星形膠質(zhì)細(xì)胞瘤課件

神經(jīng)系統(tǒng)常見腫瘤

中樞神經(jīng)系腫瘤膠質(zhì)瘤星形膠質(zhì)細(xì)胞瘤1-4少突膠質(zhì)細(xì)胞瘤1-3室管膜細(xì)胞瘤1-3脈絡(luò)叢乳頭狀瘤1原始神經(jīng)上皮源性腫瘤髓母細(xì)胞瘤4腦膜瘤1-3松果體腫瘤2-4垂體腫瘤周圍神經(jīng)腫瘤神經(jīng)鞘膜腫瘤神經(jīng)鞘瘤神經(jīng)纖維瘤神經(jīng)細(xì)胞源性腫瘤神經(jīng)母細(xì)胞瘤節(jié)細(xì)胞神經(jīng)瘤轉(zhuǎn)移性腫瘤

竺可青2011-6-13神經(jīng)系統(tǒng)常見腫瘤中樞神經(jīng)系腫瘤周圍神經(jīng)腫瘤竺可青20111中樞神經(jīng)系統(tǒng)腫瘤中樞神經(jīng)系統(tǒng)腫瘤發(fā)病率：10-12/10萬包括起源于腦、脊髓或腦膜的原發(fā)性和轉(zhuǎn)移性腫瘤兩大類。原發(fā)性腫瘤中，40%為膠質(zhì)瘤，15%為腦膜瘤，約8%為聽神經(jīng)瘤。轉(zhuǎn)移性腫瘤以轉(zhuǎn)移性肺癌為多見。兒童顱內(nèi)惡性腫瘤僅次于白血病，常見為膠質(zhì)瘤和髓母細(xì)胞瘤。共同特征：局部神經(jīng)癥狀，顱內(nèi)高壓癥狀，分化良好的腫瘤也可因壓迫重要的部位致死，分化很差的腫瘤也很少顱外轉(zhuǎn)移。中樞神經(jīng)系統(tǒng)腫瘤中樞神經(jīng)系統(tǒng)腫瘤發(fā)病率：12部位

常見腫瘤小腦星形細(xì)胞瘤、髓母細(xì)胞瘤、血管網(wǎng)織細(xì)胞瘤大腦半球星形細(xì)胞瘤、膠質(zhì)母細(xì)胞瘤、少支膠質(zhì)瘤、腦膜瘤腦干星形細(xì)胞瘤、膠質(zhì)母細(xì)胞瘤胼胝體星形細(xì)胞瘤、膠質(zhì)母細(xì)胞瘤、脂肪瘤枕大孔區(qū)腦膜瘤、表皮樣囊腫鞍區(qū)垂體瘤、顱咽管瘤、腦膜瘤、脊索瘤皮樣囊腫視交叉區(qū)星形細(xì)胞瘤、顱咽管瘤、腦膜瘤、生殖細(xì)胞瘤、表皮樣囊腫橋小腦角區(qū)聽神經(jīng)瘤、頸靜脈球瘤、室管膜瘤、表皮樣囊腫、腦膜瘤、蛛網(wǎng)膜囊腫四腦室室管膜瘤、脈絡(luò)叢乳頭狀瘤、皮樣或表皮樣囊腫三腦室室管膜瘤、膠樣囊腫部位常見腫瘤小腦星形細(xì)胞瘤、髓母3WHO中樞神經(jīng)系統(tǒng)腫瘤病理(7類)神經(jīng)上皮組織腫瘤（Neuroepithelialtumours)腦膜腫瘤(Meningealtumours)顱內(nèi)和外周神經(jīng)腫瘤(Tumoursofcranialandperipheraltumours)淋巴造血系統(tǒng)腫瘤（Tumoursoftheheamopoieticsystem)生殖細(xì)胞腫瘤(Germcelltumours)鞍區(qū)腫瘤(Tumoursofthesellarregion)轉(zhuǎn)移性腫瘤(MetastatictumoursoftheCNS)WHO中樞神經(jīng)系統(tǒng)腫瘤病理(7類)神經(jīng)上皮組織腫瘤（Neur4神經(jīng)上皮組織腫瘤（10類）星形細(xì)胞腫瘤(Astrocytictumours)少突膠質(zhì)細(xì)胞腫瘤(Oligodendroglialtumours)混合性膠質(zhì)瘤(Mixedgliomas)室管膜腫瘤(Ependymaltumours)脈絡(luò)叢腫瘤(Choroidplexustumours)起源不定的膠質(zhì)腫瘤（Neuroepithelialtumoursofuncertainorigin）神經(jīng)元和混合性神經(jīng)元-膠質(zhì)腫瘤(Neuronalandmixedneuronal-glialtumours)神經(jīng)母細(xì)胞瘤(Neuroblastoma)松果體實(shí)質(zhì)腫瘤(Pinealparenchymaltumours)胚胎性腫瘤(Embryonaltumours)神經(jīng)上皮組織腫瘤（10類）星形細(xì)胞腫瘤(Astrocytic5星形膠質(zhì)細(xì)胞瘤（astrocytictumours）

顱內(nèi)最常見的膠質(zhì)瘤G：境界不清的灰白色浸潤性腫瘤，或硬，或軟，或呈膠凍狀，可形成含有清亮液體的囊腔。M：星形細(xì)胞瘤(纖維型原漿型肥胖型混合細(xì)胞型)間變型星形細(xì)胞瘤膠質(zhì)母細(xì)胞瘤星形細(xì)胞瘤胞質(zhì)表達(dá)膠質(zhì)纖維酸性蛋白（GFAP*)，S-100和波形蛋白。

星形膠質(zhì)細(xì)胞瘤（astrocytictumours）6纖維型星形細(xì)胞瘤（fibrillaryastrocytoma）最常見，瘤細(xì)胞分化好，但呈浸潤型生長(zhǎng)。瘤細(xì)胞間可見紅染的原纖維性背景。原漿型星形膠質(zhì)細(xì)胞瘤（protoplasmicastrocytoma）為少見類型，瘤細(xì)胞體小，胞突少而短。

