臨床神經(jīng)病學(xué)課件：22-1 顱內(nèi)腫瘤

IntracranialTumors

Primary—Arisingfromintracranialtissues

Metastatic—Transferedfromothersystemororgans

Adult：Accountfor1.8%ofallcancers

Children:20%膠質(zhì)瘤

腦膜瘤垂體瘤Epidemiologystudieshavenotindicatedanyparticularfactors(viral,chemicalortraumatic)thatcausebraintumorsinhumansalthougharangeofcerebraltumorcanbeinducedinanimalsexperimentlly.Aetiology

Classificationofbraintumors(WHO,2007)

1.神經(jīng)上皮組織腫瘤Tumorsofneuroepithelialtissue

1星形細(xì)胞瘤Astrocytoma

2少枝膠質(zhì)細(xì)胞腫瘤Oligodendroglialtumors

3室管膜細(xì)胞腫瘤Ependymaltumors

2顱神經(jīng)和脊旁神經(jīng)腫瘤Tumorsofcranialandparaspinal

Nerves

雪旺氏細(xì)胞瘤(神經(jīng)鞘瘤)Schwannoma(Neurilemmoma)

3腦膜組織腫瘤TumorsoftheMeninges

腦膜瘤Meningioma

4淋巴瘤和造血細(xì)胞腫瘤

5生殖細(xì)胞腫瘤GermCellTumors

6鞍區(qū)腫瘤TumorsoftheSellarRegion

垂體腺瘤Pituitaryadenoma

7轉(zhuǎn)移性腫瘤MetastaticTumorsEpidemiologyofbraintumor

Primaryintracranialtumor

Incidence7.8--12.5/100thousands

Average10/100thousands

Metastatictumor

Incidence2.1--11.1/100thousands

RelativeIncidence

Thedistributionofbraintumoronages

Presentationofbraintumor

Themostcommonpresentationofbraintumorisprogressiveneurologicdeficit(68%),usuallymotorweakness(45%).

Symptomsandsignsofincreasedintracranialpressure：

headache

vomiting

opticdiscedema

Focalneurologicdeficitsassociatedwithbraintumors

.1thoseduetoincreasedICP

A.frommasseffectoftumorand/oredema

B.fromblockageofCSFdrainage(hydrocephalus)

Signsandsymptomsinclude2progessivefocaldeficits:includeweakness,dysphasia(which37%-58%ofpatientswithleft-sidedbraintumor)AduetodestructionofbrainparenchymabytumorinvasionBduetocompressionofbrainparenchymabymassand/orperitumoraledemaCduetocompressionofcranialnerve

3.Headache4seizures:notinfrequentlythefirstsymptomofabraintumor

Tumorshouldbeaggressivelysoughtinanidiopathicfirsttimeseizureinpatient>20years(ifnegative,thepatientshouldbefollowedwithrepeatstudiesatlaterdates）5Mentalstatuschanges

depressionlethargyapathy

6.SymptomssuggestiveofaTIAorstroke

maybedueto:

A.occlusionofavesselbytumorcells

B.hemorrhageintothetumor:anytumormayhemorrhage,

C.focalseizure7inthespecialcaseofpituitarytumorAsymptomsduetoendocrinedisturbancesBpituitaryapoplexyCCSFleakFocalneurologicdeficitsassociatedwithbraintumorInadditiontononfocalsignsandsymptoms(seizures,increasedICP)Frontallobe:

abulia,dementia,personalitychanges.Oftennonlateralizing,butapraxia,hemiparesisordysphasia(withdominanthemisphereinvolement)mayoccurTemporallobe:auditoryorolfactoryhallucinations,memoryimpairment.Contralateralsuperiorquadrantanopsiamaybedetectedonvisualfieldtesting.3.Parietallobe:

contralateralmotororsensoryimpairment,homonymoushemianopsia,Agnosias(withdominanthemisphereinvolvement)andapraxiasmayoccur4.Occipitallobe:

contralateralvisualfielddeficit,alexia(especiallywithcorpuscallosuminvolvementwithinfiltratingtumors5.Posteriorfossa:cranialnervedeficits,ataxia(truncalorappendicular)Diagnosisofabraintumor

