兒童后顱窩腦腫瘤

兒童后顱窩腦腫瘤2/64PediatricNeoplasms

ofthePosteriorFossa顱內(nèi)高壓征多呈漸進(jìn)性患兒常無典型顱高壓三聯(lián)征表現(xiàn)，視乳頭水腫少見多以頭痛和/或嘔吐為主要就診原因嘔吐易與多種消化道疾病相混淆侵犯小腦半球和小腦蚓部可導(dǎo)致軀體和四肢共濟(jì)失調(diào)但學(xué)齡兒活動范圍較大，癥狀常發(fā)現(xiàn)較晚臨床癥狀不典型3/64PediatricNeoplasms

ofthePosteriorFossa嬰兒囟門未閉，學(xué)齡兒及幼兒顱縫閉合不緊臨床以頭圍異常增大為主要體征，易被家長忽視小兒神經(jīng)系統(tǒng)發(fā)育不完善，小腦損害征不易察覺臨床表現(xiàn)無特異性，易與神經(jīng)系統(tǒng)感染相混淆早期體征不明顯4/645/646/64PediatricNeoplasmsofthePosteriorFossa

-----SeriestopicMedulloblastoma髓母細(xì)胞瘤PilocyticAstrocytomaEpendymomaChoroidPlexusPapillomaandCarcinoma7/64以神經(jīng)膠質(zhì)瘤和神經(jīng)元兩種未分化的神經(jīng)上皮細(xì)胞為基礎(chǔ)形成的腫瘤鏡下細(xì)胞形態(tài)很像胚胎期的髓母細(xì)胞1926年由Bailey和Cushing命名為髓母細(xì)胞瘤新的WHO分類將其歸為幕下原始神經(jīng)外胚層瘤（PNET）IncidenceandClinicalPresentationMedulloblastoma髓母細(xì)胞瘤8/64mostcommonpediatriccentralnervoussystemmalignancy最常見的兒童原發(fā)腦腫瘤（15%~25%）secondmostcommonpediatricbrainneoplasm最常見的兒童后顱窩原發(fā)腦腫瘤（33%）Males(60%)areslightlymorecommonly男性常見themeanageisabout7years平均好發(fā)年齡7歲，也可見于20到40歲（但成人少見）IncidenceandClinicalPresentationMedulloblastoma髓母細(xì)胞瘤9/64mostariseinthemidlinecerebellarvermis多見于小腦蚓部，以中下蚓多見laterallocationwithinthecerebellarhemisphere：olderchildren年長兒及青少年（大齡組）多見于小腦半球otherlocation:Brainstem,thefourthventricle,spinalcord其它部位：腦干、四腦室、脊髓IncidenceandClinicalPresentationMedulloblastoma髓母細(xì)胞瘤10/64clinicalpresentation：usuallylessthan3months腫瘤的侵襲性生物學(xué)特性：病程通常短于三個月commonsymptoms:Headacheandpersistentvomiting常見癥狀：頭痛或持續(xù)性嘔吐Truncalataxiaandspasticity共濟(jì)失調(diào)和痙攣狀態(tài)Abducensnervepalsy外展神經(jīng)麻痹IncidenceandClinicalPresentationMedulloblastoma髓母細(xì)胞瘤11/64PathologicFindingsMedulloblastoma髓母細(xì)胞瘤手術(shù)標(biāo)本灰紅色或粉紅色，邊緣清楚，無明確包膜，柔軟易碎尸檢標(biāo)本位于小腦上蚓部腫塊，邊界清楚12/64grossappearance:variable大體病理表現(xiàn)多樣mostcommonsubtype:classicsubtype最常見的亞型為經(jīng)典型Othersubtypes:desmoplasticsubtype,“medulloblastomawithextensivenodularityandadvancedneuronaldifferentiation”subtype,andthelargecellmedulloblastomasubtype其它亞型包括：纖維組織型、高分化型和大細(xì)胞型鏡下特點(diǎn)為細(xì)胞排列非常致密，呈菊花團(tuán)狀，主要為小圓細(xì)胞組成，胞漿少，核大且濃染PathologicFindingsMedulloblastoma髓母細(xì)胞瘤13/64免疫組織化學(xué)表現(xiàn)及應(yīng)用尿激酶型纖酶原激活劑(urokinasetypeplasminogenactivator,uPA)與腫瘤浸潤轉(zhuǎn)移關(guān)系密切血管內(nèi)皮生長因子受體(vascularendothelialgrowthfactorreceptor-2,KDR)與腫瘤血管生成及其轉(zhuǎn)移密切相關(guān)uPA或KDR高表達(dá)的患者，其平均生存時間均較低表達(dá)者短可作為預(yù)測髓母細(xì)胞瘤預(yù)后的指標(biāo)PathologicFindingsMedulloblastoma髓母細(xì)胞瘤14/64hyperattenuatedwell-defined邊緣清楚的高密度（特征性）surroundingvasogenicedema血管源性水腫fairlyuniformcontrastenhancement均勻強(qiáng)化ImagingFindingsMedulloblastoma髓母細(xì)胞瘤15/64ImagingFindings

