6533b873fe1ef96bd12d4b73

RESEARCH PRODUCT

Radical Surgery in Children and Adolescents with Rhabdomyosarcoma of the Lower Urinary Tract Using Orthotopic Bladder Substitution and Heterotopic Urinary Diversion – A Single Institution Experience

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Abstract Purpose Children with rhabdomyosarcoma(RMS)of the bladder/prostate can be treated by radical or organ sparing surgery both in combination with chemotherapy ± radiation therapy. In this retrospective study we analyzed the outcome with special attention to radical surgery and urinary diversion (UD). Material and Methods 24/32 patients with RMS of the genitourinary tract had RMS of the bladder (n=14) or prostate (n=10). 8 patients were stage II, 11 stage III and 5 stage IV. In 18/24 with RMS of the bladder/prostate, radical surgery and urinary diversion was performed. 3 had chemotherapy only, 2 in combination with radiotherapy. One patient underwent partial cystectomy. For UD: ureterosigmoidostomy n=1, Mainz-pouch II n=1, continent cutaneous Mainz-pouch diversion n=10, orthotopic ileocecal pouch n=2 (2+3 yrs. of age). In the younger child, a continent appendix stoma was additionally constructed. Results 5 patients died from metastatic disease 6 – 22 months after diagnosis. 2 addional patients with RMS and Neurofibromatosis died from fibrosarcoma 168 and 264 months after cystoprostatectomy. After a follow-up of 127 months (10-372), 18 patients have no evidence of disease, 15 after radical surgery. 8 of these 15 patients developed complications requiring operative revision. All patients with a heterotopic pouch are continent as well as the girl with the rectosigmoid pouch. The two young boys with an orthotopic pouch are continent, both are voiding by Valsalva manvoever. Conclusions Partial cystectomy can be justified in selected patients with negative surgical margins. A Mainz-pouch II can only be performed, if no radiation is planned. In patients with a RMS confined to the bladder radical cystoprostatectomy and orthotopic bladder substitution is possible. The orthotopic Mainz-pouch has the advantage that the appendix can be utilized as additional cutaneous stoma to evacuate residual urine until the patient is able to void by Valsalva. In all others cutaneous urinary diversion should be performed. The long-term complication rate in this most complicated group of patients is acceptable.