Pancreatic cancer is rare and poorly studied. What is known is that pancreatic cancer is aggressive, with a five-year survival rate of only 4 percent. A small percentage of patients with pancreatic cancer have first-degree relatives with pancreatic cancer. Smoking has been identified as the single most important risk factor in familial pancreatic cancer. Despite genetic characterization of other hereditary cancers, the genetic component of pancreatic cancer remains a mystery. With such little known about what is now called familial pancreatic adenocarcinoma (FPAC), researchers led by Ted A. James, M.D. of the Roswell Park Cancer Institute in Buffalo, New York investigated the clinical course and outcome of FPAC compared to sporadic pancreatic cancer.
Retrospective review of 826 patients with pancreatic cancer found 30 had the familial form (3.6 percent). Earlier age at diagnosis and a smoking history were marked features among the familial group. The mean age of diagnosis was younger in the hereditary cohort than among those with the sporadic form (57.1 years old versus 61 years old), and more FPAC patients were diagnosed before the age of 50 (36.7 percent versus 18.3 percent). Moreover, patients with FPAC were more likely to smoke than those who had sporadic pancreatic cancer (87 percent versus 66 percent).