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

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Changes to This Summary (05/18/2018)

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.

Executive Summary

Added text to state that extrarenal manifestations of von Hippel-Lindau disease (VHL), such as retinal hemangioblastomas, central nervous system (CNS) lesions, pheochromocytomas, and pancreatic cysts and neuroendocrine tumors, often require subspeciality evaluation and may require surgical intervention.

Von Hippel-Lindau Disease

Revised text to state that the incidence of VHL has been estimated to be between 1 per 27,000 and 1 per 43,000 live births in the general population (cited Binderup et al. and Evans et al. as references 9 and 10, respectively) and the prevalence has been estimated to be between 1 in 31,000 individuals to 1 in 91,000 individuals (cited Poulsen et al. as reference 12).

Revised Table 2, Neoplasms in von Hippel-Lindau Disease: Mean Age at Diagnosis and Cumulative Risk in Affected Patients, to state that limited data are available for cystadenomas of the broad/round ligament and epididymis.

Revised text to state that retinal involvement is one of the earliest manifestations of VHL, with a mean age at onset of 25 years.

Added text to state that the mean age at onset of CNS hemangioblastomas in patients with VHL is 29.1 years (cited Kanno et al. as reference 80).

Added text to state that the mean age at diagnosis of a pancreatic neuroendocrine tumor was 38 years in a large cohort of patients with VHL.

The Epididymal cystadenomas subsection was renamed from Epididymal cysts.

Added text to state that VHL deletions are detected mainly by using next-generation sequencing, with confirmation using targeted chromosomal microarray and/or multiplex ligation-dependent probe amplification.

Added Treatment of renal tumors as a new subsection.

Added Treatment of retinal hemangioblastomas as a new subsection.

Added Treatment of CNS hemangioblastomas as a new subsection.

Added Treatment of endolymphatic sac tumors as a new subsection.

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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