探讨家族聚集性肝细胞肝癌（肝癌）患者的临床特点及预后。

回顾性分析2001年1月至2011年12月在中山大学附属第三医院就诊的符合家族聚集性肝癌诊断标准的40例患者临床资料。其中男37例，女3例；年龄27~76岁，中位年龄是51岁。所有患者均签署知情同意书，符合医学伦理学规定。收集40例患者的肝癌家族史，嗜酒史，乙型病毒性肝炎（乙肝）或丙型病毒性肝炎（丙肝）病史，合并肝硬化情况，肝功能Child-Pugh分级，血清甲胎蛋白（AFP）水平，肿瘤直径、数目，肿瘤临床分期的临床资料。调查肝炎病毒感染情况，收集患者乙型肝炎病毒（HBV）和丙型肝炎病毒（HCV）感染的实验室资料。根据不同治疗方式将患者分为3组，手术切除组10例，术式为根治性肝切除术；综合治疗组26例，采用消融、经皮穿刺肝动脉化疗栓塞（TACE）等治疗；保守治疗组4例，应用索拉菲尼、中药等治疗。治疗后定期复查随访1~5年，分别计算3组患者的1、3、5年生存率。

40例患者男性占92%（37/40），男女比为12∶1；30岁以上患者最多，占92%（37/40）；合并肝硬化者39例，占98%（39/40）；肝功能Child-Pugh分级中的A级者占70%（28/40）；血清AFP水平>400 μg/L者占35%（14/40）；肿瘤直径≤5 cm者占55%（22/40），肿瘤单发者占50%（20/40）；巴塞罗那临床肝癌（BCLC）分期A期占20%（8/40），B~D期者占80%（32/40）。患者均合并HBV感染，占100%（40/40），乙型肝炎病毒脱氧核糖核酸（HBV-DNA）定量<10⁵ copies/ml占55%（22/40）。无患者合并HCV感染。手术切除组患者1、3、5生存率分别为70%、40%和20%，综合治疗组患者相应为50%、12%和0%，对症治疗组的4例患者生存时间均未超过1年，1年生存率为0%。

家族聚集性肝癌多发生在HBV感染或肝硬化的基础上，其治疗效果及预后不佳，提高早期诊断率是改善预后的关键。

To analyze the clinical characteristics and prognosis of patients with hepatocellular carcinoma (HCC) with familial aggregation.

Clinical data of 40 patients who met the HCC with familial aggregation diagnosis standard in the Third Affiliated Hospital of Sun Yat-Sen University from January 2001 to December 2011 were analyzed retrospectively. There were 37 males and 3 females with the age ranging from 27 to 76 years old and the median age of 51 years old. The informed consents of all patients were obtained and the ethical committee approval was received. Clinical data of 40 cases were collected including familial history of HCC, alcohol abuse history, history of hepatitis B or hepatitis C, liver cirrhosis, Child-Pugh liver function grading, serum AFP levels, tumor size, number and clinical staging. The hepatitis virus infection was investigated and the relevant laboratory data of HBV and HCV infection were collected. The patients were divided into three groups according to different treatments: 10 cases in the surgical resection group underwent radical hepatectomy, 26 cases in the comprehensive treatment group received ablation and (or) transhepatic artery chemoembolization (TACE), 4 cases in the conservative treatment group received treatment of sorafenib and traditional Chinese medicine. The patients were followed up for 1 to 5 years after treatments and the 1-, 3-, and 5-year survival rates were analyzed.

Among the patients, the percentage of male was 92% (37/40), and the ratio of male to female is 12∶1. The age of most patients was over 30 years, which accounted for 92% (37/40). Thirty-night cases were complicated with liver cirrhosis, accounting for 98% (39/40). For the Child-Pugh liver function grading, 70% (28/40) patients were grade A. Fourteen case were with over 400 μg/L of serum AFP, accounting for 35% (14/40). Patients with the tumor diameter less than 5 cm accounted for 55% (22/40). Patients with single tumors accounted for 50% (20/40). The patients of stage A of Barcelona Clinic Liver Cancer (BCLC) staging system accounted for 20% (8/40), stage B to D 80% (32/40). All the patients were combined with HBV infection, accounting for 100% (40/40), and patients with the HBV-DNA quantity<10⁵ copies/ml accounted for 55% (22/40). No case suffered from HCV infection. The 1-, 3-, 5-year survival rates were 70%, 40% and 20% respectively in the surgical resection group and were 50%, 12% and 0% in the comprehensive treatment group. Four cases in the conservative treatment group survived less than 12 months with 1-year survival rate of 0%.

The HCC with familial aggregation mostly occurs on the basis of HBV infection or liver cirrhosis. The treatment effect and prognosis of patients with HCC with familial aggregation are poor. Early diagnosis is the key to improve the prognosis.