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目的探讨家族聚集性肝细胞肝癌(肝癌)患者的临床特点及预后。方法回顾性分析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。结果 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>400μg/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<105copies/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%。结论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。
Objective To investigate the clinical features and prognosis of familial hepatocellular carcinoma (HCC). Methods The clinical data of 40 patients who met the diagnostic criteria of familial aggregated hepatocellular carcinoma at the Third Affiliated Hospital of Sun Yat-sen University from January 2001 to December 2011 were retrospectively analyzed. There were 37 males and 3 females, aged 27-76 years, with a median age of 51 years. All patients signed informed consent, in line with medical ethics rules. A total of 40 patients with family history of hepatocellular carcinoma, alcohol abuse history, history of hepatitis B (hepatitis B) or hepatitis C (hepatitis C), cirrhosis, Child-Pugh classification of liver function, AFP ) Level, tumor diameter, number, clinical stage clinical data. Investigate hepatitis virus infection and collect laboratory data on patients with Hepatitis B virus (HBV) and Hepatitis C virus (HCV) infection. The patients were divided into three groups according to different treatment methods, and the surgical resection group consisted of 10 patients underwent radical hepatectomy. Twenty-six patients in the comprehensive treatment group were treated with ablation and percutaneous transhepatic arterial chemoembolization (TACE) Group 4 cases, the application of Sorafenib, Chinese medicine and other treatment. Regular follow-up after treatment 1 to 5 years, were calculated in 3 groups of patients 1,3,5-year survival rate. Results Forty patients had 92% (37/40) males and 12 to 1 males. The patients over the age of 30 accounted for 92% (37/40), and those with cirrhosis (98%) (39/40 ); Grade A was 70% (28/40) in Child-Pugh classification, 35% (14/40) was Serum AFP> 400μg / L, 55% / 40), tumor alone accounted for 50% (20/40); Barcelona clinical liver cancer (BCLC) stage A accounted for 20% (8/40), B ~ D period accounted for 80% (32/40). All patients were infected with HBV, accounting for 100% (40/40), HBV-DNA quantifying <105copies / ml accounting for 55% (22/40). None of the patients had HCV infection. Survival rates of 1, 3, and 5 in surgical resection group were 70%, 40% and 20% respectively, while those in integrated treatment group were 50%, 12% and 0% respectively. All four patients in symptomatic treatment group had no survival time exceeding 1 Year, 1 year survival rate was 0%. Conclusions Family aggregation of HCC mainly occurs on the basis of HBV infection or cirrhosis. The treatment effect and prognosis are poor. Increasing the rate of early diagnosis is the key to improve the prognosis.