Von Hippel Lindau Disease Case Studies

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Near total replacement of the pancreas with cysts in a patient with Von Hippel Lindau Disease

Abstract: Von Hippel Lindau (VHL) disease can affect various organ systems of the body and is associated with tumors and visceral cysts. Lesions of the pancreas are fairly common leading to the occurrence of simple cysts, serous cystadenoma or solid tumors. We present a rare case of a 35 year old woman previously diagnosed with VHL disease whose pancreas on endoscopic ultrasound was found to be replaced by cysts of varying sizes with normal pancreatic tissues and duct not being identified.

VHL is an autosomal dominant genetic disorder with a birth incidence prevalence of about 1 in 36,000 live births.(1) It can manifest with as various
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