Hepatocellular Carcinoma in Budd–Chiari Syndrome

Budd–Chiari syndrome (BCS)-associated hepatocellular carcinoma (HCC) is a rare condition; however, HCC may occur during the follow-up of a chronic BCS, as well as for other liver diseases. The prevalence of HCC varies widely, according to the geographical origin of the patients and the presence of other carcinogenesis factors. Patients with BCS, especially female ones, with long segment inferior vena cava (IVC) obstruction or both hepatic vein and IVC obstruction, chronic liver disease at the cirrhosis stage, and failure recovery of hepatic venous drainage are at a higher risk of developing HCC. The diagnosis of BCS-HCC may be difficult and requires an experienced radiologist, since the typical hallmarks of HCC are often lacking. The differential diagnosis between benign nodules and HCC is also challenging, and a misdiagnosis of HCC can impact the therapeutic management and the prognosis. Thus liver biopsy may be needed to confirm the diagnosis of HCC in these patients, and it is recommended if a nodule is heterogeneous or exceeds 3 cm in diameter. Alpha-fetoprotein (AFP) level can be a useful screening tool, more valuable for HCC in the BCS setting, compared to other liver diseases. Fifteen ng/ml is considered the best cut-off value of AFP for distinction between HCC and benign nodules.