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  • Title: Cholangiocarcinoma in young individuals with and without primary sclerosing cholangitis.
    Author: Björnsson E, Angulo P.
    Journal: Am J Gastroenterol; 2007 Aug; 102(8):1677-82. PubMed ID: 17433022.
    Abstract:
    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.
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