中樞腦腫瘤教材講義

CentralNervousSystem

南京醫(yī)科大學(xué)一附院放射科洪汛寧hongxunning@2/3/20231IntracranialtumorsInthediagnosticwork-upofintracranialtumors,theprimarygoalsoftheimagingstudiesaretodetecttheabnormality,localizeanddetermineitsextent,characterizethelesion,andprovidealistofdifferentialdiagnosesor,ifpossible,thespecificdiagnosis.2/3/20232Imagingdiagnosis1.Isthereatumor?2.Ifyes,whereisitlocatedandwhatisitsfunctionaltopography?3.Whatdoesthetumorlooklikeandhowdoesitaffectthebrain?4.Coulditbesomethingelse,i.e.,anon-neoplasticlesion?5.Whatarethetherapeuticoptionsandwhatmightbetheprognosis?6.Canadvancedimagingmethodscontributetoimprovetherapyandoutcome?2/3/20233基本流程basicflowsheet核對(duì)資料checkdata臨床資料clinicaldata

影像資料imagingdata綜合分析得出診斷Analysistogetthediagnosis2/3/20234臨床資料clinicaldata姓名、性別、年齡

name、sex、age2/3/20235不同腫瘤有特定的好發(fā)年齡段新生兒最好發(fā)的腫瘤為畸胎瘤、原始神經(jīng)上皮腫瘤等2-10歲的兒童原發(fā)性腦腫瘤以后顱凹多見，多為良性。最常見的有毛細(xì)胞型星形細(xì)胞瘤、髓母細(xì)胞瘤、腦干膠質(zhì)瘤和第四腦室室管膜瘤年輕人，低度惡性星形細(xì)胞瘤多見中年人，間變型星形細(xì)胞瘤多見老年人，多形性膠母細(xì)胞瘤多見2/3/20236臨床資料clinicaldata病程course發(fā)病特點(diǎn)theonsetofillness既往病史history實(shí)驗(yàn)室資料laboratorydata

治療情況treatment返回2/3/20237影像資料imagingdata定位Localization占位效應(yīng)masseffect病灶形態(tài)theform病灶質(zhì)地（成分）Internaltexture影像診斷2/3/20238定位Localization部位location：大腦半球、鞍區(qū)、中線和幕下（腦干、橋小腦角、小腦半球、蚓部）位置position：腦內(nèi)、腦外、腦室內(nèi)2/3/20239腫瘤發(fā)病部位有其特征性腦內(nèi)腫瘤可顯示局部腦回腫脹、鄰近蛛網(wǎng)膜下腔變窄或閉塞、瘤周腦血管受壓移位等占位征象腦外腫瘤:腫塊區(qū)腦灰白質(zhì)向中央移位、局部腦血管和硬腦膜向內(nèi)移位、同側(cè)局部蛛網(wǎng)膜下腔增寬和腦膜尾征等腦膜瘤累及腦組織，腦轉(zhuǎn)移瘤合并腦膜和顱骨轉(zhuǎn)移時(shí)，鑒別較為困難2/3/202310發(fā)病部位最常見的幕上腦內(nèi)腫瘤為多形性膠母細(xì)胞瘤、轉(zhuǎn)移瘤；腦外腫瘤則為腦膜瘤最常見的幕下腦內(nèi)腫瘤為血管母細(xì)胞瘤和轉(zhuǎn)移瘤；腦外腫瘤則為神經(jīng)鞘瘤2/3/202311返回2/3/202312有或無要考慮兩類不同病變占位效應(yīng)masseffect返回2/3/202313病灶形態(tài)theform大小dimension形狀shape輪廓contour數(shù)目number邊緣margin周圍結(jié)構(gòu)Peripheralstructure返回2/3/202314病灶質(zhì)地（成分）

