Background: Epithelial-myoepithelial carcinoma (EMC) is a rare tumor of the major and minor salivary glands. Sinonasal EMC is extremely uncommon and hitherto not described within the frontal or ethmoid sinuses.
Objective: To present a novel sinonasal subsite and review the literature regarding sinonasal EMC.
Methods: A case of frontoethmoidal EMC was presented. A medical literature data base was queried from January 1, 1950, to August 8, 2017, for all reports of sinonasal EMC.
Results: A 69-year-old man underwent combined open and endoscopic craniofacial resection of a right frontoethmoidal EMC, a previously undescribed primary location for this tumor. A comprehensive review of the literature revealed 13 additional cases of sinonasal EMC.
Conclusion: EMC is an uncommon neoplasm typically found in the major salivary glands; occurrence in the nose or paranasal sinuses is extremely rare. EMC often follows an indolent clinical course, although, in a minority of cases, particularly in large tumors with nuclear atypia, more aggressive behavior may be observed.