PURPOSE: 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.
CONCLUSION: 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.
Written by:
Stein R, Frees S, Schröder A, Russo A, Gutjahr P, Faber J, Thüroff JW. Are you the author?
Division of Pediatric Urology, Department of Urology, University of Mainz, Medical School, Mainz, Germany.
Reference: J Pediatr Urol. 2013 Feb 2. pii: S1477-5131(13)00015-6.
doi: 10.1016/j.jpurol.2013.01.008
PubMed Abstract
PMID: 23385048
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