In terms of molecular characterization, common gene mutations in PEComa included TP53 (47%), ATRX (32%) and MSH3 (17%), in addition to 11% and 29% mutation rates in TSC1 and TSC2 genes, respectively, and the others were fusion of transcription factor E3 (TFE3), rearrangement of RAD51 B gene (14). Here, MSH3 is linked to neoplasm with perivascular epithelioid cell differentiation.