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92 Cards in this Set
- Front
- Back
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list gliomas
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astrocytomas
oligodendrogliomas ependymomas all usually at least partially positive for GFAP |
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3 grades of fibrillary astrocytoma
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Grade I: low grade fibrillary astrocytoma
Grade II: anaplastic astrocytoma Grade III: glioblastoma mutliforme |
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fibrillary astrocytomas can occur in children or adults but more common in
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adults
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sx of fibrillary astrocytoma
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HA
seizures focal neurologic signs depending on location of tumor |
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in children most are
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infratentorial
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adults most are
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supratentorial
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low grade lesions are:
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infiltrative
destructive but indolent mean survival > 5 yrs tendencey to evolve in higher grade over time |
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GBM is very aggressive despite all forms of therapy. death in
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8-10 months
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gliomatosis cerebri affects
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multiple regions or entire brain affected
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glioma histological marker
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glial antigen glial acid fibrillary protein (GFAP)
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grade I
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low grade fibrillary astrocytoma
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grade II
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anaplastic astrocytoma
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grade III
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glioblastoma mutliforme
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juvenile pilocytic astrocytoma can occur anywhere in CNS but most commonly in
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cerebellum
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JPA contain
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rosenthal fibers -indicated slow growing lesion
bipolar cells with long GFAP-positive processes |
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GBM is very aggressive and has characteristic/morphology
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butterfly lesions
ring enhancing more cellular mitotically active than anaplastic astrocytoma NECROSIS (defining feature) sometimes w/ peripheral pseudopalisading and vascular (endothelial) proliferation |
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Grade II anaplastic astrocytoma
morphology |
more cellular and more atypical than low-grade lesions
mitotic figures are present brightly eosinophilic cells called gemistocytic astrocytoma usually qualifies as anaplastic astrocytoma |
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JPA is more common in
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children
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JPA is usually benign or malignant?
where can they occur? contain what? |
benign
CNS, most commonly cerebellum cystic with mural nodule |
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morphology of PXA
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contain atypical astrocytes and foamy cells
Rosenthal fibers esoinophilic granular bodies (smaller red extracellular globules, probably early Rosenthal fibers) |
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morphology of PXA
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contain atypical astrocytes and foamy cells
Rosenthal fibers esoinophilic granular bodies (smaller red extracellular globules, probably early Rosenthal fibers) |
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PXA is almost always found in
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superficial temporal lobe in children to young adults with history of seizures
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PXA is almost always found in
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superficial temporal lobe in children to young adults with history of seizures
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Morphology of Subependymal giant astrocytoma (SEGA)
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large epitheloid astrocytes with mitotic activity or necrosis
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Morphology of Subependymal giant astrocytoma (SEGA)
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large epitheloid astrocytes with mitotic activity or necrosis
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SEGA has a very strong association with
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tuberous sclerosis
tubers (cerebral hamartomas) probably evolve into SEGA |
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SEGA has a very strong association with
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tuberous sclerosis
tubers (cerebral hamartomas) probably evolve into SEGA |
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SEGA can be found in
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any subependymal location
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SEGA can be found in
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any subependymal location
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what tumor has butterfly lesion?
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GBM
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What is usually benign, more common in children, rarely can transform into more aggressive
most common in cerebellum cystic with mural nodule have bipolar cells with long GFAP-positive processes, increased number of vessels, Rosenthal fibers (large red EC cigar-shaped bodies) |
JPA
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what is almost always superficial temporal lobe in children to young adults with history of seizures
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Pleomorphic xanthoastrocytoma
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Pleomorphic xanthoastrocytom has what types of cells?
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very atypical astrocytes
foamy cells Rosenthal fibers eosinophilic granular bodies |
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what has very strong association with tuberous sclerosis?
has large epitheloid astrocytes with mitotic activity or necrosis |
subependymal giant cell astrocytoma (SEGA)
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Tubers (cerebral hamartomas) probably evolve into
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SEGA
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oligodendrolgiomas are most common at what ages?
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4th-5th decade
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oligodendrogliomas often have a history of
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seizures
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oligodendroglioma is most common in
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white matter of cerebral hemispheres
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oligodendrogliomas are more ______ than astrocytomas especially if positive for _____________
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chemosensitive
deletions on chr 1p and 19q |
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what is the average survival in years of oligodendroglioma?
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5-10 yrs
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oligodendogliomas are well-circumscribed lesions with
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hemorrhage and calcifications
sheets of cells with uniform found-oval nuclei, perinuclear halos, fine capillary network, calcifications usually at least partially GFAP-positive |
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ependymoma arise in _____locations
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periventricular
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in children, ependymoma most commonly in _____
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4th ventricle
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in adults, ependymoma found in
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SC
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ependymoma gives rise to _____
mean survival? |
hydrocephalus, disseminate into CSF
mean survival of 4 yrs |
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what has well-circumscribed lesions
fairly inform cells with round-ovel nuclei and fibrillary background perivascular pseudorosettes |
ependymoma
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what arises at filum terminale
cuboidal ependymal-like cells arranged around papillary structures within myxoid background |
myxopapillary ependymoma
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solid periventricular tumors that protrude into ventricles
benign asymptomatic unless cause obstruction (hydrocephalus) |
subependymoma
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most common in kids (usually lateral ventricles)
occurs in 4th ventricle in adults exaggerated normal choroid plexus |
choroid plexus papilloma
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cystic lesion containing gelatinous material that hangs from roof of 3rd ventricle
most common in young adults hydrocephalus presents as positional headache |
colloid cyst of 3rd ventricle
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what are the two mechanisms for hydrocephalus in choroid plexus papilloma?
