Mm. Walsh et al., EPITHELIOID HEMANGIOENDOTHELIOMA OF THE LIVER MIMICKING BUDD-CHIARI-SYNDROME, Archives of pathology and laboratory medicine, 122(9), 1998, pp. 846-848
Citations number
13
Categorie Soggetti
Pathology,"Medical Laboratory Technology","Medicine, Research & Experimental
A case of epithelioid hemangioendothelioma of the liver in a 34-year-o
ld man with clinical and radiologic findings suggestive of Budd-Chiari
syndrome is reported. Despite clinical and radiologic findings, percu
taneous liver biopsy was suspicious for epithelioid hemangioendothelio
ma. The patient underwent liver transplantation 2 months later, and hi
stologic examination confirmed this diagnosis. Unusual histopathologic
features included extensive areas of capillary-thin vascular structur
es with open lumina, lack of significant cytologic atypia in the major
ity of neoplastic cells, and areas with Budd-Chiari-like features in t
he hepatic parenchyma surrounding the tumor. The neoplastic cells were
focally immunopositive for endothelial markers, such as factor VIII-r
elated antigen and CD34 antigen. The unusual clinical presentation may
have been due to tumor invasion and fibrous obliteration of terminal
hepatic venules and sublobular veins. Epithelioid hemangioendothelioma
should be considered when evaluating patients with clinical features
of Budd-Chiari syndrome or veno-occlusive disease.