中枢脑肿瘤教材讲义

CentralNervousSystem

南京医科大学一附院放射科洪汛宁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核对资料checkdata临床资料clinicaldata

影像资料imagingdata综合分析得出诊断Analysistogetthediagnosis2/3/20234临床资料clinicaldata姓名、性别、年龄

name、sex、age2/3/20235不同肿瘤有特定的好发年龄段新生儿最好发的肿瘤为畸胎瘤、原始神经上皮肿瘤等2-10岁的儿童原发性脑肿瘤以后颅凹多见，多为良性。最常见的有毛细胞型星形细胞瘤、髓母细胞瘤、脑干胶质瘤和第四脑室室管膜瘤年轻人，低度恶性星形细胞瘤多见中年人，间变型星形细胞瘤多见老年人，多形性胶母细胞瘤多见2/3/20236临床资料clinicaldata病程course发病特点theonsetofillness既往病史history实验室资料laboratorydata

治疗情况treatment返回2/3/20237影像资料imagingdata定位Localization占位效应masseffect病灶形态theform病灶质地（成分）Internaltexture影像诊断2/3/20238定位Localization部位location：大脑半球、鞍区、中线和幕下（脑干、桥小脑角、小脑半球、蚓部）位置position：脑内、脑外、脑室内2/3/20239肿瘤发病部位有其特征性脑内肿瘤可显示局部脑回肿胀、邻近蛛网膜下腔变窄或闭塞、瘤周脑血管受压移位等占位征象脑外肿瘤:肿块区脑灰白质向中央移位、局部脑血管和硬脑膜向内移位、同侧局部蛛网膜下腔增宽和脑膜尾征等脑膜瘤累及脑组织，脑转移瘤合并脑膜和颅骨转移时，鉴别较为困难2/3/202310发病部位最常见的幕上脑内肿瘤为多形性胶母细胞瘤、转移瘤；脑外肿瘤则为脑膜瘤最常见的幕下脑内肿瘤为血管母细胞瘤和转移瘤；脑外肿瘤则为神经鞘瘤2/3/202311返回2/3/202312有或无要考虑两类不同病变占位效应masseffect返回2/3/202313病灶形态theform大小dimension形状shape轮廓contour数目number边缘margin周围结构Peripheralstructure返回2/3/202314病灶质地（成分）

Internaltexture脂肪fat囊肿cyst骨骼skeleton钙化calcification液化Liquefaction坏死necrosis囊变cysticdegeneration出血hemorrhage2/3/2023152/3/2023162/3/2023172/3/202318返回2/3/202319常见脑肿瘤影像诊断神经胶质瘤glioma脑膜瘤meningioma垂体腺瘤pituitaryadenoma颅咽管瘤craniopharyngioma听神经瘤acousticneuroma脑转移瘤metastasis2/3/202320胶质瘤（星形细胞肿瘤）最常见的脑肿瘤占颅内肿瘤17%，占神经上皮源性肿瘤40%幕上多见主要症状为癫痫2/3/202321幕上Ⅰ-Ⅱ星形细胞瘤CT：好发于额叶，其次颞叶、顶叶、小脑和脑干。平扫多表现为脑内均匀的低密度病灶，边界不清，增强多数不明显。MR：T1WI略低信号，T2WI明显高信号，轮廓规则或略不规则。一般无瘤周水肿，位于白质。增强后常不增强或轻微增强。2/3/202322Astrocytoma2/3/202323Astrocytoma2/3/2023242/3/2023252/3/2023262/3/2023272/3/202328星形细胞瘤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级星形细胞瘤CT平扫密度多不均匀,以低密度或等密度为主的混合密度多见多有脑水肿增强扫描多有强化各级肿瘤均有占位征象2/3/202332幕上Ⅲ-Ⅳ级星形细胞瘤T1WI略低信号，T2WI略高信号信号可均匀或不均匀坏死、囊变、出血、钙化、血管多有强化瘤周水肿2/3/202333glioblastoma2/3/202334glioblastoma2/3/202335ABLowvsHighGradeGlioma2/3/202336LowvsHighGradeGliomaCho/NAA2.60HighGraderCBV7.72A2/3/202337LowvsHighGradeGliomaCho/NAA2.60Cho/NAA0.90BLowGraderCBV1.70HighGraderCBV7.72A2/3/202338鉴别诊断低度恶性星形细胞瘤——乏血供。脑膜瘤——脑外占位征象。单发转移瘤——环样强化，周围白质大量水肿（指样水肿），多有原发灶。淋巴瘤——较少见，多位于脑室周围，明显强化，水肿多较轻，大脑半球单发与本例难以鉴别。其他：脑血管病，脑脓肿。2/3/202339髓母细胞瘤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脑膜瘤meningiomaTypicallyhard,firm,rubberytumorsAbroadbaseofduralattachment

Thecelloforiginforthemeningiomaisactuallythe

arachnoidcell,thearachnoidcapcell

2/3/202354

脑膜瘤

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

于好发部位见圆、卵圆、扁平或分叶状稍高密度肿块密度均匀，边缘清楚，光滑多数病灶周围有脑水肿占位征明显,邻近骨质增生或破坏CT增强明显均匀强化（homogeneousenhancement），边界锐利2/3/202364脑膜瘤meningiomaTypicallyglobular,sometimeslobulated

massesattachedtotheduraComparativelylittleedemaSharpmarginsEnhancehomogeneously

Hyperostosis

15%–50%

Duralthickening,abnormal

duralenhancementextendbeyondthesiteofattachment.