肥胖型星形膠質(zhì)細(xì)胞瘤（gemistocyticastrocytoma）瘤細(xì)胞體大，胞質(zhì)豐富。間變性星形細(xì)胞瘤（anaplasticastrocytoma）瘤細(xì)胞密度增加，多形性，核異態(tài)及核分裂像等，為惡性腫瘤。此型經(jīng)常表現(xiàn)為快速進(jìn)展，最終轉(zhuǎn)變?yōu)槟z質(zhì)母細(xì)胞瘤。

纖維型星形細(xì)胞瘤（fibrillaryastrocytom7膠質(zhì)母細(xì)胞瘤(glioblastomamultiforme,GBMWHO4級(jí))鏡下:細(xì)胞密集，異型性明顯，可見怪異單核或多核巨細(xì)胞。出現(xiàn)出血壞死。發(fā)展迅速，預(yù)后極差。毛發(fā)細(xì)胞型星形膠質(zhì)細(xì)胞瘤（pilocyticastrocytomaWHOI級(jí)）多發(fā)生在兒童、青少年，生長(zhǎng)極為緩慢。多形性黃色星形細(xì)胞瘤（PleomorphicxanthoastrocytomaWHOII級(jí)）好發(fā)兒童和年輕人，大腦半球表面（顳葉）膠質(zhì)母細(xì)胞瘤(glioblastomamultiform8St.anne/mayo分級(jí)系統(tǒng)

(核的異型、分裂象、內(nèi)皮細(xì)胞增生/或壞死)

星形細(xì)胞瘤II級(jí)1個(gè)標(biāo)準(zhǔn)星形細(xì)胞瘤III級(jí)2個(gè)標(biāo)準(zhǔn)星形細(xì)胞瘤IV級(jí)3個(gè)標(biāo)準(zhǔn)St.anne/mayo分級(jí)系統(tǒng)

(核的異型、分裂象、內(nèi)皮細(xì)9Specificstructureofintracranialtumor實(shí)質(zhì)的特征性結(jié)構(gòu)間質(zhì)的特征性結(jié)構(gòu)繼發(fā)性變化：神經(jīng)元周圍的衛(wèi)星現(xiàn)象血管周圍腫瘤細(xì)胞聚集軟腦膜下腫瘤細(xì)胞聚集Specificstructureofintracra10Rosette菊形團(tuán)Rosette菊形團(tuán)11Whorl旋窩Whorl旋窩12Palisade柵狀排列Palisade柵狀排列13Honeycomb蜂窩狀Honeycomb蜂窩狀14顱內(nèi)腫瘤癥狀

局部壓迫神經(jīng)癥狀

顱壓升高癥狀顱內(nèi)腫瘤癥狀15一、中樞神經(jīng)腫瘤

（一）膠質(zhì)瘤（glioma）良惡性的相對(duì)性，無包膜，均呈浸潤性生長(zhǎng)，間變的界限反而清楚。局部浸潤轉(zhuǎn)移：①腦脊液轉(zhuǎn)移為常見方式②顱外轉(zhuǎn)移極少見一、中樞神經(jīng)腫瘤（一）膠質(zhì)瘤（glioma）16GLIOMASGliomas,derivedfromglialcells,includeastrocytomas,oligodendrogliomas,andependymomasAmongthediffusefibrillaryastrocytomas,tumorscanbewelldifferentiated(astrocytoma)orlessdifferentiated(higher-grade),rangingfromanaplasticastrocytomatoglioblastoma.GLIOMAS17WHO1-4gradePilocyticastrocytoma1Well-differentiatedfibrillaryastrocytomas1Gemistocyticastrocytoma2Anaplasticastrocytomas3Glioblastoma(glioblastomamultiforme)4WHO1-4gradePilocyticastrocy181．星形膠質(zhì)細(xì)胞瘤（astrocytoma）約占顱內(nèi)腫瘤的30%，占膠質(zhì)瘤的70%男性多見肉眼：數(shù)厘米→巨大分化好→界不清；分化差→界清灰白色，質(zhì)地或軟或硬或呈膠凍狀腦原有的結(jié)構(gòu)受壓變形1．星形膠質(zhì)細(xì)胞瘤（astrocytoma）約占顱內(nèi)腫瘤的319鏡下：形態(tài)多樣，纖維型良1原漿型

肥胖型——交界2間變性星形膠質(zhì)細(xì)胞瘤——惡性3膠質(zhì)母細(xì)胞瘤——惡性4特點(diǎn)：細(xì)胞間變，密度大，異型性多，核深染，毛細(xì)血管內(nèi)皮細(xì)胞增生。鏡下：形態(tài)多樣，纖維型20Diffusefibrillaryastrocytoma.

彌漫性纖維性星形細(xì)胞瘤

Diffusefibrillaryastrocytoma21星形膠質(zhì)細(xì)胞瘤課件22星形膠質(zhì)細(xì)胞瘤課件23星形膠質(zhì)細(xì)胞瘤課件24Diffusefibrillaryastrocytoma.

Animmunoperoxidasepreparationdemonstrateslabelingoftumorcellbodiesandprocessesbyamonoclonalantibodytoglialfibrillaryacidicprotein.

Diffusefibrillaryastrocytoma25高度惡性星形膠質(zhì)細(xì)胞瘤，又稱多形性膠質(zhì)母細(xì)胞瘤（WHO4級(jí)）。好發(fā)額、顳葉白質(zhì)。浸潤廣可至對(duì)側(cè)。鏡下：細(xì)胞密集，異型性明顯。怪異核，多核瘤巨細(xì)胞。出血壞死明顯——區(qū)別于間變型的特征毛細(xì)血管及內(nèi)皮細(xì)胞增生，腫大發(fā)展迅速，預(yù)后差，2年內(nèi)死亡高度惡性星形膠質(zhì)細(xì)胞瘤，又稱多形性膠質(zhì)母細(xì)胞瘤（WHO426Well-differentiatedastrocytoma.

