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胶质瘤。

Glioma.

机构信息

Department of Neurology & Brain Tumour Center, University Hospital Zurich & University of Zurich, Zurich, Switzerland.

Center For Neuro-Oncology, Dana-Farber/Brigham and Women's Cancer Center and Harvard Medical School, Boston, MA, USA.

出版信息

Nat Rev Dis Primers. 2024 May 9;10(1):33. doi: 10.1038/s41572-024-00516-y.

Abstract

Gliomas are primary brain tumours that are thought to develop from neural stem or progenitor cells that carry tumour-initiating genetic alterations. Based on microscopic appearance and molecular characteristics, they are classified according to the WHO classification of central nervous system (CNS) tumours and graded into CNS WHO grades 1-4 from a low to high grade of malignancy. Diffusely infiltrating gliomas in adults comprise three tumour types with distinct natural course of disease, response to treatment and outcome: isocitrate dehydrogenase (IDH)-mutant and 1p/19q-codeleted oligodendrogliomas with the best prognosis; IDH-mutant astrocytomas with intermediate outcome; and IDH-wild-type glioblastomas with poor prognosis. Pilocytic astrocytoma is the most common glioma in children and is characterized by circumscribed growth, frequent BRAF alterations and favourable prognosis. Diffuse gliomas in children are divided into clinically indolent low-grade tumours and high-grade tumours with aggressive behaviour, with histone 3 K27-altered diffuse midline glioma being the leading cause of glioma-related death in children. Ependymal tumours are subdivided into biologically and prognostically distinct types on the basis of histology, molecular biomarkers and location. Although surgery, radiotherapy and alkylating agent chemotherapy are the mainstay of glioma treatment, individually tailored strategies based on tumour-intrinsic dominant signalling pathways have improved outcome in subsets of patients.

摘要

神经胶质瘤是原发性脑肿瘤,被认为起源于携带肿瘤起始遗传改变的神经干细胞或祖细胞。根据微观外观和分子特征,它们根据世界卫生组织(WHO)中枢神经系统(CNS)肿瘤分类进行分类,并根据恶性程度从低到高分为 CNS WHO 1-4 级。成人弥漫性浸润性神经胶质瘤包括三种具有不同自然病程、对治疗反应和预后的肿瘤类型:异柠檬酸脱氢酶(IDH)突变和 1p/19q 缺失的少突胶质细胞瘤,预后最好;IDH 突变星形细胞瘤,预后中等;IDH 野生型胶质母细胞瘤,预后差。毛细胞型星形细胞瘤是儿童最常见的神经胶质瘤,其特征为局限性生长、频繁 BRAF 改变和良好的预后。儿童弥漫性神经胶质瘤分为临床惰性低级别肿瘤和侵袭性高级别肿瘤,组蛋白 3 K27 改变的弥漫性中线胶质瘤是导致儿童胶质瘤相关死亡的主要原因。室管膜肿瘤根据组织学、分子标志物和位置分为具有不同生物学和预后的类型。尽管手术、放疗和烷化剂化疗是神经胶质瘤治疗的主要方法,但基于肿瘤内在优势信号通路的个体化治疗策略已改善了部分患者的预后。

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