The surgical management of paediatric bladder and prostate rhabdomyosarcoma

BACKGROUND: The surgical management of paediatric bladder/prostate rhabdomyosarcoma (B/P RMS) continues to develop, with the goal of maximising organ preservation while achieving successful cancer control. The timing of radiotherapy and surgical excision to improve event-free survival (EFS) and overall survival (OS) remains controversial. METHODS: Previous reports in English on B/P RMS over the past 15 years were identified and reviewed, focusing on studies comparing the effects of radiotherapy and surgery for local control, the effect of local control on OS, and improved means of diagnosing viable tumour after chemotherapy. RESULTS: The concept of lowering the ‘cost of cure’ drives current protocols. Bladder-sparing surgery is possible for 80% of patients after initial chemotherapy, with a mean 5-year OS of 85%. Overall, half of the patients are continent of urine, and adding radiotherapy might increase the risk of incontinence. Previous studies suggesting that early radiotherapy achieved better EFS than delayed radiotherapy did not control for stage and size of the tumour, which are the primary determinants of EFS. Improved local control does not automatically translate into improved OS. CONCLUSIONS: The current role for the surgical management in B/P RMS is to achieve local control of tumours that do not respond to chemotherapy and radiotherapy. An improved means of detecting viable tumour after initial chemotherapy would improve the ability to decide when local therapy is necessary. The continuing challenge for urologists managing these children is knowing when bladder-sparing surgery would be the best therapy.