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影响中枢神经系统的原发性尤宁肉瘤:单中心体验和叙述性综述

Sivaraman Kumarasamy¹, Kanwaljeet Garg², Pankaj Kumar Singh¹

¹Department of Neurosurgery, All India Institute of Medical Sciences, CNC, Room No 720, New Delhi, India.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

|December 13, 2024

概括

中枢神经系统的原发性尤文肉瘤 (EWS) 是罕见的. 最多的外科切除,然后辅助疗法提供了有利的结果,尽管转移仍然是一个问题.

科学领域:

神经瘤学神经瘤学
儿科瘤学儿科瘤学
骨系统瘤骨系统瘤

背景情况:

尤文肉瘤 (EWS) 是一种罕见的恶性圆细胞瘤,主要影响儿童和年轻人骨和软组织.
主要EWS的中枢神经系统 (CNS) 参与非常罕见,发病率约为百万分之一.
在CNS EWS上存在有限的文献,需要案例系列来理解其管理和结果.

研究的目的:

介绍管理21例中枢神经系统初级EWS的临床经验.
突出临床和放射学发现,治疗策略和手术结果.
评估目前这种罕见疾病的治疗方案的疗效.

主要方法:

对经过手术治疗的初级中枢神经系统EWS患者 (2015-2023) 的医院记录的回顾性收集.
对患者人口统计,临床表现,放射学发现和治疗细节 (手术,辅助疗法) 的分析.
核准时对结果的评估和最新的后续行动.

主要成果:

16名男性和5名女性,平均年龄为18.2岁. 常见的症状包括头痛/吐 () 和背痛 (脊柱).
13名患者 (61.9%) 的头骨病变,8名患者 (38.1%) 的脊椎病变. 在18名患者 (85.71%) 实现了瘤切除,其中9名患者 (42.86%) 进行了总体切除.
15名患者接受了化疗和放射治疗; 5名KPS不良患者过早死亡. 9名患者在随访时仍然无症状;在某些情况下发生转移.

结论:

主要中枢神经系统EWS是一个罕见的实体,需要进行手术规划的详细放射学评估.
及时诊断和彻底的外科切除,然后进行辅助疗法,对于有利的结果至关重要.
辅助化疗-放射治疗可以改善疾病控制,但定期监测转移是必不可少的.

关键词:

头骨的部分是骨.欧洲警报系统 (EWS) 是一个警报系统.尤文的肉瘤是尤文的肉瘤.这是一个多学科的方法.根性切除是指进行根性切除.圆细胞瘤是一个圆细胞瘤.这种类型的肉瘤是sarcoma.脊髓脊柱的脊柱的脊柱的

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