Cholangiocarcinoma in young individuals with and without primary sclerosing cholangitis.
OBJECTIVE
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.
Björnsson
Einar
E
Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Angulo
Paul
P
D001650:Bile Duct Neoplasms / Q000150:complications / Q000401:mortality* / Q000601:surgery; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000150:complications / Q000401:mortality* / Q000601:surgery; D015209:Cholangitis, Sclerosing / Q000150:complications*; D003424:Crohn Disease / Q000150:complications; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D011379:Prognosis; D012307:Risk Factors