Familial pancreatic cancer: a review and latest advances.

Familial Pancreatic Cancer (FPC) is the autosomal dominant inheritance of a genetic predisposition to pancreatic ductal adenocarcinoma, penetrance is assumed to be high but not complete. It was first described in 1987 and since then many families have been identified, but the candidate disease gene remains elusive and the very existence of the syndrome is sometimes questioned. FPC identifies a target group for secondary screening. As well as being potentially life saving for the subjects, screening offers researchers the opportunity to elucidate the early pathogenesis of pancreatic cancer. The scientific incentive for screening should not blind us to the challenges facing clinicians in managing high risk patients. Early surgical treatment may dramatically improve the five year survival for pancreatic cancer, but this must be balanced against the risks of false positives, where healthy individuals are subjected to the mortality and morbidity of major pancreatic surgery.