We report a case of mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney. A 68-years old female consulted a primary hospital with a chief complaint of back pain. Computed tomography revealed the tumor of the left kidney, so she was referred to our department. The tumor, 45 x 42 mm in length, was slightly enhanced, and that had well-defined margins. We performed radical nephrectomy. Pathological findings suggested MTSCC. MTSCC is a rare type of renal cell carcinoma composed of a combination of low-grade tubular cuboidal cells and spindle cells in a mucinous stroma. The immunohistochemistry is effective in its diagnosis.

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