A 51-year-old man presented with a 9-month history of an injected, painful right eye, along with right eye protrusion, diplopia, and reduced visual acuity. There was no history of trauma, and there were no other clinical manifestations in the head and neck region. The patient's personal and family medical histories were unremarkable.
Physical examination revealed proptosis of the right eye, with a subconjunctival mass over its medial superior aspect and engorged vessels on its sclera (Figure 1). Some polypoid, solid masses were found in the superior aspect of the right nasal cavity on sinoscopy. There were no enlarged neck lymph nodes, cranial nerve deficits, or other anomalies in the head and neck region. Laboratory tests showed a leukocyte count of 13 300/μL (to convert to ×109/L, multiply by 0.001), a normal erythrocyte sedimentation rate, and negative results for both rheumatic factor and antinuclear antigen. A chest x-ray film showed no abnormalities. Gadolinium-enhanced T1-weighted coronal magnetic resonance imaging (MRI), with fat saturation, through the orbits demonstrated an enhancing mass with an indistinct margin involving the superior intraconal and extraconal space of the right orbit (Figure 2). Computed tomography of the sinus showed a soft-tissue mass abutting the right ophthalmic vein, the thickened sclera, and the posteromedial aspect of right orbit. In the coronal section of the computed tomogram, the mass was observed in the superior aspect of the right orbit and the right ethmoid sinus, without aggressive bony destruction in the right orbital walls (Figure 3). A biopsy specimen was obtained from the polypoid tumor in the right nasal cavity. Pathologic analysis showed “emperipolesis” (Figure 4[hematoxylin-eosin, original magnification × 400]), and an immunohistochemical stain was positive for S-100 protein but negative for CD1a.