Abstract
Renal cell carcinoma associated with Xp11.2 translocations/ TFE gene fusions (Xp11.2 RCC) has been classified as a distinct entity in the 2004 WHO classification of kidney tumors. Over the past seven years, aided by increased awareness, positive nuclear staining for TFE 3 , unique cytogenetic features, and modern molecular technology, more cases have been recognized as Xp11.2 RCC. This review summarizes the most recent advances in Xp11.2 RCC regarding to its clinical presentation, cytogenetic profile, histopathology , prognosis and treatment.
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