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No progression of multilocular cystic renal cell carcinoma is known. We present the case of a year-old male, with an accidentally discovered renal tumor due to its unusual gross genetic cancer of the kidney and a rare clinical entity. He subsequently underwent a radical right nephrectomy and further histological examination revealed the tumor to be a multilocular cystic renal clear cell carcinoma, Fuhrman grade 2.

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Histopathological diagnosis was revalued reconsidering microscopic specimens consequent to analyzed cases. Results: In After microscopic examination, most cases were classified as clear cell renal cell carcinomas and urothelial carcinomas. For clear cell renal cell carcinoma and urothelial carcinomas we registered different percentages compared with data reported in literature