PEComas are characterized by immunoexpression of both myoid (desmin, SMA, muscle-specific-actin, muscle myosin, and calponin) and melanocytic (HMB-45, Melan-A/MART-1, tyrosinase, and MiTF) markers (Folpe et al., 2005, Conlon et al., 2015, Bennett et al., 2018, Bonetti et al., 1997, Thway and Fisher, 2015). The gene discussed is SMN1; the disease is neoplasm with perivascular epithelioid cell differentiation.