| Summary: | Introduction: Renal cell carcinoma (RCC) is a heterogeneous group of cancers more and more distinguished due to the advance of diagnostic methods. Among these cancers, MiT (microphtalmia-associated transcriptional factor) translocation RCCs are rare tumors usually found in young persons, as exemplified by this case report. Case report: 15-year-old male patient underwent investigation of a complex cyst in his left kidney incidentally found by ultrasound and exhibiting features suggestive of malignancy at computerized tomography. Radical ipsilateral nephrectomy and retroperitoneal lymphadenectomy were then performed. Both results from histopathological analysis and immunohistochemistry confirmed that such lesion was a rare case of MiT family/transcription factor E3 (TFE3) translocation RCCs. Conclusion: Proper differentiation of RCCs related to translocation of MiT family requires immunostaining for TFE3, especially in the case of younger patients, among which the prevalence of this genetic mutation is frequent. Keywords: Renal cell carcinoma, TFE3 gene, Translocation of MiT family genes, WHO classification of renal cancer
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