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.
Keywords:
Familial syndromes; Genetics; Pancreatic cancer.
Copyright © 2016 Elsevier Inc. All rights reserved.
MeSH terms
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Adenomatous Polyposis Coli / complications
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Beckwith-Wiedemann Syndrome / complications
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Cystic Fibrosis / complications
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Fibrous Dysplasia, Polyostotic / complications
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Genes, Tumor Suppressor
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Genetic Predisposition to Disease / genetics*
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Hereditary Breast and Ovarian Cancer Syndrome / complications
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Humans
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Melanoma / complications
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Multiple Endocrine Neoplasia Type 1 / complications
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Neoplastic Syndromes, Hereditary / complications
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Neurofibromatosis 1 / complications
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Pancreatic Neoplasms / complications
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Pancreatic Neoplasms / etiology
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Pancreatic Neoplasms / genetics*
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Pancreatic Neoplasms / physiopathology
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Pancreatitis, Chronic / complications
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Peutz-Jeghers Syndrome / complications
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Precancerous Conditions / genetics*
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Precancerous Conditions / physiopathology
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Tuberous Sclerosis / complications
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von Hippel-Lindau Disease / complications
Supplementary concepts
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Hereditary pancreatitis
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Melanoma-Pancreatic Cancer Syndrome