Hsu FC, Roberts NJ, Childs E, et al. - An increased lifetime risk of pancreatic cancer was found in persons with a pathogenic variant in the ATM gene, and enhanced early detection surveillance may be beneficial for these individuals.

• This was a multicenter cohort analysis using pedigree data from 130 pancreatic cancer kindreds with a pathogenic germline ATM variant.

• In these 130 families with 2227 family members, 217 individuals were found to have pancreatic cancer.

• Pancreatic cancer risk in persons with a pathogenic variant in the ATM gene was 1.1% by age 50 years, 6.3% by age 70 years, and 9.5% by age 80 years.

• Relative risk of pancreatic cancer was estimated to be 6.5 in ATM variant carriers vs noncarriers.