Internaltexture脂肪fat囊腫cyst骨骼skeleton鈣化calcification液化Liquefaction壞死necrosis囊變cysticdegeneration出血hemorrhage2/3/2023152/3/2023162/3/2023172/3/202318返回2/3/202319常見腦腫瘤影像診斷神經(jīng)膠質(zhì)瘤glioma腦膜瘤meningioma垂體腺瘤pituitaryadenoma顱咽管瘤craniopharyngioma聽神經(jīng)瘤acousticneuroma腦轉(zhuǎn)移瘤metastasis2/3/202320膠質(zhì)瘤（星形細(xì)胞腫瘤）最常見的腦腫瘤占顱內(nèi)腫瘤17%，占神經(jīng)上皮源性腫瘤40%幕上多見主要癥狀為癲癇2/3/202321幕上Ⅰ-Ⅱ星形細(xì)胞瘤CT：好發(fā)于額葉，其次顳葉、頂葉、小腦和腦干。平掃多表現(xiàn)為腦內(nèi)均勻的低密度病灶，邊界不清，增強(qiáng)多數(shù)不明顯。MR：T1WI略低信號(hào)，T2WI明顯高信號(hào)，輪廓規(guī)則或略不規(guī)則。一般無瘤周水腫，位于白質(zhì)。增強(qiáng)后常不增強(qiáng)或輕微增強(qiáng)。2/3/202322Astrocytoma2/3/202323Astrocytoma2/3/2023242/3/2023252/3/2023262/3/2023272/3/202328星形細(xì)胞瘤2/3/202329Anaplastic

astrocytomas(Grade3)Anaplastic

astrocytomas(Grade3)areveryaggressivetumors,readilyinfiltrateadjacentbrainstructures,andhaveauniformlypoorprognosis.2/3/202330Glioblastomamulti-formeGlioblastomamulti-forme

(Grade4)hastheaddedhistologicfeaturesofendothelialproliferationandnecrosis.Multicentricfocioftumormaybeseenin4to6%ofglioblastomas.Gliomatosis

cerebriisanunusualconditionwithdiffusecontiguousinvolvementofmultiplelobesofthebrain.2/3/202331幕上Ⅲ-Ⅳ級(jí)星形細(xì)胞瘤CT平掃密度多不均勻,以低密度或等密度為主的混合密度多見多有腦水腫增強(qiáng)掃描多有強(qiáng)化各級(jí)腫瘤均有占位征象2/3/202332幕上Ⅲ-Ⅳ級(jí)星形細(xì)胞瘤T1WI略低信號(hào)，T2WI略高信號(hào)信號(hào)可均勻或不均勻壞死、囊變、出血、鈣化、血管多有強(qiáng)化瘤周水腫2/3/202333glioblastoma2/3/202334glioblastoma2/3/202335ABLowvsHighGradeGlioma2/3/202336LowvsHighGradeGliomaCho/NAA2.60HighGraderCBV7.72A2/3/202337LowvsHighGradeGliomaCho/NAA2.60Cho/NAA0.90BLowGraderCBV1.70HighGraderCBV7.72A2/3/202338鑒別診斷低度惡性星形細(xì)胞瘤——乏血供。腦膜瘤——腦外占位征象。單發(fā)轉(zhuǎn)移瘤——環(huán)樣強(qiáng)化，周圍白質(zhì)大量水腫（指樣水腫），多有原發(fā)灶。淋巴瘤——較少見，多位于腦室周圍，明顯強(qiáng)化，水腫多較輕，大腦半球單發(fā)與本例難以鑒別。其他：腦血管病，腦膿腫。2/3/202339髓母細(xì)胞瘤MedulloblastomaThemostcommonpediatriccentralnervoussystemmalignancyandthemostcommonprimarytumoroftheposteriorfossainchildren.Occursmorefrequentlyinmalesandusuallybefore10yearsofage.Clinicalsymptomsandsignsaregenerallybrief,typicallylessthan3monthsinduration,andreflectthestrongpredilectionofthistumortoarisewithinthecerebellum,mostofteninthevermis.2/3/202340

Medulloblastoma.Photographofanautopsyspecimensectionedinthemidlineshowsafairlywell-circumscribedmass(m)ofthesuperiorcerebellar

vermis.2/3/202341medulloblastoma.Photomicrograph(hematoxylin-eosinstain)ofaclassicmedulloblastomarevealsmonomorphicsheetsofcloselyapposedsmallcellswithahighnuclear-cytoplasmicratio,occasionallyinterruptedbyneuroblasticrosettes(arrows).2/3/202342case1Medulloblastomaina6-year-oldgirlwitha10-dayhistoryofnauseaandvomiting.2/3/202343

AxialCTimageshowsaheterogeneoushyperattenuatedmassintherightcerebellarhemisphere.

