Lymphoepithelioma-Like Carcinomas in Hepatobiliary Systems
Summary
Lymphoepithelioma-like carcinomas of the hepatobiliary system represent a rare histological variant characterised by undifferentiated epithelial tumour cells set within a dense lymphoid stroma. Two principal subtypes are recognised: lymphoepithelioma-like hepatocellular carcinoma (LEL-HCC) arising from hepatocytes, and lymphoepithelioma-like intrahepatic cholangiocarcinoma (LEL-ICC) originating from bile duct epithelium. Although most LEL-ICC cases are associated with Epstein-Barr virus (EBV) in endemic regions, LEL-HCC often lacks EBV but may coexist with hepatitis B virus infection. Histopathological hallmarks include syncytial tumour nests, prominent CD8-positive T-cell infiltration and variable expression of immune checkpoints such as PD-L1. Radiological appearance is non-specific, so definitive diagnosis rests on tissue sampling and immunohistochemistry. Clinically, these tumours tend to present as solitary masses with relatively indolent behaviour compared with conventional hepatobiliary carcinomas. Surgical resection remains the mainstay of therapy, and emerging evidence suggests that immune-based strategies may further improve outcomes by harnessing the underlying immune-rich microenvironment. Ongoing research into tumour–immune interactions promises both deeper biological insight and novel translational approaches for this uncommon cancer variant.
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Lymphoepithelioma-Like Carcinomas in Hepatobiliary Systems publication trend
The graph below shows the total number of articles in lymphoepithelioma-like carcinomas in hepatobiliary systems across all publications each year (not limited to Nature Index journals).
Technical terms
Lymphoepithelioma-like carcinoma: A carcinoma variant with undifferentiated epithelial cells admixed with dense lymphoid infiltrate.
Hepatocellular carcinoma (HCC): A primary liver malignancy arising from hepatocytes.
Cholangiocarcinoma (ICC): A carcinoma originating from intrahepatic bile duct epithelium.
Epstein-Barr virus (EBV): A herpesvirus linked to the pathogenesis of certain lymphoepithelioma-like tumours.
Programmed death-1 (PD-1) inhibitor: A therapeutic antibody that blocks the PD-1 receptor to enhance T-cell-mediated tumour immunity.
NK-CIK therapy: Adoptive immunotherapy combining natural killer cells with cytokine-induced killer cells to target malignant cells.
References
- The MR Imaging of Primary Intrahepatic Lymphoepithelioma-like Cholangiocarcinoma: A Diagnostic Challenge. Diagnostics (2023).
- Lymphoepithelioma-like hepatocellular carcinoma: a case report and a review of the literature. World Journal of Surgical Oncology (2013).
- Programmed death-ligand 1 is upregulated in intrahepatic lymphoepithelioma-like cholangiocarcinoma. Oncotarget (2016).
- Epstein-Barr virus infection-associated cholangiocarcinoma: a report of one case and the review of literature. Virology Journal (2022).
- Combined NK-CIK and PD-1 inhibitor (nivolumab), an effective immunotherapy for treating intrahepatic lymphoepithelioma-like cholangiocarcinoma unassociated with EBV infection: Two case reports and a literature review. Frontiers in Oncology (2023).
- Case Report: Primary lymphoepithelioma-like intrahepatic cholangiocarcinoma. Frontiers in Oncology (2023).
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