A 53-year-old woman presented with a 1-month history of decreased vision and progressive left periorbital fullness. Exam and radiography confirmed a left lacrimal gland mass causing mass effect on the globe and a compressive optic neuropathy. Orbitotomy with excisional biopsy confirmed carcinoma ex pleomorphic adenoma with epithelial and myoepithelial differentiation. Following a negative systemic workup, the patient underwent radiation therapy and remains free of clinical or radiographic disease 24 months following surgery. Herein the presentation, radiography, histopathology, and treatment outcome of a rare case of lacrimal gland epithelial-myoepithelial carcinoma ex pleomorphic adenoma is described. (C) 2016 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.
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