Glioma.

IF 76.9 1区医学 Q1 MEDICINE, GENERAL & INTERNAL

Nature Reviews Disease Primers Pub Date : 2024-05-09 DOI:10.1038/s41572-024-00516-y

Michael Weller, Patrick Y Wen, Susan M Chang, Linda Dirven, Michael Lim, Michelle Monje, Guido Reifenberger

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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.

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胶质瘤

胶质瘤是一种原发性脑肿瘤，被认为是由携带肿瘤诱发基因改变的神经干细胞或祖细胞发展而来。根据显微镜下的外观和分子特征，胶质瘤按照世界卫生组织（WHO）中枢神经系统（CNS）肿瘤分类法进行分类，从低度恶性到高度恶性分为 CNS WHO 1-4 级。成人弥漫浸润性胶质瘤包括三种肿瘤类型，其自然病程、对治疗的反应和预后各不相同：异柠檬酸脱氢酶（IDH）突变型和1p/19q编码缺失型少突胶质瘤预后最好；IDH突变型星形细胞瘤预后中等；IDH-Wild型胶质母细胞瘤预后较差。嗜酸性粒细胞星形细胞瘤是儿童中最常见的胶质瘤，其特点是呈环状生长、经常发生 BRAF 改变和预后良好。儿童弥漫性胶质瘤分为临床症状不明显的低级别肿瘤和具有侵袭性的高级别肿瘤，其中组蛋白3 K27改变的弥漫性中线胶质瘤是儿童胶质瘤相关死亡的主要原因。根据组织学、分子生物标志物和位置的不同，表皮神经胶质瘤在生物学和预后上被细分为不同的类型。虽然手术、放疗和烷化剂化疗是胶质瘤治疗的主要手段，但基于肿瘤内在主导信号通路的个体化治疗策略改善了部分患者的预后。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Nature Reviews Disease Primers Medicine-General Medicine

CiteScore

76.70

自引率

0.20%

发文量

期刊介绍： Nature Reviews Disease Primers, a part of the Nature Reviews journal portfolio, features sections on epidemiology, mechanisms, diagnosis, management, and patient quality of life. The editorial team commissions top researchers — comprising basic scientists and clinical researchers — to write the Primers, which are designed for use by early career researchers, medical students and principal investigators. Each Primer concludes with an Outlook section, highlighting future research directions. Covered medical specialties include Cardiology, Dermatology, Ear, Nose and Throat, Emergency Medicine, Endocrinology, Gastroenterology, Genetic Conditions, Gynaecology and Obstetrics, Hepatology, Haematology, Infectious Diseases, Maxillofacial and Oral Medicine, Nephrology, Neurology, Nutrition, Oncology, Ophthalmology, Orthopaedics, Psychiatry, Respiratory Medicine, Rheumatology, Sleep Medicine, and Urology.