Clonal Epstein-Barr virus associated cholangiocarcinoma with lymphoepithelioma-like component

Epstein-Barr virus (EBV) has been linked to several undifferentiated carcinomas of the aerodigestive tract, especially the nasopharynx, but has not been reported in the biliary tract. We here presented a case of an unusual cholangiocarcinoma harboring EBV genome in a 47-year-old Chinese woman. Physical examination in 1988 in Taiwan, including abdominal sonography of the liver, was essentially negative. She experienced three episodes of malaria in 1990 when she worked in Ghana, Africa, and felt a tumor mass in the epigastrium in October 1992. She received an extended left lobectomy for a huge hepatic tumor of 12 x 10 x 5.5 cm in February 1993. Light microscopy revealed a cholangiocarcinoma composed of both well-differentiated adenocarcinoma and lymphoepitheliomatous undifferentiated carcinoma components. Abundant EBV EBER1 was shown in both tumor components, but not in the nontumor liver. Southern blot analysis and polymerase chain reaction showed a monoclonal episomal form of EBV, with a genotype characteristic for Chinese EBV strain type 1. This finding suggests that the EBV infection preceded monoclonal EBV-harboring tumor cell expansion in this case.