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目的:探讨肝血管平滑肌脂肪瘤(AML)的临床和免疫病理学特点及鉴别诊断要点。方法:对36例手术切除肝AML的临床和病理学特点、10种免疫组化标志物的表达状况进行检测。结果:36例肝AML中女性21例,男性15例,平均年龄41.6岁(26～60岁),26例(72.2％)有肝区胀痛,10例(27.8％)为偶然发现。瘤体直径为2.5～14 cm,平均6.8 cm。光镜下肿瘤由平滑肌细胞、厚壁血管及脂肪3种成分混合组成,根据瘤组织成分的比例可分为经典型(7例,19.4％),、肌细胞为主型(24例,66.7％)、脂肪细胞为主型(4例,11.1％)、血管瘤型(1例,2.8％),有时可见髓外造血。免疫组化染色显示,瘤细胞呈HMB45(100％)、SMA(100％)和CD117(66.7％)阳性。结论:肝AML组织学变异较大,容易造成误诊,鉴别诊断需排除肝细胞癌、脂肪性肿瘤、血管源性肿瘤以及间叶性错构瘤等;免疫组化以出现HMB45阳性瘤细胞具有重要的诊断意义。
Objective: To investigate the clinical and immunopathological features and differential diagnosis of hepatic angiomyolipoma (AML). Methods: The clinical and pathological features of 36 cases of resected liver AML were examined. The expression of 10 immunohistochemical markers was detected. Results: Totally 21 female patients were enrolled in this study. The average age was 41.6 years (26-60 years). Twenty-six patients (72.2%) had liver pain and 10 (27.8%) were accidental findings. Tumor diameter 2.5 ~ 14 cm, an average of 6.8 cm. According to the proportion of tumor tissue components, the tumors were divided into three types: classic (7 cases, 19.4%), muscle cells predominant (24 cases, 66.7% ), Predominant adipocytes (4 cases, 11.1%) and hemangiomas (1 case, 2.8%). Extramedullary hematopoiesis was seen in some cases. Immunohistochemical staining showed that the tumor cells were positive for HMB45 (100%), SMA (100%) and CD117 (66.7%). Conclusion: Histopathological changes of liver AML are large and easily misdiagnosed. Hepatocellular carcinoma, fatty tumor, vasogenic tumor and mesenchymal hamartoma need to be excluded in the differential diagnosis. Immunohistochemical staining of HMB45 positive tumor cells is of great importance The diagnostic significance.