Therightfrontaltumorhasexpandedgyri,whichledtoflattening.

Well-differentiatedastrocytom27Expandedwhitematteroftheleftcerebralhemisphereandthickenedcorpuscallosumandfornices

Expandedwhitematterofthel28Computedtomographic(CT)scanofalargetumorinthecerebralhemisphereshowingsignalenhancementwithcontrastmaterialandpronouncedperitumoraledema

Computedtomographic(CT)scan29Glioblastomamultiformeappearingasanecrotic,hemorrhagic,infiltratingmass.

Glioblastomamultiformeappear30Glioblastoma.

Fociofnecrosiswithpseudopalisadingofmalignantnuclei

Glioblastoma.

Fociofnecrosi31(a)Multiform多形細(xì)胞型(a)Multiform多形細(xì)胞型32(b)Spindleform梭形細(xì)胞型(b)Spindleform梭形細(xì)胞型33(c)Smallcell小細(xì)胞型survival6.6-11.5mo,exocranialmetastasis(c)Smallcell小細(xì)胞型34毛細(xì)胞型星形膠質(zhì)細(xì)胞瘤Pilocyticastrocytoma

發(fā)生于兒童，青少年生長(zhǎng)緩慢，帶瘤存活達(dá)40年常位于小腦，第四腦室底部，第三腦室丘腦，視神經(jīng)特點(diǎn)為多極性的腫瘤細(xì)胞兩端長(zhǎng)出纖細(xì)毛發(fā)狀突起預(yù)后好GFAP為膠質(zhì)瘤的標(biāo)志原癌基因sis過度表達(dá)毛細(xì)胞型星形膠質(zhì)細(xì)胞瘤35Pilocyticastrocytomainthecerebellumwithanoduleoftumorinacyst

Pilocyticastrocytomainthec362．少突膠質(zhì)細(xì)胞瘤oligodendroglioma

占顱內(nèi)神經(jīng)上皮性腫瘤5%60-40歲成人部位：大腦半球皮質(zhì)淺層，額葉多見灰紅色邊界清楚的球形腫瘤囊性變，出血，鈣化常見。2．少突膠質(zhì)細(xì)胞瘤oligodendroglioma占顱37瘤細(xì)胞大小均勻，形態(tài)單一，彌漫排列。核深、漿空呈空暈狀。間質(zhì)富有血管，不同程度內(nèi)皮細(xì)胞增生。20%有鈣化。生長(zhǎng)慢——達(dá)10-30年。臨床多為癲癇和局部性癱瘓。少數(shù)生長(zhǎng)迅速，預(yù)后不佳。瘤細(xì)胞大小均勻，形態(tài)單一，彌漫排列。38Oligodendroglioma.Uniform,roundnucleiandclearperinuclearhalos(artefactsofdelayedfixation)typifywell-differentiatedoligodendrogliomasOligodendroglioma.Uniform,ro39星形膠質(zhì)細(xì)胞瘤課件403．室管膜細(xì)胞瘤多為兒童、青少年。占5%-6%多見于第四腦室，次為側(cè)腦室，三腦室和導(dǎo)水管。呈球形、分葉或乳頭狀，界清，膨脹性生長(zhǎng)于腦室內(nèi)，切面灰白均勻或顆粒狀。細(xì)胞大小一致，梭形或胡蘿卜狀。核膜清，有核仁胞漿豐富。有菊形團(tuán)及假菊形團(tuán)結(jié)構(gòu)，也可呈乳頭狀結(jié)構(gòu)。PTAH染色，可見纖毛體。3．室管膜細(xì)胞瘤多為兒童、青少年。41Perivascularrosette血管周菊形團(tuán)Perivascularrosette血管周菊形團(tuán)42Ependymoma.

Tumorgrowingintothefourthventricle,distorting,compressing,andinfiltratingsurroundingstructures.

Ependymoma.

Tumorgrowingi43Microscopicappearanceofependymoma

Microscopicappearanceofepen44（二）髓母細(xì)胞瘤多見于15歲以下兒童男>女來源于小腦蚓部原始神經(jīng)上皮細(xì)胞或小腦皮質(zhì)的胚胎性外顆粒層細(xì)胞。瘤組織灰紅色肉狀。細(xì)胞密集呈圓橢圓或胡蘿卜形。胞漿少而邊界不清。圍繞纖細(xì)嗜銀性纖維呈放射狀排列——菊形團(tuán)。高度惡性，預(yù)后差。（二）髓母細(xì)胞瘤多見于15歲以下兒童男>女45Medulloblastoma.

CTscanshowingacontrast-enhancingmidlinelesionintheposteriorfossa.

Medulloblastoma.

CTscansho46Sagittalsectionofbrainshowingmedulloblastomadestroyingthesuperiormidlinecerebellum.

Sagittalsectionofbrainshow47Microscopicappearanceofmedulloblastoma.

Microscopicappearanceofmedu48二、腦膜瘤(menignioma)

來源腦膜，生長(zhǎng)緩慢，大多為良性。40-50歲女性多見，顱內(nèi)腦膜瘤比脊髓多2倍。有14%無癥狀腦膜瘤好發(fā)于蛛網(wǎng)膜顆粒所在部位。球形，分葉或不規(guī)則狀。質(zhì)實(shí)，硬，邊界清，周圍腦組織受壓。切面呈灰白，顆粒狀或條索旋渦狀，有時(shí)有砂礫樣。二、腦膜瘤(menignioma)來源腦膜，生長(zhǎng)緩慢，大多49MENINGIOMASAtypicalmeningiomas(WHOgradeII/IV)Anaplastic(malignant)meningioma(WHOgradeIII/IV)MENINGIOMAS50A,Parasagittalmultilobularmeningiomaattachedtothedurawithcompressionofunderlyingbrain.

