A 76-year-old female referred by primary care physician for right eye proptosis. The patient complained of diplopia for the last six months without associated pain or irritation. External exam was significant for proptosis of the right eye and hypoglobus. Visual acuity at presentation was 20/30+1 and 20/25-1 of the right and left eye respectively. The pupils were noted to be round and reactive without APD. Patient had full extraocular movements and visual field. Hertel showed a 3 mm proptosis of the right eye compared to left at a base of 97. Slit lamp exam was largely unremarkable. CT scan showed a heterogenous mass of the right superolateral orbit with calcification. The patient then underwent orbitotomy via a temporal lid crease incision and the mass was removed in toto and the capsule was noted to be intact. Pathology was significant for carcinoma ex pleomorphic adenoma of the mucoepidermoid subtype. A review of literature revealed that carcinoma ex pleomorphic adenoma is a rare entity of the lacrimal gland without a standardized treatment. Drawing from the experience of the otolaryngology literature, the accepted treatment for similar tumor is complete surgical excision followed by radiation. However, recent study shows that chemotherapy may also be employed as an adjuvant treatment in addition to surgical excision and radiation. Given the rarity of this tumor, more study is still needed to optimize treatment and management.
Presentation Date: 08/27/2020
Issue Date: 02/05/2021