5例肝脏上皮样血管平滑肌脂肪瘤患者的临床病理资料分析  

Hepatic epithelioid angiomyolipoma: report of 5 cases

作　　者：朱亚宁[1] 吴健[1] 周武碧[1] 张媛媛[2] ZHU Yaning;WU Jian;ZHOU Wubi;ZHANG Yuanyuan(Department of Pathology, Huai'an First People's Hospital, Nanjing Medical University, Huai'an, Jiangsu 223300, P. R. China;Department of Pediatrics, Huai'an First People's Hospital, Nanjing Medical University, Huai'an, Jiangsu 223300, P. R. China)

机构地区：[1]南京医科大学附属淮安第一医院病理科,江苏淮安223300 [2]南京医科大学附属淮安第一医院儿科,江苏淮安223300

出　　处：《中国普外基础与临床杂志》2019年第6期702-705,共4页Chinese Journal of Bases and Clinics In General Surgery

摘　　要：目的总结肝脏上皮样血管平滑肌脂肪瘤(hepatic epithelioid angiomyolipoma,HEAML)的临床病理和免疫表型特征并探讨其诊断与鉴别诊断方法。方法回顾性分析 2011 年 8 月至 2017 年 12 月期间淮安市第一人民医院病理科外科活检的 5 例 HEAML 患者的临床和影像学表现、病理学形态及免疫组织化学特征。结果5 例患者中男 2 例,女 3 例;年龄 38~64 岁,平均 50 岁。肝脏左叶肿瘤 2 例,右叶肿瘤 3 例。术前影像学检查有 3 例诊断为肝癌,有 2 例诊断为肝脏错构瘤和(或)血管瘤。肿瘤直径 1.5~7.0 cm,平均 3.6 cm。镜下肿瘤由较多上皮样平滑肌细胞、薄壁血管及少量脂肪混杂而成。免疫组织化学检测结果见 melan-A、 HMB45 及 SMA均阳性,HepPar-1、AE1/AE3、EMA、CD117、Dog-1、CD10、CgA、Syn 和 Desmin 均阴性, Ki-67 增殖指数2%~10%。患者随访 2~76 个月(平均 31.4 个月),均无瘤存活,无一例复发或转移。结论HEAML 是肝脏少见的原发性间叶性肿瘤,应避免术前、术后病理检查中将其误判为肝脏其他良恶性肿瘤,影响临床处理及治疗,结合组织形态及免疫组织化学染色结果可进行诊断及鉴别诊断。Objective To summarize clinicopathologic and immunophenotypic features of hepatic epithelioid angiomyolipoma (HEAML) and to explore its diagnostic and differential diagnostic methods. Method The clinical and imaging manifestations, pathological morphology and immunohistochemical features of 5 patients with HEAML from August 2011 to December 2017 in this hospital were retrospectively analyzed. Results There were 2 males and 3 females in the 5 patients with HEAML, aged 38–64 years with an average age of 50 years. There were 2 cases of the left lobe tumors and 3 cases of the right lobe tumors. Three cases were diagnosed as the hepatocellular carcinoma and the other two cases were diagnosed as the hepatic hamartoma and (or) hemangioma by the preoperative imaging examination. The diameter of tumors ranged from 1.5 cm to 7.0 cm, with an average of 3.6 cm. Microscopically, the tumors were composed of more epithelioid smooth muscle cells, parenchyma vessels and a small amount of fat. The immunohistochemical results showed that the melan-A, HMB45, and SMA were positive, while the HepPar-1, AE1/AE3, EMA, CD117, Dog-1, CD10, CgA, Syn, and Desmin were negative. The Ki-67 proliferation index was 2%–10%. The patients were all alive without the tumor recurrence after following up for 2–76 months with an average of 31.4 months. Conclusions HEAML is a rare primary mesenchymal tumor of liver, which should be misdiagnosed for other benign or malignant tumors for influencing clinical treatment. Diagnosis and differential diagnosis can be made by histopathology and immunohistochemical staining.

关 键 词：肝脏肿瘤 上皮样血管平滑肌脂肪瘤 病理学 免疫组织化学 

分 类 号：R735.7[医药卫生—肿瘤]