Small molecule tyrosinase inhibitors such as Sorafenib, Sunitinib and Pazopanib are multi-targeted inhibitors that exert anti-angiogenic effects by inhibiting VEGFR, PDGFR and other pathways, and have achieved partial remission in several PEComa patients (22, 23). Here, KDR is linked to neoplasm with perivascular epithelioid cell differentiation.