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Renal cancer in von Hippel–Lindau disease and related syndromes

Nature Reviews Nephrology volume 9, pages 529–538 (2013)Cite this article

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Abstract

Sporadic and hereditary forms of renal cell carcinoma (RCC), von Hippel–Lindau (VHL) disease and the familial paraganglioma syndromes are closely related in terms of their clinical, molecular, and genetic aspects. Most RCCs occur sporadically and the heritable fraction of RCC is estimated to be just 2–4%. An understanding of the molecular genetic basis, the disease-specific and gene-specific biology and the clinical characteristics of these cancer syndromes is of utmost importance for effective genetic diagnosis and appropriate treatment. In addition, such insight will improve our understanding of sporadic RCCs. To date, 10 different heritable RCC syndromes have been described. VHL syndrome is the oldest known hereditary RCC syndrome. Similar to VHL disease, phaeochromocytoma is a major manifestation of the paraganglioma syndromes types 1, 3 and 4 in which RCCs have been reported. These syndromes are therefore regarded as VHL-related disorders and are included in this Review. Multifocal tumours, bilateral occurrence, a young age at diagnosis and/or family history are clinical red flags suggestive of hereditary disease and should trigger referral for genetic and molecular analysis. The identification of an underlying genetic alteration enables gene-specific risk assessment and opens up the possibility of a tailored follow-up strategy and specific surveillance protocols as the basis of effective preventive medicine. The important goals of preventive medicine are to increase the life expectancy of affected patients and to improve their quality of life. The study of seemingly rare hereditary syndromes and their susceptibility genes has consistently revealed clues regarding the aetiology and pathogenesis of these diseases, and can aid diagnosis and the development of therapeutics for patients affected by much more common sporadic counterparts.

Key Points

  • Von Hippel–Lindau (VHL) disease is the oldest known, most common hereditary renal cell carcinoma (RCC) syndrome; it is an autosomal dominant disorder caused by germline mutations in the VHL tumour suppressor gene

  • RCCs are the major neoplasm and leading cause of death in patients with VHL disease

  • A substantial amount of our knowledge regarding the tumourigenesis and treatment strategies for sporadic renal cancer results from the investigation of heritable types of RCC, particularly VHL-associated RCC

  • At least 50% of sporadic RCCs are associated with somatic inactivation of the VHL gene

  • Sporadic RCCs and the hereditary RCC syndromes, VHL disease and the paraganglioma syndromes differ in their molecular background but involve a common final pathway, the HIF–VEGF pathway

  • Improved knowledge of the molecular genetic basis, gene-specific biology and clinical characteristics of VHL disease are contributing to the improved assessment of neoplasia risk and the development of effective preventive strategies and treatment options

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Figure 1: MRI of renal lesions in von Hippel–Lindau disease.
Figure 2: Extrarenal and extra-adrenal lesions in von Hippel–Lindau disease.
Figure 3: Histopathological characteristics of simple and complex renal cysts and renal cell carcinoma in von Hippel–Lindau disease.
Figure 4: Paraganglial tumours.

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Authors and Affiliations

  1. Department of Gastroenterology, Hepatology, Endocrinology and Infectious Diseases, University Hospital, Hugstetter Strasse 55, Freiburg, D-79106, Germany

    Birke Bausch

  2. Department of Diagnostic Radiology, University Hospital, Hugstetter Strasse 55, Freiburg, D-79106, Germany

    Niklas Lützen

  3. Department of Diagnostic Radiology, and Department of Ophthalmology, University Hospital, Medical University Center, Hugstetter Strasse 55, Freiburg, D-79106, Germany

    Alexandra Anton

  4. Department of Urology, Medical University Center, Albert-Ludwigs-University, Hugstetter Strasse 55, Freiburg, D-79106, Germany

    Cordula Jilg

  5. Department of Neurosurgery, Medical University Center, Albert-Ludwigs-University, Hugstetter Strasse 55, Freiburg, D-79106, Germany

    Sven Gläsker

  6. Department of Neurosurgery, and Department of Nephrology and General Medicine, Medical University Center, Albert-Ludwigs-University, Hugstetter Strasse 55, Freiburg, D-79106, Germany

    Hartmut P. H. Neumann

  7. Division of Neuropathology, Yale University, New Haven, CT, USA

    Alexander Vortmeyer

  8. Genomic Medicine Institute, Lerner Research Institute and Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA

    Charis Eng

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B. Bausch, C. Jilg, and H. P. H. Neumann researched data for the article, provided a substantial contribution to discussion of content, wrote the article and reviewed/edited the manuscript before submission. S. Gläsker and C. Eng provided a substantial contribution to discussion of content, wrote the article and reviewed/edited the manuscript before submission. A. Vortmeyer and N. Lützen provided a substantial contribution to discussion of content and reviewed/edited the manuscript before submission. A. Anton researched data for the article.

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Bausch, B., Jilg, C., Gläsker, S. et al. Renal cancer in von Hippel–Lindau disease and related syndromes. Nat Rev Nephrol 9, 529–538 (2013). https://doi.org/10.1038/nrneph.2013.144

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