Open AccessRenal Cancer Without Primary Cancer in the Kidney: Extra-Renal TFE3 Translocation Associated Renal Cell Carcinoma
Author(s) -
Benjamin Petrinec,
Bryan Morales Vargas,
Lara R. Harik,
Viraj A. Master
Publication year - 2021
Publication title -
kidney cancer
Language(s) - English
Resource type - Journals
eISSN - 2468-4570
pISSN - 2468-4562
DOI - 10.3233/kca-200102
Subject(s) - tfe3 , tfeb , renal cell carcinoma , chromosomal translocation , kidney , medicine , pathology , kidney cancer , cancer , immunohistochemistry , biology , transcription factor , biochemistry , enhancer , gene
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.