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Diagnosis and Management of Hereditary Renal Cell Cancer

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Rare Hereditary Cancers

Part of the book series: Recent Results in Cancer Research ((RECENTCANCER,volume 205))

Abstract

Renal cell cancer (RCC) is the common denominator for a heterogeneous group of diseases. The subclassification of these tumours is based on histological type and molecular pathogenesis. Insight into molecular pathogenesis has led to the development of targeted systemic therapies. Genetic susceptibility is the principal cause of RCC in about 2–4 % of cases. Hereditary RCC is the umbrella term for about a dozen different conditions, the most frequent of which is von Hippel–Lindau disease . Here, we describe the main hereditary RCC syndromes, consider criteria for referral of RCC patients for clinical genetic assessment and discuss management options for patients with hereditary RCC and their at-risk relatives.

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Menko, F.H., Maher, E.R. (2016). Diagnosis and Management of Hereditary Renal Cell Cancer. In: Pichert, G., Jacobs, C. (eds) Rare Hereditary Cancers. Recent Results in Cancer Research, vol 205. Springer, Cham. https://doi.org/10.1007/978-3-319-29998-3_6

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