The highest life time risk of pancreatic malignancy is seen with:
Peutz-Jeghers, associated with mutations in the STK11 gene. It has the highest lifetime risk of pancreatic cancer. Genetically driven chronic causes of pancreatic cancers are hereditary pancreatitis, cystic fibrosis, and ataxia telangiectasia. Ref: Harrison, Edition -18, Page-788
Peutz-Jeghers syndrome
Hereditary nonpolyposis colorectal cancer
Li-Fraumeni syndrome
Familial adenomatous polyposis