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This Concept Map, created with IHMC CmapTools, has information related to: CNS III - Toxic, Tumors, CNS III Periph Nerve Sheath Tumors -w/ S-100 markers MALIGNANT SCHWANNOMA -not from benign S'oma -de novo or from plexiform NF -thus ass'd w/ NF-1 -can follow XRT MORPH-infiltrate parent nn. -some stain for S-100 -Triton-rhabdomyoblastic -cartilage, bone forms -Epithelioid malig s'omas -S-100, but NOT keratin, CNS III Neurocutaneous Syndromes (Phakomatoses) TUBEROUS SCLEROSIS-AD -hamartomas, benign neoplasms all over -Cortical Tubers-hamartomas in CNS -also subependymal -Renal angiolipomas -Retinal glial hamartomas -Pulm, Cardiac myomas -Cysts in liver, kidneys, pancreas -Skin: Angiofibromas, Shagreen patches, Ash-Leaf patches, subungual fibromas -genetics-TSC1 on Chrom 9q34->hamartin -TSC2 on 16p13.3->tuberin MORPH-cortical lesions like potatoes-harder -no NL organization in layers -"candle-guttering"-droplike astrocytic clusters -subependymal giant cell astrocytoma -unique to TS Tx-symptomatic - anticonvulsants for Sz, CNS III Gliomas PILOCYTIC ASTROCYTOMA -benign behavior -KIDS, young adults -CEREBELLUM, 3rd vent walls, ON -cystic or solid MORPH-hairlike processes on uscope -GFAP-positive -Rosenthal fibers, Microcysts CLINICAL-very SLOW -can live up to 40 yrs after complete resection, CNS III Periph Nerve Sheath Tumors -w/ S-100 markers NEUROFIBROMA 1. Cutaneous NF or Solitary NF (periph nn) -sporadic or w/ NF-1 -dermis or subQ fat -spindle cells 2. Plexiform NF-w/ NF-1 ONLY -difficult to remove -malignant potential -mostly on large nerve trunk -invades nerve-unlike Schwannoma -Schwann cells, MAST cells, inflam -fibroblasts, CNS III Other PARANEOPLASTIC SYNDROMES Para. CBLR Degeneration-Br, gyn CA -destroy Purkinje cells, gliosis, mild inflam -AB-mediated->CBLR S/Sx Limbic Encephalitis-SCC lung CA -subacute dementia Subacute Sensory Neuropathy-SCC lung -+/- limbic encephalitiis -lose sensory neurons from DRG -also w/ inflammation, CNS III Poorly Diff'd Neoplasms MEDULLOBLASTOMA-VERY malignant -20% of childhood brain tumors -ONLY in CEREBELLUM -can still express cell markers -genetics: chrom 17 MORPH-in midline (in kids) -can->hydrocephalus -can involve leptomeninges->SAS -dissemination thru CSF->METS -to cauda equina "drop metastases" -sheets of anaplastic cells -small blue cells -Ki-67 markers -Neuronal phenotype-Homer Wright rosettes, neurosec granues -Glial-express GFAP Tx-VERY RADIOSENSITIVE!-good survival -best Px w/ total resection and XRT, CNS III Gliomas BRAIN STEM GLIOMA ងyo (20% primary brain tumors ង) -50% -> glioblastoma -Tx-XRT-ɱ yr survival 20-40%, CNS III Acquired Metabolic VIT B12 DEFICIENCY (Subacute Combined Degen of Sp Cord) -most serious problem-neuro -also megaloblastic anemia -slight ataxia, paresthesias ->spastic weakness of legs ->complete paraplegia -poor recovery if too late MORPH-swelling of myelin layers -begins at mid-thoracic cord ->posterior columns, descending pyr tracts degrade, CNS III Acquired Metabolic HYPERGLYCEMIA-DM -can be ass'd w/ ketoacidosis, hyperosmolar coma ->dehydration, confusion -correct fluid depletion GRADUALLY -otherwise, can->cerebral edema, CNS III Other MENINGIOMAS-FEMALES, ADULTS -benign, att'd to DURA -if multiple, prob NF-2 -from meningiothelial cell of arachnoid -also in ventricles, from stromal arach -loss of chromosome 22q - also NF2 gene MORPH-can extend into bone -or "en plaque" spreading along dura -PSAMMOMA BODIES