Development of Hepatocellular Carcinoma in Type IB Abernethy Malformation

Abernethy malformation, a congenital extrahepatic portosystemic shunt, allows portal venous blood to bypass the liver and has been shown to predispose patients to hepatic tumors. We present a 29-year-old man with Type IB Abernethy malformation diagnosed in infancy who developed hepatocellular carcinoma in a noncirrhotic liver. Surgical resection, the standard curative therapy, was not feasible due to complex vascular anatomy, and he was treated with stereotactic body radiation therapy. Post-treatment imaging demonstrated sustained improvement. This case highlights the malignant potential of congenital portosystemic shunts and the importance of long-term surveillance.