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Page 1 Tumor of the Nervous System Dr Wiwit Ade FW, M.Biomed, SpPA

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Tumor of the Nervous System

Dr Wiwit Ade FW, M.Biomed, SpPA

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Normal Cells

The principal cells of the CNS : • Neurons : functional unit of nervous system.

(axon & dendrites) • Glia : derived from neuroectoderm

(macroglia: astrocytes, oligodendrocytes, ependyma) or from bone marrow (microglia),

• the cells that compose the meninges and blood vessels.

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Tumors of CNS

• Incidence : 10-17 per 100,00 person for intracranial tumor.

• 1/2 -3/4 : primary tumor, & the rest are metastatic.

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Primary Tumors of the Central Nervous System

–Glioma• Astrocytoma• Oligodendroglioma• Ependymoma

–Neuronal lineage–Meningioma–Nerve Sheath Tumors

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Primary brain tumors: Cell types

1. Neuron: Gangliocytoma, ganglioglioma medulloblastoma

2. Astrocyte: Astrocytoma, glioblastoma3. Oligodendrocyte: Oligodendroglioma4. Ependymal cell: Ependymoma5. Microglial cell: Tumors derived from

microglial cells 6. Meningeal cell: Meningiomas are derived

from arachnoidal cells and are usually dural-based.

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Primary Tumours of the CNSTumour Cells of origin Site Age Behavior

Oligodendroglioma oligodendrocyte hemisphere adulthood Low to highgrade

Astrocytoma Astrocyte HemisphereCerebellum

AdulthoodChildhood

Low to highgradeLow grade

Glioblastoma multiforme

Astrocyte Hemisphere Adulthood High grade

Ependymoma Ependyma IVth ventricleSpinal cord

AdulthoodChildhoos

High gradeLow grade

Meningioma Arachnoidal Meninges Adulthood Low grade

Medulloblastoma Neuroectoderm Cerebellum Childhood High grade

Haemangioblastoma

Unknown Cerebellum All ages Low grade

Lymphoma Lymphocyte Hemisphere Adulthood High grade

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Gliomas

• Diffusely infiltrating (not easily resected)• Histologic appearance (grade) correlates

with overall survival• May become more malignant (higher

grade) over time (especially astrocytomas which become glioblastomas)

• May spread via CSF• Rarely (never) metastasize

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GLIOMAS

• ASTROCYTOMAS

• OLIGODENDROGLIOMAS

• EPENDYMOMAS

• MIXED GLIOMAS

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• Spectrum : low grade – high grade• 2 major categories :

1. infiltrating2. non infiltrating : >> astrocytoma pylocitic

(grade I/IV).

ASTROCYTOMA

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ASTROCYTOMA

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ASTROCYTOMA

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Infiltrating Astrocytomas

• 80% of adult primary brain tumors.• Cerebral hemisphere.• Symptom : seizures, headache & focal

neurologic deficits.• Spectrum histophat diff : correlates with

clinical course & outcame.

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Spectrum of Infiltrating Astrocytomas

• Diffuse astrocytoma ( grade II/IV)

• Anaplastic astrocytoma ( grade III/IV)

• Glioblatoma ( grade IV/IV)

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Diffuse Astrocytoma

Macroscopic: • Poorly defined, gray, infiltrative tumor• Size : few cm- replace an entire hemisphere.• Cut surface : firm/ soft gelatinous, cystic degeneration. • Infiltrating beyond the outer margin : always present.

Microscopic examination :• Mild to moderate increase in glial cellularity, variable

nuclear pleomorphic, back ground a fibrillary app.• Transition between neop & normal tissue : indistinct.• Tumor cells can be seen infiltrating normal tissue.

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Diffuse Astrocytoma

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Anaplastic astrocytoma

• More densely cellular, greater nuclei pleomorphic, >> mitotic figure.

• Gemistocytic astrocytoma : astrocytoma with brightly eosinophilic cell body.

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AstrocytomaFeatures of Anaplasia

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Glioblastomas

• = glioblastoma multiforme• Variation in gross app.• Firm & white, soft & yellow (necrosis),

cystic deg, hemorrhage.• Microscopic = anaplastic, malignant tumor

cells, additional : 1. necrosis with pseudopalisading app. 2. vascular / endothelial prolif.

