Thymic Lymphoepithelial Carcinoma Associated with Epstein-Barr Virus: Experiences and Literature Review

Abstract

Thymic lymphoepithelial carcinoma (TLEC) is a primary thymic carcinoma that accounts for about 14% of all thymic epithelial tumors and is classified into 14 types. The histological morphology is similar to lymphoepithelioma, a type of undifferentiated nasopharyngeal carcinoma. It has been reported that squamous carcinoma accounts for approximately 80% of thymic carcinoma, followed by TLEC, which accounts for 6%. TLEC has been reported to be associated with Epstein-Barr virus (EBV), with EBV infection in TLEC tumor cells first noted by Lyvraz et al. in 1985. Tumors shown to be EBV-positive are classified as TLEC if lymphoplasmacytic infiltration is lacking. However, only about 50% of the cases are positive for EBV, which is lower compared to nasopharyngeal lymphoepithelioma. Instances of EBV infection in other types of thymic epithelial tumor have been reported at lower rates, which suggests that EBV infection may have an important influence on the carcinogenesis of TLEC, though the etiology is unknown. TLEC is a highly malignant tumor with poor prognosis, as affected patients have a median survival time of 22 months, according to 58 cases from the literature, while the 5-year survival rate is 34.4%. Presently, prognosis is not considered to be affected by the presence or absence of EBV positivity.

Undifferentiated Large Cell Carcinoma of the Thymus Associated with Plasma-Cell Type Castleman Disease-Like Reaction: A Case Series

“…1 , 2 The former has more pronounced lymphoepithelial lesions and demonstrates squamous cell differentiation (positive for CK5/6, p63, and P40). 5 Moreover, lymphoepithelial carcinoma has higher rates of Epstein-Barr virus infection than other types of thymic epithelial tumors. In micronodular thymic carcinoma with lymphoid hyperplasia, the lesion lacks a CD-like architecture, and the cancer cells are positive for CD5 and CD117 staining.…”

How Does Epstein–Barr Virus Interact With Other Microbiomes in EBV-Driven Cancers?

“…EBV harbors high tumorigenic potential preferentially infecting B-cells, T-cells, natural killer (NK) lymphocytes, and epithelial cells, steadily promoting the uncontrolled proliferation of infected cells ( Miller and Lipman, 1973a ; Miller and Lipman, 1973b ), leading to a wide spectrum of EBV-positive cancers ( Cai et al., 2015 ). According to the EBV-infected cell type involved in the tumorigenesis, the divergent EBV-driven cancer could be grouped into three mainly groups, including lymphoproliferative disorders (LPD) ( Heslop, 2020 ), epithelial malignancies ( Yamaguchi et al., 2018 ), and lymphoepithelioma-like carcinoma (LELC) ( Ose et al., 2021 ) ( Table 1 ).…”

Pathogenesis and therapeutic implications of EBV-associated epithelial cancers

“…The first recognition of the possible association of LELC of the lungs with EBV was by Begin et al ( 28 ), and since then, the presence of EBV has also been detected in LELCs of the salivary gland ( 29 ), gastric ( 30 , 31 ), thymus ( 32 ), colon ( 33 ), lung ( 34 ) and the intrahepatic biliary tract ( 35 ). The identification of EBV in LELC has led to an increasing amount of research exploring the role of the oncovirus in the pathogenesis of LELC ( 36 ).…”