Abstract
First recognized as a distinct entity by the World Health Organization in 2004, Xp11 translocation renal cell carcinomas (TRCCs) are a subtype of renal malignancy characterized by translocations involving the splice site Xp11.2.1 de Jong et al2 and Tomlinson et al3 first described Xp11 translocation as a previously unknown finding in the karyotype of 2 atypical renal masses presenting in infant males. A subsequent series of studies using TFE3 immunoassays have shown that Xp11 TRCCs constitute ≤40% of pediatric renal cell carcinoma (RCC) cases.
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