成人后颅窝脑肿瘤课件_1

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1、1/64Hemangioblastoma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AdultTumorsofthePosteriorFossa2/643/64IncidenceandClinicalPresentationDysplasticCerebellarGangliocytomaOriginallydescribedin1920alsocalledLhermitte-Duclosdisease(LDD)neoplastichistogenesisVs.hamar

2、tomatousoriginyoungadults:averageage34yearsnogenderpredilection1920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4/64IncidenceandClinicalPresentationDysplasticCerebellarGangliocytomacommonlysymptomsincreasedintracranialpressurehydrocephalusMegalencephalymentalretardationdurationofsympt

3、omsconsiderablevariabilityasymptomatic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5/64DysplasticCerebellarGangliocytomaVSCowdendiseaseCowdendiseaseautosomaldominanthamartomasyndromecharacterizedsyndromemucocutaneouslesionsmacrocephalyhamartomaslongarmofchromosome10Cowden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1

4、0号染色体长臂异常ClinicalPresentation8/64PathologicFindingsDysplasticCerebellarGangliocytoma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9/64ImagingFindings-CTNCCTusuallyhypoattenuatedmaybeisoattenuatedCalcificationisuncommonThinningoftheskullDysplasticCerebellarGangliocytomaCT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10/64ImagingFi

5、ndings-MRIMRI：bestimagingmodalitycharacteristicappearancewithouthistopathologicalconfirmationtypicalappearances1.unilateralcerebellarmass2.non-enhancing3.middle-agedpatient4.tiger-stripedpatternDysplasticCerebellarGangliocytomaMRI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

6、ImagingFindings-MRIcharacteristic:bandshyperintensityandisointensityonT2isointenseandhypointenseonT1hyperintensesignalonT2correspondstotheinnermolecularlayer,granularcelllayer,andlossofcentralwhitematterDysplasticCerebellarGangliocytoma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12/64Im

7、agingFindings-MRIDysplasticCerebellarGangliocytomaDysplasticcerebellargangliocytomaina47-year-oldwoman.(a)AxialT1-weightedMRimageshowsacerebellarmasswithastripedappearance.(b)AxialT2-weightedMRimageshowsthesamelaminarm