title: The treatment of hepatoblastoma: Its evolution and the current status as per the SIOPEL trials.
abstract: OBJECTIVE
  To describe the significant improvement in the diagnosis, treatment and outcome of children diagnosed with hepatoblastoma (HB) that has occurred in the past four decades. Recent findings are mainly focused on lessons learned from the experiences of the Childhood Liver Tumors Strategy Group (SIOPEL). Important milestones were the risk stratification of HB that allowed to tailor down therapy for standard-risk HB and intensify treatment for high-risk HB. The multi-institutional international cooperative SIOPEL trials are reviewed and current treatment guidelines are given. Intensified cooperation between the SIOPEL and the Children's Oncology Group (COG) and the national study groups from Germany (GPOH) and Japan (JPLT) led to the acceptance and use of one staging system (PRETEXT) and the formation of a single robust database containing data of 1605 HB patients. This will allow analysis with enough statistical power of treatment directing factors that will form one of the bases of the next-generation clinical trial that is currently designed by all four collaborating study groups.
  
  
  CONCLUSIONS
  Successive SIOPEL trials and increasing international collaboration have improved survival rates of patients with HB through risk stratification, advances in chemotherapy and increased complete resection rates including liver transplantation as a surgical option.
authors.affiliation: Department of Surgery, Division of Pediatric Surgery, Queen Elisabeth Central Hospital, University of Malawi, Blantyre, Malawi.
authors.forename: Daniel C
authors.initials: DC
authors.lastname: Aronson
authors.affiliation: Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Gdansk, Poland.
authors.forename: Piotr
authors.initials: P
authors.lastname: Czauderna
authors.affiliation: Department of statistics, International Breast Cancer Study Group (IBCSG) Coordinating Center, Berne, Switzerland.
authors.forename: Rudolf
authors.initials: R
authors.lastname: Maibach
authors.affiliation: Department of Paediatrics, Division of Paediatrics, University Hospital of Padua, Italy.
authors.forename: Giorgio
authors.initials: G
authors.lastname: Perilongo
authors.affiliation: Department of Paediatric Oncology, Birmingham Children's Hospital, Birmingham, United Kingdom.
authors.forename: Bruce
authors.initials: B
authors.lastname: Morland
keywords: Chemotherapy; PRETEXT; SIOPEL-studies; hepatoblastoma; liver transplantation; risk stratification; surgical resection