Objective: Lymphoepithelial carcinoma (LEC) accounts for 0.4% of malignant tumors of the salivary gland and 0.8% of parotid gland malignancies. Over the past 50 years, less than 300 cases have been reported in the literature. The purpose of this study was to investigate the characteristics of salivary gland LEC. Methods: We retrospectively reviewed the medical records and analyzed clinical data obtained from 10 patients seen at our hospital between 2005 and 2020 with salivary gland LEC. Results: All patients presented with a self-palpable, non-tender, hard swelling, or lump near the jaw or infra-auricular region. Most cases (n = 8) were of solitary tumors, and enhancing patterns on computed tomography mainly were homogenous (n = 8). Interestingly, eight patients tested positive for the Epstein–Barr encoding region in in situ hybridization. Still, only three patients had detectable circulating Epstein–Barr virus (EBV) DNA, and one patient had detectable EBV IgA. All patients underwent complete tumor resection, followed by radiotherapy, and six also underwent chemotherapy. Nine patients became disease-free within 5 years, and one died due to disease 4 years after surgery. Conclusion: Although rare and considered to be a high-risk malignancy, LECs have favorable treatment outcomes. Circulating EBV DNA is still not considered a marker for preoperative assessment or postoperative treatment response. The role of EBV DNA requires further investigation. Level of Evidence: 4.
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