LECOM Robbins Ch 28 Path CNS tumors
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| Tumors in children where? | posterior fossa
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| Tumors in adults where? | supratentorial
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| Most common adult CNS primary tumor | infiltrating actrocytoma
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| what tumors are gliomas? | astrocytomas
oligodendrogliomas
ependymomas
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| Four major types of astrocytoma and grade | grade 1: pilocytic astrocytoma
grade 2: diffuse fibrillary astrocytoma
grade 3: anaplastic astrocytoma
grade 4: glioblastoma
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| tumor cells infiltrate normal tissue dx? | diffuse fibrillary astrocytoma
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| foamy macrophages dx? | consider benign tumefactive lesion like acute MS plaque - otherwise ddx astrocytoma! also look for reactive gemistocytic astrocytes scattered
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| what IHC is positive in astrocytic processes of astrocytoma? | GFAP
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| diagnostic features of glioblastoma | necrosis (serpentine, pseudo-palisading)
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| endothelial cell prolif (VEGF, at least 2 layers, can be glomeruloid) |
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| what is gliomatosis cerebri? | multiple foci of infiltration by astrocytoma; aggressive (grade III)
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| low-grade astrocytoma genetics | p53 mut
PDGFa and PDGFRa overexpression
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| high grade astrocytoma genetics | RB
p16/CDKNaA
possibly chr19 TSG
MDM2
EGFR
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| what is secondary glioblastoma? | occurs in younger pts w h/o low grade astrocytoma; p53,PDGFa
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| why is there edema in astrocytoma? | leaky neovessels, espec in high grade
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| role of MGMT in astrocytoma therapy? | Methylation of the promoter for the gene encoding the DNA repair enzyme MGMT predicts responsiveness to DNA alkylating drugs
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| pilocytic astrocytoma age location grade | pediatric
cerebellum (also perventricular and optic n)
grade 1
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| pilocytic astrocytoma path | cystic, hairlike GFAP+ processes forming dense meshwork; often biphasic (microcystic and fibrillary), narrow infiltrative border (centripetal); vascular; Rosenthal bodies+, eosinophilic granular bodies+
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| pilocytic astrocytoma genetics | only rare p53 muts
if assoc'd w/NF1, has fxnl loss of neurofibromin (not in sporadic)
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| pleomorphic xanthoastrocytoma age location grade | pedi
temporal lobe (present w/seizure)
grade 2 usually
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| pleomorphic xanthoastrocytoma path | neoplastic +/-bizarre astrocytes
degree of nuclear atypia can be extreme, but abundant reticulin deposits, relative circumscription, and chronic inflam,no necrosis and no mitotic activity
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| Child, cerebellum, cystic mass Name & micro | Pilocystic Astrocytoma bipolar cells w/ GFAP+ “hairs” & Rosenthal fibers
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| BUZZ: GFAP+ “hairs” and Rosenthal fibers | Pilocystic Astrocytoma
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| BUZZ: Adult, GFAP + “feltwork” of astrocyte processes “fibrillary background” | Diffuse Astrocytoma
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| Adult, Densly cellular, nuclear pleomorphisms, mitotic bodies, | Anaplastic Astrocytoma
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| Adult, brightly eosinophilic cytoplasm w/ many “stout processes” | Gemistocytic Astrocytoma
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| Adult, densely cellular, w/ necrosis, and vascular proliferation, and pseudopalissading | Glioblastoma Multiforme
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| BUZZ: pseudopallisading | Glioblastoma
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| 20% of 0-20yo IC neoplasms | Brainstem Glioma
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| Most common Brainstem Glioma | Intrinsic Pontine
Adult, frontal lobe cystic mass w/ calcifications and seizures, name
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| Buzz: clear halo in cytoplasm | Oligodendroglioma
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| Adult, frontal lobe cystic mass w/ calcifications and seizures genetic cause | LOH 1q or 19q
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| Child, 4th ventricle mass name | ependyoma
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| Child, 4th ventricle mass genetic cause | chromosome 9
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| Adult or NF-T2 Pt, central spinal cord canal mass name | ependyoma
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| Adult or NF-T2 Pt, central spinal cord canal mass genetic cause | Chromosome 22 – NF2
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| BUZZ: 4th ventricle or spinal cord canal pseudorosettes name | ependyoma
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| Cauda equina, cuboidal cells w/ clear cytoplasm | Myxopapillary ependyoma
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| Adult w/ seizure, temporal lobe cystic mass | Ganglioglioma
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| Adult w/ seizure, temporal lobe cystic mass expected micro | irregular neurite cells in random order
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| Irregular neurite cells in random order name | ganglioglioma
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| Child, midline cerebellum, small cells in sheets, highly mitotic name | Meduloblastoma
