An increased risk of pancreatic adenocarcinoma in patients with hereditary pancreatitis was previously demonstrated in 2 multinational studies.
The pancreatic adenocarcinoma frequency in this setting is however unknown due to lack of exhaustive case collection.
|At age 75 years, the risk of pancreatic adenocarcinoma was 49% for men |
|American Journal of Gastroenterology|
Dr Vinciane Rebours and colleagues from France evaluated the standardized incidence ratio of pancreatic adenocarcinoma in an exhaustive national series of patients with hereditary pancreatitis, and searched for risk factors.
The researchers contacted all French genetic laboratories, with a 100% response rate, as well as pediatricians, and gastroenterologists with a response rate of 84%.
Inclusion criteria were mutation in the PRSS1 gene or recurrent, acute, or chronic pancreatitis.
Additional inclusion criteria were no precipitating factors in 2 first-degree relatives or 3 second-degree relatives in 2 generations.
The teams' diagnosis of pancreatic adenocarcinoma was based on histological records.
The researchers identified 78 families and 200 patients.
Of these, 181 were alive, giving 6,673 person-years, with a median of 3 generations.
The team reported that 53% were men.
PRSS1 mutations were searched for in 96% of the patients, and were detected in 68%.
The team diagnosed 10 with pancreatic adenocarcinoma.
The researchers found a standardized incidence ratio of pancreatic adenocarcinoma for the whole population.
The standardized incidence ratio was 87 for men, and 142 for women, with no influence of genetic mutation.
At ages 50 and 75 years, the cumulated risk of pancreatic adenocarcinoma was 11%, and 49% for men and 8% and 55% for women, respectively.
Smoking and diabetes mellitus were the main associated risk factors.
Dr Rebours' team concluded, "Patients with hereditary pancreatitis have a marked relative and absolute increased risk of pancreatic adenocarcinoma as compared to the general population, especially in smokers."
"There is no correlation with the type of PRSS1 mutation."