Clinicopathologic features of epithelioid haemangioendothelioma：6 cases report

LI Lan; TIAN Meng-meng; SUN Xiao-qi; DING Yi; HUANG Xiao-yuan

doi:10.3969/j.issn.2095-252X.2015.07.010

Abstract：Objective To explore the clinicopathologic, immunohistochemical features and differential diagnosis of epithelioid haemangioendothelioma ( EHE ).Methods Six cases of EHE were investigated by immunohistochemistry, and clinical and pathologic features were analyzed.Results Four of the 6 cases were male and the other 2 were female. Four cases presented as solitary lesions, locating in the right acetabulum, the eighth thoracic vertebra, the distal right femur and the sacrum repectively. Two cases presented as multifocal, one of which affected the left acetabulum and the both proximal femur, the other one affected 3 sites of the left proximal femur. The size of the tumor was 1.5-6.0 cm. One case who had a solitary lesion in the right acetabulum recurred 24 months after local excision. Three cases were alive without recurrence after 1, 30 and 31 months’ follow-up. Follow-up was lost in the other two cases. The tumors were composed of epithelioid endothelial cells in a hyalinized, chondroid, or myxoid stroma. There were intracytoplastic lumens ( vacuoles ), but no well-formed vessels. The tumor cells expressed positive at least two of endothelial markers ( include CD31, CD34, FLI-1, and Factor VIII ) ( 6 / 6 ). Local expressed CK ( 5 / 6 ), expression of SMA showed dense minute blood formation. The Ki-67 index was 3%-25%.Conclusions EHE of bone is a rare type of low-to intermediate-grade malignant angiomatous tumors with distinct clinicopathologic features. Typical manifestation: epithelioid endothelial cells were arranged in cords or small nests distributing in hyaline or myxoid stroma. Immunohistochemistry of endothelial markers may be useful for the diagnosis and differential diagnosis.