NUT Carcinoma

A patient presented with several weeks of right eye proptosis. On initial examination, his visual acuity in the right eye was 20/400, and a relative afferent pupillary defect was noted. He had 4 mm relative proptosis on the right and restricted motility in all gaze directions. CT orbits revealed a lateral orbital mass centered around the lateral orbital wall, involving the infratemporal fossa and temporalis muscle, and abutting the optic nerve. He underwent a right orbitotomy with biopsy of the lesion, which revealed a high-grade, poorly differentiated carcinoma. Immunohistochemistry was positive for a NUT rearrangement, and the patient was diagnosed with NUT carcinoma. PET scan revealed a pancreatic lesion that was biopsied and confirmed to be histologically identical to the orbital lesion. Chemotherapy was initiated, however the patient ultimately decided to transfer care to another facility closer to family.

Presentation Date: 02/09/2023

Issue Date: 02/24/2023