SummaryBackground/Aim: Primary Lymphoepithelial carcinoma (PLEC) is a rare subtype of salivary gland cancers, which comprises only 0.4% of salivary malignant neoplasms and only a few cases have been presented previously. Case report: A patient with PLEC of the parotid gland, its management and the available literature are presented. A 53-year-old woman with initial lesion of a lump in the region of the right parotid received treatment with antibiotics which did not lead to improvement. MRI was performed that recorded the presence of a tumor in the right parotid gland and the patient subsequently underwent excision biopsy. The histopathological evaluation together with additional immunohistochemical positive staining of EMA+, EGFR+, p63+, CK 5/6+, AE1/AE3+ established the diagnosis of PLEC. A PET-CT scanning has shown nor primary mucosal source neither skin lesion to account for any possible metastatic disease, consequently a course of adjuvant post-operative radiotherapy to the region of the right parotid gland was performed. Conclusions: In the differential diagnosis of a parotid gland lump should be included the possibility of a rare salivary gland neoplasm such as PLEC. Surgical excision and radiotherapy have been proposed for the treatment of early and advanced neoplasm stages. Ages of the patient, stage of the neoplasm as well as type of therapy are significant and individual variables for the prediction of the prognosis.
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