肝脏上皮样血管平滑肌脂肪瘤临床病理分析

Clinicopathologic features of hepatic epithelioid angiomyolipoma

目的:探讨肝脏上皮样血管平滑肌脂肪瘤临床病理特征、诊断及鉴别诊断要点。方法:采用常规HE染色及免疫组化方法分析病例,并复习相关文献。结果:5例患者男1例,女4例;年龄30～71岁;肝右叶3例,肝左叶1例,肝门区1例。5例均行手术切除术,其中1例复发。肿瘤最大径3. 6～12. 0 cm;肿瘤切面实性,灰褐色。HF病理结果显示,组织主要由上皮样细胞构成(60%～90%),多呈弥漫片状、梁状或结节状分布,胞质丰富、嗜酸性,部分透明状,易见核仁,有异型性。免疫组织化学显示,HMB-45、Melan-A呈弥漫阳性,不同程度表达SMA,而CK、S100、CD34、Glypican-3、Hepatocyte均不表达。结论:肝脏上皮样血管平滑肌脂肪瘤是一种具有恶性潜能的间叶源性肿瘤,其临床表现、影像学改变缺乏特异性,易误诊,确诊须依赖病理学检查,因其存在恶变可能,术后须长期严密随访。

Objective:To study the clinicopathologic features,diagnosis and differential diagnosis of hepatic epithelioid angiomylipoma(EAML).Methods:The routine HE staining and immunohistochemical method were used to analyze the cases and the relevant literatures were reviewed.Results:Five patients ranged in age from 30 to 71 years old.Three cases occurred in the right lobe of liver.One case in the left lobe of liver and one case in the hilar area.Five patients were performed surgical resection and one of them was local recurrence.EAML tended to be large,ranged in size from 3.6 cm to 12.0 cm.The cut surface was solid and pale brown.Microscopically,the tumors were predominantly composed of epithelioid cells,which were arranged in the shape of diffuse sheet,trabecular or nodular.The epithelioid cells were full of abundant eosinophilic cytoplasm.Partially transparent cells,obvious cellular atypia and nucleolus were seen.The proportion of epithelioid cells was 60%-90%.Immunohistochemical study showed that tumor cells were positive for HMB-45 and Melan-A,different degrees of expression of SMA,while CK,S100,CD34,Glypican-3 and hepatocyte were negative.Conclusion:Hepatic EAML is a potential malignant mesenchymal tumor,clinical manifestations and imaging changes are lack of specificity and easy to be misdiagnosed.Diagnosis depends on pathological examination and immunohistochemical detection.Because it may be transformed into malignant,the long-term close follow-up after operation is needed.