肝脏未分化胚胎性肉瘤1例  

Undifferentiated embryonal sarcoma of the liver:A case report

作　　者：邓茹霞 孙柯[1] DENG Ruxia;SUN Ke(Department of Pathology,First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China)

机构地区：[1]浙江大学医学院附属第一医院病理科,杭州310003

出　　处：《临床与病理杂志》2023年第7期1459-1464,共6页Journal of Clinical and Pathological Research

摘　　要：分析1例肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma,UES)的临床病理特征。患者女,17岁,半月前左下腹发生剧烈疼痛,浙江省人民医院考虑为血管平滑肌脂肪瘤并予以胃动脉栓塞治疗,患者无明显不适,为求进一步治疗于浙江大学医学院附属第一医院就诊,影像学提示肝脏第二肝门部延伸至肝胃间隙巨大占位,考虑上皮样血管平滑肌脂肪瘤而行肿瘤切除术。大体上,肿瘤呈囊实性结节,与周围肝组织分界清,切面多彩状,质软。镜下,肿瘤组织形态多样,部分区域以黏液性结缔组织小叶或结节状生长为特征,由稀疏的分化良好的间叶细胞、胆管以及肝细胞混杂组成,部分区域由未分化间叶细胞及疏松黏液样基质构成,这些区域肿瘤组织在形态上存在逐渐过渡。免疫组织化学染色结果显示:细胞角蛋白(cytokeratin,CK)7胆管阳性、INI1、BRG、结蛋白(desmin)阳性,CK、黏液蛋白-1(mucin-1,MUC-1)、磷脂酰肌醇蛋白聚糖-3(glypican-3,GPC3)、大鼠成肌分化蛋白1(myoblast determination protein 1,myoD1)、肌细胞生成蛋白(myogenin)、平滑肌肌动蛋白(smooth muscle actin,SMA)、钙调理蛋白(calponin)、神经特异性蛋白S-100、SOX-10、CD34、成红细胞转化特异性相关基因(erythroblast-specific gene,ERG)、MDM2、p16、甲胎蛋白(α-fetoprotein,AFP)均阴性,Ki-67增殖指数约60%。UES是一种罕见的肝脏原发性恶性肿瘤,主要发生于儿童,无特征性临床表现,组织学变化大,术前及术中诊断均十分困难,明确诊断依赖病理。The clinicopathological features of a case of undifferentiated embryonal sarcoma(UES)of the liver were retrospectively analyzed.The patient,a 17-year-old female,developed severe pain in the left lower abdomen half a month ago,who was diagnosed with angiomyolipoma by Zhejiang Provincial People’s Hospital and treated with gastric artery embolization.The patient had no obvious discomfort now,and was admitted to First Affiliated Hospital of Zhejiang University School of Medicine for further treatment.Imaging showed a large mass in the second hepatic hilum extending into the hepatogastric space,and tumor resection was performed for considering epithelioid angiomyolipoma.In general,the tumor was a well-demarcated and cystic-solid nodule with colorful and soft sections.Microscopically,the tumor tissue was diverse in morphology.Some areas were characterized by lobular or nodular growth of mucinous connective tissue,composed of sparse well differentiated mesenchymal cells,bile ducts,and hepatocytes,and some areas were composed of undifferentiated mesenchymal cells and loose mucoid stroma.There was a gradual transition in the morphology of tumor tissue in these areas.Immunohistochemistry staining shows:cytokeratin(CK)7 was positive for bile duct,INI1,BRG,and desmin were positive,and CK,mucin-1(MUC-1),glypican-3(GPC3),myoblast determination protein 1(myoD1),myogenin,smooth muscle actin(SMA),calponin,neurospecific protein S-100,SOX-10,CD34,erythroblast-specific gene(ERG),MDM2,p16,andα-fetoprotein(AFP)were negative.The Ki-67 proliferation index was about 60%.UES is a rare primary malignant tumor of the liver,which mainly occurs in children.It has no characteristic clinical manifestations and large histological changes.Preoperative and intraoperative diagnosis is very difficult,and definitive diagnosis depends on pathology.

关 键 词：肝脏肿瘤 胚胎性肉瘤 间叶性错构瘤 

分 类 号：R735.7[医药卫生—肿瘤]