Fatal epithelioid haemangioendothelioma presenting in the lung and liver.
Three patients with epithelioid haemangioendothelioma (EHE) are described. Two patients presented with pulmonary infiltrates and one with a hepatic tumour. All had a metastatic disease ending fatally, and all were autopsied. The diagnosis was confirmed either by immunohistological or ultrastructural analysis. All three tumours were cytokeratin-negative and vimentin-positive, while only two contained cells reacting with the antibody of factor VIII-related antigen. Electron microscopy of the third tumour revealed features indicating endothelial differentiation. A short literature review is also presented demonstrating that the outlook of EHE is worse than previously thought.
D000293:Adolescent; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006390:Hemangioendothelioma / Q000556:secondary*; D006651:Histocytochemistry; D006801:Humans; D008099:Liver / Q000473:pathology*; D008113:Liver Neoplasms / Q000556:secondary*; D008168:Lung / Q000473:pathology*; D008175:Lung Neoplasms / Q000556:secondary*; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009362:Neoplasm Metastasis