Seminar Brain Tumors

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    CLASSIFICATION ANDPRESENTATION OF

    BRAIN TUMORS

    PRESENTER: Dr. Asifa Andleeb

    MODERATOR: Dr.NAZIR AHMED KHAN

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    Anatomy of brain

    The brain is the center of thoughts, emotions,

    memory and speech.

    Brain also control muscle movements and

    interpretation of sensory information (sight,

    sound, touch, taste, pain etc)

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    MRI of brain

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    Compartments of brainSupratentorial

    compartment

    Cerebralhemispheres

    Basal

    ganglia Thalamic

    nuclei Lateral

    ventricles Hypothal

    amus Corpus

    callosum

    Infratentorial

    compartment

    Cerebellum

    Brainstem(MB/P/MO)

    4thventricle

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    GENERAL CONSIDERATIONS 0F BRAIN TUMOURS

    1. Comprise: 10% of all tumors

    2. Most common childhood neoplasms3. Peak incidence at 5th decade

    4. Supratentorial tumors in adults

    5. Infratentorial in tumors in childhood

    6. Different tumors in different ages

    7. Primary tumors infiltrative, metastatic well-demarcated

    8. Intraneural seeding occur, but no extraneuralmetastasis

    9. Produce neurologic symptoms by size,location,invasiveness, and secondary effects

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    CLASSIFICATION OF BRAIN TUMORSBrain tumors include all tumors

    inside the cranium or in the central spinal canal.

    They are created by anabnormal anduncontrolled cell division,normally either in the

    brain itselfneurons

    glial cells (astrocytes,oligodendrocytes,ependymal cells,

    myelin-producingSchwann cells),

    lymphatic tissue, bloodvessels

    in the cranialnerves,

    in the brainenvelopes

    (meninges), skull,

    pituitary and pinealgland, or

    spread from cancersprimarily located inother organs(metastatic tumors).

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    Classification of brain tumors

    The WHO approach incorporates and interrelates

    morphology,

    cytogenetics, molecular genetics, and

    immunologic markers

    in an attempt to construct a cellular classification that isuniversally applicable and prognostically valid. Earlierattempts to develop a TNM-based classification status (N)does not apply because the brain and spinal cord have nolymphatics, and metastatic spread (M) rarely applies

    because most patients with central nervous system (CNS)neoplasms do not live long enough to develop metastaticdisease.

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    .I.Glial tumors

    a.Astrocytic tumors. Pilocytic

    astrocytoma.

    Diffuse astrocytoma(including fibrillary,protoplasmic, and

    gemistocytic).

    Anaplasticastrocytoma.

    Glioblastoma(including giant cellglioblastoma, andgliosarcoma).

    Pleomorphicxanthoastrocytoma.

    Subependymal giantcell astrocytoma.

    b.Oligodendroglial

    tumors.

    Oligodendroglioma

    .

    Anaplastic

    oligodendroglioma.

    c.Mixed gliomas. Oligoastrocytoma.

    Anaplastic

    oligoastrocytoma.

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    d.Ependymal

    tumors.

    Myxopapillary

    ependymoma. Subependymoma.

    Ependymoma

    (including cellular,

    papillary, clear cell,and tanycytic).

    Anaplastic

    ependymoma.

    e.Neuroepitheli

    al tumors ofuncertain

    origin.

    Astroblastoma

    .

    Chordoid

    glioma of thethird ventricle.

    Gliomatosis

    cerebri.

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    II.Neuronal andmixed neuronal-glial tumors (some

    glial componentmay be present).

    Gangliocytoma.

    Ganglioglioma.

    Desmoplastic infantileastrocytoma/ganglioglioma.

    Dysembryoplastic

    neuroepithelial tumor. Central neurocytoma.

    Cerebellarliponeurocytoma.

    Paraganglioma.

    III.Nonglial tumors.

    a.Embryonal tumors. Ependymoblastoma.

    Medulloblastoma.

    Supratentorialprimitiveneuroectodermaltumor (PNET).

    b.Choroid plexustumors.

    Choroid plexuspapilloma.

