Lymphoepithelial-like carcinoma of the parotid gland: a case report and a brief review of the western literature

Abstract

BackgroundPrimary lymphoepithelial-like carcinoma of the parotid gland is a rare tumour with an increased incidence among Eskimos and Orientals. In these populations, it is usually associated with Epstein-Barr virus. In Western countries, salivary gland lymphoepithelial-like carcinomas are uncommon and only 14 cases have been described so far; among these, only five cases showed Epstein-Barr virus positivity.Case reportA 45-year-old woman was admitted to Siena Hospital for evaluation of a pre-existent (2 years) painless and tender submandibular mass, rapidly enlarging since two months. On physical examination, a 2.5-cm mass was found in the right parotid. It was firm, mobile and non-tender. Laboratory data were within reference range. Nuclear magnetic resonance detected a 2,5×1,5×1-cm well-circumscribed mass in the deep lobe of the right parotid. A total right paroditectomy with dissection of a satellite lymph node was performed. On the basis of morphological, immunohistochemical and molecular biology findings, a diagnosis of stage II (according to TNM7) Epstein Barr-virus positive, undifferentiated lymphoepithelial-like carcinoma of the parotid gland was made. Twenty months after surgery the patient was free of disease.ConclusionsFurther studies seem to be necessary to completely elucidate the oncogenic role of Epstein Barr-virus in these tumors, which have identical morphology but different prognosis and variable presence of the virus.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1260381551000616

Single Persistently Enlarged Lymph Node in the Level II Region of the Neck

“…Lymphoepithelial carcinoma (LEC) is a rare malignancy that has a strong ethnic predisposition for Eskimos in the northern circumpolar region, Mongolians, and South-east Asians [1][2][3][4][5][6][7][8]. The mean onset is 40 years old, and women are more frequently affected than men regardless of ethnicity [2,4].…”

“…The mean onset is 40 years old, and women are more frequently affected than men regardless of ethnicity [2,4]. For the populations described, this malignancy is often found in the nasopharynx and major salivary glands and is strongly associated with EpsteinBarr virus (EBV) [7].…”

“…This cancer is radiosensitive and is often treated with radiation therapy only or a combination of surgical resection, neck dissection, and radiation therapy. There are, however, no long-term follow-up studies demonstrating what combination is best to treat this cancer [2,6,9].…”

“…It has been proposed that two-thirds arise de novo while the other onethird arises from benign lymphoepithelial lesions [1,2]. The role of EBV in the pathogenesis of LEC is also puzzling.…”

“…Also, EBV has not been associated with all LELCs such that many non-foregutderived lymphoepithelial-like carcinomas (LELC) are usually EBV negative [1,2,7,10] compared to foregut derived LELCs. This observation further confounds the picture and as a result, there is speculation that EBV may have organ specificity in genetically predisposed patients [1,2,6,10].…”