A case of retroperitoneal fibrosarcoma is reported. A 52-year-old man with the complaint of left abdominal mass was referred to our hospital. Computed tomography showed a left retroperitoneal tumor surrounding the left kidney in the perirenal space. Angiography showed some feeding vessels from the branch of the posterior segmental artery of the left kidney. The tumor was completely resected with the left kidney, but para-aortic lymph node metestases were found. Histopathological diagnosis was a retroperitoneal fibrosarcoma with lymph node metastases. The patient died of the disease 3 months after the operation.
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