Current standards of care in bladder and prostate rhabdomyosarcoma

• Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma (STS), and half of the children with this disease are younger than 10-year old. The 5-year event-free survival (EFS) is related to age: 71% for children aged 1 to 9 years, compared with 51% for children aged older than 10 years.
• The 2 major histologic types of RMS seen in the bladder and prostate are alveolar (10%) and embryonal (90%); the former has a much poorer prognosis than the latter.
• Staging is based on preoperative findings, group on intraoperative findings and pathology, and risk is derived from both stage and group data. Bladder and prostate RMS can never be Stage 1 tumors. PAX-FOXO1 fusion abnormalities also predict clinical outcome.
• Treatment for genitourinary RMS is multimodal, including radiation therapy and chemotherapy, with a focus on bladder preservation. Survivors should be closely followed for late effects of therapy, including lower urinary tract function, renal function, reproductive health, and development of secondary malignancies.

Rhabdomyosarcoma (RMS) is the most common soft tissue tumor in children, and 15% to 20% arise from the genitourinary tract. Multicenter collaborative studies have improved survival substantially, and in addition to excellent oncologic control, current treatment focuses on organ preservation and minimization of late treatment effects. The multiple modalities needed to treat RMS dictate that treating physicians must be familiar with the disease as well as the goals and possible sequelae of treatment with chemotherapy, radiotherapy, and surgery. This article discusses the current standards of care for bladder and prostate RMS.