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A 66-year-old Japanese man was hospitalized due to epigastralgia and a 2 cm-diameter liver tumor. Laboratory data revealed hyperbilirubinemia, but there was no elevated serum transaminase. Diagnosis of NCPF was performed by a biopsy of the liver tumor. The biopsy specimen showed a fibrotic liver and the cut surface of the tumor was homogeneous. The tumor did not have a capsule and the tumor was surrounded by a smooth and regular edge in contrast to the hypervascularity of the tumor. There was no capsule formation of the tumor, showing that the tumor was a hyperplasia of the biliary epithelium. A lipiodol dye was injected to the tumor, and the tumor showed a delayed washout, which is associated with malignant tumors. Immunostaining for actin was strongly positive in fibroblasts in the fibrotic liver. No antinuclear antibody or liver-kidney microsomal antibody was observed. Mild splenomegaly was noted. For 18 months he has been on a gluten-free diet, with no recurrence of the tumor. The diagnosis of non-cirrhotic portal fibrosis (NCPF) was based on the presence of characteristics of portal fibrosis in the liver, which does not show cirrhosis. To our knowledge, this is the first reported case of NCPF. NCPF in this case may be associated with a malignant lesion of the biliary epithelium and stress-induced hepatitis.
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