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[Familial pancreatic cancer].

Abstract

Familial clustering is estimated in 5-10% of pancreatic cancers. In different countries Familial Pancreatic Cancer Registries have been established to investigate the epidemiology, and genetic background in these families and, to organize the screening programs for high-risk relatives and for follow-up. The largest such registry is found at Johns Hopkins University Hospital. Evaluating the available data revealed that familial pancreatic cancer is heterogeneous: it may occur in kindreds of pancreatic cancer patients, but it may also be associated with various familial cancer syndromes. Such syndromes include FAMMM-syndrome, hereditary breast cancer, Peutz-Jeghers syndrome, but other associations can also be taken into account. The germline mutations are also heterogeneous, and although they are not absolutely decisive, they significantly increase the risk of the affected persons, making the organ more susceptible for environmental carcinogens. High-risk family members should be screened for gene mutations (especially for BRCA2, STK11/LKB1, CDKN2A/p16, PRSS1 genes), and by using endoscopic ultrasound. These methods are useful for identifying the preneoplastic conditions, but of equal importance is the cessation of smoking. In Hungary there are no relevant data about the epidemiology of familial pancreatic cancer, but their number is estimated to be about 80-150 annually. Considering the very high (and continuously increasing) incidence, it seems to be necessary to register and screen these families. This review emphasizes the importance of these goals.

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  • Authors+Show Affiliations

    I. sz. Patológiai és Kísérleti Rákkutató Intézet, Semmelweis Egyetem AOK, Budapest, Hungary. zalatnai@korb1.sote.hu

    Source

    Magyar onkologia 50:2 2006 pg 163-8

    MeSH

    Endosonography
    Genetic Testing
    Germ-Line Mutation
    Humans
    Hungary
    Incidence
    Mutation
    Neoplastic Syndromes, Hereditary
    Pancreatic Neoplasms
    Risk Factors

    Pub Type(s)

    English Abstract
    Journal Article
    Review

    Language

    hun

    PubMed ID

    16888680

    Citation

    Zalatnai, Attila. "[Familial Pancreatic Cancer]." Magyar Onkologia, vol. 50, no. 2, 2006, pp. 163-8.
    Zalatnai A. [Familial pancreatic cancer]. Magy Onkol. 2006;50(2):163-8.
    Zalatnai, A. (2006). [Familial pancreatic cancer]. Magyar Onkologia, 50(2), pp. 163-8.
    Zalatnai A. [Familial Pancreatic Cancer]. Magy Onkol. 2006;50(2):163-8. PubMed PMID: 16888680.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - [Familial pancreatic cancer]. A1 - Zalatnai,Attila, Y1 - 2006/08/04/ PY - 2006/05/23/received PY - 2006/05/29/accepted PY - 2006/8/5/pubmed PY - 2006/10/13/medline PY - 2006/8/5/entrez SP - 163 EP - 8 JF - Magyar onkologia JO - Magy Onkol VL - 50 IS - 2 N2 - Familial clustering is estimated in 5-10% of pancreatic cancers. In different countries Familial Pancreatic Cancer Registries have been established to investigate the epidemiology, and genetic background in these families and, to organize the screening programs for high-risk relatives and for follow-up. The largest such registry is found at Johns Hopkins University Hospital. Evaluating the available data revealed that familial pancreatic cancer is heterogeneous: it may occur in kindreds of pancreatic cancer patients, but it may also be associated with various familial cancer syndromes. Such syndromes include FAMMM-syndrome, hereditary breast cancer, Peutz-Jeghers syndrome, but other associations can also be taken into account. The germline mutations are also heterogeneous, and although they are not absolutely decisive, they significantly increase the risk of the affected persons, making the organ more susceptible for environmental carcinogens. High-risk family members should be screened for gene mutations (especially for BRCA2, STK11/LKB1, CDKN2A/p16, PRSS1 genes), and by using endoscopic ultrasound. These methods are useful for identifying the preneoplastic conditions, but of equal importance is the cessation of smoking. In Hungary there are no relevant data about the epidemiology of familial pancreatic cancer, but their number is estimated to be about 80-150 annually. Considering the very high (and continuously increasing) incidence, it seems to be necessary to register and screen these families. This review emphasizes the importance of these goals. SN - 0025-0244 UR - https://www.unboundmedicine.com/medline/citation/16888680/[Familial_pancreatic_cancer]_ L2 - http://huon.hu/2006/50/2/0163/0163a.pdf DB - PRIME DP - Unbound Medicine ER -