. Author manuscript; available in PMC: 2011 Oct 1.

Published in final edited form as: Gastroenterology. 2010 Aug 19;139(4):1076–1080.e2. doi: 10.1053/j.gastro.2010.08.012

Table 1.

Inherited Syndromes Associated With Increased Risk of Pancreatic Cancer

Syndrome	Gene	Gene Function	Lifetime Risk for Pancreatic Cancer (%)
Hereditary pancreatitis⁶¹	PRSS1	Cationic trypsinogen	25–40
Familial atypical multiple mole melanoma^{37, 83}	p16/ CDKN2A	Tumor suppressor	10–17
Hereditary breast ovarian cancer syndrome^{47–48, 84}	BRCA 2 BRCA1	Tumor suppressor Tumor suppressor	5 3.6
Peutz-Jeghers syndrome²⁸	STK11/ LKB1	Tumor suppressor, serine threonine kinase	36
Lynch syndrome⁴²	MLH1, MSH2, MSH6, PMS2	Mismatch repair	3.7