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In the World Health Organization classification system, mucinous tubular and spindle cell carcinoma of the kidney (MTSCC-K) has been described as a rare pathologic subtype of renal cell cancer. This tumor is supposed to have good prognosis, though follow-up data are limited due to rarity of tumor. MTSCC-K is a rare variant of renal cell carcinoma with female predominance and favorable prognosis. We report our experience with a case of mucinous tubular spindle cell carcinoma involving the kidney. A 61-year-old female presented with a palpable left renal lump of 12 cm in size. Computed axial tomography scan revealed a well-circumscribed mass of 11 cm involving the lower pole of the left kidney. She underwent left open radical nephrectomy. The cut section revealed a well-circumscribed tumor with yellowish-white variegated areas measuring 11 cm × 8 cm × 7 cm. Histopathological diagnosis of mucinous tubular spindle cell carcinoma of the kidney was given, and the patient responded well after the treatment. An accurate diagnosis may have an impact on prognostication of tumor and understanding the biology of the tumor. Thus, the clinicians should be aware of this rare disease for prompt diagnosis.

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