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Glioblastoma Multiforme

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Glioblastoma Multiforme

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Pylocitic Astrocytoma• Relative benign behavior• Grow very slowly th/ by resection• Symptomatic recurrence : incomplete resection

cystic enlargement, rather than growth of the solid areas.

• Children & young adults• Located in :

- >> the cerebellum.- floor & wall of Ventricle III- optic nerve- cerebral hemisphere

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Pilocytic Astrocytoma

Morphology :

• >> cyctic• Solid, well demarcated, << infiltrative

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Microscopic: • Tumor is composed of bipolar cells with

long, thin “hair like” processes dense fibrillary meshworks.

• Rosenthal fibers• Eosinophilic granular bodies.• Biphasic areas : loose microcystic pattern &

fibrillary areas.• Necrosis & mitosis : uncommon.

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Pilocytic Astrocytoma

Rosenthal fibers Eosinophilic granular bodies

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OLIGODENDROGLIOMA

• 5-10% glioma• 4-5 decadce• Symptom : >> seizures• Location : >> cerebral hemisphere ( white

matter)• WHO grade II/IV

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Morphology of Oligodendroglioma• Well-circumscribed, gelatinous, gray masses, >>

cyst, focal haemorrhage, & calcification.• Microscopic :

- sheet of regular cells with spherical nuclei containing finely granular chromatin (=normal oligodendricytes) surrounded by clear halo cytoplasm.- typically contain a delicate network anastomosing capillaries (thin walled capillaries).

- calcification : > 90% tumors (foci to massive)- mitotic activity : <<

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Anaplastic Oligodendroglioma• WHO grade III/IV• Increased cell density, nuclear anaplasia,

mitotic activity >>, necrosis.

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OLIGODENDROGLIOMA

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EPENDYMOMA• Arise next to the ependyma line ventricular system.• Behave as WHO grade II/IV• Anaplastic ependymoma : WHO grade II/IV.Macroscopic :• In ventricle IV : solid/ papillary mass ascending from the

floor of the ventricle.• Well demarcated.Microscopic:• Composed of cells with regular, round to oval nuclei &

abundant granular cromathin.• Gland like round/ elongated app ( perivascular rossetes) ,

tumor cells are surrounded vessels.

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EPENDYMOMA

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NON-GLIAL TUMORS

• Medulloblastoma: Malignant cerebellar tumor of childhood

• Meningioma: Benign, superficial, well-circumscribed tumor derived from arachnoidal cells

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MEDULLOBLASTOMA• Origin : neuroectodermal• Poorly differentiated• >> children , 20% of the brain tumor in children• Location : midline of the cerebellum (children)

lateral of the cerebellum (adult) • Rapid growth occlude the flow CSF hidrocephalus.• Macros : well circumscribed, gray.• Micros :

- extremly cellular, with sheet anaplastic cells- individual tumor cells: small, scant cytop, hypercromatic nuclei.- >> mitosis- Homer Wright rossette/ neurosecretory granules.

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Medulloblastoma

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Medulloblastoma

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MENINGIOMA• Benign tumor of adult• Location : attached to dura• Arise from the meningothelial cells of arachnoid.• Low risk of recurrence.• WHO grade I/IV• Macros :

- rounded masses, well defined dural bases that comprise underlying brain.- encapsulated, thin fibrous tissue.- growth : polypoid, plaque.

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Histological pattern

• Sincytial (“meningothelial”) : whorled clusters of cells without visible cells membrane

• Fibroblastic : elongated cells and abundant collagen deposition between them.

• Transtitional :share features between sincytial and fibroblastic type.

• Psammomatous : with psammoma bodies ( calcification of the syncitial nest of meningothelial cells)

• Secretory : PAS (+) intracytoplasmic droplets & intracellular lumen (electron microscopy)

• Microcystic : with a loose, spongy app.

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MENINGIOMA

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Sincytial (“meningothelial”)

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Fibroblastic

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Secondary Involvement of the Central Nervous System

• Metastatic Tumor –Melanoma–Renal cell–Lung

• Contiguous involvement (pituitary adenoma and craniopharyngioma)

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METASTATIC MELANOMA

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