Abstract

Ewing's sarcoma/Primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney. We re-port herein the clinical, histological, and immunohisto-chemical features of a primary renal ES/PNET. A 19-year old male referred a two weeks history of constant, colic, left flank pain, and fever. A left radical nephrectomy was performed. Gross pathologic examination showed pink-tan, lobulated solid tumor, localized at the superior pole. Histologically, the tumor was solid with necrosis. The neoplastic cells showed a small amount of clear cytoplasm, and had vesicular nuclei with small nucleoli. Immunohistochemical studies showed strongly and diffusely positive staining for CD99 in a membranous pattern. This case represents a typical ES/ PNET affecting a young male patient. Adequate diagnosis is important because this neoplasm has an aggressive behaviour.

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