1. Lymphoepithelioma-like neoplasm of the biliary tract with 'probable low malignant potential'
- Author
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Binny Khandakar, Young Nyun Park, Jun-Ru Liu, Swan Thung, Irene Oi-Lin Ng, Neil D. Theise, Alexander C Kagen, Hyungjin Rhee, Ying Li, and Tommy Richard Sun-Wing Tong
- Subjects
Adult ,Male ,Pathology ,medicine.medical_specialty ,Epstein-Barr Virus Infections ,Histology ,Carcinoma, Hepatocellular ,Context (language use) ,Gene mutation ,medicine.disease_cause ,Pathology and Forensic Medicine ,Cholangiocarcinoma ,medicine ,Neoplasm ,Humans ,Lymphoepithelioma ,Aged ,business.industry ,Carcinoma ,Liver Neoplasms ,General Medicine ,medicine.disease ,Epstein–Barr virus ,Bile Duct Neoplasms ,Biliary tract ,Hepatocellular carcinoma ,Female ,KRAS ,business - Abstract
AIMS Lymphoepithelioma-like carcinomas (LELCs) are uncommon epithelial cancers characteristically showing two distinct components consisting of malignant epithelial cells and prominent dense lymphoid infiltrate. Hepatic LELCs consist of two types, the lymphoepithelioma-like hepatocellular carcinoma and lymphoepithelioma-like cholangiocarcinoma (LEL-CCA), with the latter being strongly associated with Epstein-Barr virus (EBV). METHODS AND RESULTS We present a series of three cases of intrahepatic biliary EBV-associated LEL tumours in which the biliary epithelial component showed a distinctly benign appearance, instead of the usual malignant epithelial features of a typical CCA or EBV-associated LEL-CCA. In the lesions, the biliary epithelium showed interconnecting glands or cords of cells. All had a very low proliferation (Ki-67) index. Immunohistochemistry for IDH1 and TP53 performed on two cases was negative and molecular tests for EGFR and KRAS gene mutations performed on one were negative. Prognosis was very good in all three cases, with patients alive with no evidence of disease 24-62 months after surgery. Intriguingly, all three cases had co-infection of HBV and EBV. These cases are also discussed in the context of the 63 cases of LEL-CCA available in the literature, with a focus on epidemiology, clinicopathological features and potential research interests. CONCLUSIONS Based on the distinct clinicopathological features and unique survival benefits, we believe these tumours represent the benign end of the spectrum of EBV-associated lymphoepithelial biliary carcinomas. Whether these tumours require a revision of the current nomenclature to 'lymphoepithelioma-like neoplasm of the biliary tract with probable low malignant potential' will require more detailed analysis with larger case-series.
- Published
- 2021