MRI診斷要素Medulloblastoma髓母細(xì)胞瘤兒童者多位于小腦中下蚓部，突向四腦室，軸位可見第四腦室受壓變扁，呈弧形包繞在腫瘤前方和側(cè)面，幕上腦室阻塞性腦積水發(fā)生于小腦，多呈類圓形，瘤周水腫較輕，鄰近腦實(shí)質(zhì)受擠壓壞死、囊變、出血及鈣化少見腫瘤實(shí)性部分在所有MRI序列上均與腦灰質(zhì)信號相近由于MB生長迅速，質(zhì)地軟，信號相對較均勻增強(qiáng)掃描腫瘤實(shí)性部分明顯強(qiáng)化容易發(fā)生沿腦脊液通路在蛛網(wǎng)膜下腔種植轉(zhuǎn)移16/64ImagingFindings

MRI新技術(shù)Medulloblastoma髓母細(xì)胞瘤DWI彌散受限MRS：Choline↑，NAA↓Cr↓，Lac↑（occasionally）特征性的氨基乙磺酸峰MRA可顯示起于小腦后下動脈的供血血管17/64ImagingFindingsMedulloblastoma髓母細(xì)胞瘤T1WIT2WIFLAIRGd-DTPADWIADC男性,8歲,小腦蚓部髓母細(xì)胞瘤,腫瘤呈類圓形,邊界清晰圖1

T1WI呈略低信號,腫瘤前方可見弧形四腦室腦脊液信號圖2同一層面T2WI呈略高信號圖3

FLAIR呈略高信號圖4增強(qiáng)T1WI腫瘤呈明顯對比增強(qiáng)圖5

DWI瘤體呈略高于腦實(shí)質(zhì)信號,囊變區(qū)呈低信號18/649-year-oldboywithmedulloblastoma.Ondiffusion-weightedimage,lesionshowsrestricteddiffusion

彌散受限的機(jī)制腫瘤細(xì)胞密度高細(xì)胞外間隙小腫瘤細(xì)胞的胞漿少核漿比例較大ImagingFindingsMedulloblastoma髓母細(xì)胞瘤19/64Medulloblastoma髓母細(xì)胞瘤20/64Medulloblastoma髓母細(xì)胞瘤21/64Subarachnoidseedingiscommoninmedulloblastomas髓母細(xì)胞瘤常見蛛網(wǎng)膜下腔播散，經(jīng)由腦脊液在腦室和蛛網(wǎng)膜下腔內(nèi)種植轉(zhuǎn)移，增強(qiáng)掃描，轉(zhuǎn)移灶同原發(fā)灶一樣發(fā)生顯著對比增強(qiáng)種植灶呈均勻結(jié)節(jié)狀或不規(guī)則環(huán)狀中度至明顯強(qiáng)化Post-operativehemorrhage：hamperedsubarachnoidseeding手術(shù)后出血灶常掩蓋蛛網(wǎng)膜下腔播散clinicalandcytopathologicCSFfindings臨床及CSF檢查有助于蛛網(wǎng)膜下腔播散的診斷ImagingFindingsMedulloblastoma髓母細(xì)胞瘤22/6423/64ImagingFindingsMedulloblastoma髓母細(xì)胞瘤兒童髓母細(xì)胞瘤：小腦蚓部成人髓母細(xì)胞瘤：小腦半球差異的機(jī)制髓母細(xì)胞瘤起源于四腦室頂后髓帆神經(jīng)上皮細(xì)胞的殘余這種原始細(xì)胞隨生長發(fā)育向上并向外移行形成小腦的外顆粒層MB可發(fā)生于此移行過程的任何部位，并限于后顱窩兒童MB多起自四腦室頂部后髓帆生殖中心的胚胎殘余細(xì)胞成人MB多起源于小腦軟膜下分子層表面一種較原始的小腦外顆粒層細(xì)胞24/64ImagingFindingsMedulloblastoma髓母細(xì)胞瘤CT平掃（A、B）示左側(cè)小腦半球一不規(guī)則略高密度影（↑），邊緣不清，其周圍可見帶狀低密度影；增強(qiáng)掃描（C、D）病變強(qiáng)化較明顯（↑），內(nèi)見不規(guī)則點(diǎn)、線狀低密度區(qū)，四腦室受壓、變窄，向右側(cè)移位腫瘤位于小腦半球者多見于成人25/64平均生存時間為9-11個月（不足1年）早期治療手段開顱手術(shù)和傳統(tǒng)放療為主現(xiàn)今開顱手術(shù)與輔助的放療、化療相結(jié)合的綜合治療生長迅速，細(xì)胞分裂指數(shù)高對放療中度敏感髓母細(xì)胞瘤術(shù)后強(qiáng)調(diào)早期放療全中樞神經(jīng)系統(tǒng)進(jìn)行放療治療Medulloblastoma髓母細(xì)胞瘤26/64PediatricNeoplasmsofthePosteriorFossa

