Cholangiocarcinoma in young individuals with and without primary sclerosing cholangitis.

OBJECTIVES: We aimed at characterizing the clinical presentation and prognosis of cholangiocarcinoma in youth with and without primary sclerosing cholangitis (PSC).

METHODS: Thirteen patients < or =25 yr of age with cholangiocarcinoma with or without PSC seen at the Mayo Clinic in Rochester, MN from 1980 to 2005 were identified.

RESULTS: Of the 13 patients with cholangiocarcinoma, 8 had concomitant PSC and 5 did not. PSC was the associated risk factor in 8/13 (61.5%) whereas PSC was present in only 321/2,014 (15.9%) cases with cholangiocarcinoma in patients older than 25 (P < 0.0001) seen in the same period of time. Seven of the eight (88%) patients with cholangiocarcinoma and PSC had IBD and 5 of the 7 (71%) had Crohn's disease. A mean 11 months transplant free survival was observed (range 3-20 months) in cholangiocarcinoma with PSC versus 8 months in cholangiocarcinoma patients without PSC (NS).

CONCLUSIONS: Cholangiocarcinoma is a rare disorder in young adults and it seems not to exist in children. The youth group of cholangiocarcinoma plus PSC seems to have a higher prevalence of Crohn's disease whereas the prognosis of cholangiocarcinoma in youth seems to be similarly poor as described in older patients with cholangiocarcinoma.