Ryall Scott, Tabori Uri, Hawkins Cynthia
The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G1X8, Canada.
Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, ON, Canada.
Brain Tumor Pathol. 2017 Apr;34(2):51-61. doi: 10.1007/s10014-017-0282-z. Epub 2017 Mar 25.
Gliomas are the most common central nervous system neoplasms affecting children and can be both high- and low-grade. Paediatric low-grade glioma may be either World Health Organization grade I or grade II. Despite being classified as grade II diffuse astrocytoma, these neoplasms arising in children are distinct clinically and molecularly from their adult counterparts. They do not tend to progress to higher grade lesions and only rarely harbour an IDH mutation. Here, we review the clinical, histologic and molecular features of paediatric grade II diffuse glioma, highlighting their diagnostic criteria, prevalence across brain locations, their most common molecular features and how to test for them, and lastly the current status of therapeutic options available for their treatment.
胶质瘤是影响儿童的最常见中枢神经系统肿瘤，可分为高级别和低级别。儿童低级别胶质瘤可能是世界卫生组织一级或二级。尽管被归类为二级弥漫性星形细胞瘤，但这些发生在儿童中的肿瘤在临床和分子水平上与成人同类肿瘤不同。它们通常不会进展为更高级别的病变，只有极少数携带异柠檬酸脱氢酶（IDH）突变。在此，我们综述儿童二级弥漫性胶质瘤的临床、组织学和分子特征，重点介绍其诊断标准、在脑内各部位的患病率、最常见的分子特征及其检测方法，最后介绍目前可用于其治疗的治疗方案现状。
