NEOPLASM OF THE NEOPLASM OF THE CENTRAL NERVOUS CENTRAL NERVOUS

SYSTEM.SYSTEM.

DR. AMITABHA BASU MDDR. AMITABHA BASU MD

General - Statistics vary General - Statistics vary widelywidely

Primary tumors of the CNS account Primary tumors of the CNS account for about 9% of all neoplasms. for about 9% of all neoplasms.

Of all intracranial tumorsOf all intracranial tumors, , approximately 30% are metastatic. approximately 30% are metastatic.

ANATOMICAL LOCATION OF TUMORSANATOMICAL LOCATION OF TUMORS

70% of primary 70% of primary intracranial tumors intracranial tumors in adults are in adults are supratentorial. supratentorial.

70% of primary 70% of primary intracranial tumors intracranial tumors in children are in children are infratentorialinfratentorial

INCIDENCEINCIDENCE

The most common primary The most common primary malignant intracerebral tumor in malignant intracerebral tumor in adultsadults is the is the Glioblastoma Glioblastoma MultiformeMultiforme..

TThe most frequent malignant he most frequent malignant brain tumor in brain tumor in childrenchildren is the is the MedulloblastomaMedulloblastoma..

Brain tumorsBrain tumors

1.1. Primary Neuroglial Tumor: (gliomas)Primary Neuroglial Tumor: (gliomas)1.1. AstrocytomaAstrocytoma2.2. OligodendrogliomaOligodendroglioma

2.2. Primitive Neuroepithelial TumorPrimitive Neuroepithelial Tumor1.1. MedulloblastomaMedulloblastoma

3.3. Neuronal Tumor, LymphomaNeuronal Tumor, Lymphoma4.4. MeningiomaMeningioma5.5. Metastatic TumorMetastatic Tumor

1.1. In the brain parenchymaIn the brain parenchyma2.2. In the meningesIn the meninges

ASTROCYTOMAASTROCYTOMATypesTypes

1.1. Fibrillary AstrocytomaFibrillary Astrocytoma

2.2. Pilocystic astrocytoma.Pilocystic astrocytoma.

3.3. Glioblastoma Astrocytoma (high grade)Glioblastoma Astrocytoma (high grade)

Genes and Markers:Genes and Markers:

Mutation of TP53 is important for the development of Mutation of TP53 is important for the development of this tumor.this tumor.

Marker: GFAP = Glial Fibrillary acidic ProteinMarker: GFAP = Glial Fibrillary acidic Protein

Fibrillary Fibrillary AstrocytomaAstrocytoma

Common in AdultCommon in Adult

Fibrillary astrocytic astrocytoma: fibrillary Fibrillary astrocytic astrocytoma: fibrillary appearance.appearance.

Pilocystic AstrocytomaPilocystic Astrocytoma

Common in ChildrenCommon in Children

Gross: cystic massGross: cystic mass

Micro: Rosenthal fibers.Micro: Rosenthal fibers.

Pilocystic AstrocytomaPilocystic Astrocytoma

Rosenthal fibersRosenthal fibersCystic Cystic mass in cerebellum mass in cerebellum

Pilocytic AstrocytomaPilocytic Astrocytoma Common in the childrenCommon in the children Common sites:Common sites:

Cerebellum, Cerebellum, Third ventricle, Third ventricle, Optic nerveOptic nerve

PROGNOSIS DEPENDS ON THE SITE.PROGNOSIS DEPENDS ON THE SITE. If occur in Cerebellum = It is surgically If occur in Cerebellum = It is surgically

resectable – so good prognosis.resectable – so good prognosis.

Glioblastoma MultiformeGlioblastoma Multiforme

GBMGBMGross

Micro

Clinical Features

Prognosis & treatmentPrognosis & treatment

A Glioblastoma Multiforme ; very bad A Glioblastoma Multiforme ; very bad prognosis; most aggressive neoplasmprognosis; most aggressive neoplasm

Note: this one has crossed the midline to the opposite hemisphere.

