Abstract
Individuals with a family history of pancreatic cancer have an increased risk of developing pancreatic cancer. In western countries, familial pancreatic cancer(FPC) was defined as pancreatic cancer patient having at least one pair of first-degree relatives with pancreatic cancer, comprising 5–10 % of pancreatic cancer patients overall. Some trends in correlative risk based on the number of effected family members, ethnicity and earlier onset have been shown in comparison to that in the sporadic cancer. Genes responsible for FPC have not yet been thoroughly elucidated and mutation of BRCA2 was detected only in a small fraction (8–19 %). Although the concept of FPC has not yet been accepted in Japan, nationwide registries of FPC were established in many western countries since the 1990s in order to promote early detection of pancreatic cancer by encouraging the screening the kindred of FPC patients.