    Choroid plexuscarcinoma.

    c.Pineal parenchymaltumors. Pineoblastoma.

    Pineocytoma.

    Pineal parenchymaltumor of intermediatedifferentiation.

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    2.MENINGEAL

    TUMORS. Meningioma.(be

    nign,atypical&mali

    gnant)

    Melanocyticlesion.

    5.MESENCHYMAL TUMORS BENIGN

    MESENCHYMAL TUMORSMALIGNANT

    - Hemangiopericytoma

    chondrosarcomaMalignant fibrous histiocytoma

    Rhabdomyosarcoma

    6.GERM CELL TUMORS.

    Germinoma.

    Embryonal carcinoma.

    Yolk-sac tumor (endodermal-sinustumor).

    Choriocarcinoma.

    Teratoma.

    Mixed germ cell tumor.

    3.TUMORS OR UNCERTAINHISTOGENESIS.

    Capillary hemangioblastoma

    4.HEMATOPOIETIC

    NEOPLASMA

    Malignant lymphomas(primary CNSlymphoma)

    Plasmacytoma

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    7.TUMORS OF THE SELLAR REGION.Pituitary adenoma.

    Pituitary carcinoma.

    Craniopharyngioma.

    8.CYSTS/TUMOR LIKE LESIONS

    Rathke cyst

    Epidermoid cyst

    Dermoid cyst

    9.TUMORS OF PERIPHERAL NERVES THAT AFFECT THE CNS.

    Schwannoma.

    Neurofibfoma

    10.METASTATIC TUMORS

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    supratentorial & infratentorial Tumors

    SUPRATENTORI

    AL TUMORS Meningiomas

    Gliomas

    Astrocytomas

    GlioblastomaMultiforme

    Oligodendrogli

    omas

    Germinomas

    Colloid Cysts of

    Third Ventricle

    INFRATENT

    ORIAL

    TUMORS Choroid plexus

    papillomas

    Cerebellar

    astrocytomas Medulloblastomas

    Hemangioblastomas

    Ependymomas

    Brainstem gliomas

    Schwannomas

    Pituitary adenomas

    Craniopharyngioma

    s

    WHO G d F t t di t

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    WHO Grade:Four-category tumor grading system

    Grade I tumors:Slow growing

    Nonmalignanttumors

    Patients havelong-termsurvival

    Grade III

    Malignant

    tumorsOften recur

    as highergrade

    tumors

    Grade II tumors:

    Relatively slowgrowing

    Sometimes recur ashigher grade tumors

    May benonmalignant ormalignant

    Grade IV

    Highlymalignantandaggressive

    ernohan Grade D t t

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    ernohan Grade:De nes progress ve ma gnancy or astrocytom

    Grade 1 benign astrocytoma

    Grade 2 low-grade astrocytoma

    Grade 3 anaplastic astrocytomaGrade 4 glioblastoma multiformis

    St. Anne/Mayo

    Grade Used for

    astrocytomas

    Uses fourmorphologic

    criteria1.Nuclear-

    atypia

    2.Mitosis

    3.Endothelial

    proliferation

    St. Anne/Mayo Grade Grade 1 = 0 criterion

    Grade 2 = 1criterion, usuallynuclear atypia

    Grade 3 = 2 criteria,usually nuclearatypia and mitosis

    Grade 4 = 3 or 4

    criteria

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    PRESENTATION OF BRAIN TUMORS

    Any brain tumor is inherently serious and life-threateningbecause of its

    invasive

    and infiltrative character

    in the limited space of the intracranial cavity. . Becausethe brain is well protected by the skull, the earlydetection of a brain tumor only occurs when diagnostictools are directed at the intracranial cavity. Usuallydetection occurs in advanced stages when the presence

    of the tumor has side effects that cause unexplainedsymptoms.

