Chitrakshi Nagpal, Shuvadeep Ganguly, Archana Sasi, Vivek Kumar, Bivas Biswas, Deepam Pushpam, Akash Kumar, Sandeep Agarwala, Vishesh Jain, Anjan Dhua, Devender Kumar Yadav, Shah Alam Khan, Adarsh Barwad, Asit Ranjan Mirdha, Ahitagni Biswas, Sanjay Thulkar, Sameer Bakhshi
{"title":"Ewing sarcoma among children 5 years of age or younger: Is it a different disease?","authors":"Chitrakshi Nagpal, Shuvadeep Ganguly, Archana Sasi, Vivek Kumar, Bivas Biswas, Deepam Pushpam, Akash Kumar, Sandeep Agarwala, Vishesh Jain, Anjan Dhua, Devender Kumar Yadav, Shah Alam Khan, Adarsh Barwad, Asit Ranjan Mirdha, Ahitagni Biswas, Sanjay Thulkar, Sameer Bakhshi","doi":"10.1002/pbc.31268","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Introduction</h3>\n \n <p>Children ≤5 years of age with Ewing's sarcoma (ES) possibly have a distinct disease biology, data on which are scarce. We evaluated clinical features, outcomes, and prognostic factors of ES among children with age ≤5 years.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>Children with ES registered between 2003 and 2019 were included. Baseline clinical and treatment details were retrieved from medical records. Prognostic factors were identified using multivariable Cox regression. Clinical features and outcomes of children ≤5 years were compared with those greater than 5 years by chi-square and log-rank tests. Propensity score-matched (PSM) analysis was done to evaluate the impact of age on survival in the metastatic and localized subgroups.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>Out of the 859 patients, 86 (10%) were ≤5 years of age (median age 4 years, 60 males [69.8%]). The most common location was the extremities (37.2%), followed by thorax (27.9%) and head and neck (H&N) (22.1%); baseline metastases were seen in 25 patients (29.8%). The median event-free-survival (EFS) and overall survival (OS) were 25.6 and 68.7 months, respectively. Metastatic disease predicted inferior OS (hazard ratio [HR] = 2.54, <i>p</i> = .018) and EFS (HR = 2.47, <i>p</i> = .007], symptom duration ≤3 months predicted an inferior OS (HR = 2.17, <i>p</i> = .048). Compared to age greater than 5 years, younger children had more H&N and less pelvic primaries (<i>p</i> < .001) and lesser baseline metastases (<i>p</i> = .037). PSM analysis did not reveal any significant impact of age on OS in the metastatic (HR = 1.59, <i>p</i> = .29) or localized cohort (HR = 1.77, <i>p</i> = .09).</p>\n </section>\n \n <section>\n \n <h3> Conclusions</h3>\n \n <p>Children with ES ≤5 years of age have a distinct favorable clinical presentation. However, age is not an independent prognostic factor for survival outcomes when adjusted for confounders.</p>\n </section>\n </div>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":null,"pages":null},"PeriodicalIF":2.4000,"publicationDate":"2024-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Blood & Cancer","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1002/pbc.31268","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Introduction
Children ≤5 years of age with Ewing's sarcoma (ES) possibly have a distinct disease biology, data on which are scarce. We evaluated clinical features, outcomes, and prognostic factors of ES among children with age ≤5 years.
Methods
Children with ES registered between 2003 and 2019 were included. Baseline clinical and treatment details were retrieved from medical records. Prognostic factors were identified using multivariable Cox regression. Clinical features and outcomes of children ≤5 years were compared with those greater than 5 years by chi-square and log-rank tests. Propensity score-matched (PSM) analysis was done to evaluate the impact of age on survival in the metastatic and localized subgroups.
Results
Out of the 859 patients, 86 (10%) were ≤5 years of age (median age 4 years, 60 males [69.8%]). The most common location was the extremities (37.2%), followed by thorax (27.9%) and head and neck (H&N) (22.1%); baseline metastases were seen in 25 patients (29.8%). The median event-free-survival (EFS) and overall survival (OS) were 25.6 and 68.7 months, respectively. Metastatic disease predicted inferior OS (hazard ratio [HR] = 2.54, p = .018) and EFS (HR = 2.47, p = .007], symptom duration ≤3 months predicted an inferior OS (HR = 2.17, p = .048). Compared to age greater than 5 years, younger children had more H&N and less pelvic primaries (p < .001) and lesser baseline metastases (p = .037). PSM analysis did not reveal any significant impact of age on OS in the metastatic (HR = 1.59, p = .29) or localized cohort (HR = 1.77, p = .09).
Conclusions
Children with ES ≤5 years of age have a distinct favorable clinical presentation. However, age is not an independent prognostic factor for survival outcomes when adjusted for confounders.
期刊介绍:
Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.