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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. 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 children are infratentorial INCIDENCE The most common primary malignant intracerebral tumor in adults is the Glioblastoma Multiforme. The most frequent malignant brain tumor in children is the Medulloblastoma. Brain tumors 1. Primary Neuroglial Tumor: (gliomas) 1. 2. 2. Primitive Neuroepithelial Tumor 1. 3. 4. 5. Astrocytoma Oligodendroglioma Medulloblastoma Neuronal Tumor, Lymphoma Meningioma 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 Cystic mass in cerebellum Rosenthal fibers Pilocytic Astrocytoma Common in the children Common sites: Cerebellum, Third ventricle, Optic nerve PROGNOSIS DEPENDS ON THE SITE. 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 Palisading of Neoplastic cells Necrosis Magnetic resonance imaging (MRI) scan of the head in sagittal view 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] 3. Focal abnormality: Seizers Uncontrollable shaking of his arm and leg. Prognosis & treatment Prognosis depends upon 1. Location 2. Histological Grade 3. Age [ adult : bad prognosis] Treatment Surgical removal ( if possible) and radio/chemo therapy. Oligodendroglioma 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. EPENDYMOMA EPENDYMOMA Age: Any age Common in first 2 decades Location : Ventricles Central canal of Spinal 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. Intracranial Tumor = Hydrocephalous, headache. 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 Midline vermis in young children Spread: through CSF Micro: Homer-Wright rosettes Occasionally there may be a suggestion of neuronal differentiation (Homer-Wright rosettes) . Medulloblastoma Without therapy, they are rapidly fatal. Surgical excision, chemotherapy, and radiotherapy (to the entire spinal cord), have produced 70% - 5-year survivals. Acoustic Neuromas Origin: The VIII cranial nerve. Location: In the cerebellopontine angle. Clinical: Present with hearing loss. Micro: Neurilemoma (Schwannoma). Microscopic patterns Antoni A pattern Show Verocay bodies. 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. Multiple and well circumscribed. Other site is meninges The lung is the most common primary site. Others: ALL, melanoma. Meningeal Metastasis: Leptomeningeal Carcinomatosis. Metastases of malignant cells in the leptomeninges. Breast cancer is the most likely primary. Cranial Nerve palsy is common in Meningeal Metastasis. Carcinoma Cells in the Meninges. Meningioma Derived from meningothelial cells. Female > male Attached to dura- parasagital Micro: Spindle cells in syncytial mass Arranges in whorls Psammoma body Meningioma whorls Psammoma bodies may be seen on microcopy. 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. Clinical: slow growing, weakness of the limbs. Primary Brain Lymphoma Etiology: EBV, AIDS and immunosuppression. Location of tumor cells: pervascular and peri ventricular. Type: NHL Thanks, All of you, Wish you Good luck!! BE A GOOD DOCTOR