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01 CNS Tumors 2 - Copy

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    Astrocytoma

    Most common primary intra-axial brain tumor

    12,000 cases/year Classification:

    1.fibrillary

    2.gemistocytic3.protoplasmic

    4.pilocytic

    5.microcystic cerebellar

    CNS tumors

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    SPREAD

    -tracking through the white matter(corpus

    callosum,cerebral peduncles,centrum

    semiovale,internal capsule)

    -CSF pathways

    -rarely spread systematically

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    Neuropathological Grading (WHO)

    Characteristic Astrocyto.

    (Gr.I)

    Anaplastic

    Astrocyto.

    (Gr.II)

    GBM

    (Gr.III)

    hypercellularity slight

    moderate mod.-

    marked

    pleomorphism slight moderate mod.-marked

    Vascularproliferation

    none permitted common(not req.)

    necrosis none none required

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    Neuroradiological Grading

    Grade CT features MR findings

    I Low density,no mass

    effect

    Abnormal signal,no

    mass effect

    II Low density + masseffect

    Abnornal signal +mass effect

    III Complex enhancement

    IV Necrosis(ring enhancement)

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    Approximate survival (Astrocytoma)

    Grade Median survival

    I 8-10 yrs

    II 7-8 yrs

    III 2 yrs

    IV

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    Low grade astrocytoma

    tend to occur in children and young adults

    demonstrate low deg.of cellularity

    calcifications are rare, mitosis are absent

    0.7-16% among astrocytoma

    young agegood prognosticator

    increased ICP,altered conciousness,personality

    change,significant neurologic deficit=poorprognosis

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    treatment

    Gross total excision if feasible

    surgery is not curative for most infiltratinghemispheric gliomasdue to its characteristic whichextend at least 2 cm into the normal brain

    Seizure control 15-75% will undergo malignant degeneration in 5

    years

    Relieve pressure from large tumors threateningwith hernation

    Relieve obstruction of csf pathway

    Post op Radiation for grade II astrocytomas

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    High grade astrocytoma (grade III and IV)

    Anaplastic astrocytoma:

    mean age:46 yrsmean duration of symp:15.7mos.

    Glioblastoma multiforme:

    mean age:56 yrs

    mean duration of symp:5.4mos

    GBM most common primary brain tumor

    most malignant(kernohan IV) histological feature:

    neovascularization with endothelial proliferation

    areas of necrosis

    pseudopallisading around area of necrosis

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    GBM

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    AA GBM

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    Treatment

    Functional surgery Tumor debaulking/ cytoreduction

    Prolong survival

    External beam radiation Chemotherapy

    Stereotactic biopsy:

    1.eloquent/inaccessible2.small tumors

    3.dx.

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    Cerebellar Astrocytoma

    often cystic,half have mural nodule

    better prognosis than supratentorial mean age of occurrence is lower

    27% of pediatric p-fossa tumors

    postop.survival longer

    Presentation:

    usually those of any p-fossa mass (ataxia, hydrocephalus,

    cerebellar signs)

    Pathology:

    -solid/cystic(proteinaceous fluid)

    -50% have mural nodule,cyst lining of reactive,non-neoplastic tissue

    Prognosis:

    10yr survival=94%

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    Treatment

    Excision

    Surveillance for recurrence

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    Oligodendroglioma-frequently presents with seizure

    -often calcified

    -predilection in the frontal lobes- fried egg appearance on microscopy

    - 4% of primary brain tumors

    - Average age =40yrs

    - Male:female(60:40)

    Presentation:

    -50-80% -seizure

    -often related to mass effect and less commonly to inc.ICP

    Pathology:

    -cells with monotonous round nuclei often in cellular sheets

    -fried egg-lucent perinuclear halo

    -16% of hemispheric ODG are cystic

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    Treatment

    Gross total resection if possible

    Chemotherapy

    Radiation therapy

    Prognosis:

    10 yr=10-30%

    -frontal lobe ODG survived longer

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    Meningiomas slow growing,extra-axial,usually benign

    arise from arachnoid falx,convexity,sphenoid bone

    cause hyperostosis of adjacent bone

    freq.calcified

    classic pathological finding:psammoma bodies multiple in 8% of cases

    Meningioma en plaque=diffuse type

    -14.3-19% of primary intracranial neoplasm

    -peak incidence=45 years-F:M(1.8:1)

    -1.5%=childhood

    -19-24%=adolescent

    -60-70%=falx,convexity,sphenoid bone

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    Parasagittal and falx meningiomas

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    Parasagittal and falx meningiomas:

    half invade the superior sagittal sinus

    manifest.=motor difficulty with the contralateral foot

    Olfactory groove meningioma: foster-kennedy syndrome

    mental status changes

    urinay incontinence

    visual impairment Seizure

    Pathological Classification:

