Post on 30-Oct-2014
transcript
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.
CLASSIFICATION OF BRAIN TUMORS
b.Oligodendroglial tumors. Oligodendroglioma. Anaplastic oligodendroglioma.
c.Mixed gliomas. Oligoastrocytoma. Anaplastic oligoastrocytoma.
CLASSIFICATION OF BRAIN TUMORS
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
20
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
21
WHO GRADE (CONT’D.)
• Grade III Malignant tumors Often recur as higher grade tumors
• Grade IV Highly malignant and aggressive
22
KERNOHAN GRADE
Defines progressive malignancy for astrocytoma• Grade 1 – benign astrocytoma• Grade 2 – low-grade astrocytoma• Grade 3 – anaplastic astrocytoma• Grade 4 – glioblastoma multiformis
23
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
CLASSIFICATION OF BRAIN TUMORS
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
PRESENTATION OF BRAIN TUMORS
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)
PRESENTATION OF BRAIN TUMORS
personality or emotional changes,
hemiparesis, hypoesthesia,
aphasia, ataxia,
visual field impairment,
impaired sense of smell, impaired
hearing,
facial paralysis,
double vision
PRESENTATION OF BRAIN TUMORS
And more severe symptoms like
hemiplegia impairment to swallow. A bilateral temporal visual field
defect
PRESENTATION OF BRAIN TUMORS
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