Dyskerin expression in human fetal, adult and neoplastic intrahepatic bile ducts: correlations with cholangiocarcinoma aggressiveness.

[cholangiocarcinoma]

To investigate the immunohistochemical expression of Dyskerin , a biomarker involved in ribosome production and telomeres maintenance , in human fetal , adult and neoplastic bile ducts , as well as the possible correlations with cholangiocarcinoma aggressiveness .Sixty consecutive intrahepatic cholangiocarcinomas were collected and used for a tissue micro-array construction (total 176 cores ); clinical data and follow-up were collected . Five fetal and 10 normal livers were included as controls . Automatic immunohistochemistry for Dyskerin , p 53 and Ki 67 , and nucleolar silver-staining were performed . In normal livers , Dyskerin was negative in smaller bile ducts (mean 44 .8 Â Î¼m ) and positive in the bile ducts with larger diameter (mean 116 .1 Â Î¼m ; P Â <Â 0 .001 ). Dyskerin was positive in 56 .7 % of cholangiocarcinomas , and correlated with p 53 mutation (P Â =Â 0 .008 ) and a higher proliferative index /Ki 67 (P Â =Â 0 .003 ), that were included as markers of tumor aggressiveness . Finally , Dyskerin-positive cholangiocarcinomas showed a negative trend in disease-free survival (P Â =Â 0 .078 ) at univariate analysis .The not-neoplastic biliary tree seems to progressively lose Dyskerin expression from the major branches to the peripheral portal bile ducts . Similarly , intrahepatic cholangiocarcinomas showed two patterns of Dyskerin expression , and the Dyskerin-positive phenotype seemed to characterize the more aggressive cholangiocarcinomas .

Diseases
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Diseases presenting "peripheral portal bile ducts" symptom

cholangiocarcinoma

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