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[目的]分析肝脏上皮样血管内皮细胞瘤(HEHE)的临床特征,提高对该病的诊断、治疗和转归的认识。[方法]对上海长海医院2011年2～12月间诊治的3例HEHE的影像学表现、组织病理学、免疫组化结果进行观察,结合文献讨论,详细分析该病的病因,临床影像学、病理特征,鉴别诊断、治疗及预后。[结果]该病病因不清,无特异性临床表现,影像学多表现为肝脏多发性占位,病理检查具有两个特征性的组织学表现,一是瘤组织主要由上皮样细胞和(或)树突状细胞组成,两者均可出现特征性的胞质内血管分化,即胞质内管腔形成,类似印戒细胞样,但腔内含红细胞,胞质内空泡呈黏液和PAS染色阴性;另一个特点是肿瘤含有大片黏液性或致密纤维性间质,与富细胞区形成明显对照,即肿瘤中央区为富含纤维的硬化区或少细胞区,纤维区发生透明变可类似软骨,瘤细胞萎缩,外周为富细胞区。免疫组化以CD31、CD34及Vimentin阳性率最高,第Ⅷ因子相关抗原FⅧRag阳性表达,少见表达CK及PNCA。目前尚无有效的治疗措施,预后介于良、恶性肿瘤之间。[结论]原发于肝脏的EHE是较为罕见的低度恶性肿瘤,熟悉其临床及病理特征对避免漏诊及误诊具有重要意义。主要依据组织病理学和免疫组化进行诊断。手术切除是主要的治疗手段。早期发现、局部手术扩大切除和结合术后放化疗或肝移植有助于延长患者生存期。
[Objective] To analyze the clinical features of hepatic epithelioid hemangioendothelioma (HEHE) and to improve the diagnosis, treatment and prognosis of the disease. [Methods] The imaging findings, histopathology and immunohistochemistry results of 3 cases of HEHE diagnosed and treated in Shanghai Changhai Hospital from January to December in 2011 were observed. Based on the literature review, the etiology, clinical imaging, Pathological features, differential diagnosis, treatment and prognosis. [Results] The etiology of this disease is unclear and there is no specific clinical manifestations. The imaging manifests as multiple liver space occupying lesions. The pathological examination has two characteristic histological manifestations. One is that the tumor mainly consists of epithelioid cells and / ) Dendritic cells, both characteristic cytoplasmic vascular differentiation may occur, that is, intracytoplasmic lumen, similar to the signet ring cell-like, but the cavity containing red blood cells, cytoplasmic vacuoles mucus and PAS Staining is negative; another characteristic is the tumor with large mucinous or dense fibrous interstitial, and the rich cell area formed a clear contrast, that is, the central tumor area is rich in fibrous sclerosis or less cytoplasmic zone, the transparent transflective fiber zone can be similar Cartilage, tumor cells atrophy, the periphery is rich in cells. The positive rate of CD31, CD34 and Vimentin was highest in immunohistochemistry, FⅧRag was the factor Ⅷ related antigen, and CK and PNCA were rare. There is no effective treatment, the prognosis is between good and malignant tumors. [Conclusion] EHE originating from the liver is a relatively rare low-grade malignant tumor. Being familiar with its clinical and pathological features is of great importance in avoiding misdiagnosis and missed diagnosis. Mainly based on histopathology and immunohistochemistry for diagnosis. Surgical resection is the main treatment. Early detection, local surgical resection and expansion combined with postoperative radiotherapy or chemotherapy and liver transplantation help to prolong the survival of patients.