Treatment of rhabdomyosarcoma (RMS) of the bladder and prostate has always been difficult. Despite the significant advancements that have permitted cure, there is no agreement in the choice and order of the different therapeutic options. We reviewed the available literature evaluating the role of non-radical surgery and attempts at bladder preservation especially in children. Partial resection with sufficient margin of safety is only possible in 20% of the primary bladder tumors and the 3-year survival rate is 80%, similar to the overall survival rate for tumors localized to this site. The excellent results achieved in the case described herein underscores the possibilities of a conservative treatment modality in well-selected cases. The patient, who was submitted to partial cystectomy and received VAC polychemotherapy for a period of one year, is alive, tumor free and has preserved bladder function 50 months post-operatively.

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