International registries of families at high risk of pancreatic cancer.
OBJECTIVE
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.


CONCLUSIONS
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.
Greenhalf
William
W
Division of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. greenhaf@liv.ac.uk
Malats
Nuria
N
Nilsson
Magnus
M
Bartsch
Detlef
D
Neoptolemos
John
J
D003920:Diabetes Mellitus; D005190:Family; D005819:Genetic Markers; D020022:Genetic Predisposition to Disease; D006801:Humans; D007391:International Cooperation; D009313:National Health Programs; D010190:Pancreatic Neoplasms / Q000150:complications / Q000235:genetics*; D010195:Pancreatitis / Q000150:complications / Q000235:genetics; D010375:Pedigree; D012042:Registries; D012306:Risk
D005819:Genetic Markers