Overall survival,late mortality,and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States,1975-2018  

作　　者：Peiyi Li Yujia Kong Lin Wan Jing Guo Weimin Li Hui Zhang Guang Yang Bo Zhang 

机构地区：[1]Department of Anesthesiology,West China Hospital,Sichuan University,Chengdu,Sichuan,China [2]Laboratory of Anesthesia and Critical Care Medicine,National-Local Joint Engineering Research Centre of Translational Medicine of Anesthesiology,West China Hospital,Sichuan University,Chengdu,Sichuan,China [3]Department of Public Health,Weifang Medical University,Weifang,Shandong,China [4]Department of Pediatrics,First Medical Center,Chinese PLA General Hospital,Beijing,China [5]Senior Department of Pediatrics,Seventh Medical,Center,PLA General Hospital,Beijing,China [6]Department of Health Policy and Management,School of Public Health,Peking University,Beijing,China [7]Department of Respiratory and Critical Care Medicine,West China Hospital,Sichuan University,Chengdu,Sichuan,China [8]Institute of Respiratory Health,Frontiers Science Center for Disease-related Molecular Network,West China Hospital,Sichuan University,Chengdu,Sichuan,China [9]Department of Preventive Medicine,Feinberg School of Medicine,Northwestern University,Chicago,IL [10]The Second School of Clinical Medicine,Southern Medical University,Guangzhou,China [11]Department of Neurology,Boston Children’s Hospital,Harvard Medical School,Boston,MA [12]Biostatistics and Research Design Center,Institutional Centers for Clinical and Translational Research,Boston Children’s Hospital,Harvard Medical School,Boston,MA

出　　处：《Journal of Pancreatology》2023年第2期61-66,共6页胰腺病学杂志（英文）

基　　金：Dr Peiyi Li’s research is in part supported by the National Natural Science Foundation of China(Grant No.72207174);Dr Yujia Kong’s research is in part supported by the National Nature Science Foundation of China(Grant No.20003560);Natural Science Foundation of Shandong Province(Grant No.ZR2020MH340);Undergraduate Education Reform Research Project of Shandong Province(Grant No.M2021174).

摘　　要：Background:Pediatric pancreatoblastoma is an extremely rare malignant tumor,posing diagnostic and treatment difficulties for pediatric surgeons.Using the Surveillance,Epidemiology,and End Results(SEER)database,we present an up-to-date report of the epidemiology,clinicopathological features,survival rates,and prognosis of pancreatoblastoma in pediatric patients.Methods:All pediatric patients diagnosed with pancreatoblastoma between 1975 and 2018 were identified in the SEER regis-tries(SEER 8 registries and SEER 17 registries).We conducted a survival analysis to assess overall survival and 1-and 5-year late mortality rates.Descriptive statistics and log-rank test were performed.Results:A total of 22 children and adolescents with pancreatoblastoma were identified.In this cohort,12 of 22 were male(54.55%),14 were White(63.64%),and 11 were diagnosed between the ages of 1 and 4 years(50.0%).Among the 22 patients,11(50.0%)had distant metastases,whereas 7(31.82%)had localized,and 4(18.18%)had a regional disease.A total of 5 children and adolescents died during the study period,with cumulative survival rates of 14 of 17(82.35%)and 10 of 11(90.95%)among 1-and 5-year survivors,respectively.Cancer-directed surgery was significantly associated with an increased life expectancy(log-rank test,P=.018).Conclusion:Pediatric pancreatoblastoma is a rare entity.Cases that underwent surgery had a greater likelihood of overall survival and reduced late mortality.

关 键 词：MORTALITY Overall survival Pancreatic cancer surgery PANCREATOBLASTOMA Pediatric pancreatic cancer 

分 类 号：R735.9[医药卫生—肿瘤]