Validation of the stratification for newly diagnosed hepatoblastoma: An analysis from the Children’s Hepatic tumors International Collaboration (CHIC) database

Abstract

Introduction: The Children’s Hepatic Tumors International Collaboration (CHIC) previously developed the CHIC hepatoblastoma risk stratification (CHIC-HS) model by analyzing the original CHIC dataset (development set). A validation of the results was planned with data from contemporary trials that were ongoing at the time of the initial analysis. Material and methods: Subsequent analysis was performed on the contemporary trials (validation set) SIOPEL 4, SIOPEL 6, COG AHEP0731, and JPLT 2 (2011–2013 cohort) utilizing the same methodology as in the CHIC-HS development. Results: 403 patients were available for analysis for validation of the CHIC-HS model. Clinical characteristics were comparable between the validation and development sets. Event free survival (EFS) was similar in the very low and low-risk CHIC-HS strata in both the development and validation sets. EFS was higher in the intermediate and high-risk strata in the validation set as compared with the development set consistent with improved survival of intermediate-risk patients on AHEP0731 and high-risk patients on SIOPEL 4. Conclusions: The four risk groups in the CHIC-HS retain their relevance for risk stratification in this analysis, notwithstanding improved outcomes in patients with higher risk hepatoblastoma; therefore, the CHIC-HS remains a valid model which differentiates patients into statistically distinct risk strata. The model will be further evaluated with data from the current pediatric international hepatic tumors trial (PHITT).

Keywords

• Hepatoblastoma
• Risk stratification