A multicenter prospective study on the management of hepatoblastoma in children:a report from the Chinese Children's Cancer Group  

作　　者：Meng-Jie Tang Xiao-Li Ma Xiang-Ling He Wei-Hua Pan Xiao-Hong Zhang Sha-Yi Jiang Ju Gao Fu Li Wei Yao Song Gu Wei-Ling Zhang Qiang Zhao Shi-Hao Huang Yong-Jun Fang Wei Liu Hui-Zhong Niu Chun-Mei Wang Li-Rong Sun Hui Gao Yun-Peng Dai Shun-Gen Huang Zhi-Yong Zhong Xi-Ge Wang Zhong-Rong Li Liang-Chun Yang Ye-Ming Wu Huan-Min Wang Xin Sun Xiao-Jun Yuan 

机构地区：[1]Department of Pediatric Hematology/Oncology,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,No.1665,Kongjiang Road,Yangpu District,Shanghai 200092,China [2]Medical Oncology Department,Pediatric Oncology Center,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China [3]Department of Hematology/Oncology,Children's Medical Center,Hunan Provincial People's Hospital,The First Affiliated Hospital of Hunan Normal University,Changsha 41005,China [4]Department of Pediatric Surgery,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China [5]Department of Hematology/Oncology,Guangzhou Women and Children's Medical Center,National Children's Medical Center for South Central Region,Guangzhou 510623,China [6]Department of Hematology,Shanghai Children's Hospital,Shanghai Jiao Tong University,Shanghai 200062,China [7]Department of Pediatrics,West China Second University Hospital,Sichuan University,Chengdu 610041,China [8]Department of Hematology/Oncology,Qilu Children's Hospital of Shandong University,Jinan 250022,China [9]Department of Pediatric Surgery,Children's Hospital of Fudan University,Shanghai 201102,China [10]Department of General Surgery,Shanghai Children's Medical Center(National Children's Medical Center-Shanghai),Shanghai Jiao Tong University School of Medicine,Shanghai 200127,China [11]Department of Pediatrics,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China [12]Department of Pediatric Oncology,Tianjin Medical University Cancer Institute and Hospital,Tianjin 300060,China [13]Department of Hematology/Oncology,Children's Hospital of Nanjing Medical University,Nanjing 210008,China [14]Department of Hematology/Oncology,Henan Children's Hospital,Zhengzhou 450018,China [15]Department of Pediatric General Surgery,Hebei Children's Hospital of Hebei Medical University,Shijiazhuang City 050031,China [16]Children's Hospital,the First Affliated Hospital of Zhengzhou Uni

出　　处：《World Journal of Pediatrics》2024年第6期590-601,共12页世界儿科杂志（英文版）

基　　金：supported by the Shanghai Municipal Hospital New Frontier Technology Joint Key Project,Shanghai,China(No.SHDC12019115).

摘　　要：Background This study aimed to identify survival risk factors in Chinese children with hepatoblastoma(HB)and assess the effectiveness of the new treatment protocol proposed by the Chinese Children's Cancer Group(CCCG)in 2016.Methods A multicenter,prospective study that included 399 patients with HB from January 2015 to June 2020 was con-ducted.Patient demographics,treatment protocols,and other related information were collected.Cox regression models and Kaplan-Meier curve methods were used.Results The 4-year event-free survival(EFS)and overall survival(OS)were 76.9 and 93.5%,respectively.The 4-year EFS rates for the very-low-risk,low-risk,intermediate-risk,and high-risk groups were 100%,91.6%,81.7%,and 51.0%,respec-tively.The 4-year 0S was 100%,97.3%,94.4%,and 86.8%,respectively.Cox regression analysis found that age,tumor rupture(R+),and extrahepatic tumor extension(E+)were independent prognostic factors.A total of 299 patients had complete remission,and 19 relapsed.Patients with declining alpha-fetoprotein(AFP)>75%after the first two cycles of neoadjuvant chemotherapy had a better EFS and OS than those≤75%.Conclusions The survival outcome of HB children has dramatically improved since the implementation of CCCG-HB-2016 therapy.Age≥8 years,R+,and E+were independent risk factors for prognosis.Patients with a declining AFP>75%after the first two cycles of neoadjuvant chemotherapy had better EFS and OS.

关 键 词：ALPHA-FETOPROTEIN HEPATOBLASTOMA MULTICENTER Prospective study SURVIVAL 

分 类 号：R725.8[医药卫生—儿科]