An otherwise healthy 55-year-old male presented with a history of 18 months of tearing in the right eye and progressive periocular swelling near his right brow. There was no eye pain, vision loss, or diplopia. Examination revealed best corrected visual acuity of 20/20 in both eyes with normal intraocular pressure, motility, and pupils. External exam showed proptosis and hypoglobus on the right and an elevated right upper eyelid crease. Hertel exophthalmometer measurements were 23mm on the right and 18mm on the left. Slit lamp exam was notable for mild conjunctival injection on the right but was otherwise normal and dilated fundus exam was normal.
Images from biopsy of the lesion (hematoxylin and eosin stain):
Immunostaining positive for SMA, p63, calponin, and CK5/6.
low magnification (left photo)
high magnification (right photo)