Diagnosis and Management of Genitourinary Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Genitourinary RMS accounts for 15 to 25% of all RMSs and is a heterogeneous group of soft tissue tumors that vary in presentation, distribution, and prognosis. This article reviews the pathophysiology and tumor biology of RMS. It will also describe the initial approach to its diagnosis and current tumor surveillance protocols. Furthermore, this article presents the evidence behind a number of different staging and risk stratification systems currently used to guide treatment. Lastly, this article reviews future developments of investigational studies and risk stratification under investigation by a number of large international collaborative study groups. This review contains 17 figures, 7 tables, and 68 references. Keywords: Rhabdomyosarcoma, genitourinary, staging, diagnosis, paratesticular, bladder, prostate, RMS