结节性硬化症合并室管膜下巨细胞型星形细胞瘤临床及影像学表现  

Clinical and imaging features of tuberous sclerosis combined with subependymal giant cell astrocytoma

作　　者：吴灵智 潘阿善 邱乾德 WU Lingzhi;PAN Ashan;QIU Qiande(Department of Radiology,Yueqing People's Hospital of Zhejiang Province,Yueqing 325600,P.R.China;Department of Radiology,Wenzhou People's Hospital of Zhejiang Province,Yueqing 325000,P.R.China)

机构地区：[1]浙江省乐清市人民医院放射科,浙江乐清325600 [2]浙江省温州市人民医院影像科,浙江乐清325000

出　　处：《医学影像学杂志》2021年第9期1462-1466,共5页Journal of Medical Imaging

摘　　要：目的探讨结节性硬化症合并室管膜下巨细胞型星形细胞瘤(SEGA)患者的临床与影像表现。方法选取并分析经临床确诊的10例结节性硬化症合并室管膜下巨细胞型星形细胞瘤的影像学资料,观察病变的部位、大小、形状、分布等情况。结果10例首发症状均为癫痫、智力低下。10例肿瘤均为单发位于侧脑室内,其中位于孟氏孔区4例,室间隔区3例,侧脑室前角2例,侧脑室体部1例。10例肿块最大径2.1~5.1 cm,平均(2.7±5.2)cm。CT检查示肿块呈菜花状、分叶状,肿块密度均匀7例,密度不均匀3例;肿块呈高密度8例,等密度2例,CT值32~43 HU,平均(37±7.8)HU;肿块内点状钙化4例,合并脑积水5例。MRI显示病灶信号不均匀,于T 1WI呈等、低信号,T 2WI呈等高信号,DWI呈低信号,FLAIR呈高信号;增强后明显均匀或不均匀强化。10例病例均伴有室管膜下硬化结节,其中合并皮层结节4例,纤维血管瘤和纤维瘤7例,肾血管平滑肌脂肪瘤7例,骨骼结节性硬化7例,肺淋巴管肌瘤病3例,肝脏血管平滑肌脂肪瘤1例。结论SEGA患者影像表现具有特征性,影像表现结合临床可作出较明确诊断。Objective To analyze the clinical and imaging features of subependymal giant cell astrocytomas in patients with tuberous sclerosis in order to improve its diagnosis.Methods Clinical presentation and imaging datd of 10 clinically confirmed TSC patients with subependymal giant cell astrocytoma were retrospectively analyzed.The position,size,shape and distribution of lesions of these patients were observed.Results Ten patients(7 males,3 females)were collected,with average of(23.9±19)years old.Seizure and cognitive dysfunction were found in these 10 patients.The tumor was located in four cases near the he foramen of Monro,3 cases in the ventricular septum,2 in the antecornu,and 1 in the body of lateral ventricle.The maximum size of the tumor was 2.1 to 5.1 cm,with average of(2.7±5.2)cm.CT showed that the mass was cauliflower,lobulated;the density of mass was homogeneous in 7 cases and inhomogeneous in density in 3 cases;the masses were 8 cases of high density,2 cases of equal density,and the CT value ranged from 32 to 43 HU,with an average of(37±7.8)HU;there were 4 cases of punctate calcification in the tumor,combined hydrocephalus in 5 cases.MRI showed T 1WI iso-signal or low signal,T 2WI iso-signal or high signal,DWI low signa and FLAIR high signal.The lesions can be inhomogenous.Contrast-enhanced MRI images showed obvious homogenous or in homogenous enhancement.and often had hydrocephalus.Subependymal tuber was found in 10 cases and corticaI tuber in 4 cases.There were fibrous hemangioma and fibroma in skin in 7 of the 10 patients.The renal angiomyolipoma was found in 7 patients.The tuberous sclerosis was found in bones in 7 patients.There was pulmonary lymphangiomyomatosis in 3 patients.The hepatic angiomyolipoma was found in 1 patient.Conclusion The imaging presentations of subependymal giant cell astrocvtoma(SEGA)diseases had some certain specificity.The disease can be definitely diagnosed based on imaging manifestations and clinical symptoms.

关 键 词：结节性硬化症 室管膜下 巨细胞型星形细胞瘤 影像学诊断 

分 类 号：R445[医药卫生—影像医学与核医学] R742[医药卫生—诊断学]