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Lymphoepithelioma-like neoplasm of the biliary tract with 'probable low malignant potential'

 Binny Khandakar  ;  Jun-Ru Liu  ;  Swan Thung  ;  Ying Li  ;  Hyungjin Rhee  ;  Alexander C Kagen  ;  Tommy Sun-Wing Tong  ;  Young Nyun Park  ;  Neil Theise  ;  Irene Oi-Lin Ng 
 HISTOPATHOLOGY, Vol.80(4) : 720-728, 2022-03 
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Issue Date
Epstein-Barr virus ; immunohistochemistry ; lymphoepithelioma-like cholangiocarcinoma ; lymphoepithelioma-like lesion ; molecular
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.
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1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
Yonsei Authors
Park, Young Nyun(박영년) ORCID logo https://orcid.org/0000-0003-0357-7967
Rhee, Hyungjin(이형진) ORCID logo https://orcid.org/0000-0001-7759-4458
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