A 42-year-old man presented with visual field loss in the right eye and unintentional weight loss associated with a right orbital mass. Surgical resection and biopsy revealed a diagnosis of Ewing sarcoma, and systemic evaluation showed no metastasis. Subsequent chemotherapy and radiation resulted in tumor resolution without recurrence for 3.5 years. Although systemic symptoms are associated with a poorer prognosis, longer survival may be possible in the absence of metastasis.
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