BACKGROUND: Frequency of hepatocellular carcinoma (HCC) in hepatic venous outflow tract obstruction (HVOTO) is unclear and risk factors in HVOTO associated with HCC are unknown. AIM: To assess the incidence of HCC and to identify risk factors for HCC in primary HVOTO. METHODS: In the consecutive primary HVOTO patients evaluated between 1989 to 2013, the incidence of HCC among HVOTO was assessed in a retrospective cohort study and identification of the risk factors for HCC in HVOTO patients done by a case-control study. RESULTS: Of the 421 HVOTO patients, 8 had HCC at presentation (prevalence 1.9%). Another 8 of the remaining 413 developed HCC during 2076.2 person-years follow-up (mean 5.03 + 4.65 years, range 0.08-20 years). The cumulative incidence of HCC was 3.5% (95% CI 1.28-9.2%) at 10 years. The case-control study included 16 HCC as cases and remaining 405 as controls. Controls were predominantly males (M:F - 230:175), mean age 29 ± 10.3 years. Cases were predominantly females with an older age of 36.2 ± 11.4 years (P < 0.01, OR = 1.06, CI 1.0-1.10%). Presence of cirrhosis (P < 0.001), combined inferior vena cava (IVC) and hepatic vein (HV) block (P < 0.03, OR = 5.58, CI 1.43-25.30%) and long-segment IVC block (P < 0.02, OR = 6.50, CI 1.32-32.0%) were significantly higher among cases than controls. CONCLUSIONS: Hepatic venous outflow tract obstruction is a risk factor for HCC. The cumulative incidence of HCC in HVOTO is low and progressively increases over time. Those with liver cirrhosis, combined IVC and HV block and long-segment IVC block are at risk to develop HCC and need active surveillance.
BACKGROUND: Frequency of hepatocellular carcinoma (HCC) in hepatic venous outflow tract obstruction (HVOTO) is unclear and risk factors in HVOTO associated with HCC are unknown. AIM: To assess the incidence of HCC and to identify risk factors for HCC in primary HVOTO. METHODS: In the consecutive primary HVOTO patients evaluated between 1989 to 2013, the incidence of HCC among HVOTO was assessed in a retrospective cohort study and identification of the risk factors for HCC in HVOTO patients done by a case-control study. RESULTS: Of the 421 HVOTO patients, 8 had HCC at presentation (prevalence 1.9%). Another 8 of the remaining 413 developed HCC during 2076.2 person-years follow-up (mean 5.03 + 4.65 years, range 0.08-20 years). The cumulative incidence of HCC was 3.5% (95% CI 1.28-9.2%) at 10 years. The case-control study included 16 HCC as cases and remaining 405 as controls. Controls were predominantly males (M:F - 230:175), mean age 29 ± 10.3 years. Cases were predominantly females with an older age of 36.2 ± 11.4 years (P < 0.01, OR = 1.06, CI 1.0-1.10%). Presence of cirrhosis (P < 0.001), combined inferior vena cava (IVC) and hepatic vein (HV) block (P < 0.03, OR = 5.58, CI 1.43-25.30%) and long-segment IVC block (P < 0.02, OR = 6.50, CI 1.32-32.0%) were significantly higher among cases than controls. CONCLUSIONS:Hepatic venous outflow tract obstruction is a risk factor for HCC. The cumulative incidence of HCC in HVOTO is low and progressively increases over time. Those with liver cirrhosis, combined IVC and HV block and long-segment IVC block are at risk to develop HCC and need active surveillance.