摘要
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 clinicopathologic features of hepatic epithelioid hemangioendothelioma（EHE）. Method The clinical and histopathologic features, and follow-up data of 9 patients with hepatic EHE were analyzed retrospectively. Results ① There were 4 males and 5 females in 9 patients with hepatic EHE. The age was from 25 years to 69 years. ② Five 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. ④ Histologically, 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. ⑤ Immunohistochemical stain showed that all the tumors were positive for endothelial markers（factor Ⅷ-related antigen, CD34, CD31, and so on）. Conclusions EHE 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.
出处
《中国普外基础与临床杂志》
CAS
2015年第2期224-229,共6页
Chinese Journal of Bases and Clinics In General Surgery
