Hemangioblastoma血管母細(xì)胞瘤，血管網(wǎng)狀細(xì)胞瘤（血網(wǎng)）DysplasticCerebellarGangliocytoma發(fā)育不良性神經(jīng)節(jié)細(xì)胞瘤Subependymoma室管膜下瘤

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ofthePosteriorFossa1IncidenceandClinicalPresentationDysplasticCerebellarGangliocytomaOriginallydescribedin1920alsocalledLhermitte-Duclosdisease(LDD)neoplastichistogenesisVs.hamartomatousoriginyoungadults:averageage34yearsnogenderpredilection1920年首次報(bào)道又稱(chēng)為L(zhǎng)DD早期研究認(rèn)為其為腫瘤性病變，現(xiàn)認(rèn)為其為錯(cuò)構(gòu)瘤樣改變常見(jiàn)于年輕人，中位發(fā)病年齡34歲無(wú)性別差異2IncidenceandClinicalPresentationDysplasticCerebellarGangliocytomacommonlysymptomsincreasedintracranialpressurehydrocephalusMegalencephalymentalretardationdurationofsymptomsconsiderablevariabilityasymptomatic常見(jiàn)臨床癥狀顱內(nèi)壓增高腦積水巨腦畸形精神障礙癥狀時(shí)間不定甚至也可無(wú)癥狀3PathologicFindingsdisruptionofthenormalcerebellarlaminarstructurehypertrophicganglioncellsgranularandmolecularlayersofthecerebellarcortexincreasedmyelinationinthemolecularlayerMitoticactivityandnecrosisareuncommonDysplasticCerebellarGangliocytoma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(xì)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(jiàn)5PathologicFindingsDysplasticCerebellarGangliocytoma×200×100hypertrophicganglioncellsexpandingthegranularandmolecularlayersofthecerebellarcortex

6PathologicFindingsDysplasticCerebellarGangliocytoma免疫組化染色顯示神經(jīng)元特異性蛋白酶及突觸素陽(yáng)性表達(dá)7ImagingFindings-CTNCCTusuallyhypoattenuatedmaybeisoattenuatedCalcificationisuncommonThinningoftheskullDysplasticCerebellarGangliocytomaCT平掃常為低密度但也可為等密度無(wú)特異性診斷困難鈣化少見(jiàn)偶可見(jiàn)顱板變薄8ImagingFindings-MRIcharacteristic:bandshyperintensityandisointensityonT2isointenseandhypointenseonT1hyperintensesignalonT2correspondstotheinnermolecularlayer,granularcelllayer,andlossofcentralwhitematterDysplasticCerebellarGangliocytoma特征性表現(xiàn)：條帶T2等、高信號(hào)T1等低信號(hào)T2上所見(jiàn)的高信號(hào)條帶為內(nèi)分子層、顆粒細(xì)胞層，以及白質(zhì)細(xì)胞丟失所致10ImagingFindings-MRIDysplasticCerebellarGangliocytoma虎斑征，條紋征12ImagingFindings-MRIDysplasticCerebellarGangliocytoma1.5TSWIMIP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14ImagingFindings-MRIDysplasticCerebellarGangliocytoma圖1MRI平掃軸位T1WI(A)，增強(qiáng)掃描軸位T1WI(B)，左側(cè)小腦半球可見(jiàn)一團(tuán)塊狀長(zhǎng)T1、長(zhǎng)T2異常信號(hào)影，邊界清楚，其內(nèi)信號(hào)不均勻，可見(jiàn)條紋狀等T1、T2信號(hào)影。增強(qiáng)掃描未見(jiàn)明顯強(qiáng)化。圖2各向異性分?jǐn)?shù)（fractionalanisotropic,FA）灰度圖，腫瘤區(qū)域呈低信號(hào)。圖3ADC圖，腫瘤區(qū)域呈等及稍高信號(hào)15ImagingFindings-MRIDysplasticCerebellarGangliocytoma各向異性分?jǐn)?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(jiàn)條樣信號(hào)。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。16DysplasticCerebellarGangliocytoma

GoaloftherapyDecompressionoftheventricularsystem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(guò)一段靜止期后仍可復(fù)發(fā)17Hemangioblastoma血管母細(xì)胞瘤，血管網(wǎng)狀細(xì)胞瘤（血網(wǎng)）DysplasticCerebellarGangliocytoma發(fā)育不良性神經(jīng)節(jié)細(xì)胞瘤Subependymoma室管膜下瘤PARTTWO

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ofthePosteriorFossa18IncidenceandClinicalPresentationSubependymoma1945年由Scheinker首次報(bào)道占顱內(nèi)全部腫瘤的不足1%大多數(shù)學(xué)者認(rèn)為室管膜下瘤是室管膜瘤的一種類(lèi)型室管膜下瘤、中樞神經(jīng)細(xì)胞瘤、室管膜下巨細(xì)胞星形細(xì)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(xì)胞Glialprogenitorcells20IncidenceandClinicalPresentationSubependymoma21IncidenceandClinicalPresentationSubependymomaarisesfrom:subependymalgliallayerasymptomatic,incidentallyatautopsy(0.4%)MalesmorecommonlyMostcases(82%)olderthan15years起源于室管膜下通常無(wú)癥狀常常在尸檢中發(fā)現(xiàn)男性多見(jiàn)大多數(shù)為15歲以上成人23IncidenceandClinicalPresentationSubependymomahalfofthereportedcases：fourthventricle40～45%lateralventricleseptumpellucidumthirdventriclespinalcordtotalsurgicalresectionRecurrencerare半數(shù)以上病例見(jiàn)于四腦室其余大部分位于側(cè)腦室少見(jiàn)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(jiàn)24PathologicFindingsGrosslywell-circumscribedmassfirmtexturewhitetograyishcolornarrowpediclegrowsslowavascularsmallerthan2cmindiameterSubependymoma大體觀邊界清晰質(zhì)地堅(jiān)實(shí)灰白相間有窄蒂生長(zhǎng)緩慢乏血供直徑通常小于2厘米26PathologicFindingsHistologicdensefibrillarymatrixnumeroussmallcystsisomorphicnucleiMitotic:lowWHOgradeIAdmixturewithependymomaSubependymoma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(jiàn)WHOI級(jí)可與室管膜瘤混合生長(zhǎng)27PathologicFindingsSubependymomaHEstainingImmunohistochemistry28SubependymomaSubependymoma,CTappearance.AxialnonenhancedCTimagerevealsheterogeneousmidlinemassintheposteriorfossawithmultiplefociofcalcification.

ImagingFindings30SubependymomaImagingFindings31ImagingFindingsSubependymomaMRHeterogeneouspartiallycyst-likemasseshypointenseonT1hyperintenseonT2Intra-tumoralhemorrhageEnhancement:variablenotenhanceenhanceminimallyintenseenhancement磁共振信號(hào)不均勻