Glioblastoma Multiforme (GBM): Glioblastoma Multiforme (GBM): HistologyHistology

1.1. Demonstrates marked cellularityDemonstrates marked cellularity

2.2. Pleomorphism and high mitosis. Pleomorphism and high mitosis.

3.3. Area of necrosis with neoplastic Area of necrosis with neoplastic cells palisading around it.cells palisading around it.

4.4. Vascular proliferation Vascular proliferation

Necrosis

Palisading of Neoplastic cells

Magnetic resonance imaging (MRI) scan of Magnetic resonance imaging (MRI) scan of

the head in sagittal viewthe head in sagittal view

A large A large glioblastoma glioblastoma multiforme multiforme involving the involving the left occipital left occipital lobe.lobe.

Symptoms and Signs Produced by Symptoms and Signs Produced by Brain TumorsBrain Tumors

A.A. Tumor Mass EffectTumor Mass Effect : compression of blood : compression of blood vessels and herniation.vessels and herniation.

B.B. Effect due to Surrounding EdemaEffect due to Surrounding Edema ( ( by by metastatic Tumormetastatic Tumor: herniation : herniation

4 features

Clinical Features and PrognosisClinical Features and Prognosis

3.3. Raised intracranial PressureRaised intracranial Pressure

[Headache][Headache]

3.3. Focal abnormality: SeizersFocal abnormality: Seizers

Uncontrollable shaking of his Uncontrollable shaking of his

arm and leg.arm and leg.

Prognosis & treatmentPrognosis & treatment

Prognosis depends uponPrognosis depends upon

1.1. LocationLocation

2.2. Histological GradeHistological Grade

3.3. Age [ adult : bad prognosis]Age [ adult : bad prognosis]

TreatmentTreatment Surgical removal ( if possible) and Surgical removal ( if possible) and

radio/chemo therapy.radio/chemo therapy.

OligodendrogliomaOligodendroglioma

This type of glioma tends to be well This type of glioma tends to be well circumscribed, with cystic areas and circumscribed, with cystic areas and focal calcification.focal calcification.

[Calcification = Important radiological [Calcification = Important radiological Clue]Clue]

These tumors comprise about 5% of These tumors comprise about 5% of all gliomas. all gliomas.

EPENDYMOMAEPENDYMOMA

EPENDYMOMAEPENDYMOMA AgeAge: Any age: Any age Common in first 2 Common in first 2

decadesdecades Location :Location :

VentriclesVentricles Central canal of SpinalCentral canal of Spinal

Cord.Cord.

Microscopic appearance- 2 types Microscopic appearance- 2 types

1.1. In the ventricle ( within cranium): this In the ventricle ( within cranium): this ependymoma reveals a ependymoma reveals a true true rosette rosette patternpattern with the cells arranged about a with the cells arranged about a central vascular space. central vascular space.

2.2. Myxopapillary ependymomaMyxopapillary ependymoma, which is , which is typically found arising in the typically found arising in the filum filum terminale of the spinal cordterminale of the spinal cord . .

Ependymoma reveals a rosette: Ependymoma reveals a rosette: PerivascularPerivascular

Myxopapillary ependymomaMyxopapillary ependymoma : The cells around : The cells around

papilla that have a myxoid connective tissue core.papilla that have a myxoid connective tissue core.

Found arising in the Found arising in the filum terminale filum terminale of the spinal cordof the spinal cord

Ependymoma- Clinical featuresEpendymoma- Clinical features

Depends upon the location.Depends upon the location. Intracranial Tumor = Intracranial Tumor =

HydrocephalousHydrocephalous, headache., headache.

These tumor-cell can be seen in the These tumor-cell can be seen in the CSF.CSF.

PRIMITIVE NEUROEPITHELIAL PRIMITIVE NEUROEPITHELIAL NEOPLASM (PNET)NEOPLASM (PNET)

MedulloblastomaMedulloblastoma

Other tumorsOther tumors of of PRIMITIVE PRIMITIVE NEUROEPITHELIAL cell origin….NEUROEPITHELIAL cell origin….