B,Meningiomawithawhorledpatternofcellgrowthandpsammomabodies

A,Parasagittalmultilobularm51圖腦膜瘤大體圖腦膜瘤大體52骨化的腦膜瘤引起相應(yīng)的顱骨骨質(zhì)增生骨化的腦膜瘤引起相應(yīng)的顱骨骨質(zhì)增生53星形膠質(zhì)細(xì)胞瘤課件54基本組織類型：腦膜細(xì)胞型（融合細(xì)胞型）纖維細(xì)胞型過度（混合）型（梭形細(xì)胞型）血管母細(xì)胞型，有復(fù)發(fā)播散傾向。各型腦膜瘤均可有不同程度的出血，鈣化，有些有黃色瘤細(xì)胞，軟骨、骨、黑色素及粘液樣變?；窘M織類型：55Meningioma.

Circumscription,homogeneouscontrastenhancementandanchoragetothedura(inthiscase,thetentorium)typifythemeningioma.ThisMRIalsodemonstratesthickenedandabnormallyenhancingdural“tails”extendingfromthelesionalborders—afindingsuggestive,thoughnotdiagnostic,ofthistumorentity.Meningioma.

Circumscription,56Meningioma.Thebroadduralbasedepictedhereischaracteristic.Meningioma.Thebroadduralba57Meningioma.Indistinctcytoplasmicboundaries,nuclearclearing(“pseudoinclusions”),cellularwhorls,andapsammomabodyareallapparentinthisviewofameningotheliomatous(syncytial)meningioma.Meningioma.Indistinctcytopla58星形膠質(zhì)細(xì)胞瘤課件59Microscope:

Itdivideintosubtype:1-3級(jí)(1)Meningothelial腦膜內(nèi)皮型(2)Fibrosis纖維型(3)Transitional移行細(xì)胞型(4)Psammomatous砂粒體型(5)Angiomatous血管瘤型(6)Microcystic微囊型(7)Secretory分泌型(8)Lymphoplasmacyte-rich富于淋巴漿細(xì)胞型(9)Metaplastic化生型(10)Clearcell透明細(xì)胞型(11)Chordoid脊索型(12)Atipical非典型(13)Papillary乳頭狀(14)Rhabdoid橫紋肌樣(15)Anaplastic間變型Microscope:Itdivideintosu60Meningothelial腦膜細(xì)胞型Meningothelial腦膜細(xì)胞型61(2)Transitional移行細(xì)胞型(2)Transitional移行細(xì)胞型62(3)Fibrosis纖維型(3)Fibrosis纖維型63圖腦膜瘤鏡下

(4)Psammomatous砂粒體型圖腦膜瘤鏡下(4)Psammomatous砂粒體型64(5)Angiomatous血管瘤型(5)Angiomatous血管瘤型65三、周圍神經(jīng)腫瘤

（一）神經(jīng)鞘膜瘤分類：神經(jīng)鞘瘤neurilemmoma神經(jīng)纖維瘤neurofibroma二者來源相同，但臨床表現(xiàn)和組織形態(tài)有差別。1．神經(jīng)鞘瘤多見于中青年，女:男=2:1發(fā)生部位：周圍神經(jīng)顱神經(jīng)，聽神經(jīng)瘤——發(fā)生于聽神經(jīng)

三、周圍神經(jīng)腫瘤（一）神經(jīng)鞘膜瘤66肉眼：大小不一，有完整包膜，質(zhì)實(shí)呈圓形或結(jié)節(jié)狀；常壓迫臨近組織不發(fā)生浸潤；灰白灰黃色，切面旋渦狀，可有出血，囊性變。鏡下：有兩種組織形態(tài)：束狀型（AntoniA型）網(wǎng)狀型（AntoniB型）可有過度型。臨床表現(xiàn)以其大小與部位而異。肉眼：大小不一，有完整包膜，質(zhì)實(shí)呈圓形或結(jié)節(jié)狀；67圖神經(jīng)鞘瘤圖神經(jīng)鞘瘤68Schwannoma.

A,Bilateraleighthnerveschwannomas.

B,Tumorshowingcellularareas(AntoniA),aswellaslooser,myxoidregions(AntoniB).

Schwannoma.

A,Bilateralei692．神經(jīng)纖維瘤

發(fā)生部位：皮下，單發(fā)或多發(fā)多發(fā)性——為神經(jīng)纖維瘤病肉眼：無包膜，界清，質(zhì)實(shí)，切面灰白略透明，呈旋渦狀纖維；極少有繼發(fā)改變。鏡下：神經(jīng)鞘膜細(xì)胞+纖維母細(xì)胞惡變以上兩型可發(fā)生惡變，尤為神經(jīng)纖維瘤病神經(jīng)纖維肉瘤2．神經(jīng)纖維瘤發(fā)生部位：皮下，單發(fā)或多發(fā)70星形膠質(zhì)細(xì)胞瘤課件71星形膠質(zhì)細(xì)胞瘤課件72

TumorofNervousSheathincludeneurilemmomaandneurofibroma

Neurilemmoma神經(jīng)鞘瘤Neurofibroma

神經(jīng)纖維瘤OrigincellSchwanncellSchwanncellFibroblast,nevousfiberMacroscopeWithcapsuleWithoutcapsuleMicroscopeAntoniApatternnucleusoftumorcellarrangepalisadeNervefibertwistwaveappearanceBehaviorBenignmalignantchangefewBenign,malignantchangerate10-15%

TumorofNervousSheathinclu73（二）交感神經(jīng)腫瘤

惡性——神經(jīng)母細(xì)胞瘤神經(jīng)元性腫瘤良性——節(jié)細(xì)胞神經(jīng)瘤多見于交感神經(jīng)節(jié)與腎上腺髓質(zhì)（二）交感神經(jīng)腫瘤74四、轉(zhuǎn)移性腫瘤1．腦轉(zhuǎn)移性腫瘤的來源支氣管肺癌最常見，40%發(fā)生腦轉(zhuǎn)移乳腺癌次常見25%發(fā)生腦轉(zhuǎn)移黑色素瘤15%發(fā)生腦轉(zhuǎn)移胃癌結(jié)腸癌腎癌四、轉(zhuǎn)移性腫瘤1．腦轉(zhuǎn)移性腫瘤的來源75BrainMetastasesBrainMetastases762．腦轉(zhuǎn)移瘤的形成腦實(shí)質(zhì)轉(zhuǎn)移結(jié)節(jié)軟腦膜癌病——又稱癌性腦膜炎實(shí)質(zhì)腦炎性轉(zhuǎn)移2．腦轉(zhuǎn)移瘤的形成77Metastaticcarcinoma.

