上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)是一种罕见的血管源性肿瘤,首先由Weiss及Enzinger于1982年报道并命名。目前病因尚不清楚,临床症状及体征亦无特异性,诊断需依靠病理学,尤其是免疫组织化学检查。现就上皮样血管内皮瘤的临床病理特点及诊治进展进行综述。1 临床特征EHE是一种与血管内皮增生相关的病变,组织学表现特殊,具有上皮样或组织细胞样形态,可发生转移。2002年WHO《软组织与骨肿瘤病理学和 Epithelioid hemangioendothelioma (EHE) is a rare vasculogenic tumor that was first reported and named by Weiss and Enzinger in 1982. The current etiology is not yet clear, and no specific clinical signs and symptoms, diagnosis depends on the pathology, especially immunohistochemistry. The epithelioid hemangioendothelioma is the clinicopathological features and diagnosis and treatment progress are reviewed. 1 Clinical features EHE is a vascular endothelial dysplasia-related lesions, histological manifestations, with epithelial or tissue-like morphology, the occurrence of metastasis. 2002 WHO "Soft tissue and bone tumor pathology and