In our case, the presence of sinusoidal vascular channels lacking of red blood cells and containing amorphous Alcian blue positive substance, the marked cellular atypia, the presence of necrosis and numerous atypical mitoses, the high Ki67 expression and the positive immunostaining for CD31 and D2–40 were more consistent with the diagnosis of “epithelioid angiosarcoma with lymphatic differentiation”. The gene discussed is PECAM1; the disease is epithelioid hemangioendothelioma.