We report a case of extraskeletal Ewing’s sarcoma arising in the retroperitoneal cavity. The tumor was excised en bloc with the left kidney and infiltrated portion of the left ureter. Histological diagnosis was confirmed by both electron micrograph and immunohistochemical staining using a newly developed mouse monoclonal antibody 5C11, which specifically reacts with Ewing’s sarcoma. An adjuvant chemotherapy T-11 regimen has been successfully preventing the recurrence.

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