{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T00:07:29Z","timestamp":1792714049729,"version":"4.5.0"},"reference-count":64,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,11,3]],"date-time":"2021-11-03T00:00:00Z","timestamp":1635897600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp; Cancer"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The MiT subfamily of transcription factors includes TFE3, TFEB, TFEC, and MITF. Gene fusions involving two of these transcription factors have been well\u2010characterized in renal cell carcinoma (RCC). The <jats:italic>TFE3<\/jats:italic>\u2010rearranged RCC (also known as Xp11 translocation RCC) was first officially recognized in the 2004 World Health Organization (WHO) renal tumor classification. The <jats:italic>TFEB<\/jats:italic>\u2010rearranged RCC, which typically harbor a <jats:italic>t<\/jats:italic>(6;11)(p21;q12) translocation which results in a <jats:italic>MALAT1\u2010TFEB<\/jats:italic> gene fusion, were first officially recognized in the 2016 WHO renal tumor classification. These two subtypes of translocation RCC have many similarities. Both disproportionately involve young patients, although adult translocation RCC overall outnumber pediatric cases. Both often have unusual and distinctive morphologies; the <jats:italic>TFE3<\/jats:italic>\u2010rearranged RCCs frequently have clear cells with papillary architecture and abundant psammoma bodies, while the <jats:italic>TFEB<\/jats:italic>\u2010rearranged RCCs frequently have a biphasic appearance with both small and large epithelioid cells and nodules of basement membrane material. However, the morphology of these two neoplasms can overlap, with one mimicking the other or other more common renal neoplasms. Both of these RCC underexpress epithelial immunohistochemical markers, such as cytokeratin and epithelial membrane antigen, relative to most other RCC. Unlike other RCC, both frequently express the cysteine protease cathepsin k and often express melanocytic markers like HMB45 and Melan A. Finally, TFE3 and TFEB have overlapping functional activity as these two transcription factors frequently heterodimerize and bind to the same targets. Therefore, these two neoplasms are now grouped together under the heading of \u201cMiT family translocation RCC.\u201d Approximately 50 renal cell carcinomas with gene fusions involving the anaplastic lymphoma kinase (<jats:italic>ALK<\/jats:italic>) gene have now been reported. While those with a <jats:italic>Vinculin\u2010ALK<\/jats:italic> fusion have distinctive features (predilection to affect children with sickle cell trait and to show solid architecture with striking cytoplasmic vacuolization), other <jats:italic>ALK<\/jats:italic>\u2010fusion RCCs have more varied clinical presentations and pathologic features. This review summarizes our current knowledge of these recently described RCC.<\/jats:p>","DOI":"10.1002\/gcc.23007","type":"journal-article","created":{"date-parts":[[2021,10,27]],"date-time":"2021-10-27T09:09:18Z","timestamp":1635325758000},"page":"219-227","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":66,"title":["Translocation carcinomas of the kidney"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8797-9474","authenticated-orcid":false,"given":"Pedram","family":"Argani","sequence":"first","affiliation":[{"name":"Department of Pathology Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"Departments of Oncology Johns Hopkins University School of Medicine  Baltimore Maryland 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