黏液样胶质神经元肿瘤的影像、病理表现及鉴别诊断

Imaging findings, pathological findings and differential diagnosis of myxoidglioneuronal tumor

黏液样胶质神经元肿瘤(MGT)是2021年WHO中枢神经系统肿瘤分类(第5版)中新定义的一种罕见的良性胶质神经元肿瘤,属于WHOⅠ级。过去,MGT由于在组织学形态上与胚胎发育不良性神经上皮肿瘤相似而被诊断为“透明隔中的胚胎发育不良性神经上皮瘤样肿瘤”。随着技术的发展,MGT的研究在影像学、病理学以及基因检测方面都有了新进展,尤其是PDGFRA的Lys385密码子处的热点突变,使MGT被定义为一个新的胶质瘤类型。本文对MGT的临床表现、影像学表现、病理特点以及基因检测结果进行了总结,并整合了与可能误诊的疾病的鉴别诊断,提出了该疾病可以发展的研究方向和潜在的治疗措施,旨在为MGT的诊治提供帮助,减少该疾病的漏诊、误诊。

Myxoid glioneuronal tumor(MGT)is a rare benign glioneuronal tumor newly defined in the 5th edition of 2021 WHO Classification of Central nervous System Tumors,which belongs to WHO grade I.In the past,MGT was diagnosed as"dysembryoplastic neuroepithelial tumor in the septum pellucidum"due to its histological similarity to dysembryoplastic neuroepithelial tumor.With the advancement of technology,there has been a new development in imaging,pathology,and genetic detection of the researches on MGT,especially the hot spot mutation at the Lys385 codon of PDGFRA,which defines MGT as a new type of glioma.This review summarizes the clinical manifestations,imaging manifestations,pathological characteristics and genetic test results of MGT and integrate the differential diagnosis of diseases that may be misdiagnosed,proposing research directions and potential therapeutic method of this disease,aiming to provide help for the diagnosis and treatment of MGT,and reduce the missed diagnosis and misdiagnosis of this disease.