    1.meningotheliomatous/syncitial

    -sheets of polygonal cells

    2.fibrous or fibroblastic

    -cells separated by connective tissue stroma

    3 t iti l

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    3.transitional

    -contains whorls,some of which are calcified(psammoma bodies)

    4.angiomatous-meningotheliomatous variety with closely packedblood vessels

    5.angioblastic-meningeal hemangiopericytoma

    6.malignant

    -characterized by frequent mitoses,cortical

    invasion,metastases

    Most common site of mets:

    Lung,liver,lymph nodes and heart

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    treatment

    Surgery

    Total excision

    Recurrence:

    Gross total excision=11-15% in 10 years

    Incomplete= 59% in 5 years

    Recurrence:Gross total excision=11-15%

    Incomplete=29%

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    SIMPSON GRADING

    I=macroscopically complete

    II=macroscopically complete+coagulationIII=macroscopically complete without coag.or dural

    excision

    IV=partial removal

    V=simple decompression

    Value of XRT

    Recurrence rate

    total resection:4%

    partial resection without XRT:60%

    partial resection with XRT:32%

    ACOUSTIC NEUROMA

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    ACOUSTIC NEUROMA

    are really schwannomas

    superior vestibular division of the 8th nerve

    3 common early symptoms: hearing loss(98%)

    tinnitus(70%)

    dysequilibrium(67%)

    8-10%

    Antoni A fibers

    -narrow and bipolar cells

    Antoni B fibers

    -loose reticulated

    arise from loss of a tumor suppressor gene on the long arm ofchrom.22

    Neurofibromatosis type 2

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    Neurofibromatosis type 2

    inc.incidence

    bilateral AN

    Symptoms:

    Unilateral sensorineural hearing loss,tinnitus,dysequilibrium(VIII)

    Otalgia,facial numbness and weakness,taste changes (V,VII)

    Brain stem compressionSigns:

    VII-earliest cranial invloved

    Rinne test-positive

    Weber test-lateralized to side of better hearing

    other signs:abnormal corneal reflex,nystagmus,facialhypoesthesia,facial weakness,abnormal eye movement,papilledema,babinski sign

    Radiographic evaluation:

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    Radiographic evaluation:

    MRI

    -diagnostic procedure of choice

    -sensitivity:98%-0% false positive rate

    -char.=round or oval enhancing tumor centered on IAC

    CT scan

    -second choice

    -normal in 6%

    -advantage over MRI:shows bony anatomy- trumpeting-enlarge IAC ostium

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    Treatment

    Excision

    Radiation

    Gamma knife surgery

    ACOUSTIC NEUROMA

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    ACOUSTIC NEUROMA

    Cranial nerve preservation in suboccipital removal

    Size of tumor Preserved function

    VII nerve VIII nerve

    2 cm 50-76% 6%

    Complications(morbidity/mortality)

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    Complications(morbidity/mortality)

    meningits-5.7%

    CSF leaks-7.2%

    CVA-0.7%

    shunt req.-0.7%

    Cranial nerve dysfx.=VII, VIII,IX,X,XIIbrainstem dysfx.

    Follow-up

    -CT-scan,MRI

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    PITUITARY ADENOMA

    -arise primarily from the adenohypophysis

    -10% of the intracranial tumors

    -most common in the 3rd and 4th decades of life

    -affect both sexes equally

    -incidence inc.in MEN/MEA

    Microadenoma=< 1cm

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    Presentation:

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    Presentation:

    -endocrine disturbance

    -mass effect

    -pituitary apoplexy-CSF rhinorrhea

    *Pituitary apoplexy=abrupt onset of neurologicdeterioration(HA,visualdist.,opthalmoplegia,reduced mental status)

    -Hge. and/necrosis(3% of macroade.)

    Classified:

    1.Functional

    2.Non-functional

    Functional Pituitary Tumors:

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    Functional Pituitary Tumors:

    Hormone secreted

    1.ACTH/corticotropin

    a.endogeneous hypercoticolism(cushings dse)

    b.Nelsons syndrome

    2.Prolactin

    -amenorrhea-galactorrhea

    -impotence

    -infertility

    3.Growth hormone

    -acromegaly in adults

    -gigantism in prepubertal children

    Non-Functional Pituitary Tumors:

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    Non Functional Pituitary Tumors:

    Manifestation:

    1.visual disturbance-bitemporal hemianopsia(opticchiasm)

    2.hypopituitarism

    3.cavernous sinus

    4.prolactin levels(ng/ml)

    150=prolactinoma

    5.posterior pituitary

    check adequacy of ADH

    *Dexamethasone suppression test

    Endocrine evaluation

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    Endocrine evaluation

    -may give indication of tumor type

    -needs hormone to be replaced

    -comparison following treatment1.Adrenal axis

    -cortisol

    2.thyroid axis

    -T4,TSH

    3.gonadal axis

    -FSH,LH,sex steroids(estradiol,testoterone)

