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. Author manuscript; available in PMC: 2009 Apr 7.
Published in final edited form as: Annu Rev Pathol. 2008;3:157–188. doi: 10.1146/annurev.pathmechdis.3.121806.154305

Table 1.

Genes associated with an increased risk of pancreatic cancer

Individual Gene Relative risk Risk by age 70 Cancer morphology Other cancers Reference(s)
No history None 1 0.5% NS None
Breast cancer BRCA2 3.5–10X 5% NS Breast, ovary, and prostate (10, 11)
BRCA1 2X 1% Breast cancer with basaloid features Breast, ovary, and prostate (12)
FAMMM P16 (CDKN2A) 20–34X 10%–17% NS Melanoma (14, 185)
Familial pancreatic cancer (3 FDR) Unknown 32X 16% NS Unknown (9)
Familial pancreatitis PRSS1 50–80X 25%–40% Pancreatic cancers in the background of severe diffuse chronic pancreatitis None (17)
Peutz-Jeghers STK11/LKB1 132X 30%–60% NS Gastroesophageal, small bowel, colorectal, and breast (13, 16)
HNPCC hMLH1, hMSH2, others Unknown < 5% Medullary and colloid phenotypes Colorectal, endometrial, stomach, ovarian, ureter and renal pelvis, biliary tract, and brain (18, 186)
Young-age-onset pancreatic cancer FANC-C and FANC-G Unknown Unknown NS Unknown (187, 188)

3 FDR, 3 or more first-degree relatives with pancreatic cancer; FAMMM, familial atypical multiple mole melanoma syndrome; HNPCC, hereditary nonpolyposis colorectal cancer syndrome; NS, nonspecific.