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
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Article info
Footnotes
This work was supported in part by NIH RO1 CA95785 (to ML).
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© 2005 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
