Table 1.
Cancer Susceptibility Syndromes or Inherited Disease with a Known Elevated Risk of Developing Pancreatic Cancer.
| Syndrome | Gene(s) | Risk of Pancreatic Cancer |
|---|---|---|
| Hereditary breast and ovarian cancer (HBOC) | BRCA1 BRCA2 |
RR 2–3 RR 3–10 |
| Familial cutaneous malignant melanoma (familial CMM) | CDKN2A (p16) | RR 8–45 |
| Hereditary pancreatitis | PRSS1 | SIR 60–90 |
| Hereditary non-polyposis colorectal cancer (Lynch syndrome) | MLH1/MSH2/MSH6 | RR 9 |
| Peutz-Jeghers syndrome | STK11/LKB1 | RR 75–135 |
| Familial adenomatous polyposis (FAP) | APC | RR 4.5 |
| Li-Fraumeni syndrome | p53 | RR 7.5 |
Taken from Canto et al.,15 Copyright © 2013, BMJ Publishing Group Ltd and the British Society of Gastroenterology, with permission.
RR, relative risk; SIR, standardized incidence ratio.