Brain Tumor New

CLASSIFICATION AND

PRESENTATION OF

BRAIN TUMORSMODERATOR: DR.NAZIR AHMED

KHAN

SIGNIFICANCE 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)

NORMAL ANATOMY OF BRAIN (MRI)

Supratentorial compartment:Cerebral hemispheresBasal gangliaThalamic nucleiLateral ventriclesHypothalamusCorpus callosum

Infratentorial compartment:CerebellumBrain stem (MB/P/MO)4th ventricle

Sagittal

AxialINTP - PPO, PHO, IAP.P2 – 6/27

CLASSIFICATION OF BRAIN TUMORS Brain tumors include all tumors inside the

cranium or in the central spinal canal.

They are created by an abnormal and uncontrolled cell division

, normally either in the brain itself, 1. neurons 2. glial cells (astrocytes, oligodendrocytes,

ependymal cells, myelin-producing Schwann cells),

3. lymphatic tissue, blood vessels),

CLASSIFICATION OF BRAIN TUMORS in the cranial nerves,

in the brain envelopes (meninges),

skull,

pituitary and pineal gland, or

spread from cancers primarily located in other organs (metastatic tumors).

BRAIN NEOPLASMS: GENERAL CONSIDERATIONS 1. Comprise: 10% of all tumors

2. Most common childhood neoplasms

3. Peak incidence at 5th decade

4. Supratentorial tumors in adults

5. Infratentorial tumors in childhood

BRAIN NEOPLASMS: GENERAL CONSIDERATIONS 6. Different tumors in different ages

7. Primary tumors infiltrative, metastatic well-demarcated 8. Intraneural seeding occur, but no extraneural metastasis 9. Produce neurologic symptoms by size, location, invasiveness, and secondary effects

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 is universally applicable and prognostically valid. Earlier attempts to develop a TNM-based classification status (N) does not apply because the brain and spinal cord have no lymphatics, and metastatic spread (M) rarely applies because most patients with central nervous system (CNS) neoplasms do not live long enough to develop metastatic disease.

CLASSIFICATION OF BRAIN TUMORS1.NEUROEPITHELIAL TUMORS. I.Glial tumors.

a.Astrocytic tumors. Pilocytic astrocytoma. Diffuse astrocytoma (including fibrillary,

protoplasmic, and gemistocytic). Anaplastic astrocytoma. Glioblastoma (including giant cell glioblastoma,

and gliosarcoma). Pleomorphic xanthoastrocytoma. Subependymal giant cell astrocytoma.


b.Oligodendroglial tumors. Oligodendroglioma. Anaplastic oligodendroglioma.

c.Mixed gliomas. Oligoastrocytoma. Anaplastic oligoastrocytoma.


d.Ependymal tumors.

Myxopapillary ependymoma. Subependymoma. Ependymoma (including cellular, papillary, clear

cell, and tanycytic). Anaplastic ependymoma.

CLASSIFICATION OF BRAIN TUMORS e.Neuroepithelial tumors of uncertain

origin.

Astroblastoma. Chordoid glioma of the third ventricle. Gliomatosis cerebri.

CLASSIFICATION OF BRAIN TUMORS II.Neuronal and mixed neuronal-glial

tumors (some glial component may be present).

Gangliocytoma. Ganglioglioma. Desmoplastic infantile

astrocytoma/ganglioglioma. Dysembryoplastic neuroepithelial tumor. Central neurocytoma. Cerebellar liponeurocytoma. Paraganglioma.

CLASSIFICATION OF BRAIN TUMORS III.Nonglial tumors.

a.Embryonal tumors. Ependymoblastoma. Medulloblastoma. Supratentorial primitive neuroectodermal tumor

(PNET). b.Choroid plexus tumors.

Choroid plexus papilloma. Choroid plexus carcinoma.

c.Pineal parenchymal tumors. Pineoblastoma. Pineocytoma. Pineal parenchymal tumor of intermediate

differentiation.

CLASSIFICATION OF BRAIN TUMORS2.MENINGEAL TUMORS.

Meningioma.Hemangiopericytoma.Melanocytic lesion.

CLASSIFICATION OF BRAIN TUMORS3.GERM CELL TUMORS.

Germinoma.Embryonal carcinoma.Yolk-sac tumor (endodermal-sinus tumor).Choriocarcinoma.Teratoma.Mixed germ cell tumor.

CLASSIFICATION OF BRAIN TUMORS4.TUMORS OF THE SELLAR REGION.

Pituitary adenoma. Pituitary carcinoma.Craniopharyngioma.

CLASSIFICATION OF BRAIN TUMORS5.TUMORS OF UNCERTAIN

HISTOGENESIS. Capillary hemangioblastoma

6.PRIMARY CNS LYMPHOMA.

7.TUMORS OF PERIPHERAL NERVES THAT AFFECT THE CNS.

Schwannoma.

