Table 3.
Overview of hereditary syndromes predisposing to pancreatic cancer
| Syndrome | Gene | Life-time risk | Relative risk |
| FAMMM | CDKN2A | 10%-15% | 20-34 |
| HBOC | BRCA2 | 5% | 10 |
| HBOC | BRCA1 | Not known | 2 |
| Hereditary pancreatitis | PRSS1/TRY1 | 30%-50% | 50 |
| Lynch syndrome | MLH1/MSH2 | Not known | Not known |
| Peutz–Jeghers syndrome | STK11/LKB1 | 36% | 136 |
| FAP | APC | Not known | 4 |
| Li–Faumeni syndrome | p53 | Not known | Not known |
| FPC | Not known | Up to 50% | 18-57 |
APC: Adenomatous polyposis coli; FAMMM: Familial atypical multiple mole melanoma; FAP: Familial adenomatous polyposis; FPC: Familial pancreatic cancer; HBOC: Hereditary breast and ovarian syndrome. Adapted from[72].