肝豆状核变性相关原发性肝癌的临床特征分析

肝脏 ›› 2023, Vol. 28 ›› Issue (3): 290-292.

肝豆状核变性相关原发性肝癌的临床特征分析

郭丹丹, 张英华, 高文峰, 生守鹏, 张永宏

100069 首都医科大学附属北京佑安医院肝病与肿瘤介入治疗中心

收稿日期:2022-11-03 出版日期:2023-03-31 发布日期:2023-08-28
通讯作者: 张永宏,Email: zhangyh@ccmu.edu.cn
基金资助:
北京市自然科学基金重点项目(7191004);北京市科学技术委员会(Z171 100001017078) ;2021年度院内中青年人才孵育项目(YNKTQN2021014)

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

GUO Dan-dan, ZHANG Ying-hua, GAO Wen-feng, SHENG Shou-peng, ZHANG Yong-hong

Interventional Therapy Center for Oncology, Beijing You'an Hospital, Capital Medical University, Beijing 100069, China

Received:2022-11-03 Online:2023-03-31 Published:2023-08-28
Contact: ZHANG Yong-hong,Email: zhangyh@ccmu.edu.cn

摘要/Abstract

摘要： 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。

关键词: 肝豆状核变性, 原发性肝癌, 诊断, 治疗

Abstract: 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.

Key words: Wilson's disease, Primary liver cancer, Diagnosis, Treatment

郭丹丹, 张英华, 高文峰, 生守鹏, 张永宏. 肝豆状核变性相关原发性肝癌的临床特征分析[J]. 肝脏, 2023, 28(3): 290-292.

GUO Dan-dan, ZHANG Ying-hua, GAO Wen-feng, SHENG Shou-peng, ZHANG Yong-hong. Clinical features of primary liver cancer associated with Wilson's disease[J]. Chinese Hepatolgy, 2023, 28(3): 290-292.

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