Review Article| Volume 25, ISSUE 2, P363-378, June 2005

Translocation Carcinomas of the Kidney

      A subset of renal carcinomas that most commonly affect young patients has been characterized by various translocations that involve chromosome Xp11.2; all result in gene fusions that involve the TFE3 transcription factor gene. In the past few years, several different Xp11.2-translocation carcinomas have been identified and characterized at the morphologic and molecular levels. These include a distinctive renal carcinoma that bears a translocation with the identical breakpoints and identical resulting ASPL–TFE3 gene fusion as alveolar soft part sarcoma (ASPS), a rare pediatric soft tissue neoplasm of uncertain histogenesis. These Xp11.2 translocation carcinomas are now accepted as a distinctive entity in the 2004 World Health Organization's renal tumor classification [
      • Argani P.
      • Ladanyi M.
      Renal carcinomas associated with Xp11.2 translocations/TFE3 gene fusions.
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