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. Author manuscript; available in PMC: 2017 Aug 8.
Published in final edited form as: Surg Clin North Am. 2016 Dec;96(6):1223–1233. doi: 10.1016/j.suc.2016.07.016

Table 1.

Inherited cancer syndromes associated with increased risk of pancreatic cancer

Syndrome Gene(s) Locus Lifetime Risk
of PC, Percent
Hereditary breast/ovarian cancer BRCA2, BRCA1 13q 3–5
PALB2 16p Unknown
Familial atypical multiple mole melanoma syndrome CDKN2A 9p 10–19
Peutz-Jeghers syndrome STK 11 19p 11–36
Familial adenomatous polyposis APC 5q Unknown
Hereditary nonpolyposis colon cancer (Lynch II) DNA mismatch repair genes 2p, 3p, 7p 4
Hereditary pancreatitis PRSS1, SPINK1 7q, 5q 25–40
Ataxia telangiectasia ATM 11q Unknown
Li-Fraumeni syndrome P53 17p Unknown

Adapted from Brentnall TA. Management strategies for patients with hereditary pancreatic cancer. Curr Treat Options Oncol 2005;6:437; with permission.