Hepatic and extrahepatic malignancies and primary sclerosing cholangitis

Schrumpf, E.; Boberg, K.M.
February 2003
Gut;Feb2003, Vol. 52 Issue 2, p165
Academic Journal
Background: Although primary sclerosing cholangitis (PSC) has long been described as a strong risk factor for the development of cholangiocarcinoma, there are few good epidemiological studies which have examined its frequency. In addition, the risk of developing other malignancies is unknown. Excellent health information systems in Scandinavia provide the opportunity to examine such associations in rare diseases. Objective: To assess the risk of hepatobiliary and extrahepatic malignancy in a large Swedish cohort of patients with PSC. Design: Follow up of a large case series of patients with PSC comparing the frequency of malignancy with that in the general Swedish population. Patients: A total of 609 patients with a clinical, biochemical, and cholangiographic diagnosis of PSC, identified by hepatologists from all Swedish university hospitals. Follow up was from the National Swedish Cancer Registry; clinical follow up and median follow up time was 5.7 years. Results: Twenty eight per cent of cases died during follow up, 44% of malignancy. Cholangiocarcinoma occurred in 13% of the cohort, with an incidence of hepatobiliary carcinoma of 1.5% per year after the first year post diagnosis of PSC. The risk of pancreatic cancer was increased 14-fold compared with the general Swedish population. Conclusion: The major cause of death in PSC patients (excluding those undergoing liver transplantation) was cancer. Not only was the increased risk of hepatobiliary cancer confirmed, but a new discovery of an increased risk of pancreatic cancer in patients with PSC was described.


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