The term perivascular epithelioid cell tumors [PEComa]s refers to a family of mesenchymal neoplasms composed of characteristic cells which usually express both melanocytic and myogenic markers such as human melanoma black [HMB] 45, human melanosome-associated antigen-1 [HMSA-1], MelanA/Mart1, microphthalmia transcription factor [MiTF], smooth muscle actin [SMA], pan-muscle actin, muscle myosin, calponin, sometimes h-caldesmon and, less commonly, desmin (Bonetti et al., 1997, Thway and Fisher, 2015). The gene discussed is SMN1; the disease is neoplasm with perivascular epithelioid cell differentiation.