title: Genetic Syndromes with Pancreatic Manifestations.
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.
authors.affiliation: Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, Starr 1031A, 525 East 68th Street, New York, NY 10065, USA.
authors.forename: Meredith E
authors.initials: ME
authors.lastname: Pittman
authors.affiliation: Department of Pathology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
authors.forename: Lodewijk A A
authors.initials: LA
authors.lastname: Brosens
authors.affiliation: Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, CRB2 Room 345, 1550 Orleans Street, Baltimore, MD 21231, USA. Electronic address: ldwood@jhmi.edu.
authors.forename: Laura D
authors.initials: LD
authors.lastname: Wood
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications; D003550:Cystic Fibrosis / Q000150:complications; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications; D016147:Genes, Tumor Suppressor; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D061325:Hereditary Breast and Ovarian Cancer Syndrome / Q000150:complications; D006801:Humans; D008545:Melanoma / Q000150:complications; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications; D009386:Neoplastic Syndromes, Hereditary / Q000150:complications; D009456:Neurofibromatosis 1 / Q000150:complications; D010190:Pancreatic Neoplasms / Q000150:complications / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology; D050500:Pancreatitis, Chronic / Q000150:complications; D010580:Peutz-Jeghers Syndrome / Q000150:complications; D011230:Precancerous Conditions / Q000235:genetics* / Q000503:physiopathology; D014402:Tuberous Sclerosis / Q000150:complications; D006623:von Hippel-Lindau Disease / Q000150:complications
keywords: Familial syndromes; Genetics; Pancreatic cancer