Genitourinary Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a malignant tumor with a variety of clinical presentations requiring varied therapeutic approaches. Radiotherapy plays an important role in the treatment of many children with RMS arising in genitourinary (GU) sites. Although the majority of these children present with locally advanced disease, the prognosis is relatively favorable when multimodility treatment strategies are used, with overall survival approaching 70% to 80%. Aggressive surgical procedures for local control are being replaced by bladdersparing treatments involving more intensive chemotherapy and the early use of radiotherapy, resulting in perservation of bladder function in as many as two thirds of children. Good radiotherapy technique is critical for ensuring not only the inclusion of adequate tissue volumes for treatment of tumor, but also for sparing normal, uninvolved structures. Optimal treatment factors such as dose, volume and timing of radiotherapy are still being investigated. This article discusses strategies currently being used for the management of GU RMS as well as research that is underway to answer unresolved questions about the biology and therapy of this disease.