ObjectiveTo summarize the clinicopathologic features of hepatic epithelioid hemangioendothelioma (EHE).
MethodThe clinical and histopathologic features, and follow-up data of 9 patients with hepatic EHE were analyzed retrospectively.
Results①There were 4 males and 5 females in 9 patients with hepatic EHE. The age was from 25 years to 69 years.②Five patients presented with nonspecific symptoms such as right upper quadrant pain, fatigue or weight loss. Four patients were asymptomatic.③Macroscopically, the tumors were usually multiple and ranged in size from 0.5 cm to 9.0 cm.④Histologically, the tumors appeared as cords or solid nets that were characterized by epithelioid, histiocytoid, spindle or signet-ring-like cells. The cells often contained vacuoles representing intracellular lumina, in which red blood cells could be seen. The neoplastic cells invaded the surrounding liver tissue and formed papillary or glomeruloid structure in vessels. The stroma was fibrous with myxohyaline areas.⑤Immunohistochemical stain showed that all the tumors were positive for endothelial markers (factorⅧ-related antigen, CD34, CD31, and so on).
ConclusionsEHE of liver is a very rare clinical entity. It might be easily misdiagnosed due to EHE without specific clinical and imaging changes. Its final diagnosis depends on pathological and immunohistochemical findings.
