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

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...

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Main Authors: Peiyi Li, Yujia Kong, Lin Wan, Jing Guo, Weimin Li, Hui Zhang, Guang Yang, Bo Zhang
Format: Article
Language:English
Published: Wolters Kluwer Health/LWW 2023-06-01
Series:Journal of Pancreatology
Online Access:http://journals.lww.com/10.1097/JP9.0000000000000121
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author Peiyi Li
Yujia Kong
Lin Wan
Jing Guo
Weimin Li
Hui Zhang
Guang Yang
Bo Zhang
author_facet Peiyi Li
Yujia Kong
Lin Wan
Jing Guo
Weimin Li
Hui Zhang
Guang Yang
Bo Zhang
author_sort Peiyi Li
collection DOAJ
description 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 registries (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.
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spelling doaj.art-14c70128cd8b4c86894529421c3c1d582023-06-30T01:52:02ZengWolters Kluwer Health/LWWJournal of Pancreatology2096-56642023-06-0162616610.1097/JP9.0000000000000121202306000-00004Overall survival, late mortality, and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States, 1975–2018Peiyi Li0Yujia Kong1Lin Wan2Jing Guo3Weimin Li4Hui Zhang5Guang Yang6Bo Zhang7a Department of Anesthesiology, West China Hospital, Sichuan University, Chengdu, Sichuan, Chinac Department of Public Health, Weifang Medical University, Weifang, Shandong, Chinad Department of Pediatrics, First Medical Center, Chinese PLA General Hospital, Beijing, Chinaf Department of Health Policy and Management, School of Public Health, Peking University, Beijing, Chinag Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan, Chinai Department of Preventive Medicine, Feinberg School of Medicine, Northwestern University, Chicago, ILd Department of Pediatrics, First Medical Center, Chinese PLA General Hospital, Beijing, Chinak Department of Neurology, Boston Children’s Hospital, Harvard Medical School, Boston, MABackground:. 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 registries (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.http://journals.lww.com/10.1097/JP9.0000000000000121
spellingShingle Peiyi Li
Yujia Kong
Lin Wan
Jing Guo
Weimin Li
Hui Zhang
Guang Yang
Bo Zhang
Overall survival, late mortality, and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States, 1975–2018
Journal of Pancreatology
title Overall survival, late mortality, and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States, 1975–2018
title_full Overall survival, late mortality, and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States, 1975–2018
title_fullStr Overall survival, late mortality, and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States, 1975–2018
title_full_unstemmed Overall survival, late mortality, and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States, 1975–2018
title_short Overall survival, late mortality, and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States, 1975–2018
title_sort overall survival late mortality and cancer directed surgery among children and adolescents with ultra rare pediatric pancreatoblastoma in the united states 1975 2018
url http://journals.lww.com/10.1097/JP9.0000000000000121
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