Risk of Pancreatic Cancer in Families With Lynch Syndrome

Abstract

Pancreatic cancer is the fourth leading cause of cancer deaths in the United States.¹ Although most cases are thought to be sporadic, data suggest up to 10% of ductal adenocarcinomas may be due to an inherited predisposition based on familial clustering.^2,3 For most pancreatic cancer kindreds, the causative gene has not been identified. In a subset of families, pancreatic cancer may be an integral tumor in a number of familial cancer syndromes with established germline mutations. These conditions include Peutz-Jeghers syndrome (cumulative lifetime risk of 36%),^4,5 familial atypical multiple mole melanoma syndrome (lifetime risk, 17%),⁶ hereditary breast/ovarian cancer syndrome (lifetime risks, 1.2% and 2.1%, for BRCA1 and BRCA2 carriers, respectively),^7,8 hereditary pancreatitis (lifetime risk, 40%),⁹ and the newly described familial pancreatic cancer due to mutations in the PALB2 gene (risk not specified).¹⁰