The topoisomerase II (TOP2) poison has been reported to be a causal agent of this type of RCC, for patients could have a history of TOP2 poisons (etoposide or doxorubicin) use before diagnosed with Xp11.2 tRCC [13–15]. This evidence concerns the gene TOP2A and renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.