Abstract

Nuclear protein of the testis (NUT) carcinoma (NC) (formerly known as NUT midline carcinoma) is an aggressive pleomorphic squamous cell carcinoma with a dismal prognosis. Primary NC tumors are commonly located in the chest or head and neck regions. Imaging plays an indispensable role in the staging, management, treatment response assessment, and surveillance of NC. Primary pulmonary NC usually presents as a large mass with lymphadenopathy and pleural involvement. Primary head and neck NC presents as a large expansile necrotic mass in the sinonasal region with locoregional destruction and occasional cervical lymph node involvement. These imaging features are relatively non-specific but are consistent among patients. Currently, there are no standardized guidelines for the treatment of NC. Because of its rarity, paucity of reports in the medical literature, and the lack of awareness among radiologists, NUT carcinoma (NC) has been largely underdiagnosed and misdiagnosed. Clinical aggressive features and pleomorphic/undifferentiated squamous cell carcinoma should prompt genetic evaluation for NUT translocation to diagnose NC. In this article, we discuss NC's clinicopathologic and imaging features and treatment options, including emerging new treatments.

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