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Published on: June 2, 2018
Genetic Syndromes with Pancreatic Manifestations
Meredith E Pittman1, Lodewijk A A Brosens2, Laura D Wood3
1Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, Starr 1031A, 525 East 68th Street, New York, NY 10065, USA.
Abstract:
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.
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