title: Primary orbital Ewing sarcoma.
abstract: The authors report the clinical manifestations, pathologic character, management, and prognosis of primary orbital Ewing sarcoma, and review pertinent literature. A 6-year-old boy presented with the complaint of abruptly decreasing visual acuity and right proptosis. Computed tomography scan revealed a mass in the region of the right infratemporal orbit with extension into the infratemporal fossa and masseteric space, and there was erosion of the right lateral orbital wall. An anterior orbitotomy eyelid crease was performed to remove the tumor. The tumor cells were positive in membranous pattern for CD99 immunostaining; all other reactions were negative. The EWS-FLI-1 fusion gene was not detected by reverse-transcription polymerase chain reaction. Combined radiotherapy was applied. Primary orbital Ewing sarcoma is an extremely rare malignant tumor that shows a predilection for males (ratio: 1.4 to 1). Appropriate treatment consists of local resection, radiotherapy, and adjunctive chemotherapy.
authors.affiliation: Southwest Hospital, Southwest Eye Hospital, Third Military Medical University, No. 30 Gaotanyan Main Street, Shapingba District, Chongqing 400038, China. yangyuli1979@yahoo.com.cn
authors.forename: Yuli
authors.initials: Y
authors.lastname: Yang
authors.forename: Yong
authors.initials: Y
authors.lastname: Liu
authors.forename: Zhengqin
authors.initials: Z
authors.lastname: Yin
mesh_terms: D000071443:12E7 Antigen; D015703:Antigens, CD / Q000378:metabolism; D000970:Antineoplastic Agents / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000378:metabolism; D015815:Cell Adhesion Molecules / Q000378:metabolism; D002648:Child; D003131:Combined Modality Therapy; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D013508:Ophthalmologic Surgical Procedures; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D016133:Polymerase Chain Reaction; D051789:Proto-Oncogene Protein c-fli-1 / Q000235:genetics; D034802:RNA-Binding Protein EWS / Q000235:genetics; D011878:Radiotherapy; D012512:Sarcoma, Ewing / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed; D014792:Visual Acuity
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS