| Syndrome | Gene(s) | Estimated cumulative lifetime risk of pancreatic cancer (%) | Gene locus |
| Hereditary breast/ovarian cancer | BRCA2, BRCA1 | ≤5 (BRCA1); 5 to 10 (BRCA2) | 13q |
| PALB2 | 2 to 10 | 16p | |
| Familial atypical multiple mole melanoma syndrome | CDKN2A | 10 to 21 | 9p |
| Peutz-Jeghers syndrome | STK 11 | 11 to 36 | 19p |
| Hereditary nonpolyposis colon cancer (Lynch syndrome) | DNA mismatch repair genes | <5 to 10 (highest [6.2%] for MLH1; lowest [0.5] for MSH2) | 2p, 3p, 7p |
| Hereditary pancreatitis | PRSS1, SPINK1 | 25 to 44 | 7q, 5q |
| Ataxia telangiectasia | ATM | 6 to 10 | 11q |
| Li-Fraumeni syndrome | P53 | 5 to 10 | 17p |
| Familial adenomatous polyposis | APC | Unknown | 5q |
