Lymphoepithelial carcinoma of salivary glands

Abstract

Background: Malignant lymphoepithelial lesions (MLEL) or lymphoepithelial carcinoma or undifferentiated carcinoma with lymphoid stroma, is a rare but unique malignancy of the major salivary glands. There is an exceptionally high incidence in Eskimos and native Greenlanders, but it has never been reported in the Arab population. We report the first case from Saudi Arabia in the native Arabs. These are unencapsulated tumors with a strong tendency to metastasize to the regional lymph nodes.

Methods: We reviewed English and Arabic literature and found the total number of reported cases of MLEL to be 103 with predominant involvement of the parotid gland. Parotidectomy followed by neck dissection and radiotherapy was performed, with a survey of the nasopharynx and EB virus titer.

Results: The patient underwent radical neck dissection followed by radiation after developing the neck metastasize. Three years to date he remains in remission with no distant or regional metastases.

Conclusion: Malignant lymphoepithelial lesions of the salivary gland are unencapsulated tumors with poorly differentiated epithelial cells scattered throughout fibrolymphocytic stroma. They have a strong tendency to metastasize to the regional lymph nodes with predominant involvement of the parotid gland. They are commonly associated with Epstein-Barr virus infection. The majority of them are radiosensitive. Combination therapy with surgery and radiation is desirable to control the disease. We recommend complete surgical excision with neck dissection followed by postoperative radiotherapy to the local site as well as to the neck as treatment of choice.