Von Hippel Lindau Disease Case Studies
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.
Introduction
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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Elli L, Buscarini E, Portugalli V, Reduzzi L, Reduzzi C, Brambilla G, et al. Pancreatic involvement in von Hippel-Lindau disease: report of two cases and review of the literature. The American journal of gastroenterology. 2006;101(11):2655-8.
13. Kapur V, Brower ST. Cystic Replacement of Pancreas in Patient With Von Hippel-Lindau Syndrome. Gastrointestinal Cancer Research : GCR. 2013;6(1):25-6.
14. Kursat Rahmi Serin FY, Orhan Agcaoglu, Metin Keskin, Ozgur Ceyhan, Orhan Bilge. Pancreatic Involvement in Von Hippel-Lindau Syndrome: Report of a Total Pancreatectomy Case and Literature Review. Surgical Science. 2013;4(2):3.
15. Delman KA, Shapiro SE, Jonasch EW, Lee JE, Curley SA, Evans DB, et al. Abdominal visceral lesions in von Hippel-Lindau disease: incidence and clinical behavior of pancreatic and adrenal lesions at a single center. World journal of surgery. 2006;30(5):665-9.
16. Blansfield JA, Choyke L, Morita SY, Choyke PL, Pingpank JF, Alexander HR, et al. Clinical, genetic and radiographic analysis of 108 patients with von Hippel-Lindau disease (VHL) manifested by pancreatic neuroendocrine neoplasms (PNETs). Surgery. 2007;142(6):814-8; discussion 8.e1-2.
17. Varshney N. A Review of Von Hippel-Lindau Syndrome.
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