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儿童后颅窝肿瘤:当前的认识。

Posterior fossa tumors in children: current insights.

机构信息

Division of Neurosurgery, Department of Neurology, University of Campinas, Tessalia Vieira de Camargo St., 126. 13083-887, Campinas, SP, Brazil.

Centro Infantil Boldrini, Campinas, SP, Brazil.

出版信息

Eur J Pediatr. 2023 Nov;182(11):4833-4850. doi: 10.1007/s00431-023-05189-5. Epub 2023 Sep 8.

Abstract

While in adults most intracranial tumors develop around the cerebral hemispheres, 45 to 60% of pediatric lesions are found in the posterior fossa, although this anatomical region represents only 10% of the intracranial volume. The latest edition of the WHO classification for CNS tumors presented some fundamental paradigm shifts that particularly affected the classification of pediatric tumors, also influencing those that affect posterior fossa. Molecular biomarkers play an important role in the diagnosis, prognosis, and treatment of childhood posterior fossa tumors and can be used to predict patient outcomes and response to treatment and monitor its effectiveness. Although genetic studies have identified several posterior fossa tumor types, differing in terms of their location, cell of origin, genetic mechanisms, and clinical behavior, recent management strategies still depend on uniform approaches, mainly based on the extent of resection. However, significant progress has been made in guiding therapy decisions with biological or molecular stratification criteria and utilizing molecularly targeted treatments that address specific tumor biological characteristics. The primary focus of this review is on the latest advances in the diagnosis and treatment of common subtypes of posterior fossa tumors in children, as well as potential therapeutic approaches in the future.   Conclusion: Molecular biomarkers play a central role, not only in the diagnosis and prognosis of posterior fossa tumors in children but also in customizing treatment plans. They anticipate patient outcomes, measure treatment responses, and assess therapeutic effectiveness. Advances in neuroimaging and treatment have significantly enhanced outcomes for children with these tumors. What is Known: • Central nervous system tumors are the most common solid neoplasms in children and adolescents, with approximately 45 to 60% of them located in the posterior fossa. • Multimodal approaches that include neurosurgery, radiation therapy, and chemotherapy are typically used to manage childhood posterior fossa tumors What is New: • Notable progress has been achieved in the diagnosis, categorization and management of posterior fossa tumors in children, leading to improvement in survival and quality of life.

摘要

虽然成年人的颅内肿瘤大多在大脑半球周围发展,但 45%至 60%的儿科病变发生在后颅窝,尽管该解剖区域仅占颅内体积的 10%。世界卫生组织(WHO)中枢神经系统肿瘤分类的最新版本提出了一些基本的范式转变,这些转变特别影响了儿科肿瘤的分类,也影响了那些影响后颅窝的肿瘤。分子生物标志物在儿童后颅窝肿瘤的诊断、预后和治疗中发挥着重要作用,可以用于预测患者的结局和对治疗的反应,并监测其疗效。尽管遗传研究已经确定了几种后颅窝肿瘤类型,这些肿瘤在位置、起源细胞、遗传机制和临床行为上存在差异,但最近的管理策略仍然依赖于统一的方法,主要基于切除范围。然而,在利用生物或分子分层标准指导治疗决策和利用针对特定肿瘤生物学特征的分子靶向治疗方面已经取得了重大进展。本综述的主要重点是儿童后颅窝常见肿瘤亚型的最新诊断和治疗进展,以及未来潜在的治疗方法。

结论

分子生物标志物不仅在后颅窝肿瘤的诊断和预后中起着核心作用,而且在制定治疗计划中也起着核心作用。它们可以预测患者的结局,衡量治疗反应,并评估治疗效果。神经影像学和治疗方面的进步显著提高了患有这些肿瘤的儿童的结局。

已知内容

  • 中枢神经系统肿瘤是儿童和青少年中最常见的实体肿瘤,约有 45%至 60%位于后颅窝。

  • 包括神经外科、放射治疗和化学疗法在内的多模式方法通常用于治疗儿童后颅窝肿瘤。

新内容

  • 在儿童后颅窝肿瘤的诊断、分类和管理方面取得了显著进展,导致生存率和生活质量的提高。

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