Abstract
Most pediatric liver tumors will be amenable to conventional resection; however, there is a small but real subset in whom resection is precluded by extensive liver involvement, multifocality, and/or unresectable involvement of the portal venous inflow or hepatic venous outflow to the remaining segments of the liver. In these children total hepatectomy with liver transplantation may be lifesaving and has become an integral part of current treatment algorithms for hepatoblastoma (HB). Transplantation in this setting has been particularly helpful in avoiding unnecessary attempts at intensifying chemotherapy in the vain efforts to achieve surgical resectability. As indications, and contraindications, for liver transplant in HB have become increasingly refined, outcomes from several institutional case series and multicenter cooperative studies now consistently report survival rates greater than 80%. Indications are less well developed for hepatocellular carcinoma (HCC) in children where liver transplantation is employed more sporadically. This is due in part to the heterogeneity of HCC presentations in children including incidental tumors in chronic liver conditions, large bulky de novo tumors in healthy livers, and more adult like tumors such as those arising in the context of cirrhosis and fibrolammelar tumors. In patients who have a liver transplant for other reasons and are found to have an incidental HCC, outcomes are uniformly good. More challenging are the children with otherwise normal livers who present with usually large de novo HCC tumors, especially when the size of the tumor exceeds adult established “Milan-type” criteria. Uncontrolled database analysis has suggested that in the absence of metastatic disease, transplantation could have a better outcome than conventional resection in this setting, and this is a group of patients in whom controlled research data is needed. Finally, liver transplant is used on a case-by-case basis, in the absence of any significant collaborative data, in other more rare primary pediatric primary hepatic malignancies including diffuse infantile hepatic hemangioma refractory to medical management, some hepatic sarcomas, and a few other more rare tumors. In this setting good results and survival rates have been shown when selection of patients is thoughtful and rigorous. Overall, cumulated experience with various types of tumors has shown that extrahepatic active tumor after chemotherapy is a formal contraindication, and good response of the main tumor site to preoperative chemotherapy, where applicable, is desirable.
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Meyers, R.L., de Ville de Goyet, J., Tiao, G.M. (2018). Liver Transplant for Cancer in Infants and Children. In: Dunn, S., Horslen, S. (eds) Solid Organ Transplantation in Infants and Children. Organ and Tissue Transplantation. Springer, Cham. https://doi.org/10.1007/978-3-319-07284-5_69
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