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Hereditary pancreatic cancer: a clinical perspective.

Abstract

Pancreatic cancer is an extraordinarily deadly disease and is responsible for over 220,000 deaths worldwide each year. One of the greatest risk factors for developing pancreatic cancer is a positive family history. Hereditary pancreatitis patients have a greatly elevated pancreatic cancer risk and individuals with cystic fibrosis may rarely develop this cancer, but often at very young ages. Various genetically linked cancer syndromes have been associated with pancreatic cancer in mutation-positive family members. Finally, familial pancreatic cancer-defined as families with two or more first-degree relatives who have pancreatic cancer but do not have a known cancer syndrome-is a known entity whose disease-causing mutation remains unidentified. This article describes research to date on hereditary pancreatic cancer, addresses how best clinicians should recognise hereditary forms of pancreatic cancer and explains the emotional burden of discovering a potentially lethal mutation. Many controversies and unanswered questions in hereditary pancreatic cancer remain.

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  • Authors+Show Affiliations

    ,

    University of Pittsburgh School of Medicine, Division of Gastroenterology, Hepatology and Nutrition, Medical Arts Building, 4th floor, Room 400.5, 3708 5th Ave. Pittsburgh, PA 15213, USA. greerjb@upmc.edu

    ,

    Source

    MeSH

    Early Detection of Cancer
    Gene Expression Regulation, Neoplastic
    Genetic Counseling
    Genetic Predisposition to Disease
    Genetic Testing
    Humans
    Mutation
    Neoplastic Syndromes, Hereditary
    Pancreatic Neoplasms
    Pedigree
    Risk Factors

    Pub Type(s)

    Journal Article
    Review

    Language

    eng

    PubMed ID

    19414143

    Citation

    Greer, Julia B., et al. "Hereditary Pancreatic Cancer: a Clinical Perspective." Best Practice & Research. Clinical Gastroenterology, vol. 23, no. 2, 2009, pp. 159-70.
    Greer JB, Lynch HT, Brand RE. Hereditary pancreatic cancer: a clinical perspective. Best Pract Res Clin Gastroenterol. 2009;23(2):159-70.
    Greer, J. B., Lynch, H. T., & Brand, R. E. (2009). Hereditary pancreatic cancer: a clinical perspective. Best Practice & Research. Clinical Gastroenterology, 23(2), pp. 159-70. doi:10.1016/j.bpg.2009.02.001.
    Greer JB, Lynch HT, Brand RE. Hereditary Pancreatic Cancer: a Clinical Perspective. Best Pract Res Clin Gastroenterol. 2009;23(2):159-70. PubMed PMID: 19414143.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Hereditary pancreatic cancer: a clinical perspective. AU - Greer,Julia B, AU - Lynch,Henry T, AU - Brand,Randall E, PY - 2009/5/6/entrez PY - 2009/5/6/pubmed PY - 2009/7/17/medline SP - 159 EP - 70 JF - Best practice & research. Clinical gastroenterology JO - Best Pract Res Clin Gastroenterol VL - 23 IS - 2 N2 - Pancreatic cancer is an extraordinarily deadly disease and is responsible for over 220,000 deaths worldwide each year. One of the greatest risk factors for developing pancreatic cancer is a positive family history. Hereditary pancreatitis patients have a greatly elevated pancreatic cancer risk and individuals with cystic fibrosis may rarely develop this cancer, but often at very young ages. Various genetically linked cancer syndromes have been associated with pancreatic cancer in mutation-positive family members. Finally, familial pancreatic cancer-defined as families with two or more first-degree relatives who have pancreatic cancer but do not have a known cancer syndrome-is a known entity whose disease-causing mutation remains unidentified. This article describes research to date on hereditary pancreatic cancer, addresses how best clinicians should recognise hereditary forms of pancreatic cancer and explains the emotional burden of discovering a potentially lethal mutation. Many controversies and unanswered questions in hereditary pancreatic cancer remain. SN - 1532-1916 UR - https://www.unboundmedicine.com/medline/citation/19414143/Hereditary_pancreatic_cancer:_a_clinical_perspective_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1521-6918(09)00028-6 DB - PRIME DP - Unbound Medicine ER -