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Year : 2019  |  Volume : 23  |  Issue : 1  |  Page : 159-164

Endocrine manifestations of Von Hippel–Landau disease

1 Department of Endocrine Surgery, Dr. RMLIMS, Lucknow, Uttar Pradesh, India
2 Department of Endocrine Surgery, SGPGIMS, Lucknow, Uttar Pradesh, India
3 Department of Genetics, SGPGIMS, Lucknow, Uttar Pradesh, India
4 Department of Endocrinology, SGPGIMS, Lucknow, Uttar Pradesh, India

Correspondence Address:
Amit Agarwal
Department of Endocrine Surgery, SGPGIMS, Lucknow - 226 014, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijem.IJEM_252_18

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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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