Key result
Local resection and combined radiotherapy treat extremely rare primary orbital Ewing sarcoma in a pediatric patient.
Case Report (n=1)
Primary orbital Ewing sarcoma is a rare malignancy requiring a multimodal treatment approach including local resection, radiotherapy, and adjunctive chemotherapy.
Consider Ewing sarcoma in pediatric orbital masses with proptosis and erosion; extends sparse case literature but leaves management standardization open.
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.
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Yang et al. (2011) conducted a case report in Primary orbital Ewing sarcoma (n=1). Local resection and combined radiotherapy was evaluated. Local resection and combined radiotherapy were used to treat a 6-year-old boy with primary orbital Ewing sarcoma, an extremely rare tumor with a male predilection ratio of 1.4 to 1.
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