Thediagnosisofbraintumorsincludethreesteps

AWhether

BWhat

CWhere

1Clinicaldiagnosis

historyandsigns

2Imageinvestigations

CT

MRI

DSA

PETDifferentiateDiagnosis

brainabscess

parasiticinfection

brainhemorrhage

braininfarction

benignincreasedICPTreatmentofbraintumors

1RelieveincreasedICP

2Operation

3Radiationtherapy

4Chemotherapy

5GAMMA-knifeGlioma

Gliomacomprisethemajorityofbraintumorsandarisefromtheneuroglialcellsderivedfromneuroectodermalorigin

Therearefourtypesofglialcells:

Astrocytes

Oligodendroglia

Ependymalcells

Neuroglialprecursor

Astrocytoma(WHOgradeIorII)

Themostcommonneuroepithelialtumorinvadediffuselyandhavenodistincttumormargin.

Location：mayariseinanypartofthebrain

Adult：cerebrum

Children

：cerebellum

Anaplasticastrocytoma,Glioblastoma

(Malignant,WHOIIIorIV))

growmoreaggressivelyandcaninvademorecerebrallobes,eveninvadeovermidline.

AstrocytomaGlioblastomaClinicalpresentation

RaisedICP

Focalneurologicalsigns

Epilepsy

Diagnosis

Clinicalpresentation

CT

MRI

Treatment:

Operation:primary

Radiationtherapy:prolongthesurvivaltime

Chemotherapy

Prognosis：dependonthelocationofthetumor

Mediansurvival

Astrocytoma:abouteover5years

Anaplastic:2-3years

glioblastoma:1yearsMedulloblastoma

Oneofthemostmalignantbraintumor(mayseedinthesubarachnoid

cavity)

location

：cerebelar

vermis

age：before10yearsold

Clinicalpresentation：

IncreasedICP

Ataxia

Teatment：surgery

radiationtherapy

chemotherapy

Prognosis：5-yearsurvivalisabove30%以上，thebest80%.Meningioma

Arisefromarachnoid(notdura),slowgrowing,extra-axial,usuallybenign,circumscribed(non-infiltrating).

Accountfor14.3—19%

Peakincidence45yearsage

Female:maleratio1.8:1

Meningioma

1。Mayoccuranywherethatarachnoicellsarefound,mostcommonlylocatedalongfalx,convexity,orsphenoidbone

2。Usuallycured(ifcompletelyremoved,whichisnotalwayspossible）.

3。Malignantincidence1.7%ofmeningiomas

4。Multiple8%ofcases

(morecommoninNeurofibromatosis)TreatmentSurgeryRadiationtherapy

Ineffectiveasprimarymodalityoftreatment

EfficacyinpreventingrecurrenceiscontroversialOutcome5yearsurvivalforpatientswithmeningioma91.3%PituitaryadenomaPituitaryadenomaarisefromtheanteriorgland(adenohypophysis)MaybeclassifiedbyendocrinefunctionroutinehistologicalstainingmethodelectronmicroscopicapperanceMicroadenoma:apituitaryadenoma<1cmdiameterPituitaryadenomaClinicalpresentationofpituitaryadenomaFunctionalNon-functionalFunctional

Prolactin(PRL):Prolactinomasarethemostcommonsecretoryadenoma.

Amenorrhea-falactorrhea

syndrome(Forbes-Albright)infemales

ImpotenceinmalesAdrenocorticotropichormone（ACTH)

Cushing’sdisease:endogenoushypercortisolismGrowthhormone(GH)

AcromegalyinadultsGigantisminchildrenRarepituitaryadenomassecrete

thyrotropin(TSH):producethyrotoxicosis

gonadotropins(LHorFSH):noclinicalsyndrom

Non-functionalpituitaryadenomaMasseffectsofpituitaryadenomasOpticchiasm:classicallyresultinginbitemporal

hemianopsiaPituitarygland:resultinginvaryingdegreesofhypopituitarismHypothyroidism:coldintolerance,myxedema,coarsehairHypoadrenalism:orthostatichypotension,easyfatigabilityHypogonadisn:amenorrhea,lossoflibido,infertilityDiabetesinsipidus

Hyperprolatctinemia:PRLisunderinhibitorycontrolfromthehypothalamusDiagnosisofpituitaryadenomas

HistoryandphysicalEndocrinehyperfunction

EndocrinedeficitsVisualfielddeficitsDeficitsofcranialnervesEndocrinologicevaluation

Baseline