The epidemiology of von Hippel-Lindau disease

Rachel S. van Leeuwaarde; Reut Halperin

doi:10.1007/978-3-031-53858-2_2

The epidemiology of von Hippel-Lindau disease

Rachel S. van Leeuwaarde^*, Reut Halperin

^*Corresponding author for this work

Internal Medicine

Utrecht University

Research output: Chapter in Book/Report/Conference proceeding › Chapter › peer-review

Abstract

von Hippel-Lindau disease (VHL) is an autosomal dominant disease with a penetrance of nearly 100% by age 70 years. The prevalence is about one in 39,000 to 85,000 people. VHL is characterized by the development of benign and malignant tumors, including hemangioblastomas of the retina and central nervous system, clear cell renal cell carcinomas and cysts, pheochromocytomas and paragangliomas, pancreatic neuroendocrine tumors and cysts, endolymphatic sac tumors, and epididymal and broad ligament cysts. Herein, we describe the diagnostic criteria, VHL classification, and the disease's epidemiological spectrum.

Original language	English
Title of host publication	Von Hippel-Lindau Disease
Subtitle of host publication	A Comprehensive Guide to Diagnosis, Treatment, and Management
Publisher	Springer International Publishing
Pages	27-41
Number of pages	15
ISBN (Electronic)	9783031538582
ISBN (Print)	9783031538575
DOIs	https://doi.org/10.1007/978-3-031-53858-2_2
State	Published - 5 Apr 2024

Keywords

Epidemiology
Hemangioblastoma
Morbidity
Mortality
Pancreatic neuroendocrine tumor
Pheochromocytoma
Prevalence
Renal cell carcinoma
von Hippel-Lindau

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10.1007/978-3-031-53858-2_2

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KW - Morbidity

KW - Mortality

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KW - Pheochromocytoma

KW - Prevalence

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The epidemiology of von Hippel-Lindau disease

Abstract

Keywords

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