Clinical characteristics of patients with atypical teratoid/rhabdoid tumors: a monocentric retrospective analysis.

IF 2.1 3区医学 Q2 PEDIATRICS

Frontiers in Pediatrics Pub Date : 2025-03-06 eCollection Date: 2025-01-01 DOI:10.3389/fped.2025.1463510

Zhiliang Wang, Jingchen Yang, Xing Liu, Wei Liu

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引用次数: 0

Abstract

Purpose: Atypical teratoid/rhabdoid tumors (ATRTs) are very rare, highly malignant embryonal neoplasms in central nervous system. The aim of this study was to conduct a retrospective analysis of ATRT patient survival and investigate the prognostic factors associated with ATRT.

Methods: A retrospective study was conducted using information of patients who received treatment between 2016 and 2021 in Beijing Tiantan Hospital. Kaplan-Meier curves were used for overall survival (OS) analysis. Univariate and multivariate COX analyses were applied for OS predicting.

Results: 20 histologically confirmed ATRT patients were included. The majority were male (75%) and aged over 3 years (65%). 71.4% of patients under 3 years and 46.2% of above 3 years had supratentorial tumors. All patients underwent surgery, with 60% having total resections, primarily in the supratentorial region. Subsequent treatment involved varying chemotherapy and radiation combinations, with 40% of patients receiving it, and 87.5% of those were older than 3 years, The median overall survival for ATRT patients was 180 days. Survival differed significantly between patients under and above 3 years. Radiotherapy increased overall survival for all patients. Univariate and multivariate analysis showed better survival for those diagnosed above age 3 and with adjuvant radiation.

Conclusions: Patients older than 3 years old had better prognosis and radiotherapy had a significant effect on improving patient prognosis.

查看原文本刊更多论文

非典型畸胎瘤/横纹肌样肿瘤患者的临床特征：单中心回顾性分析。

目的：非典型畸胎体/横纹肌样肿瘤是一种非常罕见的中枢神经系统高度恶性胚胎性肿瘤。本研究的目的是对ATRT患者的生存进行回顾性分析，并探讨与ATRT相关的预后因素。方法：对2016 - 2021年在北京天坛医院接受治疗的患者资料进行回顾性研究。Kaplan-Meier曲线用于总生存（OS）分析。单因素和多因素COX分析用于OS预测。结果：纳入组织学证实的ATRT患者20例。大多数是男性（75%），年龄在3岁以上（65%）。71.4%的3岁以下患者和46.2%的3岁以上患者存在幕上肿瘤。所有患者都接受了手术，60%的患者进行了全切除，主要是在幕上区域。随后的治疗包括不同的化疗和放疗组合，40%的患者接受了化疗，其中87.5%的患者年龄超过3岁，ATRT患者的中位总生存期为180天。3年以下和3年以上患者的生存率有显著差异。放疗提高了所有患者的总生存率。单因素和多因素分析显示，诊断为3岁以上并接受辅助放疗的患者生存率更高。结论：年龄大于3岁的患者预后较好，放疗对改善患者预后有显著作用。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Frontiers in Pediatrics Medicine-Pediatrics, Perinatology and Child Health

CiteScore

3.60

自引率

7.70%

发文量

2132

审稿时长

14 weeks

期刊介绍： Frontiers in Pediatrics (Impact Factor 2.33) publishes rigorously peer-reviewed research broadly across the field, from basic to clinical research that meets ongoing challenges in pediatric patient care and child health. Field Chief Editors Arjan Te Pas at Leiden University and Michael L. Moritz at the Children''s Hospital of Pittsburgh are supported by an outstanding Editorial Board of international experts. This multidisciplinary open-access journal is at the forefront of disseminating and communicating scientific knowledge and impactful discoveries to researchers, academics, clinicians and the public worldwide. Frontiers in Pediatrics also features Research Topics, Frontiers special theme-focused issues managed by Guest Associate Editors, addressing important areas in pediatrics. In this fashion, Frontiers serves as an outlet to publish the broadest aspects of pediatrics in both basic and clinical research, including high-quality reviews, case reports, editorials and commentaries related to all aspects of pediatrics.