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The Central Nervous System

The Central Nervous System. Tumors. Gliomas Neuronal tumors Poorly differentiated neoplasms Other parenchymal tumors Meningiomas Paraneoplastic syndromes Peripheral nerve sheath tumors Familial tumor syndromes. Tumors. 20% of all childhood cancers are CNS tumors

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The Central Nervous System

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  1. The Central Nervous System

  2. Tumors • Gliomas • Neuronal tumors • Poorly differentiated neoplasms • Other parenchymal tumors • Meningiomas • Paraneoplastic syndromes • Peripheral nerve sheath tumors • Familial tumor syndromes

  3. Tumors 20% of all childhood cancers are CNS tumors 70% of childhood tumors arise in the posterior fossa 70% of adult tumors arise in cerebral hemispheres, supratentorial Rarely metastasize outside of the CNS Symptoms – seizures, headaches, focal neurological deficits, hydrocephalus, ataxia

  4. Gliomas Astrocytomas Infiltrating vs non-infiltrating (pilocytic) Grade I – pilocytic – often cystic Grade II – diffuse astrocytoma Grade III – anaplastic astrocytoma Grade IV – glioblastoma (pseudopalisading) Oligodendrogliomas White matter Well-circumscribed, better prognosis Ependymomas NF2, ependyma-lined ventricular system Perivascular pseudorosettes Subepndymomas Choroid plexus papillomas/ carcinomas Colloid Cyst of third ventricle

  5. Medulloblastoma Poorly differentiated Well-circumscribed Midline of the cerebellum Desmoplastic variant Drop metastases – cauda equina

  6. Meningiomas Attached to the dura Usually benign En plaque Psammoma bodies Atypical Anaplastic

  7. Metastatic ¼ to ½ of intra-cranial tumors in hospitalized patients Five most common Lung Breast Melanoma Kidney GI

  8. Peripheral Nerve Sheath Tumors Schwannoma Neural crest-derived Schwann cell Acoustic neuroma – actually vestibular Neurofibroma Cutaneous Peripheral nerve – solitary or plexiform Malignant peripheral sheath tumors

  9. Familial Tumor Syndromes Neurofibromatosis Type I – AD, Lisch nodules, café au lait spots Neurofibromatosis Type II – AD, 8th nerve schannomas and multiple meningiomas Tuberous Sclerosis – AD, hamartomas ( cortical tubers) and benign neoplasms of the brain; seizures; liver, kidney, pancreas cysts; angifibromas, shagreen patches, ash-leaf spots, subungal fibromas Von Hippel – Lindau Disease – AD, hemangioblastomas (cerebellum and retina); cysts of liver, pancreas, kidney; renal cell carcinoma, pheochromocytomas

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