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CNS tumors

LECOM Robbins Ch 28 Path CNS tumors

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