In a 12-year-old male patient with stage IV TFE3-fusion-positive tRCC, treatment with cabozantinib demonstrated excellent disease control, including reduced pain just after 3 months and halted disease progression even 18 months later (Wedekind et al., 2017). This evidence concerns the gene TFE3 and renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.