Fatal epithelioid haemangioendothelioma presenting in the lung and liver

Abstract

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.

Epithelioid hemangioendothelioma of the liver: report of two cases

“…This difficulty can be resolved by studying vimentin and cytokeratin, proteins of the intermediate filaments of the mesenchymal and epithelial cells, respectively. Vimentin has been detected in cases of EHE (5,18), as occurred in our Case 1, while keratin is negative (5,15,18). As such, the use of UEA-I associated with vimentin and cytokeratin can be helpful in recognizing these sclerosing hepatic tumors, which can be F VIII R:Ag negative or produce results difficult to interpret.…”

“…F VIII R:Ag and UEA-I are endothelial markers that have been extensively studied in diverse tissues and tumors (13,14). F VIII R:Ag has been detected in EHE in different localisations 4, 7, 15), inluding the liver, although negative cases exist (5,15). The positivity of this antigen has been observed to be sharply reduced in the most sclerotic areas of the tumor (2), which can hinder its recognition in hepatic needle biopsy material when fibrous tissue is extracted.…”

Epithelioid hemangioendothelioma of the liver

“…Immunoreactivity of EHE for cytokeratin is controversial. Some studies did not show cytokeratin positivity, 7,10,20,75,81,82 but other studies have demonstrated coexpression of endothelial and epithelial markers, mainly in EHE located in bone. 57,58,72,73,83 In our study 14% of cases were positive for cytokeratin.…”

Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology

“…However, in a few patients, a rapidly progressive course with fatal outcome has been reported. [ 10 11 ]…”