James TA et al. (2004) Risk factors associated with earlier age of onset in familial pancreatic carcinoma. Cancer 101: 2722–2726

Around 5–10% of pancreatic adenocarcinoma patients have a familial predisposition to the disease. Little is known about the clinical characteristics of this familial form, however, and it has yet to be properly defined. A recent US study by James and colleagues has made use of registry data to compare the familial and sporadic forms of the disease.

Of 826 patients diagnosed with pancreatic adenocarcinoma between 1972 and 1999, 30 (3.6%) had at least one parent, sibling or child with the disease (the 'familial' cohort), whereas the remainder had no family history (the 'sporadic' cohort). Both groups had similarly advanced stage at presentation, poor overall survival and low resectability rates. There was no significant difference between the average age at diagnosis in the two groups, but patients in the familial cohort were significantly more likely to present at age <50 years than those with sporadic disease (36% vs 18%, P = 0.017). There was a higher proportion of smokers in the familial cohort than in the sporadic group (87% vs 66%) of those with documented history of smoking habits; although this difference was not statistically significant (P = 0.06), smoking may be an environmental trigger for familial disease and so is relevant in terms of risk assessment.

James et al. note that the younger age at onset of familial pancreatic carcinoma should be considered when developing screening strategies for at-risk patients. They also recommend that patients with a family history of the disease should be strongly advised to avoid smoking.