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©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Mar 15, 2024; 16(3): 699-715
Published online Mar 15, 2024. doi: 10.4251/wjgo.v16.i3.699
Clinical profile and outcomes of hepatocellular carcinoma in primary Budd-Chiari syndrome
Ankit Agarwal, Sagnik Biswas, Shekhar Swaroop, Arnav Aggarwal, Ayush Agarwal, Gautam Jain, Anshuman Elhence, Arun Vaidya, Amit Gupte, Ravi Mohanka, Ramesh Kumar, Ashwani Kumar Mishra, Shivanand Gamanagatti, Shashi Bala Paul, Subrat Kumar Acharya, Akash Shukla, Shalimar
Ankit Agarwal, Sagnik Biswas, Shekhar Swaroop, Arnav Aggarwal, Ayush Agarwal, Anshuman Elhence, Subrat Kumar Acharya, Shalimar, Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, New Delhi 110029, Delhi, India
Gautam Jain, Arun Vaidya, Akash Shukla, Department of Gastroenterology, Seth Gordhandas Sunderdas Medical College and KEM Hospital, Mumbai 400012, Maharashtra, India
Amit Gupte, Department of Gastroenterology, Sir HN Reliance Foundation Hospital, Mumbai 400004, India
Ravi Mohanka, Department of Liver Transplant and HPB, Sir HN Reliance Foundation Hospital, Mumbai 400004, India
Ramesh Kumar, Department of Gastroenterology, All India Institute of Medical Sciences, Patna 801507, India
Ashwani Kumar Mishra, Professor of Biostatistics, National Drug Dependence Treatment Centre (NDDTC), All India Institute of Medical Sciences, New Delhi 110029, India
Shivanand Gamanagatti, Shashi Bala Paul, Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi 110029, Delhi, India
Author contributions: Agarwal A and Biswas S designed and conducted the study, acquired the data, performed analysis and interpretation of data, and drafted the manuscript; Swaroop S, Aggarwal A, Agarwal A, Jain G, Vaidya A, Gupte A, Mohanka R, and Kumar R acquired the data and revised the manuscript; Mishra AK undertook the statistical analysis, interpretation of data, and revision of the manuscript; Elhence A, Gamanagatti S, Paul SB, Acharya SK, and Shukla A acquired the data and revised the manuscript; Shalimar conceptualized and designed the study and provided administrative, technical, or material support and study supervision, interpretation of data, critical revision, and final approval of the manuscript.
Institutional review board statement: This study was reviewed and approved by the Ethics Committee of the All India Institute of Medical Sciences, New Delhi (Approval No. IEC/NP-458/12.12.2014, RP 22-2015).
Informed consent statement: Written informed consent was waived (de-identified data).
Conflict-of-interest statement: We have no financial relationships to disclose.
Data sharing statement: No additional data are available.
STROBE statement: The authors have read the STROBE Statement—checklist of items, and the manuscript was prepared and revised according to the STROBE Statement—checklist of items.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
https://creativecommons.org/Licenses/by-nc/4.0/ Corresponding author: Shalimar, MD, Professor, Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, Sri Aurobindo Marg, Ansari Nagar, New Delhi 110029, Delhi, India. drshalimar@yahoo.com
Received: October 3, 2023
Peer-review started: October 3, 2023
First decision: December 5, 2023
Revised: December 22, 2023
Accepted: January 16, 2024
Article in press: January 16, 2024
Published online: March 15, 2024
BACKGROUND
There is scant literature on hepatocellular carcinoma (HCC) in patients with Budd-Chiari syndrome (BCS).
AIM
To assess the magnitude, clinical characteristics, feasibility, and outcomes of treatment in BCS-HCC.
METHODS
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.
RESULTS
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 (P = 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 vs 500 ng/mL, P = 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 vs 3.1 mo (P = 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.
CONCLUSION
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.
Core Tip: Hepatocellular carcinoma is not uncommon in patients with Budd-Chiari syndrome. It may be the presenting feature or may develop later during illness. Early diagnosis and intervention are the keys to improving outcomes. Strategies for surveillance include serial alpha-fetoprotein and ultrasound assessment every 6 mo with biopsy in cases with high clinical suspicion. Endovascular intervention, which improves liver functions, is usually done prior to therapy for hepatocellular carcinoma. Liver transplantation and surgical resection have curative potential, while locoregional therapy may be offered to a select group with more advanced disease, which improves outcomes in these patients.
