Inherited cancer syndromes associated with increased risk of pancreatic cancer

Syndrome	Gene(s)	Estimated cumulative lifetime risk of pancreatic cancer (%)	Gene locus
Hereditary breast/ovarian cancer	BRCA2, BRCA1	≤5 (BRCA1); 5 to 10 (BRCA2)	13q
Hereditary breast/ovarian cancer	PALB2	2 to 10	16p
Familial atypical multiple mole melanoma syndrome	CDKN2A	10 to 21	9p
Peutz-Jeghers syndrome	STK 11	11 to 36	19p
Hereditary nonpolyposis colon cancer (Lynch syndrome)	DNA mismatch repair genes	<5 to 10 (highest [6.2%] for MLH1; lowest [0.5] for MSH2)	2p, 3p, 7p
Hereditary pancreatitis	PRSS1, SPINK1	25 to 44	7q, 5q
Ataxia telangiectasia	ATM	6 to 10	11q
Li-Fraumeni syndrome	P53	5 to 10	17p
Familial adenomatous polyposis	APC	Unknown	5q

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