Hepatoblastoma state of the art: pathology, genetics, risk stratification, and chemotherapy.

PURPOSE OF REVIEW: As a rare pediatric tumor, hepatoblastoma presents challenges to the individual practitioner as no center will see more than a handful of cases each year.

RECENT FINDINGS: The Children's Hepatic tumor International Collaborative (CHIC) effort has fostered international cooperation in this rare children's tumor, leading to the establishment of a large international collaborative dataset, the CHIC database, which has been interrogated to refine risk stratification and inform treatment options. Apace with this effort has been the international collaboration of pediatric pathologists working together to establish a new international histopathologic consensus classification for pediatric liver tumors as a whole, with particular focus on the histological subtypes of hepatoblastoma.

SUMMARY: International collaborative efforts in hepatoblastoma have led to a new international histopathologic consensus classification, refinements in risk stratification, advances in chemotherapy, and a better understanding of surgical resection options forming the foundation for the development of an upcoming international therapeutic trial.