Genetics of Kidney Cancer (Renal Cell Cancer) (PDQ®)–Health Professional Version

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Changes to This Summary (03/08/2017)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Von Hippel-Lindau Disease (VHL)

The Genotype-phenotype correlations subsection was comprehensively reviewed and extensively revised.

Revised Table 3, Genotype-Phenotype Classification of Families With VHL, to summarize the defining characteristics of each VHL subtype.

Added Huntoon et al. as reference 76.

Revised text to state that levels of plasma and urine normetanephrine are typically elevated in patients with VHL, and approximately two-thirds will experience physical manifestations such as hypertension, tachycardia, and palpitations. Also added text to state that patients with a partial loss of VHL function are at higher risk of pheochromocytoma than are VHL patients with a complete loss of VHL function; the latter develop pheochromocytoma very rarely (cited Friedrich et al. as reference 81).

Added Pancreatic manifestations as a new subsection.

Revised Table 5, Practice Guidelines for Surveillance of VHL, to reflect the latest guidelines from the VHL Alliance (cited VHL Alliance as reference 100).

This summary is written and maintained by the PDQ Cancer Genetics Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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