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Familial pancreatic cancer syndromes.

Abstract

Hereditary pancreatic cancer (PC) is rare and extremely heterogeneous, and it accounts for approximately 2% of all PC cases. The major component of hereditary PC is the familial pancreatic cancer syndrome. Although up to 20% of hereditary PC cases are associated with germline mutations in the BRCA2, CDKN2A, PRSS1,STKI1, or MMR genes, the major underlying gene defect(s) is still unknown. Although hereditary PC is rare, the data on PC families that have been collected by various study groups worldwide provide a unique opportunity to evaluate the natural history, causative gene alterations, new diagnosis and chemoprevention strategies as well as treatment modalities.

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  • Authors+Show Affiliations

    ,

    Department of Surgery, Philipps-University Marburg, Baldiger Strasse, Marburg 35033, Germany.

    , ,

    Source

    MeSH

    Adenocarcinoma
    Dysplastic Nevus Syndrome
    Female
    Genes, BRCA1
    Genes, BRCA2
    Genetic Predisposition to Disease
    Genetic Testing
    Humans
    Male
    Pancreatic Neoplasms
    Pedigree
    Peutz-Jeghers Syndrome

    Pub Type(s)

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

    Language

    eng

    PubMed ID

    16632103

    Citation

    Habbe, Nils, et al. "Familial Pancreatic Cancer Syndromes." Endocrinology and Metabolism Clinics of North America, vol. 35, no. 2, 2006, pp. 417-30, xi.
    Habbe N, Langer P, Sina-Frey M, et al. Familial pancreatic cancer syndromes. Endocrinol Metab Clin North Am. 2006;35(2):417-30, xi.
    Habbe, N., Langer, P., Sina-Frey, M., & Bartsch, D. K. (2006). Familial pancreatic cancer syndromes. Endocrinology and Metabolism Clinics of North America, 35(2), pp. 417-30, xi.
    Habbe N, et al. Familial Pancreatic Cancer Syndromes. Endocrinol Metab Clin North Am. 2006;35(2):417-30, xi. PubMed PMID: 16632103.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Familial pancreatic cancer syndromes. AU - Habbe,Nils, AU - Langer,Peter, AU - Sina-Frey,Mercedes, AU - Bartsch,Detlef K, PY - 2006/4/25/pubmed PY - 2006/7/13/medline PY - 2006/4/25/entrez SP - 417-30, xi JF - Endocrinology and metabolism clinics of North America JO - Endocrinol. Metab. Clin. North Am. VL - 35 IS - 2 N2 - Hereditary pancreatic cancer (PC) is rare and extremely heterogeneous, and it accounts for approximately 2% of all PC cases. The major component of hereditary PC is the familial pancreatic cancer syndrome. Although up to 20% of hereditary PC cases are associated with germline mutations in the BRCA2, CDKN2A, PRSS1,STKI1, or MMR genes, the major underlying gene defect(s) is still unknown. Although hereditary PC is rare, the data on PC families that have been collected by various study groups worldwide provide a unique opportunity to evaluate the natural history, causative gene alterations, new diagnosis and chemoprevention strategies as well as treatment modalities. SN - 0889-8529 UR - https://www.unboundmedicine.com/medline/citation/16632103/Familial_pancreatic_cancer_syndromes_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0889-8529(06)00017-X DB - PRIME DP - Unbound Medicine ER -