case_images: PMC5362881_01
case_images: Right atrium showing severe hemorrhage (formalin-fixed sample)
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case_images: No abnormalities were detected in the shape of the heart (Figure 1), and no significant changes were apparent in the other organs examined.
case_images: Arterioles are partially to completely filled with plump spindle cells that often form glomerulus-like arrangements within the lumens. Haematoxylin and eosin (x400)
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case_images: Within the nests of spindle cells, slit-like, narrow channels were present, occasionally containing erythrocytes and eosinophilic microthrombi (Figure 2).
case_images: Many intraluminal spindle cells stained positive for von Willebrand factor (vWF) antigen, which indicates endothelial cell histogenesis. Microthrombi also stain positive for vWF. Immunohistochemistry (x 400)
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case_images: Immunohistochemical analysis revealed that many intraluminal proliferative spindle cells were positive for vWF (Figure 3).
case_images: Arteriolar smooth muscle and some intraluminal cells (pericytes) stain positive for smooth muscle actin(SMA). Immunohistochemistry (x 400)
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case_images: Intense smooth muscle actin (SMA) staining was observed on the periphery of the intraluminal proliferations (arteriolar smooth muscles), and on some intraluminal cells (pericytes; Figure 4).