胚胎发育不良性神经上皮肿瘤

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1、CASEREPORTDysembryoplasticNeuroepithelialTumor(DNET)胚胎发育不良性神经上皮肿瘤北京大学人民医院陈皓HistoryM,17yrsIDnumber:1186959Intractablecomplexpartilseizuresfor10yrs.Neurologicalexaminations:nospecialcompanyingneurologicdeficit.RadiologicalFindingsCTMRIT1WIT2WIFLAIRDWIT1WIGD-DTPAMRSRadiologicappearanceCT：Awell-dema

2、rcatedintrocorticalhypoattenuatinglesionwithnoedemaandmasseffectintherightfrontal-parietallobe.MRI：ThelesionshowsT1WIhypointense,T2WIhyperintense,FLAIRmixedisointensewitha“bright”rim,DWIisointense,T1WICEnoenhancement.Noperitumoraledemaandoccupyingeffect.MRS：NoobviouschangeofChoandCr,NAAreducessl

3、ightly.FinaldiagnosisDysembryoplasticNeuroepithelialTumor（DNET）胚胎发育不良性神经上皮瘤IntroductionConceptDysembryoplasticteuroepithelialtumor(DNET)Ararebenignintracorticalsupratentorialmixedneuronal-glialneoplasm.Mostcommonlocatedinthetemporallobe.FirstreportedbyDaumas-Duport,etalin1988.EpidemicalfeaturesF

4、requency:rare,<1%ofallprimarybraintumors.Age:adolescentsandyoungadultswithin20yearsold.Gender:M≧F.PathologyA:Hallmark:Specificglioneuronalelement(SGNE)=Columnsofheterogenerouscellslocatedinaneurofibrillarymatrix,including:oligodendrocyte-likecells+matureganglioncells+astrocytes.B:Typicalfindings

5、:Involvementofthecorticalgraymatterandamultinodulargrowthpattern.C:Accompanyingfindings:ThecortexadjacenttoaDNETshowsadisorderedarchitecture.Left：showsthenodulargrowthpatternandtheloosematrixofthistumor.Notethefocalcalcification(arrow).Right：showsmanyoligodendroglia-likecellswithsmallroundnuclei

6、(arrow)andaclearperinuclearhalo.Thesecellsarelocatedinaneurofibrillarymatrix.ClinicalfeaturesPartialchronicdrug-resistantseizuresoccurredduringearlychilodhood.Noaccompanyingneurologicdeficits.TreatmentandprognosisDrugs:tocontrolseizures.Surgicaltreatment:operationtoremovetumorsandtocureseizures.

7、Prognosis:freeofseizuresin70-81%patients,andseldomenvidenceoftumorrecurrenceandenlargementaftersurgery.RadiologicfeaturesCT:Well-demarcatedintrocorticalhypoattenuatinglesion.“pseudocystic”appearance.Minimalorsubtlecalcificat