Hepatocellular lymphoepithelioma-like carcinoma associated with epstein barr virus: a hitherto unrecognized entity

• Published in: Diagnostic molecular pathology Vol. 13; no. 3; p. 183
• Main Authors: Si, Michael W, Thorson, John A, Lauwers, Gregory Y, DalCin, Paola, Furman, Jaime
• Format: Journal Article
• Language: English
• Published: United States 01-09-2004
• Subjects: Adult; Carcinoma, Hepatocellular - genetics; Carcinoma, Hepatocellular - pathology; Carcinoma, Hepatocellular - virology; Carcinoma, Squamous Cell - genetics; Carcinoma, Squamous Cell - pathology; Carcinoma, Squamous Cell - virology; Chromosomes, Human, Pair 11 - genetics; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - pathology; Female; Gene Dosage; Herpesvirus 4, Human - isolation & purification; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Liver Cirrhosis - virology; Liver Neoplasms - genetics; Liver Neoplasms - pathology; Liver Neoplasms - virology; Liver Transplantation; Lymphatic Metastasis - pathology; Polymerase Chain Reaction; Tumor Virus Infections - complications; Tumor Virus Infections - pathology
• ISSN: 1052-9551
• DOI: 10.1097/01.pas.0000124336.90615.8d

Lymphoepithelioma-like carcinoma (LELC) is an undifferentiated carcinoma with a dense lymphoid stroma. It has been reported in diverse organs and shows variable association with Epstein-Barr virus (EBV). Only a few EBV positive cases have been observed in the hepatobiliary system, all of which were considered to be cholangiocarcinomas. We report a unique case of hepatocellular LELC arising in a cirrhotic liver with EBV demonstrated in the tumor cells.
A 39-year-old Hispanic female underwent an orthotopic liver transplant for end stage liver disease secondary to chronic hepatitis C. A high-grade hepatocellular carcinoma with a dense lymphocytic infiltrate was found in the explant as well as in a portal lymph node. Three months posttransplant, the patient developed numerous hepatic nodules with enlarged periaortic and portacaval lymph nodes. Biopsy of the hepatic nodules showed a recurrent hepatocellular carcinoma devoid of a dense lymphocytic infiltrate. Both the primary and recurrent tumors were positive for EBV by molecular studies. The patient eventually expired from liver failure over a 6-week period.
This case represents the first report of EBV-positive hepatocellular LELC. It is particularly interesting given the precipitous clinical outcome, which was possibly related to immunosuppresive therapy.