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目的总结肝脏上皮样血管内皮瘤(EHE)的临床病理特点。方法回顾性分析2012年8月至2014年9月期间四川大学华西医院确诊为肝脏EHE的9例患者的临床表现、病理组织学特点及随访结果。结果1 9例肝脏EHE患者中男4例,女5例;年龄25~69岁,平均39.11岁。2 5例表现为右上腹不适、乏力、消瘦等非特异性症状,4例无任何症状。3巨检见肿瘤表现为直径0.5~9.0 cm的多发结节。4镜下见肿瘤呈巢状、条索状排列,瘤细胞由梭形细胞、上皮样细胞、组织细胞样细胞或印戒样细胞构成,常形成胞质内血管,管腔内可见红细胞,肿瘤间质由黏液玻璃样变的纤维硬化区构成,瘤细胞浸润周围肝组织,并在脉管内形成乳头状或肾小球样结构。5免疫组织化学检测见肿瘤细胞FⅧ、CD34、CD31等内皮细胞表达阳性。结论肝脏EHE较少见,影像学和临床易误诊,其确诊依赖于病理组织学形态及免疫组织化学检查结果。
Objective To summarize the clinicopathological features of hepatic epithelioid hemangioendothelioma (EHE). Methods The clinical manifestations, histopathological features and follow-up results of 9 patients with liver EHE confirmed by West China Hospital of Sichuan University from August 2012 to September 2014 were retrospectively analyzed. Results Of the 19 patients with liver EHE, 4 were males and 5 were females, ranging in age from 25 to 69 years with an average of 39.11 years. Twenty-five cases showed nonspecific symptoms such as right upper quadrant discomfort, fatigue and weight loss, and no symptoms in 4 cases. 3 macroscopic tumor showed a diameter of 0.5 ~ 9.0 cm multiple nodules. 4, the tumor was seen in nests, arranged in the shape of a cord. The tumor cells consisted of spindle cells, epithelioid cells, tissue-like cells or signet-ring-like cells, often forming intracytoplasmic blood vessels, and red blood cells and tumors The stroma consists of mucinous glass-like fibrous sclerosis, which infiltrates surrounding liver tissue and forms papillary or glomerular-like structures within the vessel. 5 Immunohistochemical detection of tumor cells FⅧ, CD34, CD31 and other endothelial cells were positive. Conclusions Liver EHE is rare, imaging and clinical misdiagnosis, the diagnosis depends on the histopathological morphology and immunohistochemical findings.