文章：

室管膜瘤的生物学和新兴的新疗法

The biology of ependymomas and emerging novel therapies

原文发布日期：2022-01-14

DOI: 10.1038/s41568-021-00433-2

类型: Review Article

开放获取: 否

英文摘要：

Ependymomas are rare central nervous system tumours that can arise in the brain’s supratentorial region or posterior fossa, or in the spinal cord. In 1924, Percival Bailey published the first comprehensive study of ependymomas. Since then, and especially over the past 10 years, our understanding of ependymomas has grown exponentially. In this Review, we discuss the evolution in knowledge regarding ependymoma subgroups and the resultant clinical implications. We also discuss key oncogenic and tumour suppressor signalling pathways that regulate tumour growth, the role of epigenetic dysregulation in the biology of ependymomas, and the various biological features of ependymoma tumorigenesis, including cell immortalization, stem cell-like properties, the tumour microenvironment and metastasis. We further review the limitations of current therapies such as relapse, radiation-induced cognitive deficits and chemotherapy resistance. Finally, we highlight next-generation therapies that are actively being explored, including tyrosine kinase inhibitors, telomerase inhibitors, anti-angiogenesis agents and immunotherapy.

摘要翻译： 

室管膜瘤是罕见的中枢神经系统肿瘤，可发生在大脑幕上区域或后颅窝，亦可起源于脊髓。1924年，Percival Bailey发表了首篇关于室管膜瘤的系统性研究。此后特别是近十年来，我们对室管膜瘤的认识呈指数级增长。本文综述将探讨室管膜瘤分子分型研究的演进及其临床意义，同时解析调控肿瘤生长的关键致癌信号通路与抑癌通路、表观遗传失调在室管膜瘤生物学行为中的作用，以及细胞永生化、类干细胞特性、肿瘤微环境和转移等多种肿瘤发生相关的生物学特征。我们还将评述现有治疗方案的局限性，包括肿瘤复发、放疗所致认知功能障碍及化疗耐药等问题。最后重点介绍正在积极研发的新一代疗法，包括酪氨酸激酶抑制剂、端粒酶抑制剂、抗血管生成药物及免疫治疗。

原文链接：

The biology of ependymomas and emerging novel therapies