虽然成年人大多数颅内肿瘤发生在大脑半球周围，但45%到60%的儿科肿瘤位于后颅窝，尽管这个解剖区域只占颅内体积的10%。世界卫生组织对中枢神经系统肿瘤的最新分类版对儿童肿瘤的分类产生了重大影响，也影响到后颅窝肿瘤。分子生物标志物在儿童后颅窝肿瘤的诊断、预后和治疗中起着重要作用，可以用于预测患者的预后和对治疗的反应，并监测其疗效。尽管遗传研究已经确定了多种后颅窝肿瘤类型，这些类型在定位、起源细胞、遗传机制和临床行为方面存在差异，但最近的治疗策略仍然依赖于统一的方法，主要是基于切除范围。然而，在生物或分子分级标准指导下制定治疗决策以及利用分子靶向治疗针对特定肿瘤生物学特征的进展已经取得了显著进展。本综述主要关注儿童后颅窝常见亚型肿瘤的诊断和治疗的最新进展，以及未来的潜在治疗方法。结论：分子生物标志物在儿童后颅窝肿瘤的诊断、预后和个体化治疗方案中起着核心作用。它们可以预测患者的预后，衡量治疗反应，并评估治疗的有效性。神经影像学和治疗的进展显著提高了这些肿瘤患儿的预后。 已知内容： - 中枢神经系统肿瘤是儿童和青少年最常见的实体肿瘤，其中约45%到60%位于后颅窝。 - 通常采用神经外科手术、放射治疗和化疗等综合方法来处理儿童后颅窝肿瘤。 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，从而提高了生存率和生活质量。 © 2023. 作者，独家授权于德国施普林格出版社，隶属于施普林格自然出版集团。

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.© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.