PEComas typically display a myomelanocytic phenotype with expression of melanocytic markers, such as HMB-45, melan-A, and microphthalmia-associated transcription factor (MiTF), as well as myoid markers, such as smooth muscle actin (SMA) and caldesmon [3, 7]. The gene discussed is MITF; the disease is neoplasm with perivascular epithelioid cell differentiation.