Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC), originally known as HPV-related carcinoma with adenoid cystic carcinoma-like features, is a recently described neoplasm that presents only in the sinonasal tract, displays features of both a surface-derived carcinoma and a salivary gland carcinoma, and is associated with high-risk HPV, specifically HPV type 33. Majority of the cases display high-grade histologic features, but HMSC paradoxically behaves in a relatively indolent fashion. Distinguishing HMSC from other histologic mimickers is essential as the management and prognosis are significantly different. In this article, we present a unique case of HMSC and review the literature.
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