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| Primary orbital Ewing’s Sarcoma: report of a case and review of the literature |
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| 作者：Yang Yul… 文章来源：Department of Ophthalmology, West China Hospital, Sichuan University, Chengdu, 610041 Sichuan,China 点击数1345 更新时间：2006/6/28 22:35:06 文章录入：杨真2006 责任编辑：毛进 |
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| Objective: Primary orbital Ewing’s sarcoma is a very rare condition. Since the first case was reported in 1950, only 17 other cases have been reported in the English literature and 2 cases in the chinese literature. To analyse the clinic manifestations, pathologic characters, managements and prognosis of primary orbital Ewing’s sarcoma. Methods: A case of primary orbital Ewing’s sarcoma whose ophthalmologic examinations, computed tomography scan of the orbit, Chest radiogragh, radiograghs of the long bones, abdominal ultrasound, immunohistochemical and cytogenic studies were reported, and its pertinent literature was reviewed. Results: 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 inferotemporal orbit with extension into the infratemporal fossa and masseteric space and there was erosion of the right lateral orbital wall. Chest radiogragh and radiograghs of the long bones were normal, and abdominal ultrasound revealed no organ involvement. A anterior orbitotomy lid-crease was performed to remove the tumor. Regarding to immunostaining, the tumor cells were strongly and diffusely positive in membranous pattern for CD99 immunostaining. All other reactions were negative. EWS-FLI-1 fusion gene was detected by RT-PCR. Combined chemotherapy and radiotherapy are now applied. Conclusion: Primary orbital Ewing’s sarcoma is extremely rare malignant tumor. Nearly 80% of patients are younger than 20 years. And it shows a predilection for males with the ratio of 1.4 to 1. The small number of cases reported makes evaluation of the different therapeutic modalities difficult. Now it is known to all, appropriate treatment consists of local resection, radiotherapy, and adjunctive chemotherapy. |
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