PDF(Pubmed)
Abstract：
Central nervous system (CNS) tumours in neonates are relatively rare and present differently when compared with those occurring later in childhood in terms of aetiology, clinical features, location, histology and prognosis. The clinical presentation is extremely variable. Even if the most frequent clinical sign is a macrocephaly, there are many other non-specific symptoms associated. The prognosis is usually poor with overall survival of less than 30%. Surgery continues to be the primary treatment for neonatal CNS tumours, aiming for a gross total resection, directly correlated with prognosis and the overall outcome. The chemotherapy is the only adjuvant therapy whereas the radiotherapy is avoided under three years of age because of the severe sequelae. Hence the importance of molecular characterization of these neoplasms in order to improve the accuracy of the diagnosis and identify new therapeutic targets. The aim of this review is to describe the main characteristics of these tumours and the recent advances in their treatment in order to recognize these pathologies in the prenatal period and create a multidisciplinary team providing the best possible treatment while minimising the risk of long-term complications. Neonatologists play a key role in the early detection, diagnostic evaluation, management and supportive care of these neonates. Conclusion: The aim of this review is to describe the main characteristics of these tumours and the recent advances in their treatment in order to ensure the essential knowledge that will help the neonatologist identify them and create a multidisciplinary team providing the best possible treatment while minimising the risk of long-term complications. What is Known: • Neonatal CNS tumours are relatively rare and their early identification is important to identify the best diagnostic-therapeutic management. • Surgery is the main treatment of neonatal CNS tumours. The extent of surgical resection directly correlates with prognosis and outcome. What is New: • Predisposing conditions such as Cancer Predisposition Syndromes must be considered. • Targeted drugs and other therapeutic strategies can be identified through molecular characterization.
摘要：
新生儿的中枢神经系统（CNS）肿瘤相对罕见，并且在病因学方面与儿童后期发生的肿瘤相比存在差异。临床特征,location,组织学和预后。临床表现变化很大。即使最常见的临床症状是大头畸形，有许多其他非特异性症状相关。预后通常较差，总生存率低于30％。手术仍然是新生儿中枢神经系统肿瘤的主要治疗方法，旨在进行全面切除，与预后和总体预后直接相关。化疗是唯一的辅助疗法，而由于严重的后遗症，在三岁以下可以避免放疗。因此，对这些肿瘤进行分子表征以提高诊断的准确性并确定新的治疗靶标的重要性。这篇综述的目的是描述这些肿瘤的主要特征及其治疗的最新进展，以便在产前阶段认识到这些病理，并创建一个多学科团队，提供最佳治疗，同时最大限度地减少长期并发症的风险。新生儿学家在早期检测中起着关键作用，诊断评估,这些新生儿的管理和支持性护理。结论：本综述的目的是描述这些肿瘤的主要特征及其治疗的最新进展，以确保有助于新生儿学家识别它们的基本知识，并创建一个多学科团队，提供最佳治疗，同时最大限度地减少长期并发症的风险。已知:•新生儿CNS肿瘤相对罕见,并且它们的早期识别对于确定最佳诊断-治疗管理是重要的。•手术是新生儿中枢神经系统肿瘤的主要治疗方法。手术切除的程度与预后和结果直接相关。新增内容：•必须考虑诸如癌症易感性综合征之类的易感状况。•可以通过分子表征鉴定靶向药物和其他治疗策略。