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| Child, midline cerebellum, small cells in sheets, highly mitotic Genetic cause | loss of 17p or worse with MYC amplification
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| Seeded CSF with malignant cells in child, most likely tumor | meduloblastoma
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| Child, midline cerebellum, small cells in sheets, highly mitotic, w/ PALE ISLANDS name | Desmoplastic Meduloblastoma
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| Children, anywhere, large, soft, spreads across surface, vimentin + | Rhabdoid tumor
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| Most common primary CNS tumor in AIDS | B-cell lymphoma
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| Immunocomprimised Pt, multifocal primary lesions Cells are infected with what? | EBV
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| Immunocomprimised Pt, multifocal primary lesions Cells congregate around what? | vessels
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| BUZZ: BCL-6 marked tumor cells in brain | B-cell lymphoma
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| BUZZ: “hooping” of cells with silver staining reticulin fibers | primary B-cell lymphoma
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| Japanese male, midline pineal tumor name | Germ Cell Tumor
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| Japanese male, midline pineal tumor most likely primary or met? | met
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| Japanese male, midline pineal tumor. Protein Markers? | alpha-fetoprotein and B-HCG
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| BUZZ: Brain psammoma body | Meningioma
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| Meningioma age? | adults
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| Meningioma sex and ratio? | women 3:2
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| Whorled cluster of cells, psammoma bodies Gentic cause? | LOH 22long arm
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| Common tumors that met to the brain | lung, breast, skin (melanoma), kidney, GI
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| Mets favorite area | grey/white boarder
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| Purkinji cell destruction, inflammatory infiltrate, gliosis name? | Subacute cerebellar degeneration
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| Purkinji cell destruction, inflammatory infiltrate, gliosis cause? | paraneoplastic syndrome
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| Dementia, perivascular inflammatory cuffs, mostly Ant&med temporal lobe name | Limbic Encephalitis
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| Dementia, perivascular inflammatory cuffs, mostly Ant&med temporal lobes. Cause? | paraneoplastic syndrome
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| Child, seizure, temporal lobe mass, bizarre astrocytes, reticulin, chronic inflammatory cells, NO NECROSIS or mitosis | Pleomorphic Xanthoastrocytoma
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| Child, high nuclear atypia, no mitosis or necrosis Dx | Pleomorphic Xanthoastrocytoma
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| Tinnitis & hearing loss Dx | Schwannoma
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| Well circumscribed, encapsulated mass, attached to nerve, but removable. Genetic cause and lost product | NF2 gene on 22L product Merlin
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| BUZZ: Verocay body | Schwannoma
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| Tumor meshwork of cells, microcysts and myxoid stroma Name and Type | Schwannoma Antoni B
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| Schwannoma immunoreactive stain | S-100
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| Nodular Highly collegenized Spindle cells under hyperpigmented skin | Cutaneous NF
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| Plexiform NF likes where? | large nerve trunks
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| BUZZ: Shredded carrots what are they and Dx? | Collagen in myxoid stroma in Plexiform NF
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| Cowden Syndrome genetic mutation | PTEN
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| Li-Fraumeni Syndrome Genetic cause? | p53 mutation
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| Turcot syndrome genetic mutation | APC or mismatch repair
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| Gorlin syndrome genetic mutation | PTCH w/ sonic hedgehog up reg
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| Dysplastic ganglioglicytoma of the cerebellum caused by | Cowden Syndrome
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| Li-Fraumeni syndrome causes | medulloblastoma
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| Meduloblastoma or glioblastoma caused by | Turcot syndrome
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| Meduloblastoma caused by these syndromes | GTL Gorlin
Turcot
Li-Fraumeni
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| NF-Type 1 genetics | AD 17q11.2
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| NF-Type 1 associated tumors | plexiform and solitary NF, optic nerve glioma, pigmented nodules of the iris (Lisch nodules), café au lait spots
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| BUZZ: Café au lait spots & Lisch nodules | NF-1
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| Bilateral Acoustic Schwannomas, multiple meningiomas and diffuse hamartomas | NF-1
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| NF-2 genetics | AD 22q12 Merlin
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| Tuberous Sclerosis Complex genetics | AD 16q13.3 tuberin> 9q34 hamartin
Mental retardation, angiofibromas, seizures, skin shagreen or ash-leaf patches
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| VHL genetics | AD 3q25/26
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| VHL genetic pathology | normally down regulates VEG-F, erythropoietin etc
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| cerebellum, retina, brain hemangioblastomas, liver, pancreatic, kidney cysts | VHL
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