NEOPLASM OF THE CENTRAL NERVOUS SYSTEM. DR. AMITABHA BASU MD

General - Statistics vary widely Primary tumors of the CNS account for about 9% of all neoplasms. Primary tumors of the CNS account for about 9% of all neoplasms. Of all intracranial tumors, approximately 30% are metastatic. Of all intracranial tumors, approximately 30% are metastatic.

ANATOMICAL LOCATION OF TUMORS 70% of primary intracranial tumors in adults are supratentorial. 70% of primary intracranial tumors in adults are supratentorial. 70% of primary intracranial tumors in children are infratentorial 70% of primary intracranial tumors in children are infratentorial

INCIDENCE The most common primary malignant intracerebral tumor in adults is the Glioblastoma Multiforme. The most common primary malignant intracerebral tumor in adults is the Glioblastoma Multiforme. The most frequent malignant brain tumor in children is the Medulloblastoma. The most frequent malignant brain tumor in children is the Medulloblastoma.

Brain tumors 1. Primary Neuroglial Tumor: (gliomas) 1. Astrocytoma 2. Oligodendroglioma 2. Primitive Neuroepithelial Tumor 1. Medulloblastoma 3. Neuronal Tumor, Lymphoma 4. Meningioma 5. Metastatic Tumor 1. In the brain parenchyma 2. In the meninges

ASTROCYTOMA Types 1. Fibrillary Astrocytoma 2. Pilocystic astrocytoma. 3. Glioblastoma Astrocytoma (high grade) Genes and Markers: Mutation of TP53 is important for the development of this tumor. Marker: GFAP = Glial Fibrillary acidic Protein

Fibrillary Astrocytoma Common in Adult

Fibrillary astrocytic astrocytoma: fibrillary appearance.

Pilocystic Astrocytoma Common in Children Gross: cystic mass Micro: Rosenthal fibers.

Pilocystic Astrocytoma Rosenthal fibers Cystic mass in cerebellum

Pilocytic Astrocytoma Common in the children Common in the children Common sites: Common sites: Cerebellum, Third ventricle, Optic nerve Cerebellum, Third ventricle, Optic nerve PROGNOSIS DEPENDS ON THE SITE. PROGNOSIS DEPENDS ON THE SITE. If occur in Cerebellum = It is surgically resectable – so good prognosis. If occur in Cerebellum = It is surgically resectable – so good prognosis.

Glioblastoma Multiforme GBM Gross Micro Clinical Features Prognosis & treatment

A Glioblastoma Multiforme ; very bad prognosis; most aggressive neoplasm Note: this one has crossed the midline to the opposite hemisphere.

Glioblastoma Multiforme (GBM): Histology 1. Demonstrates marked cellularity 2. Pleomorphism and high mitosis. 3. Area of necrosis with neoplastic cells palisading around it. 4. Vascular proliferation

Necrosis Palisading of Neoplastic cells

Magnetic resonance imaging (MRI) scan of the head in sagittal view A large glioblastoma multiforme involving the left occipital lobe. A large glioblastoma multiforme involving the left occipital lobe.

Symptoms and Signs Produced by Brain Tumors A. Tumor Mass Effect : compression of blood vessels and herniation. B. Effect due to Surrounding Edema ( by metastatic Tumor: herniation 4 features

Clinical Features and Prognosis 3. Raised intracranial Pressure [Headache] [Headache] 3. Focal abnormality: Seizers Uncontrollable shaking of his Uncontrollable shaking of his arm and leg. arm and leg.

Prognosis & treatment Prognosis depends upon Prognosis depends upon 1. Location 2. Histological Grade 3. Age [ adult : bad prognosis] Treatment Treatment Surgical removal ( if possible) and radio/chemo therapy. Surgical removal ( if possible) and radio/chemo therapy.

Oligodendroglioma This type of glioma tends to be well circumscribed, with cystic areas and focal calcification. This type of glioma tends to be well circumscribed, with cystic areas and focal calcification. [Calcification = Important radiological Clue] These tumors comprise about 5% of all gliomas. These tumors comprise about 5% of all gliomas.

