肝脏和肺内上皮样血管内皮细胞瘤的临床病理观察

Clinical and pathological characteristics of epithelioid hemangioendothelioma

目的探讨肝脏和肺内上皮样血管内皮细胞瘤(EHE)的临床病理特点。方法收集3例EHE患者的临床病理资料并复习相关文献,分析该病的临床病理特征。结果 3例患者均为女性,其中肺EHE 1例,肝脏EHE 2例。典型病理表现为脏器多发性、边界不清的小结节;瘤组织呈多结节状;瘤细胞呈卵圆形或圆形上皮样,胞浆丰富嗜酸性,间质黏液样变性或透明变性,胞浆内有大空泡,空泡内偶见红细胞。免疫组化染色示F8、CD31、CD34为阳性表达。结论 EHE是一种低度恶性血管源性肿瘤,易误诊,诊断主要依靠病理形态学和免疫组化染色。

Objective To study the clinical and pathological characteristics of epithelioid hemangioendothelioma（EHE） in lung and liver.Methods Clinical and pathological characteristics of three cases of EHE and the related literatures,the clinical manifestations,diagnosis,differential diagnosis,treatment and prognosis were analyzed.Results Three cases were female,2 cases located in liver,and the other in lung.The classical presentation was multiple ill-circumscribed nodules in organs.Histologically,the tumors were characterized by nodulous proliferation of oval to round epithelioid cells with abundant eosinophilic cytoplasm,embedded in a myxoid or hyaline matrix.A typical feature was the presence of large intracytoplasmic vacuoles in the tumor cells in which red blood cells were sometimes identified.The tumor cells were strongly positive for vascular endothelial markers including factor VⅢ,CD31 and CD34.There was no specific treatment for EHE and prognosis was unpredicatable.Conclusion Epithelioid hemangioendothelioma is a low grade malignant tumor derived from vascular endothelial cells and often misdiagnosed as other disease.The diagnosis is depended on the pathological features and immunohistiochemical stainings.