The gene rearrangement of the MIT/TFE family of transcription factors member TFE3 (chromosome Xp11.23) has been reported in a small minority of PEComas, including SFPQ/PSF-TFE3 fusion and DVL2-TFE3 fusion (14,15). Here, DVL2 is linked to neoplasm with perivascular epithelioid cell differentiation.