儿童胶质神经元和神经元肿瘤的影像学表现

Imaging features of the glioneuronal and neuronal tumors in children

目的:探讨儿童中枢神经系统胶质神经元和神经元肿瘤的影像学征象,旨在提高对此类疾病的诊断水平。方法:回顾性分析经病理确诊的44例儿童中枢神经系统胶质神经元和神经元肿瘤的临床表现及影像学资料。结果:44例中,18例为胚胎发育不良性神经上皮瘤,病变全部位于幕上,主要累及皮层,根据MRI表现分为Ⅰ型(类囊性)9例,Ⅱ型(结节状)3例,Ⅲ型(发育异常样)6例;17例为节细胞胶质瘤,病变全部位于幕上,好发于颞叶,多呈囊实性,3例可见钙化;5例婴儿促纤维增生型节细胞胶质瘤/星形细胞瘤,病变全部位于幕上,以囊实性为主,实性成分可见强化,1例可见钙化;2例中枢神经细胞瘤,病变位于幕上脑室内,1例可见多发囊变,增强扫描肿瘤内实性成分明显强化;1例弥漫性软脑膜胶质神经元肿瘤,病变位于小脑、脑干及脊髓,幕下软脑膜多发囊变,增强扫描软脑膜弥漫性增厚、强化;1例小脑发育不良性节细胞瘤,位于左侧小脑半球及小脑蚓部,可见钙化,增强扫描呈轻度强化,可见典型“虎纹征”。结论:儿童胶质神经元和神经元肿瘤影像学具有其特征性,结合临床病史有利于提高术前诊断准确性。

Objective:To investigate the imaging features of glioneuronal and neuronal tumors in the central nervous system(CNS)in children,thus to improve the clinical diagnosis of these diseases.Methods:The clinical and preoperative imaging data of 44 children with CNS glioneuronal and neuronal tumors diagnosed by pathology were retrospectively analyzed.Results:18 cases had dysembryoplastic neuroepithelial tumors(DNETs),all of which were located in the supratentorial brain and beneath the cerebral cortex.According to MRI characteristics,there were 9 cases of typeⅠ(cystic),3 cases of typeⅡ(nodular),and 6 cases of typeⅢ(dysplasia).17 cases had gangliogliomases,all of which were located in the supratentorial brain,mainly located in the temporal lobe,and mainly showed cystic and solid,3 cases showed calcifications.There were desmoplastic infantile ganglioglioma/astrocytoma(DIG/DIA)in 5 cases,all tumors were located in the supratentorial brain,mainly showed cystic and solid,with the solid components enhanced,and one case showed calcification.Two cases of central neurocytoma(CNC),located in the supratentorial ventricle,and one case showed multiple cysts.There was diffuse leptomeningeal glioneuronal tumor(DLGNT)in one case,mildly thickened and enhanced leptomeninges were found widespread on the surface of the brain and spinal cord,as well as multiple cysts throughout the cerebellum,brainstem and spinal cord.One case had Lhermitte-Duclos disease(LDD),which located in the cerebellar hemisphere and vermis with calcification,mild enhancement,and typical"tiger spot signs".Conclusion:Glioneuronal and neuronal tumors in children have some imaging characteristics,which is helpful to improve the accuracy of preoperative diagnosis when combined with clinical data.