肝血管肉瘤的临床特点及预后总结  

Clinical features and prognosis of hepatic angiosarcoma

作　　者：窦文杉 王天怡 董驰[3] 张德奎[1] DOU Wenshan;WANG Tianyi;DONG Chi;ZHANG Dekui(Department of Gastroenterology,the Second Hospital of Lanzhou University,Lanzhou 730030;Department of Gastroenterology,Xi'an Hong Hui Hospital;Department of Pathology,the Second Hospital of Lanzhou University,China)

机构地区：[1]兰州大学第二医院消化内科,甘肃兰州730030 [2]西安市红会医院消化内科 [3]兰州大学第二医院病理科

出　　处：《胃肠病学和肝病学杂志》2023年第5期505-513,共9页Chinese Journal of Gastroenterology and Hepatology

基　　金：甘肃省卫生行业科研计划项目(GSWSKY2017-01);兰州市人才创新创业项目(2018-RC-76)。

摘　　要：目的对我院确诊的1例肝血管肉瘤(hepatic angiosarcoma,HAS)进行报道;并通过文献复习分析总结HAS的临床特点,以提高对本病的认识。方法回顾性分析我院病例及近15年文献报道中HAS病例,总结其临床特点、病理特征、实验室指标、治疗方式及预后。结果78例HAS患者中,男42例,女36例;发病年龄6周~87岁,平均年龄56.6岁。其中腹痛35例,腹胀17例,乏力8例,食欲减退8例,体质量减轻8例,无明显临床症状14例,其余表现为非特异性症状。病毒性肝炎患者10例。氯乙烯接触史2例,矿业化学品接触史1例,皮革制品接触史1例,Th232接触史1例,类固醇药物服用史2例,免疫抑制剂服用史1例,农药接触史1例。肿瘤标志物异常者8例,实验室指标肝功能异常者38例。大部分HAS病例行手术切除治疗,同时化疗、射频消融术、靶向药物、TACE、TAE、肝移植、PD-1抑制剂、同种异体RAK细胞均可用于HAS治疗。所有纳入病例中,25例患者生存期不详,其余患者生存期为2 d~48个月,中位生存期为6个月。结论HAS恶性程度高,进展快,预后很差,临床表现不典型,所以HAS的早期诊断尤为重要,通常超声、CT以及MRI可为HAS的诊断提供重要线索,确诊依赖于病理学检查。目前手术切除是主要的治疗方法,化疗可改善预后,TAE、TACE可用于HAS的辅助治疗,未来靶向分子治疗可能会成为HAS重要的治疗方式。Objective To report one case of hepatic angiosarcoma(HAS)diagnosed in our hospital and analyze the clinical characteristics of HAS through literature review to improve the understanding of this disease.Methods The case of HAS reported in our hospital and the cases reported in the literature in the past 15 years were analyzed.The clinical features,pathological features,laboratory indicators,treatment methods and prognosis were summarized.Results Among 78 HAS patients,42 were males and 36 were females.The onset age ranged from 6 weeks to 87 years,with an average age of 56.6 years old.There were 35 patients of abdominal pain,17 patients of abdominal distension,8 patients of fatigue,8 patients of loss of appetite,8 patients of weight loss,14 patients of no obvious clinical symptoms,and the rest of the symptoms were non-specific.There were 10 patients with viral hepatitis.2 patients were exposed to vinyl chloride,1 patient was exposed to mining chemicals,1 patient was exposed to leather products,1 patient was exposed to Th232,2 patients were exposed to steroid drugs,1 patient was exposed to immunosuppressive drugs,and 1 patient was exposed to pesticides.There were 8 patients with abnormal tumor markers and 38 patients with abnormal laboratory liver function.The majority of HAS patients were treated with surgical excision.What's more,chemotherapy,radiofrequency ablation,targeted drugs,TACE,TAE,liver transplantation,PD-1 inhibitors,and allogeneic RAK cells were all available for HAS.Among all the included patients,the survival time of 25 patients was unknown,and the remaining patients had a survival time range from 2 days to 48 months,with a median survival time of 6 months.Conclusion The malignant degree of HAS is high,the progression is rapid,the prognosis is poor,and the clinical manifestations are not typical.So the early diagnosis of HAS is particularly important.Usually,ultrasound,CT and MRI can provide important clues for the diagnosis of HAS,but the diagnosis depends on pathological examination.At present,surgic

关 键 词：肝肿瘤 血管肉瘤 基因检测 病例报道 

分 类 号：R735.7[医药卫生—肿瘤]