Focusing on organ preservation and function: paradigm shifts in the treatment of pediatric genitourinary rhabdomyosarcoma.

ABSTRACT

OBJECTIVE: Pediatric genitourinary rhabdomyosarcoma (RMS) accounts for 25 % of all pediatric soft tissue sarcomas. The treatment of these tumors has shifted over time from debilitating radical exenteration to organ-sparing techniques using multimodal therapy. Our review aims to summarize recent relevant literature regarding the current treatment practices of pediatric genitourinary RMS and how these practices have shifted over time. METHODS: PubMed database search was utilized to identify relevant literature from 1997 to 2015 relating to the treatment of pediatric genitourinary RMS with emphasis on organ preservation and maintaining organ function. RESULTS: A total of 31 articles from 1997 through 2015 were identified relating to current management concepts in pediatric genitourinary sarcomas. Relevant articles were reviewed in detail and discussed. CONCLUSION: The treatment of pediatric genitourinary RMS has shifted from debilitating pelvic exenteration to a multimodal treatment approach involving surgery, chemotherapy, and radiation therapy in an effort to preserve genitourinary organs and reduce treatment morbidity. Continued research is required to improve post-treatment organ function. Further studies utilizing objective urodynamic evaluation are necessary to better characterize bladder function after treatment for RMS. Exciting recent developments in RMS research of fusion proteins that induce cell transformation and inhibit apoptosis and myogenic differentiation may result in future management changes to treatment protocols.