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International registries of families at high risk of pancreatic cancer.

Abstract

PURPOSE

To describe the need for multinational registries of families at high risk of pancreatic cancer and the issues surrounding identification of such families.

RESULTS

A consensus position was published describing surveillance of individuals at high risk of pancreatic cancer. Hereditary pancreatitis patients, people with Peutz-Jeghers syndrome, individuals with CDKN2A or BRCA1/2 mutations with a family history of pancreatic cancer and kindred with multiple pancreatic cancers were considered suitable for research-based screening. Mutations responsible for familial predisposition are mostly unknown, although BRCA2 mutations have been identified in some families and a mutation in the palladin gene has been shown to segregate with pancreatic cancer in one kindred. Specific autosomal dominant inheritance of pancreatic cancer risk seems to involve anticipation; this finding aids identification of families and determination of individual risk. Diabetes mellitus is an early symptom of pancreatic cancer, but recent publications suggest that it may not be a significant predisposing factor; this remains controversial. However, in the context of hereditary pancreatitis, diabetes probably does predispose to pancreatic cancer as shown in a recent description of French families.

CONCLUSION

Appropriate inclusion of patients within registries of high-risk families provides a framework for secondary screening and research on risk stratification and early tumorigenesis. and IAP.

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  • Authors+Show Affiliations

    ,

    Division of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. greenhaf@liv.ac.uk

    , , ,

    Source

    MeSH

    Diabetes Mellitus
    Family
    Genetic Markers
    Genetic Predisposition to Disease
    Humans
    International Cooperation
    National Health Programs
    Pancreatic Neoplasms
    Pancreatitis
    Pedigree
    Registries
    Risk

    Pub Type(s)

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

    Language

    eng

    PubMed ID

    18818508

    Citation

    Greenhalf, William, et al. "International Registries of Families at High Risk of Pancreatic Cancer." Pancreatology : Official Journal of the International Association of Pancreatology (IAP) ... [et Al.], vol. 8, no. 6, 2008, pp. 558-65.
    Greenhalf W, Malats N, Nilsson M, et al. International registries of families at high risk of pancreatic cancer. Pancreatology. 2008;8(6):558-65.
    Greenhalf, W., Malats, N., Nilsson, M., Bartsch, D., & Neoptolemos, J. (2008). International registries of families at high risk of pancreatic cancer. Pancreatology : Official Journal of the International Association of Pancreatology (IAP) ... [et Al.], 8(6), pp. 558-65. doi:10.1159/000159214.
    Greenhalf W, et al. International Registries of Families at High Risk of Pancreatic Cancer. Pancreatology. 2008;8(6):558-65. PubMed PMID: 18818508.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - International registries of families at high risk of pancreatic cancer. AU - Greenhalf,William, AU - Malats,Nuria, AU - Nilsson,Magnus, AU - Bartsch,Detlef, AU - Neoptolemos,John, Y1 - 2008/09/26/ PY - 2008/9/27/pubmed PY - 2008/12/18/medline PY - 2008/9/27/entrez SP - 558 EP - 65 JF - Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] JO - Pancreatology VL - 8 IS - 6 N2 - PURPOSE: To describe the need for multinational registries of families at high risk of pancreatic cancer and the issues surrounding identification of such families. RESULTS: A consensus position was published describing surveillance of individuals at high risk of pancreatic cancer. Hereditary pancreatitis patients, people with Peutz-Jeghers syndrome, individuals with CDKN2A or BRCA1/2 mutations with a family history of pancreatic cancer and kindred with multiple pancreatic cancers were considered suitable for research-based screening. Mutations responsible for familial predisposition are mostly unknown, although BRCA2 mutations have been identified in some families and a mutation in the palladin gene has been shown to segregate with pancreatic cancer in one kindred. Specific autosomal dominant inheritance of pancreatic cancer risk seems to involve anticipation; this finding aids identification of families and determination of individual risk. Diabetes mellitus is an early symptom of pancreatic cancer, but recent publications suggest that it may not be a significant predisposing factor; this remains controversial. However, in the context of hereditary pancreatitis, diabetes probably does predispose to pancreatic cancer as shown in a recent description of French families. CONCLUSION: Appropriate inclusion of patients within registries of high-risk families provides a framework for secondary screening and research on risk stratification and early tumorigenesis. and IAP. SN - 1424-3911 UR - https://www.unboundmedicine.com/medline/citation/18818508/International_registries_of_families_at_high_risk_of_pancreatic_cancer_ L2 - https://linkinghub.elsevier.com/retrieve/pii/000159214 DB - PRIME DP - Unbound Medicine ER -