Intrahepatic Lymphoepithelioma-Like Cholangiocarcinoma Not Associated with Epstein-Barr Virus: A Case Report

Lymphoepithelioma-like cholangiocarcinomas are rare tumors and most of them are related with Epstein-Barr virus (EBV) infection. Here, a case of a patient with lymphoepithelioma-like cholangiocarcinoma not associated with EBV infection is presented. In a 79-year-old man with hepatitis B virus-associated cirrhosis, a liver mass was detected on abdominal CT. Macroscopically, the resected tumor was pale gray, rubbery and well defined. Histologically, the tumor was composed of two components: an adenocarcinoma that formed irregular small glands and a lymphoepithelioma-like carcinoma that exhibited sheets of undifferentiated epithelial cells with lymphoid stroma. Lymphoplasmacytic infiltrates were more predominant in the lymphoepithelioma-like carcinoma than in the adenocarcinoma. Both components were roughly divided, but they gradually merged. Immunohistochemically, the adenocarcinoma component was diffusely positive for AE1/AE3, cytokeratin 7, cytokeratin 19 and epithelial membrane antigen, while the lymphoepithelioma-like carcinoma component was focally positive for them. However, both components were diffusely positive for p53 protein, and in situ hybridization using EBV-encoded RNA 1 was negative in both components as well. Examination of a resected para-aortic lymph node revealed metastasis exclusively of the lymphoepithelioma-like carcinoma component.