seminar brain tumors
TRANSCRIPT
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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