間變

第246課（中樞神經(jīng)）病例探析（069）—間變性少突膠質(zhì)瘤

男，66歲，突發(fā)頭痛伴2月余

最后診斷：間變性少突膠質(zhì)瘤

病理與臨床特點(diǎn)

間變性少突膠質(zhì)瘤(anaplasticoligodendroglioma,AO)即惡性

少突膠質(zhì)瘤,2016年WHO新分類中屬于彌漫性星形細(xì)胞與少突膠質(zhì)

細(xì)胞腫瘤,包括IDH突變型與NOS型,分級(jí)為Ⅲ級(jí),占膠質(zhì)細(xì)胞腫瘤的

25%~35%及所有原發(fā)腦腫瘤的1%-2%。較少突膠質(zhì)瘤易出現(xiàn)壞死。

鏡下可見局限性或彌漫性惡性腫瘤征象,如腫瘤細(xì)胞密集、核異型性明

顯、核漿比増大,常見囊變、壞死及腫瘤細(xì)胞假柵欄狀排列,腫瘤微血管

增生明顯。免疫組織化學(xué)染色Ki-67明顯增高,超過7%-10%。發(fā)病年

齡較少突膠質(zhì)瘤大7-8歲,好發(fā)年齡為45~50歲。臨床表現(xiàn)與少突膠質(zhì)

瘤近似,最常見的癥狀為抽搐與頭痛。治療首選手術(shù)切除,化療可能有效,

放療用于治療腫瘤殘留與復(fù)發(fā)。AO預(yù)后欠佳,平均生存期為4年。

Anaplasticoligodendroglioma(anaplasticoligoden

droglioma,AO)islessmalignantglioma,in2016theWHO

classificationbelongstodiffuastrocytesandoligodendrocytes

intumor,includingIDHmutationandNOS,classforⅢ,account

for25%~35%ofglialcellsinthetumorsandthe1%2%ofall

ominentgliomasareproneto

copically,therearesignsoflocalizedordiffu

malignancy,suchasdentumorcells,prominentnuclearatypia,

enlargednuclearplasmaratio,commoncysticdegeneration,

necrosis,pudopalisadesoftumorcells,andprominenttumor

histochemicalstaining

significantlyincreadKi-67bymorethan7%-10%.Theageof

ontislessthan7-8yearsold,andtheageofontis45-50

nicalmanifestationsaresimilarto

tcommonsymptomsare

alrectionispreferredfor

treatment,chemotherapymaybeeffective,andradiotherapyis

poor

prognosiswithanaveragesurvivalof4years.

CT與MRI特點(diǎn)

①部位及形態(tài):AO與少突膠質(zhì)瘤好發(fā)部位類似,也以幕上、特別是

額葉最常見,顳葉次之。腫瘤可經(jīng)胼胝體越過中線侵犯對(duì)側(cè)大腦半球。

常為混雜密度或信號(hào)的大腫塊,瘤周水腫、出血、囊變均較常見。占位

征象明顯,如局部腦回增粗、腦溝及腦裂變窄、腦室受壓,以及中線結(jié)構(gòu)

向?qū)?cè)移位;

(1)locationandmorphology:AOandoligodendroglioma

pronesitessimilar,alsowiththesupratentorial,especiallythe

frontallobeisthemostcommon,followedbythetemporallobe.

Tumorsmaycrossthemidlinethroughthecorpuscallosumand

emixeddensityor

signalbigbump,hematomaweekisoedema,haemorrhage,

ereobvioussignsof

occupation,suchaslocalgyrithickening,narrowfissionsinsulcus

andbrain,ventricularcompression,andcontralateralmigration

ofmidlinestructure.

②CT呈等、低及高密度混雜,其中低密度可為囊變/壞死區(qū),高密度

為出血或鈣化,瘤周水腫為指狀低密度,可侵犯內(nèi)囊前后肢及外囊;

(2)CTshowedamixtureofequal,lowandhighdensity,

amongwhichthelowdensitycouldbethecystic/necroticarea,

thehighdensitycouldbethehemorrhageorcalcification,and

theperitumoredemacouldbethefingerlikelowdensity,which

couldinvadetheanteriorandposteriorlimbsoftheinnercapsule

andtheoutercapsule.

