| Summary: | Ewing sarcoma is a malignant tumor that more commonly affects the long bones. Primary Ewing sarcoma of the adrenal gland is a rare diagnosis. We report an unusual case of primary Ewing sarcoma of the adrenal gland in a 34-year-old man who initially presented with abdominal as well as flank pain and abdominal mass. Computed tomography and magnetic resonance imaging showed a heterogeneous, hemorrhagic right adrenal mass with inferior vena cava extension and thrombosis. Thus, a major open surgery including right adrenalectomy, right nephrectomy, inferior vena cava thrombectomy with resection, and a retroperitoneal lymph node dissection was necessitated. Early recognition with radiological imaging and treatment of extraosseous Ewing sarcoma of the adrenal gland with a multimodality approach reduces morbidity and mortality. Keywords: Ewing's sarcoma, Primitive neuroectodermal tumor, Abdominal mass
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