成人后顱窩腦腫瘤ppt課件

1 64 Hemangioblastoma血管母細胞瘤 血管網(wǎng)狀細胞瘤 血網(wǎng) DysplasticCerebellarGangliocytoma發(fā)育不良性神經(jīng)節(jié)細胞瘤Subependymoma室管膜下瘤 AdultTumorsofthePosteriorFossa 2 64 3 64 IncidenceandClinicalPresentation DysplasticCerebellarGangliocytoma Originallydescribedin1920alsocalledLhermitte Duclosdisease LDD neoplastichistogenesisVs hamartomatousoriginyoungadults averageage34yearsnogenderpredilection 1920年首次報道又稱為LDD早期研究認為其為腫瘤性病變 現(xiàn)認為其為錯構(gòu)瘤樣改變常見于年輕人 中位發(fā)病年齡34歲無性別差異 4 64 IncidenceandClinicalPresentation DysplasticCerebellarGangliocytoma commonlysymptomsincreasedintracranialpressurehydrocephalusMegalencephalymentalretardationdurationofsymptomsconsiderablevariabilityasymptomatic 常見臨床癥狀顱內(nèi)壓增高腦積水巨腦畸形精神障礙癥狀時間不定甚至也可無癥狀 5 64 DysplasticCerebellarGangliocytomaVSCowdendisease Cowdendiseaseautosomaldominanthamartomasyndromecharacterizedsyndromemucocutaneouslesionsmacrocephalyhamartomaslongarmofchromosome10 Cowden病常染色體顯性遺傳錯構(gòu)瘤樣病變特征性表現(xiàn)皮膚粘膜病變巨腦畸形錯構(gòu)瘤10號染色體長臂異常 ClinicalPresentation 6 64 PathologicFindings disruptionofthenormalcerebellarlaminarstructurehypertrophicganglioncellsgranularandmolecularlayersofthecerebellarcortexincreasedmyelinationinthemolecularlayerMitoticactivityandnecrosisareuncommon DysplasticCerebellarGangliocytoma 正常小腦板層結(jié)構(gòu)破壞大量增生肥大的神經(jīng)節(jié)細胞侵蝕小腦皮層的顆粒層和分子層分子層髓鞘化增加病理組織切片上有絲分裂活性及壞死少見 7 64 PathologicFindings DysplasticCerebellarGangliocytoma 200 100 hypertrophicganglioncellsexpandingthegranularandmolecularlayersofthecerebellarcortex 8 64 PathologicFindings DysplasticCerebellarGangliocytoma 免疫組化染色顯示神經(jīng)元特異性蛋白酶及突觸素陽性表達 9 64 ImagingFindings CT NCCTusuallyhypoattenuatedmaybeisoattenuatedCalcificationisuncommonThinningoftheskull DysplasticCerebellarGangliocytoma CT平掃常為低密度但也可為等密度無特異性診斷困難鈣化少見偶可見顱板變薄 10 64 ImagingFindings MRI MRI bestimagingmodalitycharacteristicappearancewithouthistopathologicalconfirmationtypicalappearances1 unilateralcerebellarmass2 non enhancing3 middle agedpatient4 tiger stripedpattern DysplasticCerebellarGangliocytoma MRI是診斷的最佳方式特征性表現(xiàn)幾乎可在不需要病理證實的條件下做出明確診斷四條特征1 單側(cè)半球腫塊2 不強化3 成年患者4 虎斑征 11 64 ImagingFindings MRI characteristic bandshyperintensityandisointensityonT2isointenseandhypointenseonT1hyperintensesignalonT2correspondstotheinnermolecularlayer granularcelllayer andlossofcentralwhitematter DysplasticCerebellarGangliocytoma 特征性表現(xiàn) 條帶T2等 高信號T1等低信號T2上所見的高信號條帶為內(nèi)分子層 顆粒細胞層 以及白質(zhì)細胞丟失所致 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 條紋征 14 64 ImagingFindings MRI DysplasticCerebellarGangliocytoma 15 64 ImagingFindings MRI DysplasticCerebellarGangliocytoma 1 5TSWIMIP圖像顯示瘤周引流靜脈 7TSWIMIP圖像顯示瘤周大量引流靜脈及齒狀核受壓 16 64 ImagingFindings MRI DysplasticCerebellarGangliocytoma 圖1MRI平掃軸位T1WI A 增強掃描軸位T1WI B 左側(cè)小腦半球可見一團塊狀長T1 長T2異常信號影 邊界清楚 其內(nèi)信號不均勻 可見條紋狀等T1 T2信號影 增強掃描未見明顯強化 圖2各向異性分數(shù) fractionalanisotropic FA 灰度圖 腫瘤區(qū)域呈低信號 圖3ADC圖 腫瘤區(qū)域呈等及稍高信號 17 64 ImagingFindings MRI DysplasticCerebellarGangliocytoma 