脑肿瘤PPT课件.ppt

Hemangioblastoma血管母细胞瘤，血管网状细胞瘤（血网）DysplasticCerebellarGangliocytoma发育不良性神经节细胞瘤Subependymoma室管膜下瘤,PARTTWOAdultTumorsofthePosteriorFossa,1,IncidenceandClinicalPresentation,DysplasticCerebellarGangliocytoma,Originallydescribedin1920alsocalledLhermitte-Duclosdisease(LDD)neoplastichistogenesisVs.hamartomatousoriginyoungadults:averageage34yearsnogenderpredilection,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,2,IncidenceandClinicalPresentation,DysplasticCerebellarGangliocytoma,commonlysymptomsincreasedintracranialpressurehydrocephalusMegalencephalymentalretardationdurationofsymptomsconsiderablevariabilityasymptomatic,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,3,DysplasticCerebellarGangliocytomaVSCowdendisease,Cowdendiseaseautosomaldominanthamartomasyndromecharacterizedsyndromemucocutaneouslesionsmacrocephalyhamartomaslongarmofchromosome10,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,ClinicalPresentation,4,PathologicFindings,disruptionofthenormalcerebellarlaminarstructurehypertrophicganglioncellsgranularandmolecularlayersofthecerebellarcortexincreasedmyelinationinthemolecularlayerMitoticactivityandnecrosisareuncommon,DysplasticCerebellarGangliocytoma,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,5,PathologicFindings,DysplasticCerebellarGangliocytoma,200,100,hypertrophicganglioncellsexpandingthegranularandmolecularlayersofthecerebellarcortex,6,PathologicFindings,DysplasticCerebellarGangliocytoma,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,7,ImagingFindings-CT,NCCTusuallyhypoattenuatedmaybeisoattenuatedCalcificationisuncommonThinningoftheskull,DysplasticCerebellarGangliocytoma,CT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,8,ImagingFindings-MRI,MRI：bestimagingmodalitycharacteristicappearancewithouthistopathologicalconfirmationtypicalappearances1.unilateralcerebellarmass2.non-enhancing3.middle-agedpatient4.tiger-stripedpattern,DysplasticCerebellarGangliocytoma,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,9,ImagingFindings-MRI,characteristic:bandshyperintensityandisointensityonT2isointenseandhypointenseonT1hyperintensesignalonT2correspondstotheinnermolecularlayer,granularcelllayer,andlossofcentralwhitematter,DysplasticCerebellarGangliocytoma,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,10,ImagingFindings-MRI,DysplasticCerebellarGangliocytoma,Dysplasticcerebellargangliocytomaina47-year-oldwoman.(a)AxialT1-weightedMRimageshowsacerebellarmasswithastripedappearance.(b)AxialT2-weightedMRimageshowsthesamelaminarmorphology,composedofalternatinghyperintenseandisointensebands.(c)Con-trast-enhancedaxialT1-weightedMRimageshowsnoenhancementofthemass.,11,ImagingFindings-MRI,DysplasticCerebellarGangliocytoma,虎斑征，条纹征,12,ImagingFindings-MRI,DysplasticCerebellarGangliocytoma,13,ImagingFindings-MRI,DysplasticCerebellarGangliocytoma,1.5TSWIMIP图像显示瘤周引流静脉，7TSWIMIP图像显示瘤周大量引流静脉及齿状核受压,14,ImagingFindings-MRI,DysplasticCerebellarGangliocytoma,图1MRI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（fractionalanisotropic,FA）灰度图，肿瘤区域呈低信号。图3ADC图，肿瘤区域呈等及稍高信号,15,ImagingFindings-MRI,DysplasticCerebellarGangliocytoma,各向异性分数（fractionalanisotropic,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(Fibertractography）。fibertractwithinthetumor肿瘤区纤维束以左右方向走行为主。,16,DysplasticCerebellarGangliocytomaGoaloftherapy,Decompressionoftheventricularsystem解除脑室系统的压迫difficultvisualization:gradualchangefromnormalcerebellartissuetotheabnormaltis