Fatal epithelioid haemangioendothelioma presenting in the lung and liver

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Abstract
Summary 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 differentation. A short literature review is also presented demonstrating that the outlook of EHE is worse than previously thought.

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