Rhabdomyosarcoma and related pelvic tumors of the genitourinary tract

Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma in infants and children, with approximately 350 new cases diagnosed in the United States each year. Of these, 15-20% arise from the genitourinary tract, the most common sites being the prostate, bladder, and paratesticular regions, followed by the vagina and uterus. A multimodal treatment approach includes systemic chemotherapy for all patients and local control involving surgical resection with or without radiation therapy. Currently, a principal goal of surgical management is organ preservation when possible. Despite improved overall survival, children with RMS continue to suffer from a wide range of treatment-related side effects and complications. Meticulous follow-up is required to assess the sequelae of current therapies.