In recent years, molecular detection studies have revealed that malignant PEComa is often accompanied by mutational inactivation of TSC1 and/or TSC2 genes, leading to activation of the mammalian target of rapamycin (mTOR) pathway (3) and partially carries TFE3 gene fusion. Here, TFE3 is linked to neoplasm with perivascular epithelioid cell differentiation.