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Published on: July 3, 2019
Endocrine Manifestations of Von Hippel-Landau Disease
Roma Pradhan1, Nelson George2, Kaushik Mandal3
1Department of Endocrine Surgery, Dr. RMLIMS, Lucknow, Uttar Pradesh, India.
Abstract:
Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder characterized by various endocrine, nonendocrine, benign, and malignant tumors in various organs. VHL tumor suppressor gene, located on short arm of chromosome 3 is responsible for this. Pheochromocytoma (PCC) is one of the important endocrine manifestations that needs to be ruled out in case of VHL suspicion. In this review, we summarize the endocrine manifestations of VHL disease and their management while giving case history of five such cases.
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