2/3/202365

MRIT1呈稍低信号或等信号，T2呈稍高信号或等信号。增强扫描肿瘤明显增强（hyperintensity），常能显示脑膜尾征（duraltail）。2/3/202366脑膜瘤纤维型2/3/202367FA：纤维受压推移，高信号，信号强度：0.81PseudoDW：低等信号ADC：高等信号ADC值：0.772-0.853DWI：稍高信号（T2效应）脑膜瘤纤维型2/3/202368病侧:无NAA峰

Cho峰出现Ala峰

Cho/对侧Cr=0.34脑膜瘤MRST1WIT1WIC+健侧常规MRI见肿瘤呈良性脑膜瘤表现。MRS也呈良性脑膜瘤表现。2/3/2023692/3/202370恶性脑膜瘤AlaT1WIT1WIC+NAA峰Cho峰出现Ala峰Cho/对Cr=3.3肿瘤边缘Cho峰常规MRI见肿瘤呈良性脑膜瘤表现。MRS呈恶性脑膜瘤表现。2/3/2023712/3/202372垂体腺瘤pituitaryadenomaCT

以冠状位为佳，大腺瘤垂体高度大于正常值（1cm），密度均匀或不均匀，边界清，上缘局限性隆起，垂体柄常倾斜，鞍底下陷。CT增强作用明显，较均匀，可有边界不清或坏死区。2/3/202373垂体大腺瘤2/3/202374PituitarymicroadenomasT1WI,usuallyhypointensecomparedwiththenormalgland.T2WI,slightlyhyperintenseThecontrastisbetteront1wi.Secondarysignsincludeasymmetricupwardconvexityoftheglandsurface,deviationoftheinfundibulum,andfocalerosionofthesellarfloor.Coronalandsagittalarethepreferredimagingplanesforpituitaryadenomas.2/3/202375Pituitary

macroadenomaIsointensetothenormalglandandbrainparenchyma,unlesscysticorhemorrhagiccomponentsarepresent.HemorrhageCentralnecrosisisnotcommonPituitaryapoplexyreferstoacutehemorrhagicnecrosisofamacroadenoma,andpatientsmaypresentwithsymptomsrelatedtomasseffectofthehematomaorassociatedsubarachnoidhemorrhage.2/3/202376垂体微腺瘤2/3/202377垂体微腺瘤动态增强2/3/202378垂体微腺瘤2/3/202379垂体腺瘤侵及海绵窦2/3/2023802/3/2023812/3/202382T1WI2/3/202383T2WI2/3/202384T1WIC+2/3/202385T1WI2/3/202386T1WI2/3/202387颅咽管瘤craniopharyngioma多见于儿童（occurmostcommonlyinpediatricagegroup）分囊性（cystic）、实性（solid）和囊实性（intermixed）三种2/3/202388Originatefromepithelialremnantsofrathke'spouch,usuallyatthejunctionoftheinfundibulumandthepituitarygland.Benignslow-growingtumorscomposedofbothsolidepithelialtissueandcysticcomponents.Thecystscontainvariableamountsofcholesterol,keratin,necroticdebris,proteinaceousfluidandhemorrhage.Calcificationispresentin75to85%ofcases.Craniopharyngioma2/3/202389

X线平片：

瘤体位于鞍上（suprasellarregion）,

显示壳状钙化（curvilinearcalcificrim）,实质性可有斑片状钙化（scatteredcalcification）CT平扫显示钙化清晰囊实性肿块增强实质区强化2/3/202390CraniopharyngiomaVariableappearanceonMR,dependingontheirsolidorcysticnatureandcystcontents.ThesolidlesionsarehypointenseonandhyperintenseonT2WI.ThecystsalsohavealongT2,butiftheyhaveahighcholesterolcontentormethemoglobin,shorteningofT1resultsinhighsignalintensityonT1WI.CalcificationisnotreliablydetectedwithMRI2/3/202391囊性颅咽管瘤2/3/202392囊性颅咽管瘤钙化2/3/202393实性颅咽管瘤2/3/202394颅咽管瘤2/3/202395颅咽管瘤2/3/2023962/3/202397颅咽管瘤2/3/202398Craniopharyngioma—Cystic2/3/202399Craniopharyngioma—solid2/3/2023100Craniopharyngioma—intermixed2/3/2023101听神经瘤Schwannomasmostcommonlyoccurinthecerebello-

pontineanglecistern(acousticneurinomas,

>80%ofextra-axialtumorsinthisregion)2/3/2023102听神经瘤

（acousticneurinoma）X线平片：内听道扩大（enlargementoftheinternalauditorycanal）CT：内听道扩大，桥小脑角区肿瘤（cerebellopontineanglemass），囊实性，四脑室移位，增强明显2/3/2023103MRI：混杂信号，增强明显，微小肿瘤更敏感2/3/20231042/3/20231052/3/20231062/3/20231072/3/20231082/3/20231092/3/2023110听神经瘤2/3/2023111女性，31岁，右耳听力