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Hereditary pancreatic cancer.

Abstract

Hereditary pancreatic cancer (PC) appears to be exceedingly heterogeneous, as evidenced by its association with a variety of integrally associated diverse cancers and/or differing mendelian inherited cancer syndromes, which include the Lynch syndrome II variant of hereditary nonpolyposis colorectal cancer, hereditary breast-ovarian cancer syndrome in families with the BRCA2 mutation, hereditary pancreatitis, Peutz-Jeghers polyposis and the familial atypical multiple-mole melanoma syndrome in families with the CDKN2A (p16) germline mutation. Because of this heterogeneity, we provide a conservative estimate that about 5% (1,460) of PC cases in the US annually are hereditary. Although this number is relatively small, members of hereditary PC families serve as excellent models for studying the etiology, natural history, biomarkers, pathogenesis, potential carcinogenic exposures and their perturbation of underlying genetic events, and treatment of PC. These individuals would benefit greatly from method(s) capable of detecting cancer at an early stage, and such knowledge would also be useful for improving the diagnosis of the much more common 'sporadic' form of PC.

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  • Authors+Show Affiliations

    ,

    Department of Preventive Medicine, Creighton University School of Medicine, 2500 California Plaza, Omaha, NE 68178, USA. htlynch@creighton.edu

    , , ,

    Source

    MeSH

    Biomarkers
    Humans
    Mutation
    Pancreatic Neoplasms
    Pancreatitis
    Registries

    Pub Type(s)

    Journal Article
    Research Support, Non-U.S. Gov't
    Research Support, U.S. Gov't, P.H.S.
    Review

    Language

    eng

    PubMed ID

    12120226

    Citation

    Lynch, H T., et al. "Hereditary Pancreatic Cancer." Pancreatology : Official Journal of the International Association of Pancreatology (IAP) ... [et Al.], vol. 1, no. 5, 2001, pp. 466-71.
    Lynch HT, Brand RE, Deters CA, et al. Hereditary pancreatic cancer. Pancreatology. 2001;1(5):466-71.
    Lynch, H. T., Brand, R. E., Deters, C. A., Shaw, T. G., & Lynch, J. F. (2001). Hereditary pancreatic cancer. Pancreatology : Official Journal of the International Association of Pancreatology (IAP) ... [et Al.], 1(5), pp. 466-71.
    Lynch HT, et al. Hereditary Pancreatic Cancer. Pancreatology. 2001;1(5):466-71. PubMed PMID: 12120226.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Hereditary pancreatic cancer. AU - Lynch,H T, AU - Brand,R E, AU - Deters,C A, AU - Shaw,T G, AU - Lynch,J F, PY - 2002/7/18/pubmed PY - 2002/8/8/medline PY - 2002/7/18/entrez SP - 466 EP - 71 JF - Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] JO - Pancreatology VL - 1 IS - 5 N2 - Hereditary pancreatic cancer (PC) appears to be exceedingly heterogeneous, as evidenced by its association with a variety of integrally associated diverse cancers and/or differing mendelian inherited cancer syndromes, which include the Lynch syndrome II variant of hereditary nonpolyposis colorectal cancer, hereditary breast-ovarian cancer syndrome in families with the BRCA2 mutation, hereditary pancreatitis, Peutz-Jeghers polyposis and the familial atypical multiple-mole melanoma syndrome in families with the CDKN2A (p16) germline mutation. Because of this heterogeneity, we provide a conservative estimate that about 5% (1,460) of PC cases in the US annually are hereditary. Although this number is relatively small, members of hereditary PC families serve as excellent models for studying the etiology, natural history, biomarkers, pathogenesis, potential carcinogenic exposures and their perturbation of underlying genetic events, and treatment of PC. These individuals would benefit greatly from method(s) capable of detecting cancer at an early stage, and such knowledge would also be useful for improving the diagnosis of the much more common 'sporadic' form of PC. SN - 1424-3903 UR - https://www.unboundmedicine.com/medline/citation/12120226/Hereditary_pancreatic_cancer_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1424-3903(01)80080-6 DB - PRIME DP - Unbound Medicine ER -