Current treatment of pediatric bladder and prostate rhabdomyosarcoma (bladder preserving vs. radical cystectomy)

To discuss alternative strategies for multimodal treatments of nonmetastatic bladder-prostate rhabdomyosarcoma performed with the aim of preserving organ function.

Bladder-prostate rhabdomyosarcomas are seldom fully resectable at presentation or after induction chemotherapy, and extensive resection might not improve survival. When an organ-sparing approach is pursued, radiotherapy might be unavoidable to achieve reliable local control of the disease. Benefits of preoperative vs. postoperative radiotherapy have yet to be investigated. Multimodal treatments may often result in bladder function impairment and erectile dysfunction. To reduce long-term side effects of radiotherapy, irradiation modalities allowing for more targeted treatment should be favoured. For this purpose, external beam proton therapy or nonradical surgery associated with brachytherapy may be viable options. Nevertheless, experience with these treatments is still limited. Advancements in lower urinary tract reconstruction make preservation of volitional voiding and erectile function possible after cystoprostatectomy. But in the context of multimodal treatment, cystoprostatectomy is reserved to patients who respond poorly to other treatments.

For the vast majority of bladder-prostate rhabdomyosarcoma, we believe that reliable local control of disease can only be achieved with the use of radiotherapy. Efforts should be made to find the best modality for targeted radiotherapy. Further studies are required to compare preoperative vs. postoperative radiotherapy and the best dose to be administered in order to reduce long-term side effects. If creation of an orthotopic continent diversion is deemed appropriate in patients undergoing cystoprostatectomy, it should be performed concurrently with extirpative surgery.