Acohesivegrowthpatternandclearlydelimitedtumor–CNSinterfacearehallmarksofneoplasmsmetastatictothebrain.Theadenocarcinoma(right),derivedfromaprimaryintheleftlung,“pushes”againstadjacentcerebralwhitematter.

Metastaticcarcinoma.

Acohes78Metastaticcarcinoma.

Thecellularcohesioncharacteristicofmostepithelialneoplasmsandforeigntogliomasandlymphomasisgenerallymaintainedincytologypreparations,facilitatingrapidintraoperativediagnosis.Notealsotheabsenceofcytoplasmicprocessesandpresenceofconspicuousnucleoli,thelatteralsoalientomostneuroepithelialtumors,inthissmearpreparationofapoorlydifferentiatedpulmonaryadenocarcinomathatpresentedasasolitary,rightfrontallobebrainmass.

Metastaticcarcinoma.

Thecel79星形膠質(zhì)細(xì)胞瘤課件80星形膠質(zhì)細(xì)胞瘤課件81星形膠質(zhì)細(xì)胞瘤課件82小結(jié)WHO中樞神經(jīng)系統(tǒng)腫瘤共7類神經(jīng)上皮組織腫瘤分10類星形膠質(zhì)細(xì)胞瘤4級(jí)星形膠質(zhì)細(xì)胞瘤St.anne/mayo分級(jí)系統(tǒng)髓母細(xì)胞瘤腦膜瘤不同亞型可分3級(jí)小結(jié)WHO中樞神經(jīng)系統(tǒng)腫瘤共7類83神經(jīng)系統(tǒng)常見腫瘤

中樞神經(jīng)系腫瘤膠質(zhì)瘤星形膠質(zhì)細(xì)胞瘤1-4少突膠質(zhì)細(xì)胞瘤1-3室管膜細(xì)胞瘤1-3脈絡(luò)叢乳頭狀瘤1原始神經(jīng)上皮源性腫瘤髓母細(xì)胞瘤4腦膜瘤1-3松果體腫瘤2-4垂體腫瘤周圍神經(jīng)腫瘤神經(jīng)鞘膜腫瘤神經(jīng)鞘瘤神經(jīng)纖維瘤神經(jīng)細(xì)胞源性腫瘤神經(jīng)母細(xì)胞瘤節(jié)細(xì)胞神經(jīng)瘤轉(zhuǎn)移性腫瘤

竺可青2011-6-13神經(jīng)系統(tǒng)常見腫瘤中樞神經(jīng)系腫瘤周圍神經(jīng)腫瘤竺可青201184中樞神經(jīng)系統(tǒng)腫瘤中樞神經(jīng)系統(tǒng)腫瘤發(fā)病率：10-12/10萬包括起源于腦、脊髓或腦膜的原發(fā)性和轉(zhuǎn)移性腫瘤兩大類。原發(fā)性腫瘤中，40%為膠質(zhì)瘤，15%為腦膜瘤，約8%為聽神經(jīng)瘤。轉(zhuǎn)移性腫瘤以轉(zhuǎn)移性肺癌為多見。兒童顱內(nèi)惡性腫瘤僅次于白血病，常見為膠質(zhì)瘤和髓母細(xì)胞瘤。共同特征：局部神經(jīng)癥狀，顱內(nèi)高壓癥狀，分化良好的腫瘤也可因壓迫重要的部位致死，分化很差的腫瘤也很少顱外轉(zhuǎn)移。中樞神經(jīng)系統(tǒng)腫瘤中樞神經(jīng)系統(tǒng)腫瘤發(fā)病率：185部位

常見腫瘤小腦星形細(xì)胞瘤、髓母細(xì)胞瘤、血管網(wǎng)織細(xì)胞瘤大腦半球星形細(xì)胞瘤、膠質(zhì)母細(xì)胞瘤、少支膠質(zhì)瘤、腦膜瘤腦干星形細(xì)胞瘤、膠質(zhì)母細(xì)胞瘤胼胝體星形細(xì)胞瘤、膠質(zhì)母細(xì)胞瘤、脂肪瘤枕大孔區(qū)腦膜瘤、表皮樣囊腫鞍區(qū)垂體瘤、顱咽管瘤、腦膜瘤、脊索瘤皮樣囊腫視交叉區(qū)星形細(xì)胞瘤、顱咽管瘤、腦膜瘤、生殖細(xì)胞瘤、表皮樣囊腫橋小腦角區(qū)聽神經(jīng)瘤、頸靜脈球瘤、室管膜瘤、表皮樣囊腫、腦膜瘤、蛛網(wǎng)膜囊腫四腦室室管膜瘤、脈絡(luò)叢乳頭狀瘤、皮樣或表皮樣囊腫三腦室室管膜瘤、膠樣囊腫部位常見腫瘤小腦星形細(xì)胞瘤、髓母86WHO中樞神經(jīng)系統(tǒng)腫瘤病理(7類)神經(jīng)上皮組織腫瘤（Neuroepithelialtumours)腦膜腫瘤(Meningealtumours)顱內(nèi)和外周神經(jīng)腫瘤(Tumoursofcranialandperipheraltumours)淋巴造血系統(tǒng)腫瘤（Tumoursoftheheamopoieticsystem)生殖細(xì)胞腫瘤(Germcelltumours)鞍區(qū)腫瘤(Tumoursofthesellarregion)轉(zhuǎn)移性腫瘤(MetastatictumoursoftheCNS)WHO中樞神經(jīng)系統(tǒng)腫瘤病理(7類)神經(jīng)上皮組織腫瘤（Neur87神經(jīng)上皮組織腫瘤（10類）星形細(xì)胞腫瘤(Astrocytictumours)少突膠質(zhì)細(xì)胞腫瘤(Oligodendroglialtumours)混合性膠質(zhì)瘤(Mixedgliomas)室管膜腫瘤(Ependymaltumours)脈絡(luò)叢腫瘤(Choroidplexustumours)起源不定的膠質(zhì)腫瘤（Neuroepithelialtumoursofuncertainorigin）神經(jīng)元和混合性神經(jīng)元-膠質(zhì)腫瘤(Neuronalandmixedneuronal-glialtumours)神經(jīng)母細(xì)胞瘤(Neuroblastoma)松果體實(shí)質(zhì)腫瘤(Pinealparenchymaltumours)胚胎性腫瘤(Embryonaltumours)神經(jīng)上皮組織腫瘤（10類）星形細(xì)胞腫瘤(Astrocytic88星形膠質(zhì)細(xì)胞瘤（astrocytictumours）

