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The genito-urinary system involves organs responsible for the production and storage of urine as well as those related to male sexual function. Cancer within this system presents a unique challenge to providers as patients often present with non-specific symptoms. However, cancer in many of these organs have genetic components that may aide in the early diagnosis and individualized treatment of patients. This chapter discusses the background and genetics of kidney, bladder, prostate, and testicular cancer. Table 11.1 provides a brief summary of the hereditary cancer syndromes.

TABLE 11.1Hereditary GU Cancer Syndromes


A hereditary component exists in a small but non-negligible proportion of patients with kidney cancer. There are a number of well-described hereditary kidney cancer syndromes with a variety of renal and extrarenal manifestations. A clinical suspicion based on young age of diagnosis, presentation with bilateral or multifocal tumors, or strong family history of kidney cancer should prompt consideration for genetic testing. Surgical intervention is guided by the principle of nephron sparing when feasible and thresholds for intervention are dependent on the hereditary syndrome and size of the tumors. In this section we describe various hereditary kidney cancer syndromes along with indications for ...

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