Hepatocellular Carcinoma Associated with Hepatic Venous Outflow Tract Obstruction: A Rare Entity

Abstract

Background and aims: Hepatocellular carcinoma (HCC) associated with hepatic venous outflow tract obstruction (HVOTO) is a rare entity that has recently gained recognition due to availability of interventional therapeutic options for HVOTO causing prolonged survival. We depict the clinical, demographical profile and management of these complex cases at our centre. Methods: Between 1989 and 2013, diagnosed patients of HVOTO-HCC were studied. They were subjected to ultrasound Doppler, multiphase CT liver (MPCT)/dynamic MRI/venography. Clinical, biochemical, imaging findings, treatment details were obtained from the case records. HVOTO treatment was based on the site/type of venous obstruction while HCC treatment was based on the Barcelona Clinic liver cancer Staging. Treatment response of HVOTO and HCC was assessed. Results: Sixteen HVOTO-HCC patients (10 females, 6 males) of mean age of 38 + 11.5 years were studied. Pain was their commonest presenting symptom 10/16 (62.5%). No signs of hepatic decompensation were noted. AFP was markedly elevated (mean 43447.1 ng/ml). On MPCT, all had cirrhosis, mean mass size was 6.2 + 4.7 cm, 14/16 (87.5%) were well-defined, had necrosis 10/16 (62.5%), capsulated 11/16 (68.7%) and predominantly exophytic 10/16 (62.5%). Combined inferior vena cava (IVC) + hepatic vein(HV) block was commonest 12/16 (75%) followed by isolated IVC or HV block in 2 (12.5%) patients each. For HVOTO, 12 patients received therapy [Transjugular intrahepatic portosystemic shunt 1, IVC angioplasty 5, IVC angioplasty + stenting1, HV angioplasty + stenting 3, IVC + HV angioplasty 2]. Complete (CR)/partial response (PR) was achieved in 10 patients, one procedure failed and in one response could not be assessed. For coexistent HCC, 12 patients were treated [Trans-arterial chemoembolization (TACE) 5, TACE plus acetic acid ablation 3, TACE followed by transarterial chemotherapy (TAC) 1, TAC 2 and oral chemotherapy 1]. Tumor response could be assessed in 9 patients (CR 5, PR 4). Conclusion: HVOTO-HCC has unique clinical and imaging features. Treatment requires great skill and expertise as it is mandatory to treat both HVOTO and HCC for an effective outcome.