成人后颅窝脑肿瘤ppt课件

1 64 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 AdultTumorsofthePosteriorFossa 2 64 3 64 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 4 64 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 5 64 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 6 64 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 7 64 PathologicFindings DysplasticCerebellarGangliocytoma 200 100 hypertrophicganglioncellsexpandingthegranularandmolecularlayersofthecerebellarcortex 8 64 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 9 64 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 10 64 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 11 64 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 12 64 ImagingFindings MRI DysplasticCerebellarGangliocytoma Dysplasticcerebellargangliocytomaina47 year oldwoman a AxialT1 weightedMRimageshowsacerebellarmasswithastripedappearance b AxialT2 weightedMRimageshowsthesamelaminarmorphology composedofalternatinghyperintenseandisointensebands c Con trast enhancedaxialT1 weightedMRimageshowsnoenhancementofthemass 13 64 ImagingFindings MRI DysplasticCerebellarGangliocytoma 虎斑征 条纹征 14 64 ImagingFindings MRI DysplasticCerebellarGangliocytoma 15 64 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 16 64 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 17 64 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 18 64 DysplasticCerebellarGangliocytomaGoaloftherapy Decompressionoftheventricularsystem解除脑室系统的压迫difficultvisualization gradualchangefromnormalcerebellartissuetotheabnormaltissue正常脑组织与瘤组织分界不清impairsacompleteresection难以完整切除mostpatientsdowellfollowingsurgicalresection大多数患者预后良好somehaverecurrenceafteraprolongeddisease freeinterval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 DysplasticCerebellarGangliocytoma发育不良性神经节细胞瘤Subependymoma室管膜下瘤 PARTTWOAdultTumorsofthePosteriorFossa 20 64 SUMMARY OftenOccursinmiddle agedmen好发于中年男性fourthventricleandlateralventricle多位于四脑室和侧脑室Mass clearlyedge团块状 边缘较清楚noorslightEnhanced增强扫描后 多无强化或轻微强化 21 64 IncidenceandClinicalPresentation 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Subependymoma centralneurocytoma subependymalgiantcellastrocytoma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Glialprogenitorcells 22 64 IncidenceandClinicalPresentation Subependymoma 23 64 IncidenceandClinicalPresentation Subependymoma rare benign slowgrowingtumorgenerallywell circumscribedsometimesmultiplelesionsmostfrequently fourthventricledistinctivehistologicappearancehistogenesis 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 IncidenceandClinicalPresentation Subependymoma arisesfrom subependymalgliallayerasymptomatic incidentallyatautopsy 0 4 MalesmorecommonlyMostcases 82 olderthan15years 起源于室管膜下通常无症状常常在尸检中发现男性多见大多数为15岁以上成人 25 64 IncidenceandClinicalPresentation Subependymoma halfofthereportedcases fourthventricle40 45 lateralventricleseptumpellucidumthirdventriclespinalcordtotalsurgicalresectionRecurrencerare 半数以上病例见于四脑室其余大部分位于侧脑室少见部位透明隔三脑室脊髓手术切除是治疗方法复发少见 26 64 IncidenceandClinicalPresentation Subependymoma clinicalpresentation non specificlocation size andintratumoralhemorrhagehydrocephalusneurologicdeficits seizures SAHgoodprognosis 临床症状不典型临床症状取决于肿瘤的部位 大小 是否合并有肿瘤内出血等常见症状为脑积水所致的症状其它也可有神经功能缺损 癫痫 蛛网膜下腔出血等手术后预后良好 27 64 PathologicFindings Grosslywell circumscribedmassfirmtexturewhitetograyishcolornarrowpediclegrowsslowavascularsmallerthan2cmindiameter Subependymoma 大体观边界清晰质地坚实灰白相间有窄蒂生长缓慢乏血供直径通常小于2厘米 28 64 PathologicFindings HistologicdensefibrillarymatrixnumeroussmallcystsisomorphicnucleiMitotic lowWHOgradeIAdmixturewithependymoma Subependymoma 组织病理学致密纤维基质大量小囊同构核有丝分裂少见WHOI级可与室管膜瘤混合生长 29 64 PathologicFindings Subependymoma HEstainingImmunohistochemistry 30 64 ImagingFindings Subependymoma CTwell circumscribedlobulatedintraventricularmasshydrocephalushypoattenuatedCalcification commoncysticdegeneration commonmost 84 enhance CT图像上边界清晰有分叶脑室内肿块脑积水低密度钙化 囊性变多见增强后大多数强化 31 64 Su