Types: syncytial, fibroblastic, transitional, psammomatous, secretory, microcystic Malignant-invade brain, mitoses CLINICAL-slow, vague Sx -progesterone receptors-grow during preg, CNS III Gliomas FIBRILLARY (DIFFUSE) ASTROCYTOMA GLIOBLASTOMA MULTIFORME -80% adult primary brain tumors -usually cerebral hemispheres -also CBLM, stem, cord -30s-50s S/Sx-Sz, HA, focal deficits MORPH-infiltration ALWAYS present ->mass effect -edema in adjacent tissues -contrast material leaks out-visualize GM-like anaplastic+necrosis+ vasc or endoth cell proliferation -VEGF->tufts (glomeruloid bodies) -Ki-67 to grade lesion Gliomatosis Cerebri-all over brain Genetics: Low-grade-p53 mut (1/3) High-RB, p16 ass'n-poor Px, CNS III Toxic ETHANOL -acute-reversible -chronic-direct effects and nutritional deficits -CBLR dysfcn-1% of drunks ->truncal ataxia, unsteady gait, nystagmus -atrophy, loss of granule cells -esp ANTERIOR VERMIS -lose Purkinje cells -astrocytes proliferate b/w layers (Bergmann gliosis) -FAS, CNS III Neurocutaneous Syndromes (Phakomatoses) NF-2-AD-less common than NF-1+ -most common tumors: acoustic SCHWANNOMA -bilateral multiple meningiomas -Schwannosis-ingrowth into cord -meningioangiomatosis -glial hamartia -chrom 22q12-NF2 gene->merlin -like cytoskeleton proteins -NONSENSE worse than missense, CNS III Other METASTATIC TUMORS -mostly carcinomas -comprise 1/2 of intracranial tumors -mostly LUNG, BREAST, skin (mel), kidney, GI tract -chorioCA often mets to brain -often the first sign of CA, mass lesions, CNS III Acquired Metabolic HYPOGLYCEMIA-like O2 depletion -large pyramidal nn more sensitive -can->pseudolaminar necrosis -layers III-V of cortex -HIPPOCAMPUS also sensitive (Sommer) -PURKINJE cells vulnerable, CNS III Gliomas OLIGODENDROGLIOMA -30s-40s-often w/ years of complaints -5-15% gliomas -mostly in cerebral hemispheres -white matter MORPH-cysts, calcificaiton, hemorrhage -sheets of cells-resemble oligos -lots of anastamosing caps CLINICAL-better Px than astrocytomas -surgery, CTX, XRT-ɱ-10 yr survival MIXED GLIOMA= oligodendroglioma + astrocytoma, CNS III Other PINEAL PARENCHYMAL TUMORS -pineocytomas or pineoblastomas -blastomas w/ mitotic figs, little neuronal diff'tion -aggressive->spread thru CSF -more in KIDS, often w/ retinoblastoma -GLIOMAS also found, CNS III Neurocutaneous Syndromes (Phakomatoses) VON HIPPEL-LINDAU DZ-AD -cap hemangioblastomas in CBLR hemispheres, retina, stem/cord -can->polycythemia -cysts in panc, liver, kidneys -STRONG PROPENSITY -> RENAL CELL CA -genetics- pVHL-tumor suppressor-inhibit elongation step of RNA synth -missense mutations->pheochromo'oma MORPH-many thin-walled vessels Tx-resection, laser therapy, nephrectomy, CARBON MONOXIDE CO ->hypoxia -selective injury of layers III-V nn. -Sommer sector HIPPOCAMPUS -PURKINJE CELLS -bilateral globus pallidus necrosis -more common in CO than other causes of hypoxia Hypoxic effects HYPOGLYCEMIA-like O2 depletion -large pyramidal nn more sensitive -can->pseudolaminar necrosis -layers III-V of cortex -HIPPOCAMPUS also sensitive (Sommer) -PURKINJE cells vulnerable, CNS III Acquired Metabolic THIAMINE (B1) DEFICIENCY ->BERIBERI ->WERNICKE ENCEPHALOPATHY -acute onset ->KORSAKOFF SYNDROME -memory disturbances, confabulation -in chronic alcoholism, gastric d/os (CA, gastritis, vomiting) Tx-always treat acute onset enceph as wernicke->administer B1 MORPH-hemorr, necrosis in MAMILLARY BODIES, adj to vent -medial dorsal nuc lesions-mem loss -hemosiderin-laden macs