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