title: Primary extraosseous Ewing sarcoma of the orbit.
abstract: A 40-year-old man presented with painless, progressive vision loss and mild proptosis of the OD. CT revealed a right intraconal mass with slight penetration of the optic canal not contiguous with any bony structure. Incisional biopsy through a transfrontal orbitotomy revealed a diffuse growth of homogeneous, small, round cells. Immunohistochemical stains were positive for vimentin and MIC2 (CD99), and the translocation at EWS gene (22q12) was detected. Metastatic workup and a full-body bone scan were negative, confirming primary orbital extraosseous Ewing sarcoma. The patient received neoadjuvant chemotherapy and an orbital exenteration with preservation of eyelids and conjunctiva. He also received adjuvant chemotherapy and local radiotherapy, and he has remained disease-free for almost 3 years.
authors.affiliation: Oculoplastic and Orbital Surgery Unit, Ophthalmology Department, Ramón y Cajal University Hospital, Madrid, Spain.
authors.forename: Jorge L
authors.initials: JL
authors.lastname: Alio
authors.forename: Marco
authors.initials: M
authors.lastname: Sales-Sanz
authors.forename: Maria A
authors.initials: MA
authors.lastname: Vaz
authors.forename: Constanza
authors.initials: C
authors.lastname: Barrancos
authors.forename: Maria E
authors.initials: ME
authors.lastname: Reguero
authors.forename: Jorge
authors.initials: J
authors.lastname: Diamantopoulus
authors.forename: Pedro
authors.initials: P
authors.lastname: Poveda
mesh_terms: D000071443:12E7 Antigen; D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D014408:Biomarkers, Tumor / Q000378:metabolism; D015815:Cell Adhesion Molecules / Q000378:metabolism; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D003131:Combined Modality Therapy; D005094:Exophthalmos / Q000175:diagnosis; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D020360:Neoadjuvant Therapy; D017233:Orbit Evisceration; D009918:Orbital Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D019651:Plastic Surgery Procedures; D012512:Sarcoma, Ewing / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed; D014178:Translocation, Genetic; D014746:Vimentin / Q000378:metabolism; D014786:Vision Disorders / Q000175:diagnosis
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; D014408:Biomarkers, Tumor; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D014746:Vimentin