Abstract

This study aimed to evaluate the prognostic value of variables used in the 2017 PRE-Treatment EXTent of tumor (PRETEXT) system and the Children’s Hepatic tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) system in pediatric patients with hepatoblastoma. A retrospective analysis of data from the pediatric hepatoblastoma registry of a tertiary referral center was conducted to evaluate the clinical and imaging variables (annotation factors) of the PRETEXT staging system. The primary outcome was event-free survival (EFS). Data from 84 patients (mean age: 2.9 ± 3.5 years) identified between 1998 and 2017 were included. Univariable Cox proportional hazards analysis revealed that PRETEXT annotation factors P (portal vein involvement), F (multifocality of tumor), and M (distant metastasis) showed a significant negative association with EFS. Multivariable Cox proportional hazard analysis showed that factor F was the strongest predictor (HR (hazard ratio), 2.908; 95% CI (confidence interval), 1.061–7.972; p = 0.038), whereas factor M showed borderline significance (HR, 2.416; 95% CI, 0.918–6.354; p = 0.074). The prediction model based on F and M (F + M) showed good performance to predict EFS (C-statistic, 0.734; 95% CI, 0.612–0.854). In conclusion, the PRETEXT annotation factor F was the strongest predictor of EFS, and the F + M model showed good performance to predict EFS in pediatric patients with hepatoblastoma.

Highlights

  • Hepatoblastoma is the predominant hepatic malignancy in children [1]

  • Serum AFP concentration was missing in two patients who had been referred from another hospital

  • Among variables derived from the 2017 PRE-Treatment EXTent of tumor (PRETEXT), we found that annotation factors P, F, and M, and one or more of VPEFR were significant prognostic factors, which is in agreement with previous studies

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Summary

Introduction

Hepatoblastoma is the predominant hepatic malignancy in children [1]. Recent advances in diagnosis and treatment have improved patient outcomes and resulted in greater interest among healthcare professionals from a range of disciplines, surgery, oncology, gastroenterology, and radiology. Advances in surgical treatment and chemotherapeutic combinations have. Orthotropic liver transplantation has broadened the range of patients that can be cured surgically [3]. Complete surgical resection of the hepatoblastoma is most likely to effect a cure, and pretreatment evaluation by imaging is crucial to define the extent of the disease, stratify risk, and plan treatment. Key treatment guidelines use the PRE-Treatment EXTent of tumor (PRETEXT) system as part of the risk stratification and treatment planning process.

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