| Group (mutated gene) | Other characteristics | Relative risk for pancreatic cancer | Lifetime risk for pancreatic cancer by age 70 years (incidence) |
| No history | 1 | 0.5% | |
| HBOC (BRCA1) | Predisposition to breast, ovarian, and prostate cancer | 2 to 3 | 1.2% |
| HBOC (BRCA2) | Predisposition to breast, ovarian, and prostate cancer; Jewish ancestry in some (refer for gene testing) | 3.5 to 10 | 2 to 5% |
| Familial PC + 1 FDR affected | Pancreatic ductal adenocarcinoma in an individual with 1 affected FDR (sibling, parent, or child) | 4.6 | |
| Familial PC + 2 FDRs affected (unknown) | Pancreatic ductal adenocarcinoma in an individual with 2 affected FDRs | 6.4 | |
| Lynch II syndrome (mismatch repair genes MLH1, MSH2, MSH6) | Predisposition to colorectal and endometrial cancer | 8 to 10 | 3.7% |
| FAMMM (CKDN2A) | Predisposition to melanoma, multiple nevi, and atypical moles (autosomal dominant) | 13 to 36 | 10 to 19% |
| Familial PC + 3 FDRs affected (unknown) | Pancreatic ductal adenocarcinoma in an individual with 3 affected FDRs | 32 | |
| Hereditary pancreatitis (PRSS1, SPINK1) | Young-onset pancreatitis (autosomal dominant) | 50 to 82 | 25 to 44% |
| Peutz-Jeghers syndrome (STK11) | 75 to 125 | 11 to 66% | |
| HBOC (PALB2) | 15 | unknown | |
| Ataxia telangiectasia (ATM) | 8 to 9 | unknown | |
| Li-Fraumeni (TP53) | 7 | unknown |