-----SeriestopicMedulloblastomaPilocyticAstrocytoma毛細(xì)胞型星形細(xì)胞瘤EpendymomaChoroidPlexusPapillomaandCarcinoma27/64Penfield于1937年根據(jù)腫瘤細(xì)胞兩端胞突起為細(xì)長的毛發(fā)樣膠質(zhì)纖維絲而命名占小腦星形細(xì)胞瘤的絕大多數(shù)（85%）好發(fā)于幕下，以小腦蚓部多見也可見于所有星形細(xì)胞所在的部位：丘腦、視交叉、小腦、腦干、大腦半球及脊髓高發(fā)年齡0-9歲無性別差異IncidenceandClinicalPresentationPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤28/64commonsymptoms：ataxia,headache,nausea,andvomiting常見癥狀：共濟(jì)失調(diào)、頭痛、惡心嘔吐prolongedclinicalcourse：slowgrowthofthetumor腫瘤生長緩慢，臨床病史較長excellentprognosis預(yù)后良好IncidenceandClinicalPresentationPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤29/64低級別的星形細(xì)胞瘤，WHOI級良性腫瘤，生長緩慢，預(yù)后好雙相性：由致密區(qū)和疏松區(qū)組成腫瘤富含豐富的血管惡性轉(zhuǎn)化及復(fù)發(fā)：均少見極高的存活率，10年生存率可達(dá)94%PathologicFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤30/64PathologicFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤鏡下特點(diǎn)：毛樣星形細(xì)胞與疏松成熟的膠質(zhì)纖維混雜排列31/64典型影像學(xué)特點(diǎn)囊性腫塊伴強(qiáng)化的壁結(jié)節(jié)瘤周水腫少見，說明其良性腫瘤的特性無彌散受限，DWI無高信號表現(xiàn)ImagingFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤32/64囊腫型病變呈囊性，沒有壁結(jié)節(jié)或?qū)嵭阅[塊囊腫結(jié)節(jié)型以囊性病變?yōu)橹?，伴有壁結(jié)節(jié)瘤囊型囊內(nèi)有瘤，瘤內(nèi)有囊腫塊型病變以實(shí)性部分為主，伴有或不伴有囊變ImagingFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤33/64腦腫瘤的強(qiáng)化程度與腫瘤的惡性程度呈正相關(guān)腫瘤的惡性程度越高腫瘤血管發(fā)育不成熟和血腦屏障破壞程度越重而毛細(xì)胞型星形細(xì)胞瘤的強(qiáng)化不代表腫瘤為惡性和血腦屏障破壞與腫瘤血管的自身特點(diǎn)（有孔型毛細(xì)血管）有關(guān)對比劑可通過腫瘤血管的內(nèi)皮間隙到達(dá)腫瘤組織所以增強(qiáng)掃描時表現(xiàn)為實(shí)性成分的均勻明顯強(qiáng)化而這在低級別星形細(xì)胞瘤中少見ImagingFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤34/64ImagingFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤囊腫結(jié)節(jié)型，壁結(jié)節(jié)明顯強(qiáng)化35/64ImagingFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤囊腫結(jié)節(jié)型，壁結(jié)節(jié)明顯強(qiáng)化瘤周水腫不明顯，說明其良性特點(diǎn)36/64ImagingFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤瘤囊型：囊內(nèi)有瘤，瘤內(nèi)有囊37/64ImagingFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤小腦蚓部毛細(xì)胞星形細(xì)胞瘤，腫瘤以實(shí)性為主，呈明顯不均勻強(qiáng)化，其內(nèi)可見多發(fā)大小不等不強(qiáng)化的囊變區(qū)腫塊型38/64ImagingFindingsPilocyticAstrocytoma

