Isolated squamous cell carcinoma of the orbital apex

A 43-year-old woman (Case 1), 63-year-old man (Case 2), and a 67-year-old man (Case 3) presented with diplopia (Cases 1, 2, and 3) and upper-eyelid ptosis (Cases 1 and 3). The cases had preceding cranial nerve V1 disturbances ranging from 3 months to 8 years. Each demonstrated complete internal ophthalmoplegia and external ophthalmoplegia. No case had a cutaneous squamous cell carcinoma (SCC) on presentation or by history. Imaging revealed isolated orbital apex masses. Tumor biopsies revealed SCCs of various differentiations. Systemic workup revealed no extraorbital malignancy. All received radiation therapy, and 2 patients underwent adjuvant chemotherapy. One patient is alive since diagnosis (49 months). In Case 2, SCC developed in the contralateral orbit, and the patient died 19 months after diagnosis; and Case 3 died 12 months after diagnosis. These isolated cases of orbital SCC may have arisen from orbital choristomatous squamous epithelium, may represent de novo or metastatic tumors, or may be manifestations of occult perineural spread.