Agarwal Ankit, Biswas Sagnik, Swaroop Shekhar, Aggarwal Arnav, Agarwal Ayush, Jain Gautam, Elhence Anshuman, Vaidya Arun, Gupte Amit, Mohanka Ravi, Kumar Ramesh, Mishra Ashwani Kumar, Gamanagatti Shivanand, Paul Shashi Bala, Acharya Subrat Kumar, Shukla Akash
Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, New Delhi 110029, Delhi, India.
Department of Gastroenterology, Seth Gordhandas Sunderdas Medical College and KEM Hospital, Mumbai 400012, Maharashtra, India.
World J Gastrointest Oncol. 2024 Mar 15;16(3):699-715. doi: 10.4251/wjgo.v16.i3.699.
There is scant literature on hepatocellular carcinoma (HCC) in patients with Budd-Chiari syndrome (BCS).
To assess the magnitude, clinical characteristics, feasibility, and outcomes of treatment in BCS-HCC.
A total of 904 BCS patients from New Delhi, India and 1140 from Mumbai, India were included. The prevalence and incidence of HCC were determined, and among patients with BCS-HCC, the viability and outcomes of interventional therapy were evaluated.
In the New Delhi cohort of 35 BCS-HCC patients, 18 had HCC at index presentation (prevalence 1.99%), and 17 developed HCC over a follow-up of 4601 person-years, [incidence 0.36 (0.22-0.57) per 100 person-years]. BCS-HCC patients were older when compared to patients with BCS alone ( = 0.001) and had a higher proportion of inferior vena cava block, cirrhosis, and long-segment vascular obstruction. The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up (13029 ng/mL 500 ng/mL, = 0.01). Of the 35 BCS-HCC, 26 (74.3%) underwent radiological interventions for BCS, and 22 (62.8%) patients underwent treatment for HCC [transarterial chemoembolization in 18 (81.8%), oral tyrosine kinase inhibitor in 3 (13.6%), and transarterial radioembolization in 1 (4.5%)]. The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years 3.1 mo ( = 0.0001). In contrast to the New Delhi cohort, the Mumbai cohort of BCS-HCC patients were predominantly males, presented with a more advanced HCC [Barcelona Clinic Liver Cancer C and D], and 2 patients underwent liver transplantation.
HCC is not uncommon in patients with BCS. Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.
关于布加综合征（BCS）患者发生肝细胞癌（HCC）的文献较少。
评估BCS-HCC的发病规模、临床特征、治疗的可行性及治疗效果。
纳入了来自印度新德里的904例BCS患者以及来自印度孟买的1140例BCS患者。确定HCC的患病率和发病率，并对BCS-HCC患者进行介入治疗的可行性及治疗效果进行评估。
在新德里队列的35例BCS-HCC患者中，18例在首次就诊时即患有HCC（患病率1.99%），17例在4601人年的随访期内发生HCC[发病率为每100人年0.36（0.22 - 0.57）]。与单纯BCS患者相比，BCS-HCC患者年龄更大（P = 0.001），下腔静脉阻塞、肝硬化及长段血管阻塞的比例更高。首次就诊时BCS-HCC患者的甲胎蛋白中位数水平高于随访期内发生HCC的患者（13029 ng/mL对500 ng/mL，P = 0.01）。35例BCS-HCC患者中，26例（74.3%）接受了针对BCS的放射介入治疗，22例（62.8%）患者接受了HCC治疗[18例（81.8%）接受经动脉化疗栓塞，3例（13.6%）接受口服酪氨酸激酶抑制剂治疗，1例（4.5%）接受经动脉放射性栓塞治疗]。接受HCC介入治疗的患者与未接受治疗的患者相比，中位生存期为3.5年对3.1个月（P = 0.0001）。与新德里队列不同，孟买队列的BCS-HCC患者以男性为主，HCC病情更严重[巴塞罗那临床肝癌分期C和D期]，2例患者接受了肝移植。
HCC在BCS患者中并不少见。放射介入治疗和肝移植在部分原发性BCS-HCC患者中是可行的，且可能改善治疗效果。
