Families are being increasingly recognized as carrying an inherited suscept
ibility for pancreatic cancer, apparently unrelated to any currently recogn
ized syndrome. The authors provide a review of the current evidence for fam
ilial susceptibility to pancreatic cancer. A formal segregation analysis of
the pattern of inheritance of pancreatic cancer in 70 families from the Na
tional Registry for Familial Pancreatic Cancer is described. This analysis
suggests a single major gene with an autosomal dominant mode of inheritance
controlling susceptibility for pancreatic cancer in these families.