Table III. Hereditary tumor predisposition syndromes associated with pancreatic cancer.

Syndrome	Features	Estimated lifetime risk of pancreatic cancer*	Gene	Locus
HNPCC	Colorectal cancers; less commonly cancers of the uterus, stomach, ovaries, genitourinary system, hepatobiliary system, brain, and small intestine	1–5%	Common
			MSH2	2p15-16
			MLH1	3p21
			Rare
			PMS1	2q31
			PMS2	2p15-16
			MSH6	2p15-16
Peutz-Jeghers syndrome	Mucocutaneous pigmentations, hamartomatous polyps of the GI tract, and increased risk of GI and non-GI malignancy	30%	STK11/LKB1 kinase	19p13.3
FAMMM	Multiple nevi, melanomas, and increased risk of pancreatic cancer	15%	CDKN2A/p16	19p13.3
Hereditary breast and ovarian cancer (BRCA2)	Increased risk of breast, ovarian, and pancreatic cancer	5–10%	BRCA2	13q12.3
Hereditary pancreatitis (HP)	Recurrent pancreatitis and pancreatic cancer	40%	PRSS1	7q35
			SPINK1	5q32
Family X	Absence of extra-pancreatic cancer and pancreatitis; pancreatic insufficiency prior to the onset of cancer	80%	Unidentified	4q32-34

Table modified from Hansel et al. ⁴. HNPCC, hereditary nonpolyposis colorectal cancer; FAMMM, familial atypical multiple mole-melanoma syndrome; GI, gastrointestinal.

*Lifetime risk for developing pancreatic cancer for all individuals in developing countries is 1% ⁶⁴.