1.1. PineoblastomaPineoblastoma

2.2. EpendymoblastomaEpendymoblastoma

MedulloblastomaMedulloblastoma

Lesion of the CerebellumLesion of the Cerebellum Midline vermisMidline vermis in young children in young children

Spread: through CSFSpread: through CSF

Micro: Micro: Homer-Wright rosettesHomer-Wright rosettes

Occasionally there may be a suggestion of Occasionally there may be a suggestion of neuronal differentiation neuronal differentiation (Homer-Wright (Homer-Wright

rosettesrosettes) .) .

MedulloblastomaMedulloblastoma

Without therapy, they are Without therapy, they are rapidly fatal. rapidly fatal.

Surgical excision, chemotherapy, Surgical excision, chemotherapy, and radiotherapy (and radiotherapy (to the entire to the entire spinal cordspinal cord), have produced ), have produced 70% 70% - 5-year survivals. - 5-year survivals.

Acoustic NeuromasAcoustic Neuromas

Origin: The VIII cranial nerve.Origin: The VIII cranial nerve. Location: In the cerebellopontine Location: In the cerebellopontine

angle.angle.

Clinical: PClinical: Present with hearing loss.

Micro: Neurilemoma (Schwannoma)Schwannoma).

Microscopic patterns Microscopic patterns Antoni A Antoni A

patternpattern Show Show VerocayVerocay bodies. bodies.

The Antoni B The Antoni B patterns: low patterns: low cellularitycellularity

Schwannoma is markedly Schwannoma is markedly positive with S-100 stainingpositive with S-100 staining

Metastatic tumorMetastatic tumor

Location: BLocation: Border of the grey and white order of the grey and white matter in the distribution of the middle matter in the distribution of the middle cerebral arterycerebral artery..

Multiple and well circumscribed.Multiple and well circumscribed. Other site is meningesOther site is meninges The The lunglung is the most common is the most common primary site.primary site. Others: ALL, melanoma.Others: ALL, melanoma.

Meningeal Metastasis: Leptomeningeal Meningeal Metastasis: Leptomeningeal Carcinomatosis.Carcinomatosis.

Metastases of malignant cells in the Metastases of malignant cells in the leptomeninges. leptomeninges.

Breast cancer is the most likely Breast cancer is the most likely primary.primary.

Cranial Nerve palsy is Cranial Nerve palsy is common in Meningeal common in Meningeal Metastasis.Metastasis.

Carcinoma Cells in the Meninges.Carcinoma Cells in the Meninges.

MeningiomaMeningioma

Derived from meningothelial cells.Derived from meningothelial cells. Female Female > male> male Attached to dura- parasagitalAttached to dura- parasagital Micro: Micro:

Spindle cells in syncytial massSpindle cells in syncytial mass Arranges in whorlsArranges in whorls Psammoma bodyPsammoma body

MeningiomaMeningioma

Psammoma bodies may be seen on microcopy.Psammoma bodies may be seen on microcopy.

whorlswhorls

Meningioma- Meningioma- Associated with Associated with

NeurofibromatosiNeurofibromatosis Type 1s Type 1

Neurofibroma, Neurofibroma, peripheral peripheral schwanomaschwanoma, , MeningiomaMeningioma, , pigment is skin and iris.pigment is skin and iris.

NeurofibromatosiNeurofibromatosis Type IIs Type II

Schwanoma of VIII cranial Schwanoma of VIII cranial nervenerve, , MeningiomaMeningioma, post lens , post lens opacificationopacification

MRI scan in axial view MRI scan in axial view

A discrete mass A discrete mass along the lateral along the lateral convexity.convexity.

Clinical: slow Clinical: slow growing, weakness growing, weakness of the limbs.of the limbs.

Primary Brain LymphomaPrimary Brain Lymphoma

Etiology: EBV, AIDS and Etiology: EBV, AIDS and immunosuppression. immunosuppression.

Location of tumor cells: pervascular Location of tumor cells: pervascular and peri ventricular. and peri ventricular.

Type: NHLType: NHL

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