③MRI呈混雜信號(hào)腫物,T1WI以等及低信號(hào)為主,囊變區(qū)為更低信

號(hào),灶周水腫呈指狀或斑片狀低信號(hào),出血為高信號(hào),壓脂后信號(hào)不下降。

T2WI及FLAIR以高信號(hào)為主,出血及鈣化低信號(hào);

(3)MRIshowedmixedsignalmass,T1WIwasdominatedby

equalandlowsignal,thecysticareawaslowersignal,perifocal

edemashowedfingerlikeorpatchylowsignal,bleedingwashigh

signal,andthesignaldidnotdecreaafterlipidcompression.

T2WIandFLAIRhavehyperintensity,bleedingandlow

calcification.

④CT與MRI増強(qiáng)掃描呈不同程度強(qiáng)化,實(shí)性部分強(qiáng)化明顯,呈塊狀、

斑片狀、環(huán)形,以環(huán)狀為主。但也可不岀現(xiàn)強(qiáng)化。總的來說,AO強(qiáng)化率

為62%~100%,明顯高于少突膠質(zhì)瘤。且強(qiáng)化程度更明顯,腫瘤增強(qiáng)比

大(如1.30);

(4)CTandMRIenhancedscanswereenhancedtodifferent

degrees,andthesolidpartwanhancedobviously,whichwas

lumpy,patchy,annular,ydon'tintensify

ral,theAOenhancementratewas

62%~100%,whichwassignificantlyhigherthanoligodendyma.

Theenhancementdegreewasmoreobvious,andthetumor

enhancementratiowaslarger(e.g.1.30).

⑤功能成像:CBV可增高。MRS顯示Cho峰及Cho/Cr明顯增高

(后者>2.33)、NAA降低。T2*WI及SWI顯示出血及鈣化為低信號(hào)。

(5)Functionalimaging:wedthat

ChopeakandCho/Crincreadsignificantly(>2.33),andNAA

decread.T2*WIandSWIshowedlowsignalofhemorrhageand

calcification.

鑒別診斷

①少突膠質(zhì)瘤,僅從影像學(xué)上難以與AO鑒別,前者水腫、囊變、壞

死及出血少見,而鈣化較多見,MRS顯示Cho峰及Cho/Cr較低,增強(qiáng)

T1WI對(duì)鑒別有一定價(jià)值,AO強(qiáng)化更明顯,典型者為厚壁、不規(guī)則花環(huán)

狀,但兩者均可不出現(xiàn)強(qiáng)化,因此最終往往需病理學(xué)檢查鑒別;

(1)gliomas,lesswithAOonimagingaloneisdifficultto

identify,edemaoftheformer,capsule,necrosisandhemorrhage

rare,andcalcificationismoree,MRSshowedlowerpeakand

ChoCho/Cr,enhancedT1WIofidentificationhasacertainvalue,

theAOstrengthenedmoreapparent,typicalforthickwall,

irregularflowerring,butmaynotappearbothreinforcement,so

oftenendtopathologyinspectionidentification;

②少突-星形細(xì)胞混合膠質(zhì)瘤及間變性星形細(xì)胞瘤或膠質(zhì)母細(xì)胞瘤,

僅從部位、影像學(xué)征象上難以鑒別,常需病理學(xué)檢查確定診斷。

(2)oligodendrogate-astrocytomamixedgliomaand

anaplasticastrocytomaorglioblastomaaredifficultto

distinguishonlyfromthesiteandimagingsigns,and

pathologicalexaminationisoftenneededtoconfirmthe

diagnosis.

簡(jiǎn)要討論

間變性少突膠質(zhì)瘤較少見,其特點(diǎn)為發(fā)病年齡較大,瘤周水腫、瘤內(nèi)

壞死/囊變、出血較多見,而鈣化較少,增強(qiáng)掃描腫瘤強(qiáng)化比増大,以不規(guī)

則環(huán)狀較有特征,MRS顯示Cho峰明顯增高。但因其發(fā)病部位及形態(tài)

特點(diǎn)類似于更常見的少突膠質(zhì)瘤,因此術(shù)前診斷有一定難度,需綜合多種

征象甚至Ki-67才能定性診斷。

Anaplasticoligodendrogenicgliomaisrelativelyrare,

characterizedbyolderontage,moreperi-tumoredema,intra-

tumornecrosis/cysticdegeneration,andbleeding,andless

calcification,whichischaracterizedbyirregularringand

r,duetoits

locationandmorphologicalcharacteristicssimilartothemore

commonoligodendroglioma,preoperativediagnosisisdifficult,

andqualitativediagnosiscanonlybemadebyintegrating

multiplesignsandevenki-67.

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