毛細(xì)胞型星形細(xì)胞瘤偏良性的腫瘤實(shí)質(zhì)部分細(xì)胞密度小細(xì)胞間隙大水分子彌散受限較輕良性腫瘤:彌散不受限39/64PediatricNeoplasmsofthePosteriorFossa

-----SeriestopicMedulloblastomaPilocyticAstrocytomaEpendymomaChoroidPlexusPapillomaandCarcinoma40/646–12%ofallpediatricbraintumors占所有兒童腦腫瘤的6–12%one-thirdofallbraintumorsinpatientsyoungerthan3years占所有嬰幼兒腦腫瘤的1/358%fourthventricle;42%lateralandthirdventricles58%見于四腦室，42%見于側(cè)腦室及三腦室meanage:6yearsold平均發(fā)病年齡：6歲IncidenceandClinicalPresentationEpendymoma室管膜瘤41/64病程較長，呈緩慢發(fā)展過程臨床表現(xiàn)早期因腫瘤體積小、活動性大，體位變化時可堵塞導(dǎo)水管或四腦室側(cè)孔或正中孔，而引起暫時性腦脊液循環(huán)障礙，表現(xiàn)為發(fā)作性頭痛、惡心、嘔吐；當(dāng)腫瘤增大充滿四腦室時可造成持續(xù)性腦脊液循環(huán)障礙，引起腦積水，出現(xiàn)持續(xù)的頭痛、惡心、嘔吐。IncidenceandClinicalPresentationEpendymoma室管膜瘤42/64起源于已分化的室管膜細(xì)胞起自四腦室頂或底結(jié)節(jié)狀或分葉狀、膨脹性生長病理特征：血管旁假菊形團(tuán)生長緩慢的膠質(zhì)瘤WHOII級腫瘤出血、壞死、囊變和鈣化PathologicFindingsEpendymoma室管膜瘤43/64PathologicFindingsEpendymoma室管膜瘤病理切片血管旁假菊形團(tuán)結(jié)構(gòu)尸檢標(biāo)本起自四腦室底44/64ImagingFindingsEpendymoma室管膜瘤等密度或稍高密度其內(nèi)常有點(diǎn)狀鈣化及低密度囊變無瘤周水腫，周圍可見CSF間隙均勻明顯強(qiáng)化CT45/64ImagingFindingsEpendymoma室管膜瘤T1WI等-低信號，T2WI高信號生長時間長，可發(fā)生鈣化或囊性變，其T1WI及T2WI的信號不均勻中腦導(dǎo)水管擴(kuò)張-“茄莢征”起源于四腦室底，因此常將四腦室推向后部呈裂隙狀無瘤周水腫，周圍可見CSF間隙明顯強(qiáng)化MRI46/64ImagingFindingsEpendymoma室管膜瘤MRI茄莢征47/64ImagingFindingsEpendymoma室管膜瘤MRIT2WI（A）示四腦室區(qū)高信號為主的異常信號（↑），矢狀面T1WI（B）腫瘤呈不均勻略低信號（↑），幕上腦室擴(kuò)張、積水；增強(qiáng)掃描（C、D）腫瘤呈斑片狀明顯強(qiáng)化（↑）

48/64ImagingFindingsEpendymoma室管膜瘤49/64ImagingFindingsEpendymoma室管膜瘤ShortTEandlongTEMRSofanependymomaverylowlevelsofNAAandCr50/64PediatricNeoplasmsofthePosteriorFossa

-----SeriestopicMedulloblastomaPilocyticAstrocytomaEpendymomaChoroidPlexusPapillomaandCarcinoma51/64IncidenceandClinicalPresentationChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤

