Progress in the surgical treatment of malignant liver tumors in children☆

Summary 

During the last decade, important progress has been made in the surgical treatment of malignant liver tumors in children. For hepatoblastoma, there is a general consensus for combining surgical resection with neoadjuvant (and adjuvant) chemotherapy. Long-term disease-free survival of around 85–90% can be achieved for resectable HB involving no more than three sections of the liver (PRETEXT I–III). For unresectable HB without extrahepatic invasion (PRETEXT IV with involvement of all four sections and some cases of PRETEXT III with invasion of, or close contact with major venous structures), similar results can be obtained with total hepatectomy and liver transplantation.

For hepatocellular carcinoma, most often without underlying liver disease in children of the western world, results of resection with partial hepatectomy remain dismal, due to a high rate of recurrence. In contrast, remarkable survival rates have been obtained during the last decade with liver transplantation. There is no argument, either biological or based on evidence, that the selection of pediatric candidates for transplantation should be based on the same criteria as in adult patients (the Milan criteria).

Optimization of results require to concentrate children with a malignant liver tumors in specialized, multidisciplinary pediatric centers with expertise in chemotherapy and in both major liver resections and transplantation.

Enrolling these children in prospective trials should be encouraged, as well as prospective registration of transplanted patients in PLUTO (Pediatric Liver Unresectable Tumor Observatory-http://Pluto.cineca.org) in order to clarify issues unresolved by retrospective studies.

Keywords: Malignant liver tumors, Hepatoblastoma, Hepatocellular carcinoma, Chemotherapy, Surgical resection, Liver transplantation