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Genetic testing by cancer site: pancreas.

Abstract

It is estimated that 5% to 10% of pancreatic cancer is familial. Although there is evidence of a major pancreatic cancer susceptibility gene, the majority of families with multiple cases of pancreatic cancer do not have an identifiable causative gene or syndrome. However, a subset of pancreatic cancer is attributable to known inherited cancer predisposition syndromes, including several hereditary breast cancer genes (BRCA1, BRCA2, and PALB2), CDKN2A, hereditary pancreatitis, hereditary nonpolyposis colorectal cancer, and Peutz-Jeghers syndrome. In addition to explaining a proportion of familial pancreatic cancer, individuals with these conditions are at increased risk for pancreatic cancer. Relatives from familial pancreatic cancer kindreds without one of these identifiable syndromes may have as high as a 32-fold risk of pancreatic cancer, depending on the number of affected first-degree relatives. Such high-risk individuals may benefit from increased surveillance, and strategies for early detection of pancreatic cancer are under evaluation.

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  • Authors+Show Affiliations

    ,

    Department of Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. axilbund@jhmi.edu

    Source

    MeSH

    Carcinoma
    Colorectal Neoplasms, Hereditary Nonpolyposis
    Genes, BRCA1
    Genes, BRCA2
    Genetic Counseling
    Genetic Predisposition to Disease
    Genetic Testing
    Humans
    Pancreatic Neoplasms
    Pancreatitis, Chronic
    Peutz-Jeghers Syndrome
    Risk Assessment

    Pub Type(s)

    Journal Article
    Review

    Language

    eng

    PubMed ID

    22846737

    Citation

    Axilbund, Jennifer E., and Elizabeth A. Wiley. "Genetic Testing By Cancer Site: Pancreas." Cancer Journal (Sudbury, Mass.), vol. 18, no. 4, 2012, pp. 350-4.
    Axilbund JE, Wiley EA. Genetic testing by cancer site: pancreas. Cancer J. 2012;18(4):350-4.
    Axilbund, J. E., & Wiley, E. A. (2012). Genetic testing by cancer site: pancreas. Cancer Journal (Sudbury, Mass.), 18(4), pp. 350-4. doi:10.1097/PPO.0b013e3182624694.
    Axilbund JE, Wiley EA. Genetic Testing By Cancer Site: Pancreas. Cancer J. 2012;18(4):350-4. PubMed PMID: 22846737.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Genetic testing by cancer site: pancreas. AU - Axilbund,Jennifer E, AU - Wiley,Elizabeth A, PY - 2012/8/1/entrez PY - 2012/8/1/pubmed PY - 2013/1/31/medline SP - 350 EP - 4 JF - Cancer journal (Sudbury, Mass.) JO - Cancer J VL - 18 IS - 4 N2 - It is estimated that 5% to 10% of pancreatic cancer is familial. Although there is evidence of a major pancreatic cancer susceptibility gene, the majority of families with multiple cases of pancreatic cancer do not have an identifiable causative gene or syndrome. However, a subset of pancreatic cancer is attributable to known inherited cancer predisposition syndromes, including several hereditary breast cancer genes (BRCA1, BRCA2, and PALB2), CDKN2A, hereditary pancreatitis, hereditary nonpolyposis colorectal cancer, and Peutz-Jeghers syndrome. In addition to explaining a proportion of familial pancreatic cancer, individuals with these conditions are at increased risk for pancreatic cancer. Relatives from familial pancreatic cancer kindreds without one of these identifiable syndromes may have as high as a 32-fold risk of pancreatic cancer, depending on the number of affected first-degree relatives. Such high-risk individuals may benefit from increased surveillance, and strategies for early detection of pancreatic cancer are under evaluation. SN - 1540-336X UR - https://www.unboundmedicine.com/medline/citation/22846737/Genetic_testing_by_cancer_site:_pancreas_ L2 - http://Insights.ovid.com/pubmed?pmid=22846737 DB - PRIME DP - Unbound Medicine ER -