Notably, it is a type of perivascular epithelioid cell tumor (PEComa), characterized by aberrant proliferation of smooth muscle-like cells that express melanocytic (HMB45 and Melan-A) and smooth muscle markers (smooth muscle actin, desmin, and caldesmon), as well as melanocyte inducing transcription factor/ Microphthalmia-Associated Transcription Factor (MITF) [7]. The gene discussed is DES; the disease is neoplasm with perivascular epithelioid cell differentiation.