Pediatric Central Nervous System Embryonal Tumors: Presentation, Diagnosis, Therapeutic Strategies, and Survivorship—A Review

IF 3.2 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology Pub Date : 2024-10-09 DOI:10.1016/j.pediatrneurol.2024.09.031

Rebecca Ronsley MD , Bonnie Cole MD , Tyler Ketterl MD , Jason Wright MD , Ralph Ermoian MD , Lindsey M. Hoffman MD , Ashley S. Margol MD , Sarah E.S. Leary MD

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引用次数: 0

Abstract

Central nervous system (CNS) embryonal tumors represent a diverse group of neoplasms and have a peak incidence in early childhood. These tumors can be located anywhere within the CNS, and presenting symptoms typically represent tumor location. These tumors display distinctive findings on neuroimaging and are staged using magnetic resonance imaging of the brain and spine as well as evaluation of cerebrospinal fluid. Diagnosis is made based on an integrated analysis of histologic and molecular features via tissue sampling. Risk stratification is based on integration of clinical staging and extent of resection with histologic and molecular risk factors. The therapeutic approach for these tumors is multimodal and includes surgery, chemotherapy, and radiation, tailored to the individual patient factors (including age) and specific tumor type. Comprehensive supportive care including management of nausea, nutrition support, pain, fertility preservation, and mitigation of therapy-related morbidity (including hearing protection) is imperative through treatment of CNS embryonal tumors. Despite advances in therapy and supportive care, the long-term consequences of current treatment strategies are substantial. Integration of less toxic, molecularly targeted therapies and a comprehensive, multidisciplinary approach to survivorship care are essential to improving survival and the overall quality of life for survivors.

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小儿中枢神经系统胚胎性肿瘤：小儿中枢神经系统胚胎性肿瘤：表现、诊断、治疗策略和生存期--综述。

中枢神经系统（CNS）胚胎性肿瘤是一类多种多样的肿瘤，其发病高峰期在儿童早期。这些肿瘤可位于中枢神经系统的任何部位，出现的症状通常代表肿瘤的位置。这些肿瘤在神经影像学上有独特的表现，可通过脑部和脊柱的磁共振成像以及脑脊液评估进行分期。诊断是通过组织取样对组织学和分子特征进行综合分析后做出的。风险分层基于临床分期和切除范围与组织学和分子风险因素的整合。这些肿瘤的治疗方法是多模式的，包括手术、化疗和放疗，并根据患者的个体因素（包括年龄）和特定的肿瘤类型量身定制。在中枢神经系统胚胎性肿瘤的治疗过程中，必须提供全面的支持性治疗，包括控制恶心、营养支持、疼痛、生育力保护以及减轻治疗相关的发病率（包括听力保护）。尽管在治疗和支持性护理方面取得了进步，但目前的治疗策略仍会造成严重的长期后果。整合毒性较低的分子靶向疗法以及全面、多学科的幸存者护理方法，对于提高幸存者的生存率和整体生活质量至关重要。

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

Pediatric neurology 医学-临床神经学

CiteScore

4.80

自引率

2.60%

发文量

176

审稿时长

78 days

期刊介绍： Pediatric Neurology publishes timely peer-reviewed clinical and research articles covering all aspects of the developing nervous system. Pediatric Neurology features up-to-the-minute publication of the latest advances in the diagnosis, management, and treatment of pediatric neurologic disorders. The journal''s editor, E. Steve Roach, in conjunction with the team of Associate Editors, heads an internationally recognized editorial board, ensuring the most authoritative and extensive coverage of the field. Among the topics covered are: epilepsy, mitochondrial diseases, congenital malformations, chromosomopathies, peripheral neuropathies, perinatal and childhood stroke, cerebral palsy, as well as other diseases affecting the developing nervous system.