Genitourinary rhabdomyosarcoma: Which treatment, how much, and when?

ObjectiveTo review the current management of pediatric genitourinary rhabdomyosarcoma (RMS).MethodsStudies performed by the Intergroup Rhabdomyosarcoma Study Group, Children's Oncology Group (COG), International Society of Paediatric Oncology (SIOP) and others over the past 10 years were reviewed to compare the use of surgery, chemotherapy, and radiotherapy for treatment of RMS and their associated outcomes.ResultsEquivalent overall survival rates were reported in the last COG and SIOP trials, with worse event-free survival rates for bladder/prostate RMS in SIOP trials. The use of radiotherapy for local control was the main difference between current COG and SIOP protocols. Surgery is used to diagnose RMS, and for local control after chemotherapy. Chemotherapy is used for systemic control of RMS, but metastatic RMS will require new approaches.ConclusionRisk stratification and risk-based therapy are being studied to decrease morbidity from treatment of RMS. The proper role of surgery vs radiotherapy for local control and whether additional treatment with second-line chemotherapy outweighs the avoidance of radiotherapy remain to be defined.