各向異性分數(shù) fractionalanisotropic FA 方向彩色編碼圖上正常小腦區(qū)域呈綠色 表示前后走行 腫瘤區(qū)以紅色為主 表示左右走行 未見條樣信號 圖5纖維束追蹤成像像 Fibertractography fibertractwithinthetumor腫瘤區(qū)纖維束以左右方向走行為主 18 64 DysplasticCerebellarGangliocytomaGoaloftherapy Decompressionoftheventricularsystem解除腦室系統(tǒng)的壓迫difficultvisualization gradualchangefromnormalcerebellartissuetotheabnormaltissue正常腦組織與瘤組織分界不清impairsacompleteresection難以完整切除mostpatientsdowellfollowingsurgicalresection大多數(shù)患者預(yù)后良好somehaverecurrenceafteraprolongeddisease freeinterval少數(shù)患者經(jīng)過一段靜止期后仍可復(fù)發(fā) 19 64 Hemangioblastoma血管母細胞瘤 血管網(wǎng)狀細胞瘤 血網(wǎng) DysplasticCerebellarGangliocytoma發(fā)育不良性神經(jīng)節(jié)細胞瘤Subependymoma室管膜下瘤 PARTTWOAdultTumorsofthePosteriorFossa 20 64 SUMMARY OftenOccursinmiddle agedmen好發(fā)于中年男性fourthventricleandlateralventricle多位于四腦室和側(cè)腦室Mass clearlyedge團塊狀 邊緣較清楚noorslightEnhanced增強掃描后 多無強化或輕微強化 21 64 IncidenceandClinicalPresentation Subependymoma 1945年由Scheinker首次報道占顱內(nèi)全部腫瘤的不足1 大多數(shù)學(xué)者認為室管膜下瘤是室管膜瘤的一種類型室管膜下瘤 中樞神經(jīng)細胞瘤 室管膜下巨細胞星形細胞瘤Subependymoma centralneurocytoma subependymalgiantcellastrocytoma具有相同的起源 均起源于室管膜下的具有雙向分化潛能的神經(jīng)膠質(zhì)祖細胞Glialprogenitorcells 22 64 IncidenceandClinicalPresentation Subependymoma 23 64 IncidenceandClinicalPresentation Subependymoma rare benign slowgrowingtumorgenerallywell circumscribedsometimesmultiplelesionsmostfrequently fourthventricledistinctivehistologicappearancehistogenesis controversial 良性腫瘤 生長緩慢通常邊界清楚可為多發(fā)常見于四腦室病理學(xué)表現(xiàn)典型組織學(xué)發(fā)生有爭議 24 64 IncidenceandClinicalPresentation Subependymoma arisesfrom subependymalgliallayerasymptomatic incidentallyatautopsy 0 4 MalesmorecommonlyMostcases 82 olderthan15years 起源于室管膜下通常無癥狀常常在尸檢中發(fā)現(xiàn)男性多見大多數(shù)為15歲以上成人 25 64 IncidenceandClinicalPresentation Subependymoma halfofthereportedcases fourthventricle40 45 lateralventricleseptumpellucidumthirdventriclespinalcordtotalsurgicalresectionRecurrencerare 半數(shù)以上病例見于四腦室其余大部分位于側(cè)腦室少見部位透明隔三腦室脊髓手術(shù)切除是治療方法復(fù)發(fā)少見 26 64 IncidenceandClinicalPresentation Subependymoma clinicalpresentation non specificlocation size andintratumoralhemorrhagehydrocephalusneurologicdeficits seizures SAHgoodprognosis 臨床癥狀不典型臨床癥狀取決于腫瘤的部位 大小 是否合并有腫瘤內(nèi)出血等常見癥狀為腦積水所致的癥狀其它也可有神經(jīng)功能缺損 癲癇 蛛網(wǎng)膜下腔出血等手術(shù)后預(yù)后良好 27 64 PathologicFindings Grosslywell circumscribedmassfirmtexturewhitetograyishcolornarrowpediclegrowsslowavascularsmallerthan2cmindiameter Subependymoma 大體觀邊界清晰質(zhì)地堅實灰白相間有窄蒂生長緩慢乏血供直徑通常小于2厘米 28 64 PathologicFindings HistologicdensefibrillarymatrixnumeroussmallcystsisomorphicnucleiMitotic lowWHOgradeIAdmixturewithependymoma Subependymoma 組織病理學(xué)致密纖維基質(zhì)大量小囊同構(gòu)核有絲分裂少見WHOI級可與室管膜瘤混合生長 29 64 PathologicFindings Subependymoma HEstainingImmunohistochemistry 30 64 ImagingFindings Subependymoma CTwell circumscribedlobulatedintraventricularmasshydrocephalushypoattenuatedCalcification commoncysticdegeneration commonmost 84 enhance CT圖像上邊界清晰有分葉腦室內(nèi)腫塊腦積水低密度鈣化 囊性變多見增強后大多數(shù)強化 31 64 Su