000293:Adolescent; D000328:Adult; D001003:Anal Canal / Q000601:surgery; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D003609:Dactinomycin / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000401:mortality; D011471:Prostatic Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D054047:Surgical Stomas; D016896:Treatment Outcome; D001749:Urinary Bladder Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D014547:Urinary Diversion / Q000379:methods* / Q000401:mortality; D016476:Urinary Reservoirs, Continent; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide
keywords: Bladder & prostate; Continent anal diversion; Continent cutaneous urinary diversion; Orthotopic bladder substitution; Rhabdomyosarcoma