Undifferentiated carcinoma with lymphoid stroma or lymphoepithelial carcinoma of the major salivary glands is a demographically and histopathologically unique malignancy. Although whites may have the disease, it is preponderantly a carcinoma of North American Eskimos and native Greenlanders. The carcinoma shares many features with undifferentiated nasopharyngeal carcinomas, from which it must be distinguished: histologic appearance, putative relationship with Epstein-Barr virus, predilection for mongoloid races, and response to therapy. In some cases, the carcinoma appears to have evolved from a lymphoepithelial lesion.
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