Abstract
A case of a 15-year-old girl with sudden vision loss left eye, left relative afferent pupillary defect, and normal fundus both eyes. Magnetic resonance imaging (MRI) brain and orbits showed a heterogeneous enhancing mass lesion in the left posterior ethmoid sinus with bony invasion and crowding of the optic canal. She underwent left optic nerve decompression with removal of the lesion and was started on systemic steroids. Biopsy was suggestive of NUT carcinoma (NC) and immunohistochemistry (IHC) confirmed the same. Despite chemotherapy, the tumor progressed rapidly in six months invading the left orbit, extending to right side of the midline and left anterior cranial fossa, with subsequent complete loss of vision of right eye also. She eventually underwent a globe sparing orbital exenteration with anterior cranial base resection along with postoperative radiotherapy. A month later, patient developed extensive skeletal and hepatic metastasis and succumbed to the disease; 11 months after the initial diagnosis.
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