2/3/202344OnanaxialT1-weightedMRimage,themasshashomogeneoushypointensitycomparedwithnormalcerebellarsignalintensity.2/3/202345OnanaxialT2-weightedMRimage,themassisheterogeneouswithsurroundingvasogenicedema.2/3/202346Contrast-enhancedaxialT1-weightedMRimageshowsheterogeneousenhancementofthemass.2/3/202347Case2Medulloblastomaina3-year-oldboywitha1-monthhistoryofprogressivelyworseningclumsiness,ataxia,headache,nausea,andvomiting.Developmentaldelayinspeechandmotorskillswasalsopresent.Papilledemawasnotedonphysicalexamination.2/3/202348AxialCTimageshowsanearlyhomogeneoushyperattenuatedmassintheposteriorfossamidline.Athincrescentofthefourthventricle(arrowheads)isnotedalongtheanteriormarginofthemass.2/3/202349OnanaxialT1-weightedMRimage,themassishypointensecomparedwiththesurroundingnormalcerebellum.2/3/202350OnanaxialT2-weightedMRimage,themassshowsmildhyperintensitycomparedwithsurroundingnormalbraintissue.2/3/202351Contrast-enhancedaxialT1-weightedMRimageshowsintensebutmildlyheterogeneousenhancementofthemass.2/3/202352Photographoftheresectedspecimenhighlightsthesoftfriablenatureofthemass,characteristicofamedulloblastoma.2/3/202353腦膜瘤meningiomaTypicallyhard,firm,rubberytumorsAbroadbaseofduralattachment

Thecelloforiginforthemeningiomaisactuallythe

arachnoidcell,thearachnoidcapcell

2/3/202354

腦膜瘤

2/3/2023552/3/2023562/3/2023572/3/2023582/3/2023592/3/2023602/3/2023612/3/202362TheDuralTailSignTheduraltailsignisseenoncontrastmaterial–enhancedmagneticresonanceimagesasathickeningoftheenhancedduramaterthatresemblesatailextendingfromamass.2/3/202363腦膜瘤meningiomaCT平掃

于好發(fā)部位見圓、卵圓、扁平或分葉狀稍高密度腫塊密度均勻，邊緣清楚，光滑多數(shù)病灶周圍有腦水腫占位征明顯,鄰近骨質(zhì)增生或破壞CT增強(qiáng)明顯均勻強(qiáng)化（homogeneousenhancement），邊界銳利2/3/202364腦膜瘤meningiomaTypicallyglobular,sometimeslobulated

massesattachedtotheduraComparativelylittleedemaSharpmarginsEnhancehomogeneously

Hyperostosis

15%–50%

Duralthickening,abnormal

duralenhancementextendbeyondthesiteofattachment.

2/3/202365

MRIT1呈稍低信號(hào)或等信號(hào)，T2呈稍高信號(hào)或等信號(hào)。增強(qiáng)掃描腫瘤明顯增強(qiáng)（hyperintensity），常能顯示腦膜尾征（duraltail）。2/3/202366腦膜瘤纖維型2/3/202367FA：纖維受壓推移，高信號(hào)，信號(hào)強(qiáng)度：0.81PseudoDW：低等信號(hào)ADC：高等信號(hào)ADC值：0.772-0.853DWI：稍高信號(hào)（T2效應(yīng)）腦膜瘤纖維型2/3/202368病側(cè):無NAA峰

Cho峰出現(xiàn)Ala峰

Cho/對(duì)側(cè)Cr=0.34腦膜瘤MRST1WIT1WIC+健側(cè)常規(guī)MRI見腫瘤呈良性腦膜瘤表現(xiàn)。MRS也呈良性腦膜瘤表現(xiàn)。2/3/2023692/3/202370惡性腦膜瘤AlaT1WIT1WIC+NAA峰Cho峰出現(xiàn)Ala峰Cho/對(duì)Cr=3.3腫瘤邊緣Cho峰常規(guī)MRI見腫瘤呈良性腦膜瘤表現(xiàn)。MRS呈惡性腦膜瘤表現(xiàn)。2/3/2023712/3/202372垂體腺瘤pituitaryadenomaCT

