Extraskeletal Ewing sarcoma of the duodenum is a relatively uncommon entity tumor. We report a case of extraskeletal Ewing sarcoma in a 21-year-old woman. She complained of abdomen pain and melena. 18 F-FDG PET/CT showed an intense uptake by the duodenum mass along with multiple FDG-avid enlarged lymph nodes in the mesentery, which was pathologically proven as extraskeletal Ewing sarcoma.
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