{"title":"Malignant Peripheral Nerve Sheath Tumors (MPNST) in children and adolescents: a population-based study.","authors":"Hongjun Wu, Zhenqi Liao, Yinuo Wu, Liangxing Li","doi":"10.1016/j.wneu.2025.123934","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong>This study aims to analyze the prognosis of Malignant Peripheral Nerve Sheath Tumors (MPNST) in children using the Surveillance, Epidemiology, and End Results (SEER) database to identify significant prognostic factors affecting survival.</p><p><strong>Methods: </strong>A retrospective cohort analysis was conducted using the SEER database, identifying pediatric patients diagnosed with MPNST from 2000 to 2019. Demographic data, tumor characteristics, treatment modalities, and survival outcomes were extracted and analyzed. The main outcome measure was overall survival (OS), analyzed using Kaplan-Meier methods and Cox proportional hazards models to assess the impact of clinical and demographic factors on survival. Furthermore, we constructed a nomogram to predict OS in pediatric MPNST patients.</p><p><strong>Results: </strong>The study included 208 pediatric patients with MPNST, with a near-equal distribution across gender and a majority being white. The most common sites for MPNST were the trunk & core areas (38.9%), and the limbs (34.1%). A majority of the patients (87.0%) underwent surgical treatment and radiation therapy was administered to 44.7% of the patients. The overall 5-year survival rate for the entire cohort was 59.9%. When compared to no surgery, surgery had better survival outcomes. According to the results of Cox proportional hazard regression, only the SEER stage was an important independent predictor of OS in this model.</p><p><strong>Conclusions: </strong>Pediatric MPNST presents with challenging prognosis, significantly influenced by the SEER stage at diagnosis. Surgery is crucial as first-line treatment for MPNST, especially if the tumor is localized at diagnosis.</p>","PeriodicalId":23906,"journal":{"name":"World neurosurgery","volume":" ","pages":"123934"},"PeriodicalIF":1.9000,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"World neurosurgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.wneu.2025.123934","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Malignant Peripheral Nerve Sheath Tumors (MPNST) in children and adolescents: a population-based study.
Purpose: This study aims to analyze the prognosis of Malignant Peripheral Nerve Sheath Tumors (MPNST) in children using the Surveillance, Epidemiology, and End Results (SEER) database to identify significant prognostic factors affecting survival.
Methods: A retrospective cohort analysis was conducted using the SEER database, identifying pediatric patients diagnosed with MPNST from 2000 to 2019. Demographic data, tumor characteristics, treatment modalities, and survival outcomes were extracted and analyzed. The main outcome measure was overall survival (OS), analyzed using Kaplan-Meier methods and Cox proportional hazards models to assess the impact of clinical and demographic factors on survival. Furthermore, we constructed a nomogram to predict OS in pediatric MPNST patients.
Results: The study included 208 pediatric patients with MPNST, with a near-equal distribution across gender and a majority being white. The most common sites for MPNST were the trunk & core areas (38.9%), and the limbs (34.1%). A majority of the patients (87.0%) underwent surgical treatment and radiation therapy was administered to 44.7% of the patients. The overall 5-year survival rate for the entire cohort was 59.9%. When compared to no surgery, surgery had better survival outcomes. According to the results of Cox proportional hazard regression, only the SEER stage was an important independent predictor of OS in this model.
Conclusions: Pediatric MPNST presents with challenging prognosis, significantly influenced by the SEER stage at diagnosis. Surgery is crucial as first-line treatment for MPNST, especially if the tumor is localized at diagnosis.
期刊介绍：
World Neurosurgery has an open access mirror journal World Neurosurgery: X, sharing the same aims and scope, editorial team, submission system and rigorous peer review.
The journal''s mission is to:
-To provide a first-class international forum and a 2-way conduit for dialogue that is relevant to neurosurgeons and providers who care for neurosurgery patients. The categories of the exchanged information include clinical and basic science, as well as global information that provide social, political, educational, economic, cultural or societal insights and knowledge that are of significance and relevance to worldwide neurosurgery patient care.
-To act as a primary intellectual catalyst for the stimulation of creativity, the creation of new knowledge, and the enhancement of quality neurosurgical care worldwide.
-To provide a forum for communication that enriches the lives of all neurosurgeons and their colleagues; and, in so doing, enriches the lives of their patients.
Topics to be addressed in World Neurosurgery include: EDUCATION, ECONOMICS, RESEARCH, POLITICS, HISTORY, CULTURE, CLINICAL SCIENCE, LABORATORY SCIENCE, TECHNOLOGY, OPERATIVE TECHNIQUES, CLINICAL IMAGES, VIDEOS
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