肝豆状核变性相关原发性肝癌的临床特征分析

Clinical features of primary liver cancer associated with Wilson’s disease

目的总结肝豆状核变性(Wilson disease,WD)相关原发性肝癌患者的临床特点。方法回顾性分析2002年1月至2022年9月北京佑安医院WD相关原发性肝癌患者的临床资料,总结临床特征、实验室检查、诊断及治疗方式。结果WD相关肝癌患者12例,其中男性5例,女性7例。发生肝癌时均合并肝硬化,其中8例患者为Child-Pugh B/C级。甲胎蛋白(AFP)阴性患者4例,AFP阳性患者8例。巴塞罗那(Barcelona clinic liver cancer,BCLC)分期A期4例,B期1例,C期4例,D期3例。治疗方式:1例患者内科保守治疗,5例患者行单纯肝动脉化疗栓塞(transcatheter arterial chemoembolization,TACE),2例患者TACE联合消融治疗,4例患者行肝移植术。结论WD相关肝癌临床较为罕见,发现时大部分为中晚期。对WD进展到肝硬化患者应密切随访肝癌的发生,以提高早期诊断率。

Objective To investigate the clinical features of primary liver cancer associated with Wilson’s disease(WD).Methods Patients with primary liver cancer admitted to our hospital were retrospectively enrolled.The clinical manifestations,laboratory tests,diagnosis and treatments were collected.Results Among the 12 patients with primary liver cancer associated with WD,5 cases were males and 7 were females.All cases were combined with liver cirrhosis,8 cases were Child-Pugh B/C.Four patients with alpha fetoprotein(AFP)negative,while 8 patients with AFP positive.There were 4 cases in BCLC stage A,1 case in stage B,4 cases in stage C and 3 cases in stage D.One patients received conservative treatment,5 patients treated by transcatheter arterial chemoembolization(TACE),2 patients received TACE combined with ablation and 4 patients received liver transplantation.Conclusion WD-associated primary liver cancer is rare clinically,and most of them are in middle or late BCLC stage.The occurrence of liver cancer should be closely followed up in patients with WD-associated cirrhosis,in order to improve the early diagnosis rate.