Hepatocellular carcinoma in Budd-Chiari syndrome: A single center experience with long-term follow-up in South Korea

Table 1 Baseline clinical characteristics of patients with Budd-Chiari syndrome (n = 67)

Characteristics	Budd-Chiari syndrome
Age (yr)	47 ± 16
Gender (male)	34 (50.7)
Obstruction site
IVC	56 (83.6)
Hepatic vein	5 (7.5)
Combined	6 (9.0)
Alcohol consumption
None/social/heavy (> 80 g/d)	32 (47.8)/15 (22.3)/20 (29.9)
Positive viral marker
HBsAg/anti-HCV	3 (4.5)/0 (0)
Liver cirrhosis at diagnosis	54 (80.6)
Child-Pugh A/B/C	25/23/6 (37.3/34.3/9.0)
MELD score	11 ± 6
Decompensate LC symptoms	23 (34.3)
Length of obstruction (cm)	2.0 ± 4.0
HVPG (mmHg)	15 ± 10
Laboratory data
ALT (IU/L)	19 ± 17
Bilirubin (mg/dL)	1.4 ± 1.3
Albumin (g/dL)	3.8 ± 0.7
Hemoglobin (g/dL)	12.3 ± 2.6
Platelet count (k/μL)	109 ± 78
Creatinine (mg/dL)	0.9 ± 0.2
PT (%)	73 ± 27
Treatment modality
Angioplasty	27 (40.3)
Shunt operation	4 (5.9)
TIPS	3 (4.5)
Thrombolysis	1 (1.5)
Symptomatic medical treatment	32 (47.7)
Median follow up period (mo)	103 ± 156

Data were expressed as median ± IQR or n (%). IVC: Inferior vena cava; HCV: Hepatitis C virus; HbsAg: Hepatitis B surface antigen; MELD: Model for End-Stage Liver Disease; LC: Liver cirrhosis; HVPG: Hepatic venous pressure gradient; ALT: Alanine aminotransferase; PT: Prothrombin time; TIPS: Transjugular intrahepatic portosystemic shunt; IQR: Interquartile range.

Table 2 Characteristics of the patients with hepatocellular carcinoma at the time point of diagnosis (n = 17)

Variables	HCC
Age (yr)	53 ± 12
Time period from BCS to HCC (mo)	51 ± 115
Child-Pugh class
A/B/C	6 (35.3)/8 (47.1)/3 (17.6)
Tumor stage (AJCC 6th)1
I/II/III/IV	8 (47.1)/6 (35.3)/3 (17.6)/0 (0)
Treatment modality
TACE/TACI	9 (52.9)
Intra-arterial chemotherapy	3 (17.6)
Conservative management	3 (17.6)
Operation	2 (11.9)
Prognosis
Alive	12 (70.5)
Death	3 (17.6)
F/U loss	2 (11.9)

Data were expressed as median ± IQR or n (%). BCS: Budd-Chiari syndrome; HCC: Hepatocellular carcinoma; TACE: Transarterial chemo-embolization; TACI: Transarterial chemo-infusion; F/U: Follow up; IQR: Interquartile range.

¹AJCC 6th, American Joint Committee on Cancer staging system, 6th edition.

Table 3 Comparison of baseline clinical characteristics between hepatocellular carcinoma group and non hepatocellular carcinoma group

Variables	HCC(n = 17)	Non HCC(n = 50)	P value
Age (yr)	47 ± 11	47 ± 18	0.863
Gender (male)	10 (58.8)	24 (48)	0.441
Obstructive site			0.264
IVC	15 (88.2)	41 (82)
Hepatic vein	2 (11.8)	3 (6)
Combined	0 (0)	6 (12)
Alcohol consumption			0.329
None	6 (35.3)	26 (52.0)
Social	4 (23.5)	11 (22.0)
Heavy (> 80 g/d)	7 (41.2)	13 (26.0)
Positive for HBsAg	0 (0)	3 (6.0)	0.554
LC at diagnosis	14 ( 82.4)	40 (80.0)	0.832
Decompensate LC symptoms	8 (47.1)	15 (28.0)	0.148
Child Pugh A/B/C	5/7/2	20/16/4	0.647
MELD score	11 ± 6	11 ± 6	0.778
Follow up period (mo)	103 ± 146	103 ± 160	0.648
Length of obstruction (cm)	1.0 ± 2.5	2.0 ± 4.4	0.144
HVPG (mmHg)	21 ± 12	14 ± 7	0.019
Laboratory data
ALT (IU/L)	21 ± 21	18 ± 14	0.160
Bilirubin (mg/dL)	1.5 ± 1.9	1.3 ± 1.3	0.521
Albumin (g/dL)	3.6 ± 0.85	3.8 ± 0.72	0.245
Hemoglobin (g/dL)	12.5 ± 4.1	12.3 ± 2.3	0.897
Platelet count (k/μL)	97 ± 59.5	113 ± 80.7	0.559
Creatinine (mg/dL)	0.9 ± 0.14	0.85 ± 0..24	0.798
PT (%)	71 ± 31	74 ± 22	0.756
Survival (%)			< 0.001
5 yr	79	93.4
10 yr	43.1
15 yr	21.5	74.7

Data were expressed as median ± IQR or n (%). HCC: Hepatocellular carcinoma; IVC: Inferior vena cava; LC: Liver cirrhosis; MELD: Model for End-Stage Liver Disease; HVPG: Hepatic venous pressure gradient; ALT: Alanine aminotransferase; PT: Prothrombin time; IQR: Interquartile range.

Table 4 Prevalence and characteristics of hepatocellular carcinoma in patients with Budd-Chiari syndrome from the literature

Ref.	Matsui et al[3]	Shin et al[24]	Moucari et al[7]	Gwon et al[6]
Year, country	2000, Japan	2004, South Korea	2008, France	2010, South Korea
Study design	Retrospective	Retrospective	Retrospective	Retrospective
Study period	Apr 1968-Feb 1999	Mar 1989-Aug 2001	1987-2005	Mar 1990-Nov 2008
No. of patient with BCS	12	73	97	98
HCC (%)	3 (25)	15 (20.5)	11 (11.3)	23 (23)
Cumulative incidence of HCC	-	-	4 yr 3%	1 yr 7.3%
			7 yr 6%	5 yr 13.5%
			14 yr 12%	10 yr 31.8%
Tx. for HCC	Resection (1)	TACE (11)	TACE (7)	TACE (20)
	TAE (1)	Resection (2)	LT (3)	TACE + LT (3)
	iA chemotherapy (1)	Conservative tx. (2)	Conservative tx. (1)
Survival rate of HCC in patients with BCS
Median survival period (mo)	-	58 (range, 3-59)	-	-
Cumulative survival	-	1 yr 93%	-	1 yr 90%
		2 yr 84%		2 yr 85%
		3 yr 72%		3 yr 61%
				4 yr 61%
				5 yr 46%
Risk factors for HCC in patients with BCS
Risk factors	Chronic congestion in the liver, caused by an outflow block of hepatic veins	-	Male gender,	Female gender2
			Coagulopathy1,
			IVC obstruction
Analysis method	No statistical analysis (mere presumption)	-	Univariate analysis	Multivariate analysis

HCC: Hepatocellular carcinoma; BCS: Budd-Chiari syndrome; Tx.: Treatment; TAE: Transarterial embolization; TACE: Transarterial chemoembolization; iA: Intra-arterial; LT: Liver transplantation.

¹Coagulopathy harbored factor V Leiden;

²Female gender showed an odds ratio of 6.02 with P < 0.001 in Cox regression analysis; IVC: Inferior vena cava.