14歲以下兒童多見于側(cè)腦室發(fā)生于四腦室者占兒童CPP的30%脈絡(luò)叢乳頭狀瘤（80%）、癌（20%）起源于腦室脈絡(luò)叢上皮細(xì)胞后顱窩的CPP除可見于第四腦室外側(cè)隱窩或第四腦室內(nèi)外，亦可見于腦橋小腦三角區(qū)52/64IncidenceandClinicalPresentationChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤

Hydrocephalus腦積水overproductionofCSF產(chǎn)生過多impairedresorptionofCSFsecondarytohemorrhage出血所致的CSF吸收障礙obstructionofnormalCSFflow梗阻性腦積水Prognosis預(yù)后choroidplexuspapilloma:excellent脈絡(luò)叢乳頭狀瘤：好choroidplexuscarcinoma:lesspromising脈絡(luò)叢乳頭狀癌：差53/64Grosslysoftcauliflower-likemassesperipherallobulationsfrequenthemorrhagecystformationNecrosisandparenchymalinvasionPathologicFindingsChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤

大體觀菜花狀軟組織腫塊周圍分葉常有出血囊變壞死及周圍組織侵犯54/64Histologicexamination:choroidplexuspapillomas脈絡(luò)叢乳頭狀瘤鏡下觀quitesimilartonormalnonneoplasticchoroidplexustissue與正常脈絡(luò)叢組織類似fibrovascularconnectivetissuesurroundedbycolumnarorcuboidalcells柱狀及立方細(xì)胞環(huán)繞纖維血管結(jié)締組織withoutsignificantmitoticactivity核分裂像少見PathologicFindingsChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤

55/64Histologicexamination:choroidplexuspapillomas脈絡(luò)叢乳頭狀瘤鏡下觀柱狀及立方細(xì)胞環(huán)繞纖維血管結(jié)締組織56/64Histologicexamination:choroidplexuscarcinoma脈絡(luò)叢乳頭狀癌鏡下觀hypercellularity,nuclearpleomorphism細(xì)胞密度高，核異型多見highnucleuscytoplasmratio核漿比高conspicuousmitoticactivity核分裂像多見invasionintotheadjacentbrainparenchyma易侵犯周圍腦組織PathologicFindingsChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤

57/64Histologicexamination:choroidplexuscarcinoma脈絡(luò)叢乳頭狀癌鏡下觀Poorlydifferentiatedchoroidplexuscarcinoma.Areaofvascularendothelialproliferationandthickeningofaconnectivetissueseptum.

58/64PathologicFindingsChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤

Transformationofpapillomatocarcinoma:uncommonly脈絡(luò)叢乳頭狀瘤與脈絡(luò)叢乳頭狀癌的轉(zhuǎn)化：少見CSFseeding:bothcouldoccur腦脊液播散：均可發(fā)生59/64nonenhancedCTstudiesiso-tohyperdensityintraventricularmasses等低密度腦室內(nèi)腫塊withoutbraininvasion無明確周圍組織侵潤Hydrocephalusisverycommon常見腦積水Calcification:uncommon鈣化：少見CPAextension:characteristicfeature橋小腦角區(qū)侵犯：特征性表現(xiàn)ImagingFindings---CTChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤60/64ImagingFindings---CTChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤Contrast-enhancedCTscanshowingthetumorobviouslyenhanced,aswellasCPAextension.61/64OnMRimagingT1WI:iso-tohypointenseintraventricularmassT1WI：等低信號T2WI:variableT2WI：多樣intenseenhancement明顯強(qiáng)化Flowvoids:common常見流空血管Post-contrastMRimagingofthespine:excludethepossibilityofseeding診斷脈絡(luò)叢乳頭狀瘤/癌后，須行全脊柱檢查除外CSF轉(zhuǎn)移ImagingFindings---MRIChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤62/64ImagingFindings---MRIChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤PreoperativeT1-weightedcontrast-enhancedmagneticresonanceimaging(MRI)oftheposteriorfossashowingafourthventriclemass(A)andpostoperativeMRIshowingtotalremovaloftumor(B).MRItaken6yearsafteroperationshowingarecurrenttumor(C)andspinaldropmetastasisattheleveloftheL3-S1vertebra(D).Spinaldropmetastasisofposteriorchoroidplexuspapilloma63/64ImagingFindings---PETChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤A:AxialT1-weightedMRimageshowingarightcerebellopontineangletumorasiso-intensity.B:MRimagewithgadoliniumshowingenhancement.C:PETwith18F-fluoro-2-deoxy-D-glucose(FDG)showinglowglucoseconsumptioninthetumor.葡萄糖代謝正常D:PETwithmethionineshowinghighaminoacidconsumptioninthetumor.蛋氨酸代謝增高64/64Aberrationsdiffersignificantlybetweenchoroidplexuspapillomasandchoroidplexuscarcinomas脈絡(luò)叢乳頭狀瘤及癌之間存在明顯基因差異pediatricandadultchoroidplexuspapillomas兒童與成人脈絡(luò)叢乳頭狀腫瘤存在明顯基因差異indicating:distinctgeneticpathwaysmaybeinvolvedinthedevelopmentofthesetumors提示基因差異可能參與本病的發(fā)生ChromosomalImbalancesinChoroidPlexusTumorsChoroidPlexusPapilloma脈絡(luò)叢乳頭狀瘤65/64SUMMARY66/6467/64Hemangioblastoma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

