Immunohistochemically, PEComas are positive for both smooth muscle markers (e.g., SMA, h-caldesmon, desmin) and melanocytic markers (e.g., HMB45, melan-A, tyrosinase, MITF) [224,225,226] (Figure 7c,d). Here, DES is linked to neoplasm with perivascular epithelioid cell differentiation.