Clinicopathological consultation lymphoepithelial carcinoma of the larynx, hypopharynx, and trachea

Lymphoepithelial carcinoma of the larynx, hypopharynx, and trachea is a rare neoplasm composed of large, poorly differentiated, nonkeratinized cells intermingled with small nonneoplastic lymphocytes and plasma cells. It is histologically similar to its more common counterpart occurring in the nasopharynx. In contrast to nasopharyngeal carcinoma, most cases have not been associated with Epstein-Bart virus (EBV), although rare cases have been reported to be EBV-positive. The diagnosis often requires immunohistochemistry or electron microscopy for confirmation. The neoplasm seems to behave in a fashion reminiscent of nasopharyngeal carcinoma. Lymph node metastasis occurs in the majority of patients, and eventual visceral dissemination occurs in one fourth. Radiotherapy is the main treatment for the primary tumor and regional metastases, but chemotherapy is indicated for more advanced disease. The initial stage is the primary determinant of prognosis. Death from disease occurs in about one third of patients.