Synonyms: EOE | Extraosseous Ewing sarcoma | Extraosseous Ewing tumor | Extraskeletal Ewing tumor
Extraskeletal Ewing sarcoma is a rare poorly differentiated highly malignant soft tissue tumor derived from neuroectoderm that is morphologically indistinguishable from skeletal Ewing sarcoma but is located in extraosseous locations with the most common being: chest wall paravertebral region abdominopelvic area (with predilection for the retroperitoneal space) gluteal region and lower extremities. Clinical presentation is highly variable and depends on tumor localization. Local recurrence is common and metastatic disease most frequently involves the bones and lungs.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2026
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Extraskeletal Ewing sarcoma?
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