We evaluated and compared the therapeutic efficacy of sunitinib, sorafenib, and everolimus, which are known to be effective in treatments of Xp11.2 tRCC in addition to LY294002, the PI3K pathway inhibitor, which targets the overexpression of PIK3R2 in aggressive Xp11.2 tRCC patients. This evidence concerns the gene PIK3R2 and renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.