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3 - Familial and inherited renal cancers

Published online by Cambridge University Press:  08 August 2009

By
Tristan Barrett  and
Peter L. Choyke
Edited by
Uday Patel
Tristan Barrett
Affiliation:
Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland, USA
Peter L. Choyke
Affiliation:
Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland, USA
Uday Patel
Affiliation:
St George's Hospital, London
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Summary

Introduction

Although familial and inherited renal cancers account for only 1%–4% of all renal tumors, they have had a disproportionate impact on our understanding of renal cancer biology. Unlike sporadic renal cancer, hereditary forms tend to be multiple, bilateral, develop earlier in life, and occur with similar frequency between the sexes. The discovery of the von Hippel–Lindau (VHL) tumor suppressor gene in 1993 was the first definitive genetic evidence for hereditary renal cancer and it has subsequently been shown to be important in the formation of sporadic clear cell carcinomas of the kidney. The advance of genomics over the last 15 years has led to the discovery of a number of new genes and new inherited renal cancer syndromes. Inheritable diseases with an increased risk of developing renal cancer in adults include VHL (von Hippel–Lindau), BHD (Birt–Hogg–Dubé), HPRC (hereditary papillary renal carcinoma), HLRCC (hereditary leiomyomatosis renal cell cancer), TS (tuberous sclerosis), and FRO (familial renal oncocytoma). The proportion of renal tumors attributed to inherited disease may well increase as the understanding of these syndromes improves. Knowledge of these syndromes is of practical importance to the radiologist, who may be the first to suggest a hereditary basis on typical imaging findings.

The diagnosis of a genetic predisposition to cancer can lead to screening of close family members, the early detection of cancer in these individuals, and earlier, potentially more successful, treatment.

Type
Chapter
Information
Carcinoma of the Kidney , pp. 38 - 63
Publisher: Cambridge University Press
Print publication year: 2007

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  • Familial and inherited renal cancers
    • By Tristan Barrett, Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland, USA, Peter L. Choyke, Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland, USA
  • Edited by Uday Patel, St George's Hospital, London
  • Book: Carcinoma of the Kidney
  • Online publication: 08 August 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511545436.005
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To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

  • Familial and inherited renal cancers
    • By Tristan Barrett, Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland, USA, Peter L. Choyke, Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland, USA
  • Edited by Uday Patel, St George's Hospital, London
  • Book: Carcinoma of the Kidney
  • Online publication: 08 August 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511545436.005
Available formats
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  • Familial and inherited renal cancers
    • By Tristan Barrett, Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland, USA, Peter L. Choyke, Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland, USA
  • Edited by Uday Patel, St George's Hospital, London
  • Book: Carcinoma of the Kidney
  • Online publication: 08 August 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511545436.005
Available formats
×