17 Years of Pediatric Liver Transplantation Experience for Cirrhosis and Hepatocellular Carcinoma
Figen Ozcay1, Oya Balci Sezer1, Faik Sarialioglu2, Fatih Boyvat3, Mehmet Coskun4, Nihan Haberal Reyhan5, Mehmet Haberal6.
1Department of Pediatric Gastroenterology, Baskent University, Ankara, Turkey; 2Department of Pediatric Oncology, Baskent University, Ankara, Turkey; 3Department of Interventional Radiology, Baskent University, Ankara, Turkey; 4Department of Radiology, Baskent University, Ankara, Turkey; 5Department of Pathology, Baskent University, Ankara, Turkey; 6Department of General Surgery, Division of Transplantation, Baskent University, Ankara, Turkey
Objectives: The aim of the study was a retrospective analysis of the pediatric patients who had liver cirrhosis accompanied with hepatocellular carcinoma and were treated with liver transplantation.
Methods: A total of 14 children, 8 males and 6 females with chronic liver disease and hepatocellular carcinoma underwent a liver transplant at Baskent University Medical Faculty between 2004-2021. Pre-existing liver diseases were tyrosinemia in six, PFIC-2 in two, PFIC-3 in two, cryptogenic cirrhosis in two, Hepatitis B in one, and biliary atresia in one.
Results: The patients underwent LT at the age of 9.43 ± 4.9 years (13 months-16 y). Eight children had Child A,4 Child B and 2 had Child C cirrhosis. Two children received cadaveric grafts and 12 children received living donor grafts. Three children had solitary tumors,4 had 2 tumors,7 had multiple (3 or more) lesions. AFP levels were elevated in 11/14 patients. Six patients were classified as PRETEXT stage IV, three as II, five as I.HCC histologically classified as grade grade-1 in 10, and grade-2 in four patients. None of the patients had lymph node or vascular involvement.
Systemic chemotherapy combinations were given to 4 children before and to 3 children after LT. One child received trans-arterial chemoembolization and microwave ablation before LT. 3 children were converted to sirolimus. HCC recurrence was observed 23-47-108 months following LT in 3/14 (21%) patients. Recurrence sites were omentum in one, and graft liver in two. Hepatic resection-RFA-RT and RFA-CT were the treatment modalities in two patients for graft HCC recurrence. Relapse-free patient survival was 92%, 82.5%, and 72.2% at 2,4, and 10 years respectively. The death occurred in 4 out of 14 recipients (28.5%). Two of them were unrelated to HCC, due to infection, 19 and 188 months after LT. Two deaths after 79 and 165 months of LT were related to HCC recurrence.10 patients have been followed median of 178 months (range 13-204 months). The mean estimated survival time was 171.25 ±16.6 months. Overall patient survival was 100%, 92.3%, 92.3%, 83%, 72% at one-two-five-10- and 15 years after LT respectively.
Conclusion: HCC was mainly associated with inherited liver diseases in our pediatric series. Liver transplantation offered a remarkable survival advantage to children with cirrhosis and HCC in the long term.