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Inherited Renal Tumour Syndromes

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Primer on Nephrology

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Abstract

Approximately 3–5% of all cases of kidney cancer are inherited. These familial kidney cancer syndromes can cause disease in a wide range of organ systems in addition to the kidney. They include von Hippel-Lindau disease, tuberous sclerosis, Birt-Hogg-Dube syndrome, and hereditary leiomyomatosis. Screening and management guidelines have been designed to reduce mortality and morbidity by early detection of tumours. MRI is now the preferred modality of imaging, and follow-up is best achieved by close multidisciplinary team cooperation.

The genes involved in different types of inherited kidney cancer are all involved in related cellular pathways. Importantly, most sporadic kidney cancers also involve the same genes and dysregulation of the same cellular pathways. This has allowed scientists to develop targeted therapies, which have revolutionised the treatment of this cancer.

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Authors and Affiliations

  1. Oxford Kidney Unit, Churchill Hospital, Oxford, UK

    Thomas M. F. Connor

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  1. Thomas M. F. Connor
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Correspondence to Thomas M. F. Connor .

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Editors and Affiliations

  1. Department of Renal Medicine, UCL, London, UK

    Mark Harber

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Connor, T.M.F. (2022). Inherited Renal Tumour Syndromes. In: Harber, M. (eds) Primer on Nephrology. Springer, Cham. https://doi.org/10.1007/978-3-030-76419-7_59

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  • DOI: https://doi.org/10.1007/978-3-030-76419-7_59

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-76418-0

  • Online ISBN: 978-3-030-76419-7

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