Epithelial–Myoepithelial Carcinoma Presenting as a Pseudo Veno-Lymphatic Malformation

A 41-year-old woman presented with several days of right eye pain and blurred vision. Examination and radiologic workup were consistent with a veno-lymphatic malformation, including demonstration of cystic fluid–fluid levels on imaging. Histopathology supported the presence of cystic spaces but revealed the final diagnosis of lacrimal epithelial–myoepithelial carcinoma, a rare neoplasm typically associated with the salivary gland. This represents the first reported case of lacrimal epithelial–myoepithelial carcinoma presenting with pain and evidence of intralesional cysts on radiographic and histopathologic evaluation. Better characterization will enable recognition, complete surgical excision, and potential adjuvant radiation therapy. Accepted for publication June 5, 2018. The authors have no financial or conflicts of interest to disclose. Supported by the National Institutes of Health Research Grant CA 16359 from the National Cancer Institute. Presented at the New York Orbital Society Spring Meeting, New York, NY on April 18, 2018. Address correspondence and reprint requests to Emily Li, M.D., 40 Temple Street, Suite 3D, New Haven, CT 06510. E-mail: emily.li@yale.edu © 2018 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.