顱內(nèi)最常見的膠質(zhì)瘤G：境界不清的灰白色浸潤性腫瘤，或硬，或軟，或呈膠凍狀，可形成含有清亮液體的囊腔。M：星形細(xì)胞瘤(纖維型原漿型肥胖型混合細(xì)胞型)間變型星形細(xì)胞瘤膠質(zhì)母細(xì)胞瘤星形細(xì)胞瘤胞質(zhì)表達(dá)膠質(zhì)纖維酸性蛋白（GFAP*)，S-100和波形蛋白。

星形膠質(zhì)細(xì)胞瘤（astrocytictumours）89纖維型星形細(xì)胞瘤（fibrillaryastrocytoma）最常見，瘤細(xì)胞分化好，但呈浸潤型生長(zhǎng)。瘤細(xì)胞間可見紅染的原纖維性背景。原漿型星形膠質(zhì)細(xì)胞瘤（protoplasmicastrocytoma）為少見類型，瘤細(xì)胞體小，胞突少而短。

肥胖型星形膠質(zhì)細(xì)胞瘤（gemistocyticastrocytoma）瘤細(xì)胞體大，胞質(zhì)豐富。間變性星形細(xì)胞瘤（anaplasticastrocytoma）瘤細(xì)胞密度增加，多形性，核異態(tài)及核分裂像等，為惡性腫瘤。此型經(jīng)常表現(xiàn)為快速進(jìn)展，最終轉(zhuǎn)變?yōu)槟z質(zhì)母細(xì)胞瘤。

纖維型星形細(xì)胞瘤（fibrillaryastrocytom90膠質(zhì)母細(xì)胞瘤(glioblastomamultiforme,GBMWHO4級(jí))鏡下:細(xì)胞密集，異型性明顯，可見怪異單核或多核巨細(xì)胞。出現(xiàn)出血壞死。發(fā)展迅速，預(yù)后極差。毛發(fā)細(xì)胞型星形膠質(zhì)細(xì)胞瘤（pilocyticastrocytomaWHOI級(jí)）多發(fā)生在兒童、青少年，生長(zhǎng)極為緩慢。多形性黃色星形細(xì)胞瘤（PleomorphicxanthoastrocytomaWHOII級(jí)）好發(fā)兒童和年輕人，大腦半球表面（顳葉）膠質(zhì)母細(xì)胞瘤(glioblastomamultiform91St.anne/mayo分級(jí)系統(tǒng)

(核的異型、分裂象、內(nèi)皮細(xì)胞增生/或壞死)

星形細(xì)胞瘤II級(jí)1個(gè)標(biāo)準(zhǔn)星形細(xì)胞瘤III級(jí)2個(gè)標(biāo)準(zhǔn)星形細(xì)胞瘤IV級(jí)3個(gè)標(biāo)準(zhǔn)St.anne/mayo分級(jí)系統(tǒng)

(核的異型、分裂象、內(nèi)皮細(xì)92Specificstructureofintracranialtumor實(shí)質(zhì)的特征性結(jié)構(gòu)間質(zhì)的特征性結(jié)構(gòu)繼發(fā)性變化：神經(jīng)元周圍的衛(wèi)星現(xiàn)象血管周圍腫瘤細(xì)胞聚集軟腦膜下腫瘤細(xì)胞聚集Specificstructureofintracra93Rosette菊形團(tuán)Rosette菊形團(tuán)94Whorl旋窩Whorl旋窩95Palisade柵狀排列Palisade柵狀排列96Honeycomb蜂窩狀Honeycomb蜂窩狀97顱內(nèi)腫瘤癥狀

局部壓迫神經(jīng)癥狀

顱壓升高癥狀顱內(nèi)腫瘤癥狀98一、中樞神經(jīng)腫瘤

（一）膠質(zhì)瘤（glioma）良惡性的相對(duì)性，無包膜，均呈浸潤性生長(zhǎng)，間變的界限反而清楚。局部浸潤轉(zhuǎn)移：①腦脊液轉(zhuǎn)移為常見方式②顱外轉(zhuǎn)移極少見一、中樞神經(jīng)腫瘤（一）膠質(zhì)瘤（glioma）99GLIOMASGliomas,derivedfromglialcells,includeastrocytomas,oligodendrogliomas,andependymomasAmongthediffusefibrillaryastrocytomas,tumorscanbewelldifferentiated(astrocytoma)orlessdifferentiated(higher-grade),rangingfromanaplasticastrocytomatoglioblastoma.GLIOMAS100WHO1-4gradePilocyticastrocytoma1Well-differentiatedfibrillaryastrocytomas1Gemistocyticastrocytoma2Anaplasticastrocytomas3Glioblastoma(glioblastomamultiforme)4WHO1-4gradePilocyticastrocy1011．星形膠質(zhì)細(xì)胞瘤（astrocytoma）約占顱內(nèi)腫瘤的30%，占膠質(zhì)瘤的70%男性多見肉眼：數(shù)厘米→巨大分化好→界不清；分化差→界清灰白色，質(zhì)地或軟或硬或呈膠凍狀腦原有的結(jié)構(gòu)受壓變形1．星形膠質(zhì)細(xì)胞瘤（astrocytoma）約占顱內(nèi)腫瘤的3102鏡下：形態(tài)多樣，纖維型良1原漿型

