Orbital Chordoma: An Extremely Rare Tumor

A 77-year-old man presented with painless, progressive, proptosis and diminution of vision in the right eye of 30 years duration. Examination of the right eye (OD) revealed (A) no light perception, proptosis with inferior displacement of the globe, a dilated and fixed pupil, and pallor of the optic disc. (B) Computed tomography of the orbit demonstrated an osteolytic lesion centered in the frontal process of zygomatic bone. On biopsy the lesion showed (C) epithelial cells in a cord-like arrangement with small, round, nuclei and abundant vacuolated cytoplasm.