小脑发育不良性神经节细胞瘤的影像表现与临床特征  

Imaging Findings and Clinical Features of Cerebellar Dysplastic Gangliocytoma

作　　者：陈明 陈荣 李勇 郑海龙 李维民[2] 杨国庆 陈洪 Chen Ming;Chen Rong;Li Yong;Zheng Hai-long;Li Wei-min;Yang Guo-qing;Chen Hong(Radiology Department of Suining Municipal Hospital of TCM,Sichuan Suining 629000;Neurosurgery Department of Suining Central Hospital,Sichuan Suining 629000)

机构地区：[1]遂宁市中医院放射科,四川遂宁629000 [2]遂宁市中心医院神经外科,四川遂宁629000

出　　处：《中国CT和MRI杂志》2024年第5期61-63,共3页Chinese Journal of CT and MRI

基　　金：四川省科技厅重点研发项目(2019YFQ0028)。

摘　　要：目的探究小脑发育不良性神经节细胞瘤(DGC)CT、MRI影像学特点,同时分析患者的临床治疗、预后以及与Cowden综合征的关系,从而提高对该肿瘤的诊断准确性及鉴别诊断。方法回顾分析7例确诊DGC的影像学资料,确定术前肿瘤的部位、大小、影像学特点及强化方式等,并结合患者的临床资料判断是否诊断Cowden综合征。结果7例患者中2例病灶累及左侧小脑半球、小脑蚓部及桥小脑结合臂,1例位于左侧小脑半球及小脑扁桃体,1例位于左侧小脑半球,3例位于右侧小脑半球。其中1例CT显示明显钙化,7例T_(1)WI均表现为层状等、低信号,T_(2)WI呈层状等、高信号,6例呈明显“虎斑征”改变,增强显示5例呈条纹状轻度强化。随访中2例患者出现肿瘤复发、1例诊断Cowden综合征。结论DGC影像学特征性表现为“虎斑征”,多数呈轻度条纹状强化,预后良好,术前全面检查有助于做出Cowden综合征诊断。Objective To investigate the CT and MRI imaging features of cerebellar dysplastic gangliocytoma,and to analyze the clinical treatment,prognosis and relationship with Cowden syndrome,so as to improve the understanding and differential diagnosis of the disease.Methods The clinical and imaging datas of cerebellar dysplastic gangliocytoma confirmed by surgery and pathology were retrospectively analyzed.The location,size,imaging characteristics and enhancement of the tumor before operation were determined.Results Two of the seven patients were involved in the left cerebellar hemisphere,cerebellar vermis and pontine cerebellar binding arm,one was in the left cerebellar hemisphere and cerebellar tonsil,one was in the left cerebellar hemisphere,and three were in the right cerebellar hemisphere.Among them,one case showed obvious calcification on CT,seven cases showed laminar isointensity and low signal intensity on T_(1)WI,and T_(2)WI showed laminar isointensity and high signal intensity,six cases showed obvious"tabby sign"change,and five cases showed striped mild enhancement on enhanced MRI.During follow-up,two patients had tumor recurrence and one patient was diagnosed with Cowden syndrome.Conclusion The imaging findings of cerebellar dysplastic gangliocytoma are characteristic of"tabby sign",most of them show mild striated enhancement,and mostly have favourable prognosis.Comprehensive preoperative examination is helpful to make the diagnosis of Cowden syndrome.

关 键 词：小脑发育不良神经节细胞瘤 体层摄影术 X线计算机 磁共振成像 Cowden综合征 

分 类 号：R445.2[医药卫生—影像医学与核医学]