Undifferentiated pleomorphic sarcoma (UPS) originating from the kidney is rare in clinical practice. Its symptoms are not obvious, and the auxiliary examination is non-specific. It mainly depends on pathomorphology and immunohistochemistry. The tumor has high malignancy, poor prognosis, and is insensitive to radiotherapy and chemotherapy. The treatment method is mainly early surgery. The diagnosis and treatment of this tumor requires the accumulation of cases and long-term observation and follow-up. A case of primary UPS of the kidney was reported.

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