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Clinicopathologic Reports, Case Reports, and Small Case Series
July 2003

Primary Orbital Extraskeletal Ewing Sarcoma

Arch Ophthalmol. 2003;121(7):1049-1052. doi:10.1001/archopht.121.7.1049

Extraskeletal Ewing sarcoma is a rare soft tissue sarcoma histologically indistinguishable from osseous Ewing sarcoma. Tefft et al1 first described the tumor in children with paravertebral soft tissue masses. Subsequent larger case series have shown that the tumor has a predilection for the paravertebral area and lower extremities.2 The tumor most often occurs in the extremities and paraspinal areas and has been reported to occur in the head and neck region in 5% to 11% of patients.3-5 While osseous Ewing sarcoma, both primary and metastatic, has been reported in the orbit, 6 to our knowledge, ours is the first case of extraskeletal Ewing sarcoma to occur in the orbit. Furthermore, extraskeletal Ewing sarcoma manifesting as late as the sixth decade of life has not been reported, to our knowledge. We report a case of primary orbital extraskeletal Ewing sarcoma in a 56-year-old man, which was diagnosed using monoclonal antibody 013 to the p30/32 MIC2 gene product, CD99.