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In contrast to nasopharyngeal carcinoma, most cases have not been associated with Epstein-Barr 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. 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