CLASSIFICATION AND PRESENTATION OF BRAIN TUMORS

PRESENTER: Dr. Asifa Andleeb MODERATOR: Dr.NAZIR AHMED KHAN

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)

MRI of brain

Compartments of brain
Supratentorial compartment
Cerebral hemisphe res Basal ganglia Thalamic nuclei Lateral ventricles Hypothal amus Corpus callosum

Infratentorial compartment
Cerebel lum Brain stem (MB/P/ MO) 4th ventricle

GENERAL CONSIDERATIONS 0F BRAIN TUMOURS

1. 2. 3. 4. 5. 6. 7. Comprise: 10% of all tumors Most common childhood neoplasms Peak incidence at 5th decade Supratentorial tumors in adults Infratentorial in tumors in childhood Different tumors in different ages Primary tumors infiltrative, metastatic welldemarcated 8. Intraneural seeding occur, but no extraneural metastasis 9. Produce neurologic symptoms by size,location, invasiveness, and secondary effects

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 neurons glial cells (astrocytes, oligodendrocytes, ependymal cells, myelin-producing Schwann cells), lymphatic tissue, blood vessels

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).

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.

1) 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.

e.Neuroepitheli al tumors of uncertain origin.

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

 II.Neuronal and mixed neuronalglial tumors (some glial component may be present).
Gangliocytoma. Ganglioglioma. Desmoplastic infantile astrocytoma/gangliogli oma. 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.(be
nign,atypical&mali gnant)

5.MESENCHYMAL TUMORS BENIGN MESENCHYMAL TUMORS MALIGNANT

- Hemangiopericytoma
chondrosarcoma Malignant fibrous histiocytoma Rhabdomyosarcoma 6.GERM CELL TUMORS.
Germinoma. Embryonal carcinoma. Yolk-sac tumor (endodermal-sinus tumor). Choriocarcinoma. Teratoma. Mixed germ cell tumor.

Melanocytic lesion. 3.TUMORS OR UNCERTAIN HISTOGENESIS.

Capillary hemangioblastoma

4.HEMATOPOIETIC NEOPLASMA
Malignant lymphomas(primary CNS lymphoma) Plasmacytoma

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

supratentorial & infratentorial Tumors

SUPRATENTORI AL TUMORS
Meningiomas Gliomas
Astrocytomas Glioblastoma Multiforme Oligodendrogli omas

INFRATENT ORIAL TUMORS

Choroid plexus papillomas Cerebellar astrocytomas Medulloblastomas Hemangioblastoma s Ependymomas Brainstem gliomas Schwannomas Pituitary adenomas Craniopharyngioma s

Germinomas Colloid Cysts of Third Ventricle

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

Grade III
Malignant tumors Often recur as higher grade tumors

Grade IV
Highly malignant and aggressive

Kernohan Grade:Defines progressive malignancy for astrocytoma

Grade 1 benign astrocytoma Grade 2 low-grade astrocytoma Grade 3 anaplastic astrocytoma Grade 4 glioblastoma multiformis
St. Anne/Mayo Grade Used for astrocytomas Uses four morphologic criteria
1.Nuclearatypia 2.Mitosis 3.Endothelial proliferation

St. Anne/Mayo Grade 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

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: I.tumor size (volume) and II. tumor location
Symptoms of solid neoplasms of the brain (primary brain tumors and secondary tumors alike) can be divided in 3 main categories 1. Consequences of intracranial hypertension 2. Dysfunction 3. 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 brainstructures), 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
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 (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

Infratentorial vs Supratentorial Tumors

SUPRATEN TORIAL TUMORS
Meningiomas Gliomas
Astrocytomas Glioblastoma Multiforme Oligodendrogli omas

INFRATENT ORIAL TUMORS

Choroid plexus papillomas Cerebellar astrocytomas Medulloblastomas Hemangioblastoma s Ependymomas Brainstem gliomas Schwannomas Pituitary adenomas Craniopharyngioma s

Germinomas Colloid Cysts of Third Ventricle

Epi:

2nd most common primary brain tumor after gliomas, incidence of ~ 6/100,000 Usual age 40-70 F>M Arise from arachnoidal cap cell type from the arachnoid membrane Usually non-invasive Associated with NF-2 Parasagittal region Sphenoid wing Parasellar region Asymptomatic Symptomatic: focal or generalized seizure or gradually worsening neurologic deficit

MENINGIOMA

Facts:

Location:

Presentation:

Gliomas
Astrocytes- astrocytomas Fibrillary Pilocytic Oligodendrocytes- oligodendrogliomas Ependyma- ependymomas

GLIOMAS
Arise from Glial Cells

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

ASTROCYTOMA
Diffuse Low Grade Astrocytoma
Epi:
15% of Astrocytomas Young Adults

Facts:
Widely Infiltrate surrounding tissue

Location:
Frontal Region Subcortical white matter

Cyst

Presentation:
Seizures Headache Slowly progressive neurologic deficits
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:

Location:

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
Frontal & Temporal Lobes Basal Ganglia Seizures, Headache Slowly progressive neurologic deficits

Presentation:

Astrocytomas
ADULTS Supratentorial Solid Malignant; fibrillary. CHILDHOOD Infratentorial Cystic Benign ; pilocytic ,

astrocytomas

OLIGODENDROGLIOMA
Epi:

5-10% of primary brain tumors Mean 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 common Headache Slowly progressive neurologic deficits

Oligodendroglioma

Slow growing tumor Potentially malignant Calcifications

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

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

 Epi

CHOROID PLEXUS PAPILLOMAS

Facts

Represents 2% of gliomas One of the most common brain tumors in patients < 2 years of age; Benign tumor; Headache Hydrocephalus secondary to CSF overproduction Occur in decreasing frequency: 4th, lateral, and 3rd ventricles;

Presentation Location
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

Cyst

Tumor arising from vermis or cerebellar hemispheres

 Epi

MEDULLOBLASTOMAS

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 Facts
2% of primary intracranial tumors and 10% of posterior fossa tumors Most found in young adults and children 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 Usually present with neurologic deficits by direct compression or hemorrhage Neurologic deficits may include cerebellar ataxia, oculomotor nerve dysfunction, motor weakness, or sensory deficits

Presentation

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

EPENDYMOMAS
Imaging Usually well demarcated
with frequent areas of calcification,

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

hemorrhage, and cysts;

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);;

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

 Epi

SCHWANNOMAS

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)

Presentation

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

Location

SCHWANNOMAS

PITUITARY ADENOMAS
Epi
Most common tumors of pituitary gland Represent 8% of primary brain tumors

Imaging:
Plain x-ray may show an enlarged sella turcica;

MRI is the imaging of choice;

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.

CRANIOPHARYNGIOMAS
Epi
Represent 1-3% of primary brain tumors Bimodal distribution: first peak infants and children; second peak 55-65 year old

Imaging
Cystic calcified parasellar lesion could be seen on radiograph;

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;

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.

thankyou