肝血管肉瘤18例临床病理学特征分析  

Analysis of clinicopathological features of 18 cases of hepatic angiosarcoma

作　　者：周颖颖 王瀚 冼志红 丛文铭 董辉 Zhou Yingying;Wang Han;Xian Zhihong;Cong Wenming;Dong Hui(Department of Pathology,Eastern Hepatobiliary Surgery Hospital,Naval Medical University,Shanghai 200438,China)

机构地区：[1]海军军医大学东方肝胆外科医院病理科,上海200438

出　　处：《中华肝脏病杂志》2023年第7期729-735,共7页Chinese Journal of Hepatology

基　　金：孟超青年人才发展计划基金。

摘　　要：目的探讨肝血管肉瘤的临床病理特征和治疗预后。方法回顾性收集18例肝血管肉瘤患者的临床病理信息和预后情况,Kaplan-Meier法计算无复发生存率和总生存率,Cox回归分析探索生存相关风险因素。结果患者男性12例,女性6例,平均年龄57(37~70)岁。肿瘤平均直径8.40(2.00~18.00)cm,多发肿瘤7例,2例有大血管瘤栓。镜下肿瘤组织排列呈不规则吻合状血管腔隙样或实性束状编织状,组织形态上可模拟毛细血管瘤、海绵状血管瘤或血管外皮瘤。瘤细胞可呈梭形或上皮样,呈靴钉样衬覆于管腔或在管腔内形成乳头状结构。高、中、低分化肿瘤占比为4∶8∶6,6例肿瘤边界清晰,8例有微血管瘤栓,16例见血湖形成。全部病例不同程度表达CD31、CD34、成红细胞转化特异性相关基因、Fli-1标志物,P53突变病例4例,Ki-67>10%病例6例。随访0.23~114.20个月,5年无复发生存率为16.7%,5年总生存率为37.2%。Cox回归多因素分析提示,术前有症状、多发肿瘤为无复发生存的显著风险因素,术前有症状、Ki-67>10%为总生存的显著风险因素。结论肝血管肉瘤是一种罕见的肝脏间叶源性肿瘤,恶性程度高,预后差,确诊需结合病理形态和免疫组织化学标志物情况。复杂的组织和细胞学构象以及与良性血管源性肿瘤、肉瘤、癌在病理特征上的重叠是血管肉瘤鉴别诊断的难点。早期发现和根治性手术切除是延长生存期仅有的有效方法。Objective To investigate the clinicopathological features,treatment,and prognosis of hepatic angiosarcoma.Methods Clinicopathological data and prognostic conditions of 18 cases with hepatic angiosarcoma were collected retrospectively.The recurrence-free survival rate and overall survival rate were calculated by the Kaplan-Meier method.A Cox regression analysis was used to explore the survival-related risk factors.Results There were 12 male and 6 female patients,with an average age of 57(37~70)years.The tumor’s average diameter was 8.40(2.00~18.00)cm.Seven cases had multiple tumors,while two cases had large vessel tumor thrombuses.Microscopically,the tumor tissues were irregularly anastomosed,with vascular lacunar or solid bundle-like weaving,and the tissue morphology mimicked capillary hemangioma,cavernous hemangioma,or angioepithelioma,while tumor cells were spindle-shaped or epithelioid,lined with hobnails in the lumen,or formed papillary structures in the lumen.The proportion of highly,moderately,and poorly differentiated tumors was 4:8:6,with six cases having clear tumor boundaries,eight having microvascular tumor thrombi,and sixteen having blood lake formation.Different levels of expression of CD31,CD34,erythroblast transformation-specific related genes,and Fli-1 markers were demonstrated in all of the cases.Four cases had a P53 mutation,and six cases had Ki-67>10%.During the follow-up period of 0.23-114.20 months,the five-year recurrence-free survival rate and overall survival rate were 16.7%and 37.2%,respectively.Cox regression multivariate analysis showed that preoperative symptoms and multiple tumors were significant risk factors for recurrence-free survival,while preoperative symptoms and Ki-67>10%were significant risk factors for overall survival.Conclusion Hepatic angiosarcoma is a rare hepatic mesenchymal tumor with high malignancy and a poor prognosis.Pathological morphology and immunohistochemical marker combinations are needed for a definite diagnosis.However,the complexity of angiosarcomas'hist

关 键 词：肝脏 血管肉瘤 病理学 临床 预后 

分 类 号：R735.7[医药卫生—肿瘤]