Abstract
Nuclear protein in testis (NUT) midline carcinoma (NMC) is a rare malignant tumor defined by a unique chromosomal translocation of the NUT gene on chromosome 15q14. This very rare tumor is usually located in midline structures, especially in the head and neck region. However, there have been no previous reports of NMC arising from the eyes. Here, we first describe a case of NMC originating in the right orbit of a 60-year-old woman. A multimodality approach, including surgery, chemotherapy, radiotherapy, and target therapy, was adopted in clinical practice.