Ewing sarcoma of the small intestine

This report describes a rare case of Ewing sarcoma (ES) of the small intest ine. The patient was a 9-year-old girl with progressive abdominal distensio n. Computed tomography showed a large mass in the small bowel. Histopatholo gic examination of the resected tumor showed ES with typical histologic, im munohistochemical, and ultrastructural features. The tumor recurred in the pelvic cavity 18 months after the original surgery. Molecular study of the recurrent tumor confirmed a diagnostic EWS-FL11 gene fusion. This patient i llustrates the unique occurrence of ES in the small intestine.