Cholangiocarcinoma and Its Association with Primary Sclerosing Cholangitis in Young Adults

Primary sclerosing cholangitis (PSC) is an uncommon form of chronic liver disease, and approximately 6% to 23% of PSC patients develop cholangiocarcinoma (CCA). In addition, PSC has been associated with inflammatory bowel disease (IBD). Although most PSC patients who develop CCA do so in their 40s, risk factors for CCA are not related to duration of PSC, sex, age, or histologic stage. PSC and CCA in younger patients have not been described well. In this retrospective study, researchers evaluated the occurrence of CCA in young patients (age, ≤25) and described clinical presentations and prognoses.

A total of 13 young patients with CCA were identified from 1980 through 2005 at Mayo Clinic in Rochester, Minnesota. CCA diagnoses were based on hi…