Open AccessThe treatment of hepatoblastoma: Its evolution and the current status as per the SIOPEL trials
Author(s) -
Daniël C. Aronson,
Piotr Czauderna,
Rudolf Maibach,
Giorgio Perilongo,
Bruce Morland
Publication year - 2014
Publication title -
journal of indian association of pediatric surgeons
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.306
H-Index - 17
eISSN - 1998-3891
pISSN - 0971-9261
DOI - 10.4103/0971-9261.142001
Subject(s) - hepatoblastoma , medicine , liver transplantation , risk stratification , clinical trial , transplantation , pediatrics , oncology , intensive care medicine
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.