TFE3 is highly sensitive for TFE3-rearranged RCC (positive in 95% of cases), though it may also be overexpressed in other tumor types, such as epithelioid angiomyolipoma, paragangliomas, melanomas, perivascular epithelioid cell tumors, and adrenocortical carcinomas [13,14,15,16,17,18]. This evidence concerns the gene TFE3 and adrenal cortex carcinoma.