Objective: We present a case of clear cell sarcoma of the kidney (CCSK) in 53-year-old white man who was treated with surgery. This case represents the oldest patient with CCSK published in the English literature. Methods: Right radical nephrectomy with lymphadenectomy and thrombectomy was performed. Results: Histological findings indicated a CCSK. Tumor cells showed positive vimentin staining. Conclusion: CCSK is considered a rare and highly malignant renal tumor. The malignant nature may relate not only to the biological features of these tumor cells, but also to the high resistance against radiation and chemotherapy. The treatment of CCSK has been a subject of controversy.
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