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    PRESENTATION OF BRAIN TUMORSThe visibility

    of signs andsymptoms ofbrain tumorsmainly

    depends ontwo factors:

    I.tumor size

    (volume)and

    II. tumorlocation

    Symptoms of solidneoplasms of the

    brain (primarybrain tumors andsecondary tumorsalike) can be

    divided in 3 maincategories

    1. Consequences of

    intracranialhypertension

    2. Dysfunction

    3. Irritation

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    I)CONSEQUENCES OF INTRACRANIAL

    HYPERTENSION :

    The symptoms thatoften occur first arethose that are theconsequences ofincreased intracranial

    pressure: Largetumors or tumorswith extensiveperifocal swelling(edema) inevitablylead to elevatedintracranial pressure(intracranialhypertension), whichtranslates clinicallyinto;

    Headaches,

    vomiting (sometimes

    without nausea), altered state ofconsciousness(somnolence, coma),

    dilatation of the pupil othe side of the lesion(anisocoria),

    papilledema (prominenoptic disc at thefunduscopic eyeexamination)

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    small tumors obstructing the passage ofcerebrospinal fluid (CSF) may cause early signs ofincreased intracranial pressure.

    Increased intracranial pressure may result in

    herniation (i.e. displacement) of certain parts ofthe brain, such as the cerebellar tonsils or thetemporal uncus, resulting in lethal brainstemcompression.

    In very young children, elevated intracranialpressure may cause an increase in the diameterof the skull and bulging of the fontanelles.

    ) d di l i d ( i

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    II)DYSFUNCTION : depending on tumor location, damage( it may

    have caused to surrounding brainstructures), either through

    compressionor infiltration,

    any type of focal

    neurologic

    symptoms may

    occur, such ascognitive and

    behavioral

    impairment

    (including

    impaired judgment

    memory loss,

    lack of recognition,

    spatial orientation

    personality or emotional

    changes,

    hemiparesis,

    hypoesthesia,

    aphasia, ataxia,

    visual field impairment,

    impaired sense of smell,

    impaired hearing,

    facial paralysis,

    double vision

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    And more severe symptoms like

    Hemiplegia impairment to swallow. A bilateral temporal visual field defect

    (bitemporal hemianopiadue to compression

    of the optic chiasm), often associated with

    endocrine disfunction

    either hypopituitarism or

    hyperproduction of pituitary hormones

    and hyperprolactinemia is suggestive of a

    pituitary tumor.

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    III)IRRITATION :

    signs abnormal fatigue,

    weariness,

    absences and tremors, also epileptic seizures

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    Infratentorial vs Supratentorial TumorsSUPRATEN

    TORIAL

    TUMORS

    Meningiomas

    Gliomas

    Astrocytomas Glioblastoma

    Multiforme

    Oligodendrogli

    omas

    Germinomas

    Colloid Cysts of

    Third Ventricle

    INFRATENT

    ORIAL

    TUMORS Choroid plexus

    papillomas

    Cerebellar

    astrocytomas Medulloblastomas

    Hemangioblastomas

    Ependymomas

    Brainstem gliomas

    Schwannomas

    Pituitary adenomas

    Craniopharyngioma

    s

    Epi:

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    MENINGIOMA

    Epi: 2nd most common primary brain

    tumor after gliomas, incidence of ~6/100,000

    Usual age 40-70 F>M

    Facts: Arise from arachnoidal cap cell type

    from the arachnoid membrane

    Usually non-invasive Associated with NF-2

    Location: Parasagittal region

    Sphenoid wing Parasellar region

    Presentation: Asymptomatic Symptomatic: focal or generalized

    seizure or gradually worseningneurolo ic deficit

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    Astrocytes- astrocytomas

    Fibrillary

    Pilocytic

    Oligodendrocytes- oligodendrogliomas

    Ependyma- ependymomas

    Gliomas

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    GLIOMASArise from Glial Cells

    AstrocytomasAstocytomas fall on a gradient that ranges from benign to malignant

    Oligodendrogliomas

    Low Grade Pilocytic

    Astocytomas

    Glioblastoma

    multiforme

    Benign Malignant

    Diffuse Low Grade

    Astrocytomas

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    ASTROCYTOMADiffuse Low Grade Astrocytoma

