Abstract
Recent advances in molecular genetics have elucidated the biological mechanisms of familial renal cell carcinoma (RCC), which have been responsible for the development and introduction of novel targeted agents that continue to change and refine the management of advanced disease. The genetic pathways have been well characterized and defined for several hereditary kidney cancer syndromes, including von Hippel–Lindau, hereditary papillary RCC, Birt–Hogg–Dubè, and hereditary leiomyomatosis RCC. Each of these hereditary kidney cancer syndromes has distinct clinical manifestations and treatment challenges. In this context, our objective is to provide an overview of the molecular genetics, clinical syndromes, and management strategy of hereditary kidney cancer syndromes.
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Kim, S.P., Leibovich, B.C. (2013). Familial Renal Cell Carcinoma. In: Campbell, S., Rini, B. (eds) Renal Cell Carcinoma. Current Clinical Urology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-062-5_3
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