8.METASTATIC TUMORS

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WHO GRADE

Four-category tumor grading system• Grade I tumors:

Slow growing Nonmalignant tumors Patients have long-term survival

Grade II tumors: Relatively slow growing Sometimes recur as higher grade tumors May be nonmalignant or malignant

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WHO GRADE (CONT’D.)

• Grade III Malignant tumors Often recur as higher grade tumors

• Grade IV Highly malignant and aggressive

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KERNOHAN GRADE

Defines progressive malignancy for astrocytoma• Grade 1 – benign astrocytoma• Grade 2 – low-grade astrocytoma• Grade 3 – anaplastic astrocytoma• Grade 4 – glioblastoma multiformis

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ST. ANNE/MAYO GRADE Used for astrocytomas Uses four morphologic criteria

• Nuclear atypia• Mitosis• Endothelial proliferation• Necrosis

Grade 1 = 0 criterion Grade 2 = 1 criterion, usually nuclear

atypia Grade 3 = 2 criteria, usually nuclear

atypia and mitosis Grade 4 = 3 or 4 criteria

•

Primary (true) brain tumors are commonly located in the

posterior cranial fossa in children and

in the anterior two-thirds of the cerebral hemispheres in adults, although they can affect any part of the brain


Primary (true) brain tumors are commonly located in the

posterior cranial fossa in children and

in the anterior two-thirds of the cerebral hemispheres in adults, although they can affect any part of the brain

INFRATENTORIAL VS SUPRATENTORIAL TUMORS

SUPRATENTORIAL TUMORS Meningiomas Gliomas

AstrocytomasGlioblastoma MultiformeOligodendrogliomas

Germinomas Colloid Cysts of Third Ventricle

INFRATENTORIAL TUMORS1

Choroid plexus papillomas Cerebellar astrocytomas Medulloblastomas Hemangioblastomas Ependymomas Brainstem gliomas Schwannomas Pituitary adenomas Craniopharyngiomas

PRESENTATION OF BRAIN TUMORS Any brain tumor is inherently serious and life-

threatening because of its• invasive and infiltrative character in the limited space

of the intracranial cavity. . Because the brain is well protected by the skull, the early detection of a brain tumor only occurs when diagnostic tools are directed at the intracranial cavity. Usually detection occurs in advanced stages when the presence of the tumor has side effects that cause unexplained symptoms.

•

PRESENTATION OF BRAIN TUMORS

The visibility of signs and symptoms of brain tumors mainly depends on two factors:

tumor size (volume) and tumor location

PRESENTATION OF BRAIN TUMORS Symptoms of solid neoplasms of the

brain (primary brain tumors and secondary tumors alike) can be divided in 3 main categories

Consequences of intracranial hypertension

Dysfunction Irritation


I.CONSEQUENCES OF INTRACRANIAL

HYPERTENSION : The symptoms that

often occur first are those that are the

consequences of increased intracranial

pressure: Large tumors or tumors with

extensive perifocal swelling (edema)

inevitably lead to elevated intracranial

pressure (intracranial hypertension),

PRESENTATION OF BRAIN TUMORS which translates clinically into

headaches, vomiting (sometimes without nausea), altered state of consciousness

(somnolence, coma), dilatation of the pupil on the side of

the lesion (anisocoria), papilledema (prominent optic disc at

the funduscopic eye examination)

PRESENTATION OF BRAIN TUMORS small tumors obstructing the passage of

cerebrospinal fluid (CSF) may cause early signs of increased intracranial pressure. Increased intracranial pressure may result in herniation (i.e. displacement) of certain parts of the brain, such as the cerebellar tonsils or the temporal uncus, resulting in lethal brainstem compression. In very young children, elevated intracranial pressure may cause an increase in the diameter of the skull and bulging of the fontanelles.

PRESENTATION OF BRAIN TUMORSII.DYSFUNCTION : depending on

tumor location damage( it may have caused to

surrounding brain structures), either through compression or infiltration,

any type of focal neurologic symptoms may occur,

PRESENTATION OF BRAIN TUMORS such as cognitive and behavioral

impairment (including impaired judgment,

memory loss,

lack of recognition,

spatial orientation disorders)


personality or emotional changes,

hemiparesis, hypoesthesia,

aphasia, ataxia,

visual field impairment,

impaired sense of smell, impaired

hearing,

facial paralysis,

double vision


And more severe symptoms like

hemiplegia impairment to swallow. A bilateral temporal visual field

defect


A bilateral temporal visual field defect (bitemporal hemianopia—due 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.

PRESENTATION OF BRAIN TUMORSIII.IRRITATION : signs abnormal fatigue, weariness, absences and tremors, also epileptic seizures

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 worsening neurologic deficit

GLIOMAS Astrocytes- astrocytomas

FibrillaryPilocytic

Oligodendrocytes- oligodendrogliomas

Ependyma- ependymomas

GLIOMAS ARISE 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

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

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

HIGH GRADE ASTROCYTOMA: GLIOBLASTOMA

ASTROCYTOMAS

Adults:

Childhood:

SupratentorialSolidMalignant; fibrillary.