    Epi: 15% of Astrocytomas

    Young Adults

    Facts: Widely Infiltrate surrounding tissue

    Location: Frontal Region

    Subcortical white matter

    Presentation: Seizures

    Headache

    Slowly progressive neurologic deficits

    Cyst

    T1 weighted T2 weighted

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    ASTROCYTOMA:High Grade Astrocytoma: Glioblastoma

    Epi: Most common type of primary brain tumor in adults Age of presentation: 40-60, M>F

    Facts: May arise de novo or evolve from a low-grade glioma Tumor infiltrates along white matter tract and can cross

    corpus callosum Poor Prognosis Can look like a butterfly lesion

    Location: Frontal & Temporal Lobes Basal Ganglia

    Presentation: Seizures, Headache Slowly progressive neurologic deficits

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    Astrocytomas

    ADULTS

    Supratentorial

    Solid

    Malignant; fibrillary.

    CHILDHOOD

    Infratentorial

    Cystic

    Benign ; pilocytic ,

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    astrocytomas

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    OLIGODENDROGLIOMA

    Epi:

    5-10% of primary brain tumors Mean age of onset 40 years

    Facts:

    Distinguished pathologically from astrocytomas by thecharacteristic fried egg appearance. Arises from Myelin

    Location: Superficially in Frontal Lobes

    Presentation: Seizures most common Headache Slowly progressive neurologic deficits

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    Oligodendroglioma Slow growing tumor

    Potentially malignant

    Calcifications

    GERMINOMA

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    GERMINOMA

    Facts: Germ Cell Tumors

    Causes Parinauds Syndrome

    disorder characterized by fixed upward gaze

    Location:

    Commonly in Pineal Region (>50%)

    Overlies tectum of midbrain

    Presentation: Obstructive Hydrocephalus due to aqueductal stenosis

    T1 Images

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    COLLOID CYST OF THE VENTRICLE

    Epi:

    Usually in Adults

    1% of all intracranial tumors

    Facts:

    Managed Surgically

    Causes hydrocephalus

    by obstructive flow Endodermal origin

    Location:

    Foramen of Monro

    Anterior aspect of third

    ventricle Presentation:

    Headaches

    Vertigo

    Memory deficits

    CHOROID PLEXUS PAPILLOMAS

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    CHOROID PLEXUS PAPILLOMAS Epi

    Represents 2% of gliomas One of the most common

    brain tumors in patients

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    CEREBELLAR ASTROCYTOMA

    Epi: Most often occurs in

    childhood

    Facts: Most potentially curable

    of the astrocytomas

    Location: Posterior Fossa

    Presentation: Headaches

    Nausea/Vomiting

    Gait Unsteadiness Posterior head tilt with

    caudal

    tonsillar herniation

    Cyst

    Tumor arising from vermis or cerebellar

    hemispheres

    MEDULLOBLASTOMAS

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    MEDULLOBLASTOMAS Epi

    Represent 7% of primary brain tumors

    2

    nd

    most common posterior fossa tumor in children 70% of patients are diagnosed prior to age 20 with peak incidence

    between 5-9 years of age;

    Facts Primitive neuroectodermal tumors (PNET)

    Soft, friable tumors, often necrotic Can metastasize via CSF tracts

    Highly radiosensitive

    Location About 75% arise within the cerebellar vermis

    Presentation Most frequently present with signs of intracranial pressure

    May cause hydrocephalus

    Cranial nerve deficits may also occure

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    HEMANGIOBLASTOMA

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    HEMANGIOBLASTOMA

    Epi 2% of primary intracranial tumors and 10% of posterior fossa

    tumors Most found in young adults and children

    Facts Characterized by abundant capillary blood vessels If found in cerebellum and retina, may represent part of von

    Hippel-Lindau syndrome. Acute hemorrhage can be fatal 15-20% of patients with hemangioblastomas can present

    with erythrocytosis

    Presentation

    Usually present with neurologic deficits by directcompression or hemorrhage Neurologic deficits may include cerebellar ataxia, oculomotor

    nerve dysfunction, motor weakness, or sensory deficits

    Location

    Most often found in cerebellum and spinal cord

    Epi

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    EPENDYMOMASImaging Usually well demarcatedwith frequent areas of calcification,

    hemorrhage, and cysts;

    Epi

    Accounts for 10% of CNS lesions;

    Male=Female

    Median age at diagnosis is 5 yearsold

    Facts

    Derived from primitive glia

    Overall survival at 10 years is 45-55%

    Presentation

    Most patients present withsymptoms of increased intracranialpressure

    Location

    Typically arise within or adjacent tothe ependymal lining of theventricular system.

