肝脏血管周上皮样细胞肿瘤的临床病理分析  

Perivascular epithelioid cell tumor of liver: a clinicopathologic analysis

作　　者：龙卫国[1] 郑芳[1] 李梅[1] 陈凯[1] 张云兴[1] 吴建农[1] 

机构地区：[1]江苏大学附属医院病理科,江苏镇江212000

出　　处：《安徽医药》2018年第3期445-449,共5页Anhui Medical and Pharmaceutical Journal

基　　金：镇江市卫生科技重点专项项目(SHW2015002)

摘　　要：目的探讨肝脏血管周上皮样细胞肿瘤(PEComa)的临床病理学特征、诊断、鉴别诊断及预后。方法报道2例发生于肝脏的PEComa,对2例少见部位PEComa行HE、免疫组化染色,并进行相关文献复习。结果 2例肝脏PEComa均发生于中年女性,45~53岁,临床均无特异性症状。CT分别显示肝右叶后下段和左顶叶部界限清楚的类圆形肿块,初诊考虑肝恶性肿瘤。镜下肿瘤主要由上皮样细胞和丰富血窦组成,1例无脂肪细胞成分,另1例伴多灶性脂肪细胞分化成分。免疫表型:肿瘤细胞均Melan-A、HMB-45、SMA阳性。结论发生于肝脏的PEComa临床较少见,组织起源仍然不清,临床需与原发性肝癌及转移性恶性黑色素瘤鉴别,大多生物学行为偏良性,诊断恶性需足够的证据支持和长期的随访观察。Objective To explore the clinical pathologic features,diagnosis,differential diagnosis and prognosis of perivascular epithelioid cell tumors(PEComa) of the liver.Methods Clinical data of two PEComa patients were collected and analyzed.Conventional HE staining and immunohistochemical staining were performed,with review of the literature.Results Both patients are middle-aged females,45 to 53 years old.There were no specific clinical symptoms.CT scan showed a clear circular mass of the right posterior lobe and the left parietal lobe of the liver,hepatic malignancies considered preliminarily.Microscopically,the tumors were mainly composed of epithelioid cells and abundant sinusoids vessels.One case had no adipose component,the other case with multifocal adipocyte differentiation components.Immunohistochemical phenotype showed that tumor cells were steadily positive with Melan-A,HMB-45 and SMA.Conclusions PEComa occurring in the liver is less common,histogenesis of which is still unclear.Clinically,it needs to be differentiated from primary hepatocellular carcinoma and metastatic malignant melanoma.Most of PEComas have a benign biological behavior.The diagnosis of malignancy requires adequate evidence and long-term follow-up.

关 键 词：肝脏肿瘤 血管周上皮样细胞肿瘤 免疫组化 

分 类 号：R735.7[医药卫生—肿瘤]