肥胖型——交界2間變性星形膠質(zhì)細(xì)胞瘤——惡性3膠質(zhì)母細(xì)胞瘤——惡性4特點(diǎn)：細(xì)胞間變，密度大，異型性多，核深染，毛細(xì)血管內(nèi)皮細(xì)胞增生。鏡下：形態(tài)多樣，纖維型103Diffusefibrillaryastrocytoma.

彌漫性纖維性星形細(xì)胞瘤

Diffusefibrillaryastrocytoma104星形膠質(zhì)細(xì)胞瘤課件105星形膠質(zhì)細(xì)胞瘤課件106星形膠質(zhì)細(xì)胞瘤課件107Diffusefibrillaryastrocytoma.

Animmunoperoxidasepreparationdemonstrateslabelingoftumorcellbodiesandprocessesbyamonoclonalantibodytoglialfibrillaryacidicprotein.

Diffusefibrillaryastrocytoma108高度惡性星形膠質(zhì)細(xì)胞瘤，又稱多形性膠質(zhì)母細(xì)胞瘤（WHO4級(jí)）。好發(fā)額、顳葉白質(zhì)。浸潤廣可至對(duì)側(cè)。鏡下：細(xì)胞密集，異型性明顯。怪異核，多核瘤巨細(xì)胞。出血壞死明顯——區(qū)別于間變型的特征毛細(xì)血管及內(nèi)皮細(xì)胞增生，腫大發(fā)展迅速，預(yù)后差，2年內(nèi)死亡高度惡性星形膠質(zhì)細(xì)胞瘤，又稱多形性膠質(zhì)母細(xì)胞瘤（WHO4109Well-differentiatedastrocytoma.

Therightfrontaltumorhasexpandedgyri,whichledtoflattening.

Well-differentiatedastrocytom110Expandedwhitematteroftheleftcerebralhemisphereandthickenedcorpuscallosumandfornices

Expandedwhitematterofthel111Computedtomographic(CT)scanofalargetumorinthecerebralhemisphereshowingsignalenhancementwithcontrastmaterialandpronouncedperitumoraledema

Computedtomographic(CT)scan112Glioblastomamultiformeappearingasanecrotic,hemorrhagic,infiltratingmass.

Glioblastomamultiformeappear113Glioblastoma.

Fociofnecrosiswithpseudopalisadingofmalignantnuclei

Glioblastoma.

Fociofnecrosi114(a)Multiform多形細(xì)胞型(a)Multiform多形細(xì)胞型115(b)Spindleform梭形細(xì)胞型(b)Spindleform梭形細(xì)胞型116(c)Smallcell小細(xì)胞型survival6.6-11.5mo,exocranialmetastasis(c)Smallcell小細(xì)胞型117毛細(xì)胞型星形膠質(zhì)細(xì)胞瘤Pilocyticastrocytoma

發(fā)生于兒童，青少年生長(zhǎng)緩慢，帶瘤存活達(dá)40年常位于小腦，第四腦室底部，第三腦室丘腦，視神經(jīng)特點(diǎn)為多極性的腫瘤細(xì)胞兩端長(zhǎng)出纖細(xì)毛發(fā)狀突起預(yù)后好GFAP為膠質(zhì)瘤的標(biāo)志原癌基因sis過度表達(dá)毛細(xì)胞型星形膠質(zhì)細(xì)胞瘤118Pilocyticastrocytomainthecerebellumwithanoduleoftumorinacyst

Pilocyticastrocytomainthec1192．少突膠質(zhì)細(xì)胞瘤oligodendroglioma

占顱內(nèi)神經(jīng)上皮性腫瘤5%60-40歲成人部位：大腦半球皮質(zhì)淺層，額葉多見灰紅色邊界清楚的球形腫瘤囊性變，出血，鈣化常見。2．少突膠質(zhì)細(xì)胞瘤oligodendroglioma占顱120瘤細(xì)胞大小均勻，形態(tài)單一，彌漫排列。核深、漿空呈空暈狀。間質(zhì)富有血管，不同程度內(nèi)皮細(xì)胞增生。20%有鈣化。生長(zhǎng)慢——達(dá)10-30年。臨床多為癲癇和局部性癱瘓。少數(shù)生長(zhǎng)迅速，預(yù)后不佳。瘤細(xì)胞大小均勻，形態(tài)單一，彌漫排列。121Oligodendroglioma.Uniform,roundnucleiandclearperinuclearhalos(artefactsofdelayedfixation)typifywell-differentiatedoligodendrogliomasOligodendroglioma.Uniform,ro122星形膠質(zhì)細(xì)胞瘤課件1233．室管膜細(xì)胞瘤多為兒童、青少年。占5%-6%多見于第四腦室，次為側(cè)腦室，三腦室和導(dǎo)水管。呈球形、分葉或乳頭狀，界清，膨脹性生長(zhǎng)于腦室內(nèi)，切面灰白均勻或顆粒狀。細(xì)胞大小一致，梭形或胡蘿卜狀。核膜清，有核仁胞漿豐富。有菊形團(tuán)及假菊形團(tuán)結(jié)構(gòu)，也可呈乳頭狀結(jié)構(gòu)。PTAH染色，可見纖毛體。3．室管膜細(xì)胞瘤多為兒童、青少年。124Perivascularrosette血管周菊形團(tuán)Perivascularrosette血管周菊形團(tuán)125Ependymoma.