    In children, 90% are intracranial with60% arising in posterior fossa (4thventricle is the most commoninfratentorial site)

    Most common spinal cord glioma (inadults, 75% arise within spinalcord);;

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    BRAINSTEM GLIOMAS

    Epi Male=Female

    Account for 10-20% on all CNS tumors

    More common in children (account for 20% of all intracranialneoplasms under the age 15);

    In children, median age at diagnosis is 5-9 years of age.

    Facts NF-1 is the only known risk factor

    Mostly benign (but range from benign to very aggressive);

    Long term survival for low-grade gliomas is near 100%.

    Location In peds, 80% arise in pons, with 20% arise in medula, midbrain, and

    cervicomedulary junction;

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    BRAINSTEM GLIOMAS

    Presentation Most patients with low-grade

    brainstem gliomas have along history of minor signsand symptoms;

    May present with neck painor torticollis;

    Medulary tumors maypresent with cranial nervepalsies, dysphagia, nasalspeech and apnea, n/v,ataxia,or weakness;

    May cause locked-insyndrome

    SCHWANNOMAS

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    SCHWANNOMAS Epi

    Female>male Median age at diagnosis is 50

    Account for 80-90% of cerebellopontine angle tumors Comprise 8% of intracranial tumors in adults; rare in children

    (except with NF-2) Facts

    Unilateral in 90% of cases (R=L); Bilateral acoustic neuromas are diagnostic of NF-2;

    Presentation Patients may present with asymmetric sensorineural hearing

    loss, tinnitus Fluctuating unsteadiness while walking, vertigo (although only

    1% of patients with vertigo had schwannomas);

    If CN V nerve is affected, facial numbness, pain, andhyperesthesia may be present; If CN VII is affected, facial paresis may be present. Tumor progression may lead to compression of brainstem or

    cerebellum leading to ataxia, tonsil herniation, andhydrocephalus

    Location Arise from vestibular division of CN VIII; majority benign

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    SCHWANNOMAS

    PITUITARY Imaging:

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    PITUITARYADENOMAS

    Epi

    Most common tumors ofpituitary gland

    Represent 8% of primary braintumors

    Facts Out of pituitary adenomas,

    prolactinomas are the mostcommon;

    Presentation

    May cause hypopituitarism andvisual field defects;

    Patients should have endocrine,radiographic, andophthalmologic assessments.

    Imaging:

    Plain x-ray may show an enlargedsella turcica;

    MRI is the imaging of choice;

    Imaging

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    CRANIOPHARYNGIOMAS

    Epi

    Represent 1-3% of primary braintumors

    Bimodal distribution: first peak infantsand children; second peak 55-65 yearold

    Facts

    Derived from epithelial remnants ofRathkes pouch; slow growing; benign

    Tend to recur even after completeremoval

    20-year survival rate of children withcraniopharyngiomas is about 60%.

    Location Located in suprasellar fossa and

    inferior to optic chiasm

    Presentation Cause bitemporal hemianopsia and

    hypopituitarism;

    frequently present with headache;

    g g

    Cystic calcified

    parasellar lesion could

    be seen on radiograph;

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    Metastatic brain tumors

    Most common brain tumor in adults.

    Common primary sites: melanoma, lung, breast, GItract, kidney.

    Most are in cerebrum (MCA territory).

    In gray-white junctions due to rich capillarityDiscrete, globoid, sharply demarcated tumors.

    Amenable to surgical resection.

    Single or multiple.

    Brain edema frequent.

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    thankyou