InfratentorialCysticBenign ; pilocytic ,

Fibrillary astrocytomas

Pilocytic astrocytoma

OLIGODENDROGLIOMA Epi:

5-10% of primary brain tumorsMean age of onset 40 years

Facts: Distinguished pathologically from astrocytomas

by the characteristic “fried egg” appearance.Arises from Myelin

Location:Superficially in Frontal Lobes

Presentation:Seizures most commonHeadacheSlowly progressive neurologic deficits

OLIGODENDROGLIOMA

Slow growing tumor

Potentially malignant

Calcifications

GERMINOMA

Facts:

Germ Cell Tumors

Causes Parinaud’s 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

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

INFRATENTORIAL TUMORS1

Choroid plexus papillomas Cerebellar astrocytomas Medulloblastomas Hemangioblastomas Ependymomas Brainstem gliomas Schwannomas Pituitary adenomas Craniopharyngiomas

CHOROID PLEXUSPAPILLOMAS

Epi Represents 2% of gliomas One of the most common

brain tumors in patients < 2 years of age;

Facts Benign tumor;

Presentation Headache Hydrocephalus secondary

to CSF overproduction Location

Occur in decreasing frequency: 4th, lateral, and 3rd ventricles;

Imaging CT: Often calcified &

enhanced with contrast

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

Tumor arising from vermis or cerebellar

hemispheres

Cyst

MEDULLOBLASTOMAS Epi

Represent 7% of primary brain tumors 2nd 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

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 direct compression 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

EPENDYMOMAS Epi

Accounts for 10% of CNS lesions; Male=Female Median age at diagnosis is 5 years old

Facts Derived from primitive glia Overall survival at 10 years is 45-55%

Presentation Most patients present with symptoms of increased intracranial

pressure Location

Typically arise within or adjacent to the ependymal lining of the ventricular system.

In children, 90% are intracranial with 60% arising in posterior fossa (4th ventricle is the most common infratentorial site)

Most common spinal cord glioma (in adults, 75% arise within spinal cord);;

EPENDYMOMA Imaging

Usually well demarcated with frequent areas of calcification, hemorrhage, and cysts;

BRAINSTEM GLIOMAS Epi

Male=Female Account for 10-20% on all CNS tumors More common in children (account for 20% of all

intracranial neoplasms 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;

BRAINSTEM GLIOMAS Presentation

Most patients with low-grade brainstem gliomas have a long history of minor signs and symptoms;

May present with neck pain or torticollis;

Medulary tumors may present with cranial nerve palsies, dysphagia, nasal speech and apnea, n/v, ataxia,or weakness;

May cause “locked-in” syndrome

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, and hyperesthesia

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, and hydrocephalus Location

Arise from vestibular division of CN VIII; majority benign

SCHWANNOMAS

PITUITARY ADENOMAS Epi

Most common tumors of pituitary gland Represent 8% of primary brain tumors

Facts Out of pituitary adenomas, prolactinomas are the

most common; Presentation

May cause hypopituitarism and visual field defects;

Patients should have endocrine, radiographic, and ophthalmologic assessments.

PITUITARY ADENOMAS Imaging:

Plain x-ray may show an enlarged sella turcica;

MRI is the imaging of choice;

CRANIOPHARYNGIOMAS Epi

Represent 1-3% of primary brain tumors Bimodal distribution: first peak infants and children; second

peak 55-65 year old Facts

Derived from epithelial remnants of Rathke’s pouch; slow growing; benign

Tend to recur even after “complete” removal 20-year survival rate of children with craniopharyngiomas is

about 60%. Location

Located in suprasellar fossa and inferior to optic chiasm Presentation

Cause bitemporal hemianopsia and hypopituitarism; frequently present with headache;

CRANIOPHARYNGIOMAS

ImagingCystic calcified

parasellar lesion could be seen on radiograph;

EpiRepresent 1-3% of primary brain tumorsBimodal distribution: first peak infants and children; second peak 55-65 year old

FactsDerived from epithelial remnants of Rathke’s pouch; slow growing; benignTend to recur even after “complete” removal20-year survival rate of children with craniopharyngiomas is about 60%.

LocationLocated in suprasellar fossa and inferior to optic chiasm

PresentationCause bitemporal hemianopsia and hypopituitarism; frequently present with headache;

METASTATIC BRAIN TUMORS Most common brain tumor in adults. Common primary sites: melanoma,

lung, breast, GI tract, kidney. Most are in cerebrum (MCA territory). In gray-white junctions due to rich

capillarity Discrete, globoid, sharply demarcated

tumors. Amenable to surgical resection.

Single or multiple. Brain edema frequent.

PHAKOMATOSIS: DEFINITION Phakos (Greek): lentil mole or freckle. Neurologic abnormalities combined with

defects of skin or retina, explained by their common ectodermal origin.

Involvement of visceral organs

1. Neurofibromatosis (von Recklinghausen's dis.)

2. Tuberous Sclerosis

3. Sturge-Weber disease (Encephalofacial

Angiomatosis)

4. von Hippel-Lindau Disease

5. Neurocutaneous Melanosis

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