Introduction: Non-hepatoblastoma pediatric liver tumors represent a diverse group of neoplasms with variable outcomes. Unlike hepatoblastoma, this heterogeneous group may not have clearly defined prognostic indicators, evidence-based therapeutic regimens, or data on liver transplantation. Extrahepatic disease adds complexity and increased risk. The decision for transplantation should be guided by a comprehensive multidisciplinary approach.  

Methods: We present two cases of pediatric patients with non-hepatoblastoma tumors, each with extrahepatic disease, posing significant challenge to the feasibility of liver transplantation. The first case is a pediatric patient with fibrolamellar hepatocellular carcinoma (HCC) refractory to standard chemotherapy who was initially listed for liver transplantation but found to have locoregional lymph node invasion on exploration. After treatment with triple chemoimmunotherapy and transarterial chemoembolization, favorable response led to reevaluation for transplant. The second case is a pediatric patient with hepatocellular neoplasm NOS with tumor extension into the inferior vena cava (IVC) and right atrium. Management with a hepatoblastoma-based treatment protocol led to a decrease in tumor burden but persistent and extensive vascular invasion. Liver transplant evaluation required comprehensive planning with cardiothoracic considerations and analysis of risks associated with vascular invasion.  

Results: Both patients underwent successful liver transplantation without known disease recurrence.

Conclusion: These cases highlight the importance of a comprehensive evaluation and a multidisciplinary approach to the consideration of liver transplant for pediatric non-hepatoblastoma liver cancers with extrahepatic involvement.