Pediatric vascular tumors of the liver: Review from the pathologist’s point of view

Figure 1  Histologic classification of hepatic hemangioendothelioma.

Figure 2 Hepatic congenital hemangioma. A: A relatively well-demarcated vascular lesion; B: Lobules of variable sized, mostly small thin-walled vascular spaces and more abundant larger vessels at the periphery; C: Necrotic and hemorrhagic areas in the central part (area of involution); D: Entrapment of hepatocytes and bile ducts in interface areas.

Figure 3 Hepatic congenital hemangioma. A: Erythroblast transformation-specific-related gene expression of endothelial cells; B: No GLUT1 expression of endothelial cells.

Figure 4 Hepatic infantile hemangioma. A: A well-demarcated vascular lesion; B: Lobular, small-sized vascular spaces.

Figure 5 Hepatic infantile hemangioma. A: Erythroblast transformation-specific-related gene expression of endothelial cells; B: Smooth muscle actin expression of the pericytic cells; C: GLUT1 expression of endothelial cells.

Figure 6 Hepatic epithelioid hemangioendothelioma. A: Nests, cords, strands and single infiltrative epithelioid cells with intracytoplasmic vacuoles; B: Nests, cords, strands and single infiltrative epithelioid cells with intracytoplasmic vacuoles; C: Nuclear calmodulin-binding transcription activator1 (CAMTA1) expression; D: Nuclear CAMTA 1 expression.

Figure 7  Hepatic angiosarcoma, macroscopical features.

Figure 8 Hepatic angiosarcoma. A: Unencapsulated vascular lesion; B: Infiltrative growth pattern; C: Anastomosing vascular spaces lined by endothelial cells with marked cytological atypia and multilayering. D: Anastomosing vascular spaces lined by endothelial cells with marked cytological atypia and multilayering.

Figure 9 Hepatic angiosarcoma. A: CD31 expression of the endothelial cells; B: Erythroblast transformation-specific-related gene expression of the endothelial cells.

Figure 10  Overview pediatric vascular tumors of the liver and their immunohistochemistry. ¹Positive immunohistochemical staining； ²Negative immunohistochemical staining； ³Occasionally positive immunohistochemical staining. WT-1: Wilms’ tumor 1; FVIII: factor VIII; ERG: Erythroblast transformation-specific-related gene; GLUT-1: glucose transporter-1; D2-40: podoplanin; CAMTA1: calmodulin-binding transcription activator1; TFE3: transcription factor E3; CK8-18: cytokeratin 8-18.