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目的观察肝内胆管肉瘤样癌(SICC)的临床病理、免疫表型和预后特征。方法对16例SICC的临床病理特征、免疫表型进行观察,并结合文献探讨该肿瘤的病理诊断和鉴别诊断。结果 16例患者中男性15例,女性1例,年龄35～72岁,中位年龄51岁。临床主要表现为腹痛及局部肿块,病变部位以右叶为主,呈浸润性生长。镜下见胆管癌细胞由不同分化程度的腺癌细胞组成,肉瘤样成分按其形态特征分为梭形细胞型、巨细胞型和多种肉瘤分化。免疫组化示肿瘤细胞CK19和vimentin(+),HepPar 1和AFP(-),并表达多种间叶分化性标记。16例均行术后随访,中位生存时间为6个月。结论肝内胆管肉瘤样癌为罕见高度恶性的上皮性肿瘤,组织学结构复杂,表现为多种成分并存,肿瘤细胞CK19和vimentin(+),HepPar 1和AFP(-)模式具有重要诊断价值。
Objective To observe the clinicopathological features, immunophenotype and prognosis of intrahepatic cholangiocarcinoma of the liver (SICC). Methods The clinicopathological features and immunophenotypes of 16 cases of SICC were observed. The pathological diagnosis and differential diagnosis of SICC were also discussed. Results Of the 16 patients, 15 were male and 1 female, aged 35-72 years with a median age of 51 years. The main clinical manifestations of abdominal pain and local mass, the lesion to the right lobe, was invasive growth. Microscopically, cholangiocarcinoma cells were composed of adenocarcinoma cells with different degrees of differentiation. The sarcomatoid cells were divided into spindle cell type, giant cell type and multiple sarcoma according to their morphological characteristics. Immunohistochemistry showed that the tumor cells CK19 and vimentin (+), HepPar 1 and AFP (-), and expressed a variety of mesenchymal markers. All 16 patients were followed up for a median of 6 months. Conclusions Intrahepatic choriocarcinoma of the liver is a rare and highly malignant epithelial tumor. The histological structure of the intraepithelial cholangiocarcinoma is complicated and its components are coexistent. The patterns of CK19 and vimentin (+), HepPar 1 and AFP (-) in tumor cells have important diagnostic value.