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

作　　者：李宁波 申发燕[1] 张海芳[1] 陈安敏[1] 贺峥 白冬雨[1] Li Ningbo;Shen Fayan;Zhang Haifang;Chen Anmin;He Zheng;Bai Dongyu(Department of Pathology,The First affiliated hospital to Xiamen University,Xiamen 361003,China)

机构地区：[1]厦门大学附属第一医院病理科,厦门361003

出　　处：《中国组织化学与细胞化学杂志》2021年第3期271-275,共5页Chinese Journal of Histochemistry and Cytochemistry

摘　　要：目的探讨肝脏上皮样血管平滑肌脂肪瘤的临床病理特征、免疫组织化学表型、鉴别诊断及预后情况。方法回顾性分析厦门大学附属第一医院2014年1月至2019年12月10例肝上皮样血管平滑肌脂肪瘤,并复习相关文献。结果本院收治10例患者,其中9例为女性,1例为男性,年龄35~61岁,中位年龄47.9±10岁。8例患者肿瘤位于肝右叶,1例位于左叶,1例位于尾状叶。肉眼观肿瘤最大径2.1cm~14.5cm,平均直径7.93±4.2cm,10例均为单发,境界较清楚,实性,灰白、灰黄或灰褐色,质地中等或质软,部分病例切面伴出血。镜下见多边形或类圆形的上皮样细胞弥漫成片排列,胞浆丰富,胞质嗜酸或透亮,可见核仁,部分病例可见多核或奇异形核,核分裂像少见,部分区域可见肿瘤细胞围绕血管排列。免疫组化结果显示HMB-45和Melan-A均弥漫性阳性,SMA部分阳性,CK、Hepar1、glypican-3、desmin、CD34均阴性,其中仅1例(1/10)肿瘤细胞TFE3弱阳性,Ki-67指数(3%~10%)。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性肿瘤,没有典型的临床症状和影像学征象,与发生在肝的其他良恶性肿瘤较难鉴别,需结合病理形态及免疫组织化学表型确诊。Objective To investigate the clinicopathological characteristics,immunohistochemical phenotypes,differential diagnosis and prognosis of hepatic epithelioid angiomyolipoma.Methods A retrospective review was performed on 10 cases of hepatic epithelioid angiomyolipoma in the First Affiliated Hospital of Xiamen University from January 2014 to December 2019,and the relevant literature was reviewed.Results 10 patients were admitted,of which 9 cases were female and one was male,aged from 35 to 61 years with a median age of 47.9±10 years.The lesions were located in the right lobe of the liver in 8 patients,the left lobe in one patient,and the caudate lobe in one patient.The maximum diameter of the tumor was 2.1cm~14.5cm,and the average diameter was 7.93±4.2cm.All 10 cases were single.The masses had clear boundaries and solid content,and showed grey,sallow,or greyish brown in color and medium or soft in texture.Some cases were showed hemorrhage in sections.Microscopically,polygonal or circular epithelioid cells arranged in sheets were diffusely distributed,with abundant eosinophilic or translucent cytoplasm.Nucleoli were visible and multinuclear cells and odd-shaped nuclei could be observed in some cases,and mitotic figures were rare.In some areas,tumor cells were arranged around the blood vessels.Immunohistochemistry showed that HMB-45 and Melan-A were diffusely positive,and SMA was partially positive,while CK,Hepar1,glypican-3,desmin,and CD34 were all negative.Only 1 case(1/10)showed TFE3 weakly positive,and Ki-67 index(3-10%).Conclusion Hepatic epithelioid angiomyolipoma is a mesenchymal tumor with malignant potential.It has no typical clinical symptoms and imaging signs.It is difficult to be distinguished from other benign and malignant tumors in the liver.Pathological morphology and immunohistochemical phenotypes are needed for diagnosis.

关 键 词：肝脏上皮样血管平滑肌脂肪瘤 肝细胞癌 鉴别诊断 预后 

分 类 号：R732.2[医药卫生—肿瘤]