Clinical features of hepatolenticular degeneration related primary hepatocellular carcinoma

WANG Liping; HE Tingting; WANG Zhongxia; YAN Liping; YU Simiao; JING Jing; ZHANG Aozhe; SUN Yongqiang; WANG Xin; REN Yuebo; WANG Ruilin

doi:10.12122/j.issn.1674-4500.2026.01.04

Abstract：Objective To explore the clinical features of hepatocellular carcinoma associated with Wilson's disease,providing reference for its clinical diagnosis and treatment.Methods A retrospective analysis was conducted on the clinical data of 9 patients with hepatocellular carcinoma associated with Wilson's disease admitted to the Fifth Medical Center of PLA General Hospital from January 2022 to December 2024.The data included demographic information,clinical characteristics,laboratory indicators,treatment methods,and prognosis.Results Among the 9 patients,there were 6 males and 3 females,aged 22-66(43.6±15.2)years old at the time of hepatocellular carcinoma diagnosis.All patients had concurrent liver cirrhosis(Child-Pugh class A in 3 cases,class B in 4 cases,and class C in 2 cases).Alpha-fetoprotein was negative in 8 cases and positive in 1 case;Kayser-Fleischer rings on the cornea were positive in 6 cases.The tumor staging was mainly early-stage(Barcelona Clinic Hepatocellular Carcinoma staging A in 6 cases,Chinese Hepatocellular Carcinoma staging Ia in 6 cases).Individualized treatment plans were adopted:4 patients underwent ablation(1 case of radiofrequency ablation and 3 cases of microwave ablation),3 patients underwent transcatheter arterial chemoembolization(TACE),and 2 patients received conservative treatment.Three patients recurred at 5,13 and 18 months postoperatively,respectively,and all received TACE treatment after the first recurrence.Among them,1 patient who recurred a second time received combined TACE and microwave ablation treatment.In terms of prognosis,only 1 late-stage patient died,and the remaining 8 patients improved.Conclusion Hepatocellular carcinoma associated with Wilson's disease occurs secondary to liver cirrhosis.It has a wide age range of onset,hidden course,and mostly negative alpha-fetoprotein,making early diagnosis difficult.The risk of postoperative recurrence is high,and there is a significant difference in recurrence intervals.Clinicians should strengthen long-term follow-up for patients with Wilson's disease progressing to liver cirrhosis,and it is recommended to use enhanced MRI for dynamic monitoring to improve the early diagnosis rate.Early diagnosis and treatment are key to improving the prognosis of this disease.