肝上皮样血管内皮瘤的诊断与治疗:附1例报告及文献复习

Diagnosis and treatment of hepatic epithelioid hemangioendothelioma:one case report and literature review

目的探讨肝上皮样血管内皮瘤(hepatic epithelioid haemangioendothelioma,HEHE)的临床诊断及治疗特点。方法报道天津市第一中心医院1例HEHE患者,标本经苏木素-伊红(HE)染色后光镜观察,同时采用En Vision免疫组化二步法检测CD31、CD34、Vim、CK19、AE1/AE3蛋白的表达情况,随访患者,结合其临床表现及病理形态并复习相关文献。结果切除该例HEHE患者的肝左外叶和肝右后叶肿块,切面可见5 cm×2 cm×2 cm,有灰白、灰红结节数数,边界不清,质稍硬。镜检穿刺组织、肿瘤细胞呈卵圆形或不规则形,排列成条索状,胞质丰富呈嗜酸性,部分区域可见片状坏死及钙化,肿瘤与周围肝组织的界限不清。免疫组化结果为肿瘤细胞CD31、CD34、Vim阳性,HMB45、S-100、Desmin、SMA阴性。该患者随访5年,患者健在,但肝、肺多发转移灶。结论 HEHE是一种少见的低度恶性肿瘤,易误诊,了解该肿瘤的临床及病理特点有助于鉴别其他易混淆的肿瘤类型。

ObjectiveTo summarize and analyze the clinical characteristic of HEHE in Chinese patients.Methods A patent with HEHE underwent liver transplant in Tianjin First Center Hospital,samples stained by hematoxylin eosin(HE) the morphological characteristics of this cases of hepatic EH were observed. At the same time,detect the expressionof CD31,CD34 protein with En Vision two step immunohistochemical method. According to the clinical manifestations,pathological morphology and the review of the literature.Results The tumor occupied left lateral lobe and right posterior of the liver,Section5 cm×2 cm×2 cm gray, gray red nodule count, the boundary is not clear, slightly hard matter. Microscopic examination of biopsy, tumor cells were oval or irregular in shape, arranged in cords, abundant cytoplasm is eosinophilic, necrosis and calcification can be seen in some areas, the boundaries of tumor and surrounding liver tissue is unclear. The results of immunohistochemistry for tumor cell CD31, CD34, HMB45, S-100, Vim are positive, Desmin negative, SMA. The patients were followed up for5 years, alive, but liver,multiple metastases were foung in the liver ans lungs.Conclusion HEHE is arare low-grade malignant tumor, which is easy misdiagnosed, understand the clinical and pathological characteristic of the tumors can help identify other tumor types confusing.