AdultTumors

ofthePosteriorFossa68/64IncidenceandClinicalPresentationHemangioblastoma

血網(wǎng)1to2.5%ofallCNStumors占所有中樞神經(jīng)系統(tǒng)腫瘤的1到2.5%7%ofthosearisingwithintheposteriorfossa占所有后顱窩腦腫瘤的7%vastmajority(75%)inthecerebellum,spinalcord(20%)多見于小腦半球，也可見于脊髓alwayslocatedperipherally,nearthecerebellarpialsurface常位于小腦半球周邊部位，近軟脊膜下Malesareslightlymorecommonly男性略常見69/64IncidenceandClinicalPresentationHemangioblastoma

血網(wǎng)Clinicalmanifestations臨床癥狀increasedintracranialpressure顱內(nèi)壓增高癥狀Headache,nausea,vomiting,ataxia頭痛、惡心、嘔吐、共濟(jì)失調(diào)longclinicalcourse(average,25weeks)臨床病史較長Polycythemiaoccursinabout20%ofcases腫瘤可分泌促紅細(xì)胞生成素，導(dǎo)致RBC升高70/64HemangioblastomaandvonHippel-LindauHemangioblastoma

血網(wǎng)HemangioblastomaisthemostfrequentmanifestationofVHL血網(wǎng)是最常見的VHL表現(xiàn)類型VHL:autosomaldominantphakomatosisVHL為常染色體顯性遺傳斑痣性錯構(gòu)瘤病1/3hemangioblastomaestablishthediagnosisofVHL1/3的血網(wǎng)病人見于VHL80%ofallVHLpatientswillhavehemangioblastoma80%的VHL病人有血網(wǎng)diagnosisofVHLdiseaseVHL的診斷Hemangioblastoma,andoneotherVHL-associatedtumor(retinalhemangioblastoma,benigncysts,benignorlow-gradetumors,malignanttumors),orfamilyhistory血網(wǎng)及其它VHL相關(guān)腫瘤（如視網(wǎng)膜血網(wǎng)、良性囊腫、良性或低級別腫瘤或惡性腫瘤），或家庭史71/64PathologicFindingsHemangioblastoma

血網(wǎng)agradeItumorintheWHOclassificationWHO1級腫瘤well-circumscribedmass,lacksatruecapsule邊界清楚，無真正的囊壁cysticcomponent+smallerhighlyvascularnodule囊性成份及實(shí)性結(jié)節(jié)cystwall:compressedbrainparenchymaorreactiveneuroglialcells囊壁：受壓腦組織或反應(yīng)性膠質(zhì)細(xì)胞72/64PathologicFindingsHemangioblastoma

血網(wǎng)Low-powerviewofahemangioblastoma,showinglargevascularchannels(openarrows)andcysticchanges(closedarrows).Thetumoriswellcircumscribedagainstarimofadjacentcompressedcerebellarcortex(arrowheads).

73/64PathologicFindingsHemangioblastoma

血網(wǎng)1.largelipid-filledvacuolatedstromalcells較大的空泡狀間質(zhì)細(xì)胞theneoplasticcomponentofthelesion腫瘤的實(shí)性部分2.abundantcapillarynetwork豐富的毛細(xì)血管網(wǎng)絡(luò)propensityofthesetum