脑肿瘤1 neoplasms of the posterior 课件

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1、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PARTTWOAdultTumorsofthePosteriorFossaIncidenceandClinicalPresentationDysplasticCerebellarGangliocytomaOriginallydescribedin1920alsocalledLhermitte-Duclosdisease(LDD)neoplastichistogenesisVs.hamartomatou

2、soriginyoungadults: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IncidenceandClinicalPresentationDysplasticCerebellarGangliocytomacommonlysymptomsincreasedintracranialpressurehydrocephalusMegalencephalymentalretardationdurationofsymptomsconsidera

3、blevariabilityasymptomatic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DysplasticCerebellarGangliocytomaVSCowdendiseaseCowdendiseaseautosomaldominanthamartomasyndromecharacterizedsyndromemucocutaneouslesionsmacrocephalyhamartomaslongarmofchromosome10Cowden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

4、PresentationPathologicFindingsdisruptionofthenormalcerebellarlaminarstructurehypertrophicganglioncellsgranularandmolecularlayersofthecerebellarcortexincreasedmyelinationinthemolecularlayerMitoticactivityandnecrosisareuncommonDysplasticCerebellarGangliocytoma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

5、鞘化增加病理组织切片上有丝分裂活性及坏死少见PathologicFindingsDysplasticCerebellarGangliocytoma×200×100hypertrophicganglioncellsexpandingthegranularandmolecularlayersofthecerebellarcortexPathologicFindingsDysplasticCerebellarGangliocytoma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ImagingFindings-CTNCCTusuallyhypoattenuatedmaybeisoattenu

6、atedCalcificationisuncommonThinningoftheskullDysplasticCerebellarGangliocytomaCT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ImagingFindings-MRIMRI：bestimagingmodalitycharacteristicappearancewithouthistopathologicalconfirmationtypicalappearances1.unilateralcerebellarmass2.non-enhancing3.middle-agedpatient4.ti

7、ger-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ImagingFindings-MRIcharacteristic:bandshyperintensityandisointensityonT2isointenseandhypointense