S5953 De Novo Hepatocellular Carcinoma 30 Years Post-Living Donor Liver Transplant in a Patient With Biliary Atresia

Introduction: De-novo hepatocellular carcinoma (HCC) following liver transplantation is extremely rare, typically occurring in the context of viral hepatitis, cirrhosis or recurrence of underlying liver disease. We present the first known case of de novo HCC occurring 30 years after pediatric living donor liver transplant (LDLT) for biliary atresia, in the absence of hepatitis B or C and without established cirrhosis. Case Description/Methods: A 30-year-old woman with a history of LDLT from her biological mother at 3 months of age for biliary atresia following failed Kasai procedure, presented with nausea, vomiting, unintentional weight loss and abdominal pain for a few weeks. Her post-transplant course was complicated by early multiple vascular thromboses requiring shunting procedures, splenectomy, chronic rejection and autoimmune hepatitis. She was being monitored with routine lab tests every 6 months that had been normal previously. At the time of this presentation, Lab results showed elevated alpha-fetoprotein of 105 ng/mL and total bilirubin of 3.9 mg/dL, and features of cholestatic hepatitis. MRI demonstrated a new 4.3 cm arterially enhancing mass in hepatic segment VIII concerning for adenoma vs HCC. Given her long term use of Immunosuppression and elevated AFP, though she lacked any other risk factors, a biopsy of the mass was performed that showed HCC; biopsied of non-mass allograft showed features of biliary obstruction, neutrophilic abscesses and stage 2-3 fibrosis. After completing antibiotics which were initiated for the abscesses noted on biopsy, her bilirubin normalized. Surgical resection was not feasible due to complex vascular anatomy. The patient is planned to undergo Y-90 radioembolization and was evaluated and approved for repeat liver transplantation. Discussion: While HCC recurrence occurs in up to 18.3% of liver transplant recipients, de novo HCC is exceedingly rare with only a few cases reported in the literature. It is usually associated with viral hepatitis or cirrhosis of the allograft. This case represents the longest reported interval between LDLT and the development of de novo HCC (30 years) that we are aware of. It underscores the need for high clinical suspicion when patients present with sudden onset of constitutional symptoms and abnormal imaging, even in the absence of traditional risk factors.