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Wegert J et al. – Wilms tumor (WT) is one of the most common solid tumors in childhood. Mutations in WT1 and CTNNB1 are well established as causal alterations in about 10–15% of cases. Recently, WTX (WT gene on the X–chromosome), a gene implicated in WNT signaling, has been identified as a third WT gene. WTX alteration is not an essential and early mutation needed to drive tumorigenesis, but rather a later event that may affect only a fraction of cells with unclear clinical relevance.

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