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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
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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
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Varieties of brain tumors
Meninges: meningioma, hemangiopericytoma Astrocytes: astrocytoma (various types) Oligodendrocytes: oligodendroglioma Ventricles: ependymoma, choroid plexus papilloma, colloid cyst Vascular: hemangioblastoma Primitive cells: germinoma, medulloblastoma, neuroblastoma, pineoblastoma, retinoblastoma Neuronal: ganglioglioma, gangliocytoma Pituitary: adenoma, craniopharyngioma Nerves: schwannoma, neuroblastoma
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Incidence of Intracranial Gliomas (All ages)
Glioblastomas Astrocytomas Ependymomas Medulloblastomas Oligodendrogliomas Choroid plexus papillomas Colloid cysts 55.0% 20.5% 6.0% 5.0% 2.0%
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Incidence of Primary Intraspinal Intramedullary Gliomas
63.0% 24.5% 7.5% 3.0% 2.0% Ependymomas Astrocytomas (grades 1 and 2) Glioblastomas (Astrocytomas grades 3 and 4) Oligodendrogliomas Other tumors
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Frequent brain tumors Meningioma Astrocytoma/glioblastoma
Oligodendroglioma Ependymoma Medulloblastoma Schwannoma/neurofibroma Phakomatosis
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Meningioma Arachnoid cells origin Attached to dura, subdural
Common sites Changes in cranium Hyperostosis Invasion Microscopic: whorls and psammoma bodies
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Gliomas Astrocytes- astrocytomas Fibrillary Pilocytic
Oligodendrocytes- oligodendrogliomas Ependyma- ependymomas
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Astrocytomas Adults: Childhood: Supratentorial Solid Malignant
Infratentorial Cystic Benign
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Adult vs childhood astrocytomas
Adult: fibrillary. Grading varies from low grade malignancy to one of most malignant brain tumor. Childhood: pilocytic. Very low grade tumor (benign).
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Fibrillary astrocytomas
Grossly solid Common in cerebral hemispheres Low grade in young, higher grade in older Grading astrocytoma (low grade) Anaplastic astocytoma glioblastoma multiforme
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Fibrillary astrocytoma: microscopic
Low grade- hypercellularity, pleomorphism Anaplastic- as above plus mitosis, vascular endothelial proliferation Glioblastoma multiforme- as above plus necrosis and pseudopalisades. Grossly variegated appearance (multiforme)
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Pilocytic astrocytoma
Common in childhood Most slow growing of the gliomas Sites: cerebellum, around III V., optic nerve Grossly cystic with mural nodule Microscopic elongated hair-like (pilo) elongated cells Rosenthal fibers
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Rosenthal fiber definition
Dense, eosinophilic fibers within cytoplasmic processes of astrocytes. Correspond to aggregate accumulation of intermediate filaments in these processes.
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Oligodendroglioma Slow growing tumor Potentially malignant
Calcifications
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Tumors in Ventricles 1. Ependyma: Ependymoma
2. Choroid Plexus: Papilloma
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Ependymomas Arise from ependymal lining- ventricles and central canal of spinal cord Common in childhood 4th V. common in cerebrum Most common tumor of spinal cord parenchyma in adult Microscopic perivascular pseudorosettes ependymal rosettes
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Primitive neuroectodermal tumors
Neuroblastoma- cerebral hemispheres Medulloblastoma- cerebellum Ependymoblastoma- ventricles Pineoblastoma- pineal region
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Medulloblastoma Origin: primitive neuroectodermal cells
Age: 1st decade of life Site: vermis of cerebellum May cause hydrocephalus Subarachnoid dissemination
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Histologic patterns: definitions
Whorls: onion-skinning pattern of tumor cells Psammoma bodies: laminated calcium Pseudopalisading: lining up of the tumor cells around a central necrotic area Palisade: lining up of tumor cells around their own cytoplasmic processes. No necrosis. Pseudorosette: tumor cells around blood vessels, cells equidistant from vessel walls. Rosettes: tumor cells around central lumen or fibrillary area of cellular processes
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Brain Tumors: Microscopic
Meningioma Whorls and psammoma bodies Glioblastoma Pseudopalisades Oligodendroglioma Mosaic/poached-egg Ependymoma Perivascular pseudorosettes Medulloblastoma Rosettes
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Tumors of Nerve Roots and Peripheral Nerves
1. Schwannoma viii Cranial nerve (Acoustic sch.) Spinal roots, posterior Peripheral nerves 2. Neurofibroma Spinal Roots, rare 3. Malignant variants Rare
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Peripheral nerve tumors
Schwannoma Schwann cells Compress the nerve trunk Encapsulated Easily resectable without nerve damage Microscopic: Antony A and B fibers Verocay bodies Neurofibroma Schwann cells, neurites, fibroblasts Fusiform and involves nerve trunk Not encapsulated Not resectable without sacrificing nerve Micro- Intermingled cells with wavy nuclei
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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.
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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
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Phakomatosis (Neurocutaneous dysplasia)
1. Neurofibromatosis (von Recklinghausen's dis.) 2. Tuberous Sclerosis 3. Sturge-Weber disease (Encephalofacial Angiomatosis) 4. von Hippel-Lindau Disease 5. Neurocutaneous Melanosis
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Neurofibromatosis 1. Dominant inheritance 2. Multiple neurofibromas
Central - CNS peripheral nerves 3. Increased incidence of: meningioma glioma schwannoma - bilateral VIII N. 4. Cafe-au-lait (melanosis) in skin 5. Elephantiasis: increased connective tissue
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Tuberous Sclerosis 1. Dominant inheritance 2. Clinical triad: seizures
mental retardation adenoma sebaceum 3. Retinal hamartoma (phakoma) 4. Tubers in cerebral cortex 5. Subependymal giant cell astrocytoma 6. Hamartomas in other organs: heart, kidney
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Venice
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