Copyright ©The Author(s) 2021.
World J Gastrointest Oncol. Apr 15, 2021; 13(4): 223-230
Published online Apr 15, 2021. doi: 10.4251/wjgo.v13.i4.223
Table 1 Clinicopathologic characteristics of pediatric hepatic angiosarcoma and epithelioid hemangioendothelioma
AngiosarcomaEpithelioid hemangioendothelioma
DemographicsFemale predominance; mean age at diagnosis: 40 moFemale predominance; median age at diagnosis: 12 yr
Clinical presentationAbdominal pain, and distension due to rapid liver enlargement. Lung metastasis is commonMostly present with multiorgan involvement, most frequently involving liver, lungs, bone and soft tissue
Associated conditionsCutaneous and hepatic infantile hemangiomas, cutaneous vascular malformations, dyskeratosis congenitaNone reported
MolecularUnknown (ROS1-GOPC fusion reported in an adult hepatic angiosarcoma)[29]WWTR1-CAMTA1 fusion (90%) YAP1-TFE3 fusion (10%)
HistologyWell-formed, anastomosing vessels to more solid areas composed of pleomorphic spindle cells. Hypercellular whorls of spindled cells (glomeruloid bodies). Intracytoplasmic eosinophilic globules (periodic acid-Schiff -positive and diastase-resistant)Cords and nests of epithelioid cells in a variable fibromyxoid stroma. Occasional intracytoplasmic vacuoles/lumina. Minimal cytologic atypia with low mitotic rates
ImmunohistochemistryCD31, CD34, factor VIII, FLI-1, ERG, occasionally podoplanin and GLUT-1CD31, CD34, factor VIII, FLI-1, ERG, variable podoplanin, smooth muscle actin and keratin