Abstract

ObjectiveThe aim of this study was to compare the accuracy of the Children’s Oncology Group (COG) risk stratification system to the Children’s Hepatic tumor International Collaboration (CHIC) risk stratification system for predicting the prognosis of Chinese children with hepatoblastoma (HB).MethodsClinicopathological data of 86 patients diagnosed with HB between January 2014 and December 2017 were retrieved. The study endpoints were the 1- and 3-year overall survival (OS) and disease-free survival (DFS) were analyzed to evaluate the predictive value.ResultsThe 1-, 3-year OS and DFS of the 86 patients were 86.0%, 76.3%, and 74.4%, 54.0%, respectively. Univariate analyses revealed that age at diagnosis had a significant role in prognosis for both OS and DFS, along with PRETEXT staging and metastasis at diagnosis. Multivariate analysis showed that metastasis at diagnosis (HR 3.628, 95% CI 1.404-9.378, P=0.008), PRETEXT staging system (HR 2.176, 95% CI 1.230-3.849, P=0.008) and age at diagnosis (HR 2.268, 95% CI 1.033-4.982, P=0.041) were independent factors for OS. For DFS, the independent factors were the PRETEXT staging system (HR 2.241, 95% CI 1.533-3.277, P<0.001) and age at diagnosis (HR 1.792, 95% CI 1.018-3.154, P=0.043). Both COG and CHIC risk stratification systems could effectively predict the prognosis of children with HB for OS. For DFS, the CHIC risk stratification system was more effective. In addition, the CHIC risk stratification system had a higher c-index (OS 0.743, DFS 0.730), compared to the COG risk stratification system (OS 0.726, DFS 0.594).ConclusionAge at diagnosis played a significant role in prognosis. Compared to the COG risk stratification system, the CHIC risk stratification system was superior in predicting the survival of Chinese children with HB.

Highlights

  • HB is a rare childhood tumor with an annual incidence of 1.5 cases per million children, occurring predominantly in the first 2 years of life [1]

  • After accumulating and analyzing large amounts of data, these organizations proposed that the small cell undifferentiated (SCU) histology and low serum AFP at diagnosis could be associated with adverse outcomes in survival [9, 10] and have been constantly investigating novel prognostic factors [11,12,13,14]

  • Multivariate analysis showed that metastasis at diagnosis (HR 3.628, 95% CI 1.404-9.378, P=0.008), PRETEXT staging (HR 2.176, 95% CI 1.230-3.849, P=0.008) and age at diagnosis (HR 2.268, 95% CI 1.033-4.982, P=0.041) were independent prognostic factors for overall survival (OS) (Table 3)

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Summary

Introduction

HB is a rare childhood tumor with an annual incidence of 1.5 cases per million children, occurring predominantly in the first 2 years of life [1]. Its overall survival in the past three decades has increased from ~30% to ~70% [2, 3]; largely contributed due to improvement in imaging technology, surgical methods, timely surgeries, and chemotherapy prescription [4,5,6]. Milestone developments for the risk stratification of HB still lag behind more common pediatric cancers [7]. Four separate cooperative multicenter trial groups have performed systematic treatment studies in HB, namely, the International Childhood Liver Tumor Strategy Group (SIOPEL); the Children’s Oncology Group (COG), and its legacy groups the Children’s Cancer Group (CCG) and the Pediatric Oncology Group (POG); the German Society for Pediatric Oncology and Haematology (GPOH); and the Japanese Study Group for Pediatric Liver Tumors (JPLT) [8]. As the stratification criteria used in each group are different, it is not possible to compare the prognosis of each group clearly

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