Transcription factor E3 (TFE3) belongs to the basic helix-loophelix zipper transcription factor family. The TFE3 gene is located on the short arm of the X chromosome, is expressed in various cells of the human body, and participates in the regulation of various genes. The rearrangement of this gene is associated with a variety of tumors, and it is highly expressed in acinar soft tissue sarcoma with TFE3 fusion gene, Xp11.2 translocation/TFE3 gene fusion-related renal cell carcinoma, and in perivascular epithelioid cell tumors.