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Risk of Pancreatic Cancer in the Long-Term Prospective Follow-Up of Familial Pancreatic Cancer Kindreds.

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BACKGROUND A family history of pancreatic cancer is associated with increased pancreatic cancer risk. However, risk estimates for individuals in kindreds with an aggregation of pancreatic cancer (>1 relative) are… Click to show full abstract

BACKGROUND A family history of pancreatic cancer is associated with increased pancreatic cancer risk. However, risk estimates for individuals in kindreds with an aggregation of pancreatic cancer (>1 relative) are imprecise due to small samples sizes or potentially impacted by biases inherent in retrospective data. OBJECTIVE Determine the age-specific pancreatic cancer risk as a function of family history using prospective data. METHODS We compared pancreatic cancer incidence (n = 167) in 21,141 individuals from 4,433 families enrolled in the National Familial Pancreatic Cancer Registry to that expected based upon Surveillance Epidemiology and End Results (SEER) data and estimated the cumulative probability of pancreatic cancer using competing risk regression. RESULTS Familial pancreatic kindred members (FPC, kindreds with pancreatic cancer in two first-degree relatives or a pathogenic variant) had a standardized incidence ratio (SIR) of 4.86 (95% CI 4.01-5.90), and NFPTR sporadic kindreds members (SPC, kindreds not meeting familial criteria) had an SIR of 2.55 (95% CI 1.95-3.34). Risk in FPC kindreds increased with an increasing number of first-degree relatives with pancreatic cancer, with SIR of 3.46 (95% CI 2.52-4.76), 5.44 (95% CI 4.07-7.26), 10.78 (95% CI 6.87-16.89) for 1, 2 and 3 or more first-degree-relatives with pancreatic cancer, respectively. Risk was also higher among individuals with a family history of young-onset(<50) pancreatic cancer. CONCLUSION Pancreatic cancer risk is strongly dependent on family history, including both the degree-of-relationship(s) and age-of-onset of pancreatic cancer in relatives. These risk estimates will help inform the design of early detection studies and the risk/benefit analysis of screening trials.

Keywords: pancreatic cancer; risk; familial pancreatic; family history; cancer

Journal Title: Journal of the National Cancer Institute
Year Published: 2022

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