Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery.

Abstract

To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968. From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5-18) had RMS of bladder/prostate or vagina. Twenty of the patients received primary polychemotherapy (vincristine, actinomycin D, cyclophosphamide, adriamycin, and more recently including etoposide and ifosfamide), two had primary surgery and seven had additional radiotherapy. Fourteen patients had radical cystoprostatectomy, with continent cutaneous urinary diversion with an ileocaecal pouch in seven, in one each a transverse colonic pouch, orthotopic ileocaecal bladder substitution, a rectal reservoir and rectosigmoid pouch and a colonic conduit diversion in two patients. After a mean (range) follow-up of 8.6 (1.0-26) years, 17 patients had no evidence of disease. Five patients presenting initially with advanced tumour stages died from progressive RMS. Two patients with a continent urinary diversion required ureteric reimplantation for stenosis. In two patients severe bladder contraction after radiotherapy required bladder augmentation. Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.