Table 1.

Clinical settings associated with an increased risk of inherited pancreatic cancer

Clinical settingGeneRisk
Familial multiorgan cancer syndromes
    Peutz-Jeghers syndromeSTK11/LKB1RR = 132
CLR = 36%
    Familial atypical multiple mole melanomaCDKN2aCLR = 17%
    Familial breast-ovarian cancerBRCA2RR = ∼5%
BRCA1?
    Hereditary nonpolyposis colorectal cancerMLH1, MSH2, MSH6, PMS1, PMS2?
    Familial adenomatous polyposisAPC?
Genetically driven chronic diseases
    Hereditary pancreatitisPRSS1CLR = 40%
    Cystic fibrosisCFTRRR = 3.5
    Fanconi anemiaFA?
    Ataxia telangiectasiaATM?
FPC
    Pancreatic cancer in ≥3 first-degree relativesRR = 32
    Pancreatic cancer in 2 first-degree relativesRR = 6.4
    Pancreatic cancer in 1 first-degree relativesRR = 4.5
  • Abbreviations: RR, relative risk; CLR, cumulative lifetime risk.