以冠狀位為佳，大腺瘤垂體高度大于正常值（1cm），密度均勻或不均勻，邊界清，上緣局限性隆起，垂體柄常傾斜，鞍底下陷。CT增強(qiáng)作用明顯，較均勻，可有邊界不清或壞死區(qū)。2/3/202373垂體大腺瘤2/3/202374PituitarymicroadenomasT1WI,usuallyhypointensecomparedwiththenormalgland.T2WI,slightlyhyperintenseThecontrastisbetteront1wi.Secondarysignsincludeasymmetricupwardconvexityoftheglandsurface,deviationoftheinfundibulum,andfocalerosionofthesellarfloor.Coronalandsagittalarethepreferredimagingplanesforpituitaryadenomas.2/3/202375Pituitary

macroadenomaIsointensetothenormalglandandbrainparenchyma,unlesscysticorhemorrhagiccomponentsarepresent.HemorrhageCentralnecrosisisnotcommonPituitaryapoplexyreferstoacutehemorrhagicnecrosisofamacroadenoma,andpatientsmaypresentwithsymptomsrelatedtomasseffectofthehematomaorassociatedsubarachnoidhemorrhage.2/3/202376垂體微腺瘤2/3/202377垂體微腺瘤動(dòng)態(tài)增強(qiáng)2/3/202378垂體微腺瘤2/3/202379垂體腺瘤侵及海綿竇2/3/2023802/3/2023812/3/202382T1WI2/3/202383T2WI2/3/202384T1WIC+2/3/202385T1WI2/3/202386T1WI2/3/202387顱咽管瘤craniopharyngioma多見于兒童（occurmostcommonlyinpediatricagegroup）分囊性（cystic）、實(shí)性（solid）和囊實(shí)性（intermixed）三種2/3/202388Originatefromepithelialremnantsofrathke'spouch,usuallyatthejunctionoftheinfundibulumandthepituitarygland.Benignslow-growingtumorscomposedofbothsolidepithelialtissueandcysticcomponents.Thecystscontainvariableamountsofcholesterol,keratin,necroticdebris,proteinaceousfluidandhemorrhage.Calcificationispresentin75to85%ofcases.Craniopharyngioma2/3/202389

X線平片：

瘤體位于鞍上（suprasellarregion）,

顯示殼狀鈣化（curvilinearcalcificrim）,實(shí)質(zhì)性可有斑片狀鈣化（scatteredcalcification）CT平掃顯示鈣化清晰囊實(shí)性腫塊增強(qiáng)實(shí)質(zhì)區(qū)強(qiáng)化2/3/202390CraniopharyngiomaVariableappearanceonMR,dependingontheirsolidorcysticnatureandcystcontents.ThesolidlesionsarehypointenseonandhyperintenseonT2WI.ThecystsalsohavealongT2,butiftheyhaveahighcholesterolcontentormethemoglobin,shorteningofT1resultsinhighsignalintensityonT1WI.CalcificationisnotreliablydetectedwithMRI2/3/202391囊性顱咽管瘤2/3/202392囊性顱咽管瘤鈣化2/3/202393實(shí)性顱咽管瘤2/3/202394顱咽管瘤2/3/202395顱咽管瘤2/3/2023962/3/202397顱咽管瘤2/3/202398Craniopharyngioma—Cystic2/3/202399Craniopharyngioma—solid2/3/2023100Craniopharyngioma—intermixed2/3/2023101聽神經(jīng)瘤Schwannomasmostcommonlyoccurinthecerebello-

pontineanglecistern(acousticneurinomas,

>80%ofextra-axialtumorsinthisregion)2/3/2023102聽神經(jīng)瘤

（acousticneurinoma）X線平片：內(nèi)聽道擴(kuò)大（enlargementoftheinternalauditorycanal）CT：內(nèi)聽道擴(kuò)大，橋小腦角區(qū)腫瘤（cerebellopontineanglemass），囊實(shí)性，四腦室移位，增強(qiáng)明顯2/3/2023103MRI：混雜信號(hào)，增強(qiáng)明顯，微小腫瘤更敏感2/3/20231042/3/20231052/3/20231062/3/20231072/3/20231082/3/20231092/3/2023110聽神經(jīng)瘤2/3/2023111女性，31歲，右耳聽力