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.

RMMJ Rambam Maimonides Medical Journal Rambam Health Care Campus 2018 October; 9(4): e0029. ISSN: 2076-9172
Published online 2018 October 4. doi: 10.5041/RMMJ.10353
Copyright: © Lachter et al.