类脑膜炎表现的弥漫性软脑膜胶质神经元肿瘤1例

A case of diffuse leptomeningeal glioneuronal tumor resembling meningitis

弥漫性软脑膜胶质神经元肿瘤是一种2016年世界卫生组织分类新列入的罕见神经元-混合神经元胶质肿瘤。当前影像个案报道多为广泛软脑(脊)膜异常增厚、强化结节及脑表面小囊状改变。文中报道1例酷似脑膜炎表现的弥漫性软脑膜胶质神经元肿瘤患者,影像学表现为多脑叶皮质肿胀、囊变不明显的均匀软脑膜增厚强化及脑回样钙化;临床上表现为反复急性发作的脑膜刺激征表现,且抗炎治疗有效;病理学证实为弥漫性软脑膜胶质神经元肿瘤。通过报道1例非特异表现的弥漫性软脑膜胶质神经元肿瘤患者,分析其影像和临床诊断思路,并结合文献复习,更新对该病的认识,提高临床鉴别诊断能力。

Diffuse leptomeningeal glioneuronal tumor(DLGNT)is a rare,low-grade neoplasm,which is newly categorized into the neuronal and mixed neuro-glial tumor in 2016.The most characteristic imaging findings are diffuse leptomeningeal thickening and enhancement with multiple minor cysts.This article described a case with DLGNT mimicking meningitis,whose cystic lesions were not obvious,with swollen multiple lobes cortex,gyri form cortical calcification and enhanced meninges.Meningeal irritation sign repeated attacks and the clinical symptoms gradually improved after steroid pulse therapy.The biopsy and immunohistochemistry staining were diagnosed as DLGNT.The imaging features and clinical data of this case were analyzed to improve the understanding of the disease in clinical practice.