Genetic Syndromes with Pancreatic Manifestations

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• 作者：Meredith E. Pittman ; MD^a ; Lodewijk A.A. Brosens ; MD ; PhD^b ; Laura D. Wood ; MD ; PhD^c ; ^{ldwood@jhmi.edu" class="auth_mail" title="E-mail the corresponding author}
• 关键词：Pancreatic cancer ; Genetics ; Familial syndromes
• 刊名：Surgical Pathology Clinics
• 出版年：2016
• 出版时间：December 2016
• 年：2016
• 卷：9
• 期：4
• 页码：705-715
• 全文大小：5260 K

文摘

Although the pancreas is affected by only a small fraction of known inherited disorders, several of these syndromes predispose patients to pancreatic adenocarcinoma, a cancer that has a consistently dismal prognosis. Still other syndromes are associated with neuroendocrine tumors, benign cysts, or recurrent pancreatitis. Because of the variability of pancreatic manifestations and outcomes, it is important for clinicians to be familiar with several well-described genetic disorders to ensure that patients are followed appropriately. The purpose of this review was to briefly describe the hereditary syndromes that are associated with pancreatic disorders and neoplasia.