Abstract
Intrahepatic lymphoepithelioma-like cholangiocarcinoma (LELCC) is a rare variant of cholangiocarcinoma. Here, we report the largest single series of LELCC cases yet studied (n = 13). We retrospectively analyzed the clinical data of the 13 patients and measured the expression of programmed death-ligand 1 (PD-L1) in tumors using immunohistochemical staining. We also analyzed 15 cases of conventional intrahepatic cholangiocarcinoma (IHCC) for comparison. We found that eight patients with LELCC were infected with Epstein-Barr Virus (EBV), and EBV infection correlated with poor prognosis in LELCC. Four patients among the five (80.0%) without EBV had a history of chronic viral hepatitis B. None of the 15 cases of conventional cholangiocarcinoma were positive for EBV. PD-L1 was expressed in both the tumor cells and tumor-infiltrating immune cells in LELCC patients at higher levels than in IHCC patients (P < 0.05). These observations suggest that EBV infection may promote the development of LELCC, and that PD-L1 may be a potential therapeutic target for treatment of EBV-associated LELCC.
Highlights
Lymphoepithelioma-like carcinomas (LELCs) are tumors characterized by undifferentiated carcinomas with prominent tumor-infiltrating lymphocytes (TILs) identical to undifferentiated nasopharyngeal carcinoma (NPC)
We found that eight patients with like cholangiocarcinoma (LELCC) were infected with Epstein-Barr Virus (EBV), and EBV infection correlated with poor prognosis in LELCC
Lymphoepithelioma-like cholangiocarcinoma is a rare variant of intrahepatic cholangiocarcinoma (IHCC), displaying distinct clinicopathological characteristics
Summary
Lymphoepithelioma-like carcinomas (LELCs) are tumors characterized by undifferentiated carcinomas with prominent tumor-infiltrating lymphocytes (TILs) identical to undifferentiated nasopharyngeal carcinoma (NPC). Because of the limited number of cases reported (only 26 in the literature in English [8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23]), further research is needed to better characterize the clinicopathological features of LELCC. Programmed death-1 (PD-1) is an immunosuppressive receptor expressed in active T cells [24, 25] that has been found in TILs in several types of cancer [26,27,28,29]. To better understand the clinicopathological features of LELCC, we analyzed 13 new cases, measuring the expression pattern of PD-L1 and analyzing the relationship between EBV infection and PD-L1 expression
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