EPENDYMOMA

EPENDYMOMA Age: Any age Age: Any age Common in first 2 Common in first 2decades Location : Location : Ventricles Ventricles Central canal of Spinal Central canal of Spinal Cord. Cord.

Microscopic appearance- 2 types 1. In the ventricle ( within cranium): this ependymoma reveals a true rosette pattern with the cells arranged about a central vascular space. 2. Myxopapillary ependymoma, which is typically found arising in the filum terminale of the spinal cord.

Ependymoma reveals a rosette: Perivascular

Myxopapillary ependymoma : The cells around papilla that have a myxoid connective tissue core. Found arising in the filum terminale of the spinal cord

Ependymoma- Clinical features Depends upon the location. Depends upon the location. Intracranial Tumor = Hydrocephalous, headache. Intracranial Tumor = Hydrocephalous, headache. These tumor-cell can be seen in the CSF. These tumor-cell can be seen in the CSF.

PRIMITIVE NEUROEPITHELIAL NEOPLASM (PNET) Medulloblastoma Other tumors of PRIMITIVE NEUROEPITHELIAL cell origin…. 1.Pineoblastoma 2.Ependymoblastoma

Medulloblastoma Lesion of the Cerebellum Lesion of the Cerebellum Midline vermis in young children Midline vermis in young children Spread: through CSF Spread: through CSF Micro: Homer-Wright rosettes Micro: Homer-Wright rosettes

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

Medulloblastoma Without therapy, they are rapidly fatal. Without therapy, they are rapidly fatal. Surgical excision, chemotherapy, and radiotherapy (to the entire spinal cord), have produced 70% - 5-year survivals. Surgical excision, chemotherapy, and radiotherapy (to the entire spinal cord), have produced 70% - 5-year survivals.

Acoustic Neuromas Origin: The VIII cranial nerve. Origin: The VIII cranial nerve. Location: In the cerebellopontine angle. Location: In the cerebellopontine angle. Clinical: P Clinical: Present with hearing loss. Schwannoma) Micro: Neurilemoma (Schwannoma).

Microscopic patterns Antoni A pattern Show Verocay bodies. Antoni A pattern Show Verocay bodies. The Antoni B patterns: low cellularity The Antoni B patterns: low cellularity Schwannoma is markedly positive with S-100 staining

Metastatic tumor Location: Border of the grey and white matter in the distribution of the middle cerebral artery. Location: Border of the grey and white matter in the distribution of the middle cerebral artery. Multiple and well circumscribed. Multiple and well circumscribed. Other site is meninges Other site is meninges The lung is the most common primary site. The lung is the most common primary site. Others: ALL, melanoma. Others: ALL, melanoma.

Meningeal Metastasis: Leptomeningeal Carcinomatosis. Metastases of malignant cells in the leptomeninges. Metastases of malignant cells in the leptomeninges. Breast cancer is the most likely primary. Breast cancer is the most likely primary. Cranial Nerve palsy is common in Meningeal Metastasis. Cranial Nerve palsy is common in Meningeal Metastasis.

Carcinoma Cells in the Meninges.

Meningioma Derived from meningothelial cells. Derived from meningothelial cells. Female > male Female > male Attached to dura- parasagital Attached to dura- parasagital Micro: Micro: Spindle cells in syncytial mass Spindle cells in syncytial mass Arranges in whorls Arranges in whorls Psammoma body Psammoma body

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

Meningioma- Associated with Neurofibromatosis Type 1 Neurofibroma, peripheral schwanoma, Meningioma, pigment is skin and iris. Neurofibromatosis Type II Schwanoma of VIII cranial nerve, Meningioma, post lens opacification

MRI scan in axial view A discrete mass along the lateral convexity. A discrete mass along the lateral convexity. Clinical: slow growing, weakness of the limbs. Clinical: slow growing, weakness of the limbs.

Primary Brain Lymphoma Etiology: EBV, AIDS and immunosuppression. Etiology: EBV, AIDS and immunosuppression. Location of tumor cells: pervascular and peri ventricular. Location of tumor cells: pervascular and peri ventricular. Type: NHL Type: NHL

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