    ACROMEGALY

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    ACROMEGALY

    1.growth hormone

    >10ng/ml

    2.borderline cases

    somatomedine-C (IGF-I)

    glucose suppression test

    growth hormone releasing hormone stimulation test

    3.PRL

    PITUITARY TUMORS

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    U U O S

    Radiographic evaluation:

    MRI-imaging of choice

    -information about invasion of cav. sinus-location/inv.of parasellar carotids

    CT

    Contrast enhancement:(time dependent)

    1.normal pituitary

    2.macroadenomas enhance more

    3.microadenomas enhance less

    Angiography-localize the parasellar carotids

    Treatment

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    Treatment1.Acromegaly

    surgery

    medical tx:bromocriptine,octreotideradiation

    Medical

    1.bromocriptine2.Octreotide

    Radiation

    -not routinely useSurgery

    PITUITARY TUMORS

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    PITUITARY TUMORS

    Indications for surgery

    1.prolactinomas

    2.primary cushings dse.

    3.acromegaly

    4.macroadenomas5.pituitary apoplexy

    6.histopathologic dx

    *Diabetic Insipidus

    PITUITARY TUMORS

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    Post-op. comlications:

    1.hypopituitarism

    2.secondary empty-sella syndrome3.infection-pituitary abscess,meningitis

    4.CSF rhinorrhea

    5.Carotid artery rupture

    6.nasal septum perforation

    Outcome(transphenoidal surgery)

    endocrinologic cureprolactinoma=25%

    growth hormone=20%

    Gross total unusual in tumors>2cm

    recurrence=12%

    CRANIOPHARYNGIOMA

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    CRANIOPHARYNGIOMA-arise from anterior superior margin of the pituitary

    -lined with with stratified squamous epithelium-solid and cystic component

    -cholesterol crystal

    -calcification (50%)

    -2.5%-4%

    -50%occur in childhood(5-10yrs)

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    CRANIOPHARYNGIOMA

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    CRANIOPHARYNGIOMA

    Treatment:

    surgery

    outcome=5-10% mortality

    5 year survival=55-85%

    Radiationcontroversial

    Recurrence

    most occur in

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    CRANIOPHARYNGIOMA

    Comparison of Craniopharyioma:Rathkes cleft cyst

    Feature Craniopharyngio. Rathkes cleft cyst

    Site of origin Anterior superior

    margin of pit.

    Pars intermedia of

    pit.Cell lining Stratified

    squamous epith.Single layer

    cuboidal epith.

    Cysts contents Cholesterolcrystals

    Resembles motoroil

    Surgical

    treatment

    Total removal Partial excision

    And drainage

    CEREBRAL METASTASIS

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    CEREBRAL METASTASIS-most common brain tumor seen clinically

    -15-20% of patient with Ca

    -15% as initial presenting symptom in pt.with no CA hx.

    -43-60% will have an abnormal CXR

    CEREBRAL METASTASIS

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    CEREBRAL METASTASIS

    Incidence inc.:

    1.increasing length of survival

    2.enhanced ability to diagnose CNS tumors

    3.many chemotherapeutic agents cant cross the BBB

    4.some chemotx.agent weakens the BBB

    CEREBRAL METASTASIS

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    CEREBRAL METASTASIS

    Sources of Cerebral mets.in adults

    Primary %

    Lung CA 44%

    breast 10%

    kidney 7%

    GI 6%

    melanoma 3%

    undetermined 10%

    CEREBRAL METASTASIS

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    Specific Types of Cerebral mets:

    1.melanoma

    -classically inv.the pia and arachnoid

    -primary site cannot be identified in 14% of cases

    2.Lung CA

    -commonest source-radiosensitive

    3.Renal Cell CA

    Signs and symp.

    -slowly progrssive

    Evaluation:

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    CT

    -solitary in 50-65%

    -arise in the gray-white matter junction

    -profound white matter edema

    -enhance

    MRI

    -more sensitive

    Lumbar puncture

    -useful in carcinomatous meningitis

    Met.workup:

    1.CXR

    2.CT of the chest/abdomen

    3.stool exam

    4.mammogram

    Management:

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    -median survival=32 wks

    -mostly palliative

    Medical:

    anticonvulsant

    corticosteroid

    H2 antagonist

    Surgical mngt:

    Solitary lesion

    1.primary dse. Is quiescent

    2.lesion accesible

    3.symptomatic or life-threatening

    4.dx.unknown

    Multiple lesion

    -irradiation

    CEREBRAL METASTASIS

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    Outcome:

    resection of single mets+postop radiotx=8 months

    Radiotx=15 wks

    Steroids=2 months

    Not tx=1 month


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