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1. overproduction of CSF
2. obstruction of ventricular flow |
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what is the marker for schwann cells
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S-100
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what is the marker for neuronal tumors?
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synaptophysin
neurofilaments |
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primitive neuroectodermal tumors
cerebellum--> cerebral cortex--> pineal --> adrenal medulla --> retina --> elsewhere --> |
medulloblastoma
central neuroblastoma pineoblastoma neuroblastoma retinablastoma PNET (primitive neuroectodermal tumor) |
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morphological features of medulloblastoma
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very cellular with little cytoplasm
hyperchromatic nuclei high mitotic rate Homer Wright rosettes |
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medulloblastoma is usually found in ____exclusively in what part of the brain
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children
cerebellum |
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medulloblastoma is associated with loss of material from
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Chr 17q
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CNS lymphoma is most common in
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immunosuppressed pts
and old people, 60 or p;der |
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Bcell marker
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CD20
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what tumor is more common in females?
F:M = 2:1 1-:1 in spinal _____ |
meningioma
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meningiomas arise from
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meningothelial cells of arachnoid
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meningiomas express what receptors and can grow rapidly in pregnancy
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progesterone
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with meningiomas, increased risk of what?
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Neurofibromatosis type II
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what has rounded dural-based lesions that compress underlying brain but easily separated from it?
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meningiomas
can present meningeal plaques can invade overlying bone |
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what is positive for EMA?
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meningiomas
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where are CNS germ cell tumors usually found?
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midline
pineal or suprasellar area |
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CNS germ cell tumors are most common in
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adolescents and young adults
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what is positive for EMA?
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meningiomas
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5 most likely to metastasize to the CNS
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1. lung
2. breast 3. melanoma 4. kidney 5. GI tract except for melanoma, all carcinomas usually sharply circumscribed often at gray-white junction |
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where are CNS germ cell tumors usually found?
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midline
pineal or suprasellar area |
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schwannomas are associated with with what other tumors?
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NF type II
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CNS germ cell tumors are most common in
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adolescents and young adults
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if extradural schwannoma, arise from
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large peripheral nerve trunks
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5 most likely to metastasize to the CNS
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1. lung
2. breast 3. melanoma 4. kidney 5. GI tract except for melanoma, all carcinomas usually sharply circumscribed often at gray-white junction |
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if intracranial schwannoma, most commonly arise at
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cerebellopontine angle: attached to CN8
presented with tinnitus, hearing loss, acoustic neuroma |
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schwannomas are associated with with what other tumors?
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NF type II
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schwannoma are a mixture of 2 growth patterns
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cellular Antoni A areas with palisading nuclei Verocay bodies
Antoni B areas of less dense cellularity in myxoid stroma |
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if extradural schwannoma, arise from
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large peripheral nerve trunks
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Name the neurocutaneous syndrome: phalomatoses
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NF types I and II
tuberous sclerosis Von Hippel-Lindau syndrome |
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if intracranial schwannoma, most commonly arise at
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cerebellopontine angle: attached to CN8
presented with tinnitus, hearing loss, acoustic neuroma |
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NF type I is due to what mutation?
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tumor suppressor gene NF1 on ch 17 which codes for neurofibromin
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schwannoma are a mixture of 2 growth patterns
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cellular Antoni A areas with palisading nuclei Verocay bodies
Antoni B areas of less dense cellularity in myxoid stroma |
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Name the neurocutaneous syndrome: phalomatoses
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NF types I and II
tuberous sclerosis Von Hippel-Lindau syndrome |
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NF type I is due to what mutation?
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tumor suppressor gene NF1 on ch 17 which codes for neurofibromin
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NF2 gene mutation is
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on chr 22 for merlin, a cytoskeletal protein
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what tumor?
cortical hamartomas tubers --> SEGA renal angiomyolipoma various organ cysts subungual fibroma skin lesions angriofibromas leathery thickening "shagreen patch" hypopigmented areas ash-lead patches |
tuberous sclerosis
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what the firm areas with haphazardly arranged neurons and large eosinophilic cells which express both neuronal and glial antigens
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tubers
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subependymal lesions that buldge into ventricles
"candle guttering" |
tuberous sclerosis
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what is hemangioblastoma of cerebellum, retina, various organ cysts
great risk of renal cell carcinoma |
Von-Hippel-Lindau
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Von-Hippel Lindau has mutation of
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pVHL inhibits elongation phase of RNA synthesis
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what has multiple plexiform and cutaneous neurofibromas, optic nerve gliomas, pigmented nodules of iris
Lisch nodules hyperpigmented macules called cafe au lait spots |
NF type I
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what has bilateral acoustic neuroma and multiple meningiomas
sometimes glial hamartomas |
NF type II
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