国内成人肝母细胞瘤37例临床特点分析

Analysis on the domestic clinical characteristics of 37 cases with adult hepatoblastoma

目的探索国内成人肝母细胞瘤（HB）的临床特点,为该疾病的临床诊断与治疗提供参考与帮助。方法检索我国生物医学文献各数据库,对截止至2013年12月31日已发表的关于成人HB的文献中经病理证实的临床病例进行汇集分析,主要内容包括病例的性别、年龄、主要临床表现、实验室检查、影像学检查、治疗及预后等。结果共汇集文献24篇,成人HB病例37例。男女比约为1.64：1（23/14）,相对多发于40～60岁年龄段（18/37,48.65%）。上腹疼痛（54.05%,20/37）、腹胀（48.65%,18/37）和腹部包块（22.22%,8/37）是最常见的临床表现,但缺乏特异性。约半数病例（48.39%,15/31）血AFP升高,治疗后AFP水平逐渐降低。乙肝并不是成人HB的高危因素。B型超声、CT是诊断成人HB最常用的影像学检查,但其影像学表现缺乏特异性。肿瘤好发于肝右叶（54.05%,20/37）,约半数患者在诊断时发生了不同程度的浸润转移。成人HB误诊率较高,易被误诊为肝癌。手术是最常用的治疗方法,化疗是重要的辅助治疗手段。成人HB预后较肝癌略好,其中位生存时间为6个月。结论成人HB的临床及影像学表现缺乏特异性,手术是治疗成人HB的首选,化疗是重要的辅助治疗手段。

Objective To investigate the clinical features of domestic adult hepatoblastoma（hepatoblastoma,HB）and provide a reference for clinical diagnosis and treatment.Methods Retrieval search biomedical Chinese literatures of the database,the deadline was December 31,2013,searched all the published adult HB literature,collected and analyzed the literature with clinical pathological demonstrated cases,analysis items including gender,age,the main clinical manifestations,laboratory examination,imaging examination,treatment and prognosis.Results There were 24 papers,37 cases of adult HB were enrolled.Men and women about 1.64-＇ 1（23/14）,relatively more commonly found in the age of 40 ～60 years（18/37,48.65%）.Abdominal pain（54.05%,20/37）,abdominal distension（48.65%,18/37） and abdominal mass（22.22%,8/37） are the most common clinical manifestations,but lack of specificity.About half of the cases（48.39%,15/31） with elevated plasma AFP,AFP levels after treatment were decreased.Risk factor for hepatitis B is not adult HB.Ultrasound and CT examination are the most commonly used image methods in the diagnosis of adult HB,but lack of specificity.Tumors are more commonly found in the right lobe of the liver（54.05%,20/37）,about half of the patients had different degree of infiltration and metastasis at diagnosis.Adult HB misdiagnosis rate is high,is easy to be misdiagnosed as hepatocellular carcinoma.Operation is the most commonly used method of treatment,chemotherapy is an important therapeutic tool.Adult HB＇s prognosis slightly better than liver cancer,the median survival time was 6 months.Conclusion Adult HB studies show a lack of specificity of the clinical and imaging character,in clinical work should be alert to the presence of adult HB.Operation is the first choice of treatment of adult HB,chemotherapy is an important therapeutic tool.