Key result
An isolated para-aortic retroperitoneal renal cell carcinoma without a primary kidney tumor demonstrated TFE3 and TFEB positivity, suggesting it represents a rare form of translocation RCC.
Why the study?
Single case reports have described extra-renal RCC in different locations with no evidence of primary renal tumor, warranting further reporting and characterization of such rare presentations.
Case Report (n=1)
Extra-renal RCCs may represent rare forms of translocation RCCs associated with TFE3 and TFEB positivity.
Answers are grounded in this paper and cite their sources.
Extra-renal RCC merits consideration in retroperitoneal masses; this case extends TFE3/TFEB reports but remains hypothesis-generating.
We report a case of an isolated para-aortic retroperitoneal renal cell carcinoma (RCC) in the absence of a primary cancer in the kidney. Single case reports in the literature have described extra-renal RCC in different locations with no evidence of primary renal tumor. We present the initial presentation, diagnostic imaging, surgical treatment, and pathologic evaluation. Immunohistochemistry demonstrated positivity for TFE3 and TFEB, both of which are Microphthalmia associated transcription factors (MiT) associated with translocation RCCs. We hypothesize these few cases of extra-renal RCC represent rare forms of translocation RCC.
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Petrinec et al. (2021) conducted a case report in Extra-renal renal cell carcinoma (n=1). An isolated para-aortic retroperitoneal renal cell carcinoma without a primary kidney tumor demonstrated TFE3 and TFEB positivity, suggesting it represents a rare form of translocation RCC.
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