Table 1

Definition of high-risk individuals eligible for pancreatic cancer surveillance.

Gene mutationPDAC family history criteriaAgreementGrade
LKB1/STK11
(Peutz-Jeghers syndrome)
Regardless of family history99%1
CDKN2A p16* (FAMMM)With at least one affected FDR99%1
CDKN2A p16* (FAMMM)Regardless of family history77%1
BRCA2 If at least one affected FDR, or at least two affected relatives† of any degree93%2
PALB2 If at least one affected FDR83%2
MLH1/MSH2/MSH6 (Lynch)If at least one affected FDR84%2
ATM If at least one affected FDR88%2
BRCA1 If at least one affected FDR69.6%‡3
Regardless of gene mutation statusIf at least three affected relatives† on the same side of the family, of whom at least one is an FDR to the individual considered for surveillance97%2
Regardless of gene mutation statusIf at least two affected relatives† who are FDR to each other, of whom at least one is an FDR to the individual considered for surveillance93%2
Regardless of gene mutation statusIf at least two affected relatives† on the same side of the family, of whom at least one is an FDR to the individual considered for surveillance88%2
  • *Only encompassing CDKN2A mutations leading to changes in the p16 protein.

  • †Wherever relative is stated, this indicates blood relatives only.

  • ‡An additional 20.3% somewhat agreed with surveillance (total 89.9%).

  • ATM, ataxia telangiectasia mutated; BRCA2, breast cancer 2; CDKN2A, cyclin-dependent kinase inhibitor 2A; FAMMM, familial atypical multiple mole melanoma; FDR, first-degree relative; GRADE, Grading of Recommendations, Assessment, Development, and Evaluations; HBOC, hereditary breast and ovarian cancer; LKB1/STK11, liver kinase B1/serine/threonine kinase 11; Lynch syndrome, MLH1, mutL homolog 1; MSH2, mutS homolog 2; MSH6, mutS homolog 6; PALB2, partner and localizer of BRCA2; PDAC, pancreatic ductal adenocarcinoma.