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CNS tumors
neuro module 2
27
Medical
Professional
01/17/2010

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Term
clinical symptoms of CNS mass lesions
Definition
1. altered level of consciousness
2. focal deficits, depending on location
3. seizure
4. elevated ICP and CSF protein
5. headache
6. papilledema
Term
axial tumor
Definition
anywhere within the brain parenchyma
Term
extra-axial
Definition
outside the brain parenchyma
Term
metastatic carcinoma tumors
Definition
1. usually at the gray/white junction when axial
2. may be extra-axial (dural)
3. most common intracranial adult tumor
4. most commonly small cell carcinomas from epithelial tissue, i.e. lung, breast, kidney, GI, skin
5. sarcomas uncommon
6. predominance of location relates to blood flow (cerebrum>cerebellum>spinal cord); vertebral bodies more common than cord
Term
gross/radiology features of metastatic carcinoma
Definition
1. tumor emboli most commonly found at the grey/white junction - appears as hemorrhage
2. may be more than one lesion
3. marked edema surrounding tumor (more edema than primary cancers)
Term
Meningioma epidemiology
Definition
1. 20% of primary CNS tumors
2. 40-65 yrs old
3. female 2:1
4. some have progesterone or estrogen receptors, can accelerate growth during pregnancy
Term
clinical features of meningioma
Definition
1. benign, slow progression but can be locally aggressive and invade bone
2. clinical symptoms depend on location
3. may be found on external surface of brain, ventricular symptom, or on bony ridges where the dura is thick and prominent
4. prognosis is good with 25% recurrence b/c of incomplete excision
Term
pathological features of meningioma
Definition
1. well circumscribed
2. may be focally mineralized
3. arachnoid-like cells with vacuolated nucleus
4. syncytium - sheets of cells without distinct borders
5. spindle-shaped cells - elongated cells with abundant cytoplasm
6. cellular whorls of arachnoid cells
7. psammoma bodies: concentric laminated calcifications
Term
schwannoma epidemiology
Definition
1. 10% of primary tumors
2. 35-60 years - younger age and multiple if part of neurofibromatosis complex
3. cranial nerves (VIII), sensory spinal nerves, peripheral nerves involved
4. favorable prognosis - benign and slow growing
Term
gross findings in schwannoma
Definition
1. nerve focally enlarged and rubbery
2. tumor grows by expansion and is encased within the nerve - displaces nerve
3. usually can be shelled out sparing remaining nerve
Term
pathologic features of schwannoma
Definition
1. spindle-cell proliferation with wavy appearance
2. Antoni A: cellular with palisading nuclei, very pink
3. Antoni B: mixed with loose, less cellular areas, bluish
4. Verocay body: palisading nuclei in parallel rows with intervening anuclear zone
Term
classification of circumscribed astrocytoma
Definition
1. relatively well-circumscribed or cystic
2. usually low grade (I)
3. low tendency for anaplastic progression
4. favorable prognosis
Term
classification of diffuse astrocytoma
Definition
1. infiltrative margin
2. 3 histologic grades (II-IV)
3. tendency to progress to more anaplastic forms
4. unfavorable prognosis
Term
epidemiology of circumscribed astrocytoma
Definition
1. 10% of primary CNS tumors
2. childhood tumor
3. location in midline, especially the cerebellum or optic nerve
Term
Juvenile Pilocytic Astrocytoma: gross features
Definition
1. well-demarcated, effacing adjacent structures
2. grey-white, rubbery to gelatinous
3. may be cystic, especially when in cerebellum
4. encapsulated congealed proteinacious content
Term
Juvenile Pilocytic Astrocytoma: pathologic features
Definition
1. elongated spindle-shaped astrocytes (piloid)
2. biphasic pattern of hyper and hypodence areas with microcysts
3. condensed pink areas surrounding blood vessels
Term
rosenthal fibers
Definition
pink beaded arrangement of astrocyte glial filaments common in circumscribed astrocytomas
Term
pathological features of Glioblastoma Multiforme (GBM) grade IV
Definition
1. stains positively for the presence of the intermediate cytoskeletal filaments, GFAP (glial fibrillary acidic protein)
2. high cellularity
3. pleomorphism
4. hyperplastic vascularity
5. mitoses and necrosis
Term
clinical behavior of GBM
Definition
-poor prognosis, usually 6-12 months
-transformation of low grade tumor to high grade can be traced by cumulative gene abnormalities including loss of function p53 mutations and LOH of chromosome 10
-treatment is surgical debulking, radiation, and chemo (poor response)
Term
epidemiology of oligodendroglioma
Definition
1. 5% of CNS tumors
2. 25-50 years
3. affects cerebral hemispheres
4. 40% have 5 year survival; progressive course over several years
Term
gross features of oligodendroglioma
Definition
poor circumscription, infiltrative, hemorrhage, focal calcification
Term
pathologic features of oligodendroglioma
Definition
1. uniform, cellular pattern of oligodendrocytes with "fried egg" perinuclear halo appearance and calcifications
2. chicken-wire vascular pattern can be identified with factor VIII antibody against endothelial cells
3. if halos present, negative for GFAP
Term
Ependymoma epidemiology
Definition
1. 5% of CNS tumors
2. 5-25 (brain) 20-50 (spinal cord)
3. present with symptoms of CSF obstruction
4. occur in 4th ventricle or distal spinal cord
5. 40-60% 5 year survival
Term
gross features of ependymoma
Definition
intraventricular, granular, friable, seeds CSF pathways
Term
pathologic features of ependymoma
Definition
1. vascular tumor with uniform sheets of cells with small dark round nuclei in a glial fibrillary background
2. perivascular pseudorosettes - nuclei clustered around blood vessel with adjacent anuclear zone
Term
medulloblastoma epidemiology
Definition
1. 5% CNS tumors
2. 5-20 yrs
3. rapid progession of cerebellar symptoms
4. 75% 5 yr survival, CSF seeding common
Term
pathologic features of medulloblastoma
Definition
1. small, round blue cell tumor
2. primitive neuroectodermal cell precursors of glia and neurons (PNET)
3. Homer Wright rosettes
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