Case ReportsOrbital Nerve Sheath Myxoma: A Case ReportSánchez-Orgaz, Margarita M.D., Ph.D.*; Grabowska, Anna M.D.*; Arbizu-Duralde, Álvaro M.D.*; Romero-Martín, Ricardo M.D.*; Patrón, Mercedes M.D., Ph.D.†; Diéguez, Elisa M.D.‡; García-Cabezas, Miguel A. M.D., Ph.D.† Author Information *Service of Ophthalmology, University Hospital “La Paz,” Madrid; †Department of Anatomic Pathology, University Hospital “La Paz,” Madrid; and ‡Service of Radiology, Clínica Nuestra Señora de América, Madrid, Spain Accepted for publication July 5, 2010. Address correspondence and reprint requests to Margarita Sánchez-Orgaz, M.D., Ph.D., Service of Ophthalmology, University Hospital “La Paz,” Paseo de la Castellana 261, 28046 Madrid, Spain. E-mail: [email protected] Ophthalmic Plastic & Reconstructive Surgery: July 2011 - Volume 27 - Issue 4 - p e106-e108 doi: 10.1097/IOP.0b013e3181f29e74 Buy Metrics Abstract A 72-year-old man was referred to the Service of Ophthalmology due to a 2-year history of ptosis and a painless mass in the lateral orbital margin of the right eye. Orbital MRI revealed a well-demarcated lesion in the superotemporal quadrant of the orbit. After surgical excision, histopathological examination led to the diagnosis of nerve sheath myxoma, a tumor composed of myxoid nodules separated by fibrous septa with spindle-shaped and stellate cells. Many of these cells were immunostained with antibodies to S-100 protein. This is the first case reported in the literature of such a tumor located in the orbit, and, though extremely rare, it should be considered in the differential diagnosis of orbital tumors. ©2011The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.