Tumorgrowingintothefourthventricle,distorting,compressing,andinfiltratingsurroundingstructures.

Ependymoma.

Tumorgrowingi126Microscopicappearanceofependymoma

Microscopicappearanceofepen127（二）髓母細(xì)胞瘤多見于15歲以下兒童男>女來源于小腦蚓部原始神經(jīng)上皮細(xì)胞或小腦皮質(zhì)的胚胎性外顆粒層細(xì)胞。瘤組織灰紅色肉狀。細(xì)胞密集呈圓橢圓或胡蘿卜形。胞漿少而邊界不清。圍繞纖細(xì)嗜銀性纖維呈放射狀排列——菊形團(tuán)。高度惡性，預(yù)后差。（二）髓母細(xì)胞瘤多見于15歲以下兒童男>女128Medulloblastoma.

CTscanshowingacontrast-enhancingmidlinelesionintheposteriorfossa.

Medulloblastoma.

CTscansho129Sagittalsectionofbrainshowingmedulloblastomadestroyingthesuperiormidlinecerebellum.

Sagittalsectionofbrainshow130Microscopicappearanceofmedulloblastoma.

Microscopicappearanceofmedu131二、腦膜瘤(menignioma)

來源腦膜，生長(zhǎng)緩慢，大多為良性。40-50歲女性多見，顱內(nèi)腦膜瘤比脊髓多2倍。有14%無癥狀腦膜瘤好發(fā)于蛛網(wǎng)膜顆粒所在部位。球形，分葉或不規(guī)則狀。質(zhì)實(shí)，硬，邊界清，周圍腦組織受壓。切面呈灰白，顆粒狀或條索旋渦狀，有時(shí)有砂礫樣。二、腦膜瘤(menignioma)來源腦膜，生長(zhǎng)緩慢，大多132MENINGIOMASAtypicalmeningiomas(WHOgradeII/IV)Anaplastic(malignant)meningioma(WHOgradeIII/IV)MENINGIOMAS133A,Parasagittalmultilobularmeningiomaattachedtothedurawithcompressionofunderlyingbrain.

B,Meningiomawithawhorledpatternofcellgrowthandpsammomabodies

A,Parasagittalmultilobularm134圖腦膜瘤大體圖腦膜瘤大體135骨化的腦膜瘤引起相應(yīng)的顱骨骨質(zhì)增生骨化的腦膜瘤引起相應(yīng)的顱骨骨質(zhì)增生136星形膠質(zhì)細(xì)胞瘤課件137基本組織類型：腦膜細(xì)胞型（融合細(xì)胞型）纖維細(xì)胞型過度（混合）型（梭形細(xì)胞型）血管母細(xì)胞型，有復(fù)發(fā)播散傾向。各型腦膜瘤均可有不同程度的出血，鈣化，有些有黃色瘤細(xì)胞，軟骨、骨、黑色素及粘液樣變?；窘M織類型：138Meningioma.

Circumscription,homogeneouscontrastenhancementandanchoragetothedura(inthiscase,thetentorium)typifythemeningioma.ThisMRIalsodemonstratesthickenedandabnormallyenhancingdural“tails”extendingfromthelesionalborders—afindingsuggestive,thoughnotdiagnostic,ofthistumorentity.Meningioma.

Circumscription,139Meningioma.Thebroadduralbasedepictedhereischaracteristic.Meningioma.Thebroadduralba140Meningioma.Indistinctcytoplasmicboundaries,nuclearclearing(“pseudoinclusions”),cellularwhorls,andapsammomabodyareallapparentinthisviewofameningotheliomatous(syncytial)meningioma.Meningioma.Indistinctcytopla141星形膠質(zhì)細(xì)胞瘤課件142Microscope:

Itdivideintosubtype:1-3級(jí)(1)Meningothelial腦膜內(nèi)皮型(2)Fibrosis纖維型(3)Transitional移行細(xì)胞型(4)Psammomatous砂粒體型(5)Angiomatous血管瘤型(6)Microcystic微囊型(7)Secretory分泌型(8)Lymphoplasmacyte-rich富于淋巴漿細(xì)胞型(9)Metaplastic化生型(10)Clearcell透明細(xì)胞型(11)Chordoid脊索型(12)Atipical非典型(13)Papillary乳頭狀(14)Rhabdoid橫紋肌樣(15)Anaplastic間變型Microscope:Itdivideintosu143Meningothelial腦膜細(xì)胞型Meningothelial腦膜細(xì)胞型144(2)Transitional移行細(xì)胞型(2)Transitional移行細(xì)胞型145(3)Fibrosis纖維型(3)Fibrosis纖維型146圖腦膜瘤鏡下

(4)Psammomatous砂粒體型圖腦膜瘤鏡下(4)Psammomatous砂粒體型147(5)Angiomatous血管瘤型(5)Angiomatous血管瘤型148三、周圍神經(jīng)腫瘤

（一）神經(jīng)鞘膜瘤分類：神經(jīng)鞘瘤neurilemmoma神經(jīng)纖維瘤neurofibroma二者來源相同，但臨床表現(xiàn)和組織形態(tài)有差別。1．神經(jīng)鞘瘤多見于中青年，女:男=2:1發(fā)生部位：周圍神經(jīng)顱神經(jīng)，聽神經(jīng)瘤——發(fā)生于聽神經(jīng)

三、周圍神經(jīng)腫瘤（一）神經(jīng)鞘膜瘤149肉眼：大小不一，有完整包膜，質(zhì)實(shí)呈圓形或結(jié)節(jié)狀；常壓迫臨近組織不發(fā)生浸潤；灰白灰黃色，切面旋渦狀，可有出血，囊性變。鏡下：有兩種組織形態(tài)：束狀型（AntoniA型）