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International registries of families at high risk of pancreatic cancer.
Pancreatology 2008; 8(6):558-65P

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.

Authors+Show Affiliations

Division of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. greenhaf@liv.ac.ukNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

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 -