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1、1/64,Hemangioblastoma 血管母细胞瘤，血管网状细胞瘤（血网） Dysplastic Cerebellar Gangliocytoma 发育不良性神经节细胞瘤 Subependymoma 室管膜下瘤,Adult Tumors of the Posterior Fossa,2/64,3/64,Incidence and Clinical Presentation,Dysplastic Cerebellar Gangliocytoma,Originally described in 1920 also called Lhermitte-Duclos disease (LDD) n
2、eoplastic histogenesis Vs. hamartomatous origin young adults: average age 34 years no gender predilection,1920年首次报道 又称为LDD 早期研究认为其为肿瘤性病变，现认为其为错构瘤样改变 常见于年轻人，中位发病年龄34岁 无性别差异,4/64,Incidence and Clinical Presentation,Dysplastic Cerebellar Gangliocytoma,commonly symptoms increased intracranial pressure h
3、ydrocephalus Megalencephaly mental retardation duration of symptoms considerable variability asymptomatic,常见临床症状 颅内压增高 脑积水 巨脑畸形 精神障碍 症状时间 不定 甚至也可无症状,5/64,Dysplastic Cerebellar Gangliocytoma VS Cowden disease,Cowden disease autosomal dominant hamartoma syndrome characterized syndrome mucocutaneous le
4、sions macrocephaly hamartomas long arm of chromosome 10,Cowden病 常染色体显性遗传 错构瘤样病变 特征性表现 皮肤粘膜病变 巨脑畸形 错构瘤 10号染色体长臂异常,Clinical Presentation,6/64,Pathologic Findings,disruption of the normal cerebellar laminar structure hypertrophic ganglion cells granular and molecular layers of the cerebellar cortex inc
5、reased myelination in the molecular layer Mitotic activity and necrosis are uncommon,Dysplastic Cerebellar Gangliocytoma,正常小脑板层结构破坏 大量增生肥大的神经节细胞 侵蚀小脑皮层的颗粒层和分子层 分子层髓鞘化增加 病理组织切片上有丝分裂活性及坏死少见,7/64,Pathologic Findings,Dysplastic Cerebellar Gangliocytoma,200,100,hypertrophic ganglion cells expanding the g
6、ranular and molecular layers of the cerebellar cortex,8/64,Pathologic Findings,Dysplastic Cerebellar Gangliocytoma,免疫组化染色显示神经元特异性蛋白酶及突触素阳性表达,9/64,Imaging Findings-CT,NCCT usually hypoattenuated may be isoattenuated Calcification is uncommon Thinning of the skull,Dysplastic Cerebellar Gangliocytoma,C
7、T平扫 常为低密度 但也可为等密度 无特异性 诊断困难 钙化少见 偶可见颅板变薄,10/64,Imaging Findings-MRI,MRI：best imaging modality characteristic appearance without histopathological confirmation typical appearances 1. unilateral cerebellar mass 2. non-enhancing 3. middle-aged patient 4. tiger-striped pattern,Dysplastic Cerebellar Gang
8、liocytoma,MRI是诊断的最佳方式 特征性表现 几乎可在不需要病理证实的条件下做出明确诊断 四条特征 1. 单侧半球肿块 2. 不强化 3. 成年患者 4. 虎斑征,11/64,Imaging Findings-MRI,characteristic: bands hyperintensity and isointensity on T2 isointense and hypointense on T1 hyperintense signal on T2 corresponds to the inner molecular layer, granular cell layer, and
9、loss of central white matter,Dysplastic Cerebellar Gangliocytoma,特征性表现：条带 T2等、高信号 T1等低信号 T2上所见的高信号条带为内分子层、颗粒细胞层，以及白质细胞丢失所致,12/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,Dysplastic cerebellar gangliocytoma in a 47-year-old woman. (a)Axial T1-weighted MR image shows a cerebellar mass
10、with a striped appearance. (b)Axial T2-weighted MR image shows the same laminar morphology, composed of alternating hyperintense and isointense bands. (c) Con-trast-enhanced axial T1-weighted MR image shows no enhancement of the mass.,13/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,虎斑
11、征，条纹征,14/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,15/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,1.5T SWI MIP图像显示瘤周引流静脉，7T SWI MIP图像显示瘤周大量引流静脉及齿状核受压,16/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,图1 MRI平扫轴位T1WI(A)，增强扫描轴位T1WI(B)，左侧小脑半球可见一团块状长T1、长T2异常
12、信号影，边界清楚，其内信号不均匀，可见条纹状等T1、T2信号影。增强扫描未见明显强化。图2 各向异性分数（fractional anisotropic, FA）灰度图，肿瘤区域呈低信号。图3 ADC图，肿瘤区域呈等及稍高信号,17/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,各向异性分数（fractional anisotropic, FA）方向彩色编码图上正常小脑区域呈绿色(表示前后走行)，肿瘤区以红色为主(表示左右走行)，未见条样信号。图5 纤维束追踪成像像(Fiber tractography）。fiber tr
13、act within the tumor肿瘤区纤维束以左右方向走行为主。,18/64,Dysplastic Cerebellar Gangliocytoma Goal of therapy,Decompression of the ventricular system 解除脑室系统的压迫 difficult visualization: gradual change from normal cerebellar tissue to the abnormal tissue 正常脑组织与瘤组织分界不清 impairs a complete resection 难以完整切除 most patient
14、s do well following surgical resection 大多数患者预后良好 some have recurrence after a prolonged disease-free interval 少数患者经过一段静止期后仍可复发,19/64,Hemangioblastoma 血管母细胞瘤，血管网状细胞瘤（血网） Dysplastic Cerebellar Gangliocytoma 发育不良性神经节细胞瘤 Subependymoma 室管膜下瘤,PART TWO Adult Tumors of the Posterior Fossa,20/64,SUMMARY,Ofte
15、n Occurs in middle-aged men 好发于中年男性 fourth ventricle and lateral ventricle 多位于四脑室和侧脑室 Mass, clearly edge 团块状、边缘较清楚 no or slight Enhanced 增强扫描后，多无强化或轻微强化,21/64,Incidence and Clinical Presentation,Subependymoma,1945年由Scheinker首次报道 占颅内全部肿瘤的不足1% 大多数学者认为室管膜下瘤是室管膜瘤的一种类型 室管膜下瘤、中枢神经细胞瘤、室管膜下巨细胞星形细胞瘤Subependy
16、moma, central neurocytoma, subependymal giant cell astrocytoma具有相同的起源，均起源于室管膜下的具有双向分化潜能的神经胶质祖细胞Glial progenitor cells,22/64,Incidence and Clinical Presentation,Subependymoma,23/64,Incidence and Clinical Presentation,Subependymoma,rare, benign, slowgrowing tumor generally well-circumscribed sometimes multiple lesions most frequently: fourth ventricle distinctive histologic appearance histogenesis: controversial,良性肿瘤，生长缓慢 通常边界清楚 可为多发 常见于四脑室 病理学表现典型 组织学发生有争议,24/64,Incidence and Clinical Presentation,
