Pediatric hepatoblastoma: diagnosis and treatment

Hepatoblastoma (HBL) is the most common primary liver tumor in children, and is usually diagnosed during the first 3 years of life. Collaborative multicenter studies have led to improved diagnostic and treatment strategies. The pretreatment extent of disease (PRETEXT) staging system has become a consensus classification, and an international pathological classification system has also been developed. Clinical trials examining multimodal therapy, which consists of complete surgical resection plus liver transplantation and chemotherapy, have led to improved outcomes for children with HBL. Recently, the Children's Hepatic Tumors International Collaboration (CHIC), which includes major multicenter study groups, created a shared database that merges data on all children underwent therapy in the clinical trials of these groups until 2008. CHIC has developed a global approach to risk stratification of pediatric HBL for use in future global clinical trials. The aim of this review is to report the recent developments on the diagnosis and treatment of pediatric HBL.