Abstract
INTRODUCTION: Hepatoblastoma with tumor thrombi involving three hepatic veins or extending into the inferior vena cava (IVC) toward the right atrium is mostly considered unresectable. In the current era of liver transplantation for unresectable hepatoblastoma with stable outcomes, it is challenging to choose extreme hepatectomy with hypothermic perfusion under total hepatic vascular exclusion. We herein report a successful case of extreme liver resection with in situ hypothermic perfusion. CASE PRESENTATION: A 10-month-old girl was referred for an unresectable hepatoblastoma (alpha-fetoprotein [AFP] 592037 ng/mL), approximately 12 cm in size, occupying the right lobe with a tumoral thrombus extending into the IVC toward the right atrium. The tumor continued to involve three hepatic veins and the IVC after intensive chemotherapy, which was categorized into POSTTEXT III (P1, V3, M0) staging. We planned an ante situm technique or in situ hypothermic perfusion to accomplish the complete removal of the large tumor and venous reconstruction. The adopted surgical technique was as follows: (1) hepatic partition on the line of extended right hepatectomy, including segment Iva; (2) mobilization of the suprahepatic IVC with tumor thrombi; (3) insertion of a cannula for hypothermic perfusion was from the stump of the right portal vein to the left lobe; (4) complete occlusion of the hepatic inflow following systemic heparinization with cutting the left hepatic vein and hypothermic perfusion with cold preservation solution using crushed ice; (5) complete tumor removal with the IVC tumoral thrombi; and (6) reconstruction of the IVC and left hepatic vein using an artificial vessel graft without requiring ante situm position, followed by reperfusion of the remnant liver. Time for cooling and preservation of the remaining liver in the body was 40 minutes, and time for IVC reconstruction was 21 minutes. The histological examination was margin-negative, and the AFP level was normalized. CONCLUSIONS: Resection of liver tumors invading the IVC or hepatic veins has become possible with technical lessons learned from liver transplantation skills. This procedure is a realistic option for achieving surgical cure and improving the quality of life in select pediatric patients with otherwise unresectable hepatoblastomas.