MODERATOR: DR.

NAZIR AHMED KHAN

 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)

 Supratentorial

compartment:
Sagittal

Cerebral hemispheres Basal ganglia Thalamic nuclei Lateral ventricles Hypothalamus Corpus callosum

Infratentorial

compartment:

Cerebellum Brain stem (MB/P/MO) 4th ventricle

INTP - PPO, PHO, IAP. P2 6/27

Axial

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),

in the cranial nerves,

in the brain envelopes (meninges), skull, and pineal gland, or

pituitary

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

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

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

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.

1.NEUROEPITHELIAL TUMORS.
I.Glial

tumors.
tumors.

a.Astrocytic

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.

e.Neuroepithelial tumors of uncertain origin.

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

 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.

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.

2.MENINGEAL TUMORS.

Meningioma. Hemangiopericytoma. Melanocytic lesion.

3.GERM CELL TUMORS.

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

4.TUMORS OF THE SELLAR REGION.

Pituitary

adenoma. Pituitary carcinoma. Craniopharyngioma.

5.TUMORS OF UNCERTAIN HISTOGENESIS.

Capillary hemangioblastoma

6.PRIMARY CNS LYMPHOMA.

7.TUMORS OF PERIPHERAL NERVES THAT AFFECT THE CNS.

Schwannoma.

8.METASTATIC TUMORS

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

20

Grade III

Malignant tumors Often recur as higher grade tumors

Grade IV

Highly malignant and aggressive

21

Defines progressive malignancy for astrocytoma

Grade 1 benign astrocytoma Grade 2 low-grade astrocytoma Grade 3 anaplastic astrocytoma Grade 4 glioblastoma multiformis

22

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

23

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

 Meningiomas
Gliomas

Astrocytomas Glioblastoma Multiforme Oligodendrogliomas

Germinomas Colloid

Cysts of Third Ventricle

 Choroid

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

 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.

The visibility of signs and symptoms of brain tumors mainly depends on two factors: size (volume) and tumor location

tumor

 Symptoms

of solid neoplasms of the brain (primary brain tumors and secondary tumors alike) can be divided in 3 main categories
of intracranial hypertension

Consequences 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),

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)

 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.

II.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,

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 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.

III.IRRITATION : signs abnormal fatigue,

weariness, absences and tremors, also epileptic seizures

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

 Astrocytes

astrocytomas

Fibrillary Pilocytic

Oligodendrocytes-

oligodendrogliomas Ependyma- ependymomas

 Astrocytomas

Astocytomas fall on a gradient that ranges from benign to malignant

Benign Low Grade Pilocytic Astocytomas Diffuse Low Grade Astrocytomas

Malignant Glioblastoma multiforme

Oligodendrogliomas

Epi:

15% of Astrocytomas Young Adults

Widely Infiltrate surrounding tissue

Facts:

Location:

Cyst

Frontal Region Subcortical white matter

T1 weighted T2 weighted

Presentation:
Seizures Headache Slowly progressive neurologic deficits

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

Adults:

Supratentorial Solid Malignant; fibrillary.

Infratentorial Childhood: Cystic Benign ; pilocytic ,

Fibrillary astrocytomas

Pilocytic astrocytoma

Facts: Distinguished pathologically from astrocytomas by the characteristic fried egg appearance. Arises from Myelin Location: Superficially in Frontal Lobes Presentation: Seizures most common Headache Slowly progressive neurologic deficits

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

Slow growing tumor

Potentially malignant Calcifications

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

Epi:

Facts:

Usually in Adults 1% of all intracranial tumors Managed Surgically Causes hydrocephalus by obstructive flow Endodermal origin Foramen of Monro Anterior aspect of third ventricle Headaches Vertigo Memory deficits

Location:

Presentation:

 Choroid

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

Epi

Facts

Represents 2% of gliomas One of the most common brain tumors in patients < 2 years of age;

Presentation

Benign tumor;
Headache Hydrocephalus secondary to CSF overproduction

Location

Imaging

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

CT: Often calcified & enhanced with contrast

Epi:

Facts:

Most often occurs in childhood Most potentially curable of the astrocytomas Posterior Fossa Cyst

Location:

Presentation:

Headaches Nausea/Vomiting Gait Unsteadiness Posterior head tilt with caudal tonsillar herniation

Tumor arising from vermis or cerebellar hemispheres

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

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 HippelLindau 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

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);;

Imaging

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

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. 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%. In peds, 80% arise in pons, with 20% arise in medula, midbrain, and cervicomedulary junction;

Facts

Location

Presentation

Most patients with lowgrade 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

Epi

Facts

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)
Unilateral in 90% of cases (R=L); Bilateral acoustic neuromas are diagnostic of NF-2; 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 Arise from vestibular division of CN VIII; majority benign

Presentation

Location

Epi

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

Out of pituitary adenomas, prolactinomas are the most common; May cause hypopituitarism and visual field defects; Patients should have endocrine, radiographic, and ophthalmologic assessments.

Facts

Presentation

Imaging:

Plain x-ray may show an enlarged sella turcica; MRI is the imaging of choice;

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 Rathkes 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;

Epi

Facts

Represent 1-3% of primary brain tumors Bimodal distribution: first peak infants and children; second peak 55-65 year old Derived from epithelial remnants of Rathkes pouch; slow growing; benign Tend to recur even after complete removal 20-year survival rate of children with craniopharyngiomas is about 60%. Located in suprasellar fossa and inferior to optic chiasm Cause bitemporal hemianopsia and hypopituitarism; frequently present with headache;

Location

Imaging

Presentation

Cystic calcified parasellar lesion could be seen on radiograph;

 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.

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