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