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Neuro > CNS Tumors > Flashcards

CNS Tumors Flashcards

1
Q

Where do most adult CNS tumors occur?

A

in cerebral hemispheres, most freq in frontal lobes

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2
Q

Where do most childhood CNS tumors occur?

A

posterior fossa

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3
Q

List some key general features of astrocytomas

A

most common glial tumor
80% are glioblastomas
present with seiuzres, focal neuro deficits (gradual), headaches
diffuse tend to become anaplastic over time

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4
Q

What are the various grades/names for astrocytomas?

A

grade 1-pilocytic astrocytoma
grade 2- diffuse astrocytoma
grade 3-anaplastic astrocytoma
grade 4-glioblastoma multiforme

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5
Q

Describe diffuse astrocytoma histology

A

cellularity moderately increased and occasional nuclear atypia

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6
Q

Describe anaplastic astrocytoma histology

A

increased cellularity distinct nuclear atypia, marked mitotic activity

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7
Q

Describe glioblastoma multiforme histology

A

pleomorphic astrocytic cells, brisk mitotic activity, prominent microvascular proliferation
has necrosis with pseudopalisading

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8
Q

Describe pilocytic astrocytoma histology

A

biphasic pattern: densely fibrillary (pilocytic) areas alternating with microcystic component
rosenthal fibers

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9
Q

What are key clinical features of pilocytic astrocytoma?

A

cerebellum, also optic nerve, 3rd ventricle, hypothalamus, brainstem and occasionally cerebral hemispheres
1st 2 decades
presents with focal neurologic deficits, seizures, or S/S of increased intracranial pressure

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10
Q

What does pilocytic astrocytoma look like on imaging?

A

well demarcated, often cystic contrast-enhancing tumor

mural nodule

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11
Q

What is a significant mutation realted to oligodendromglioma?

A

loss of 1p and 19q are predictors of prolonged survival and susceptibility to chemotherapy in anaplastic oligodendrogliomas

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12
Q

What are some key clinical features of oligodendroglioma?

A

5th to 6th decade
long history of progressive neuro sx (seizures, headache, focal signs)
better survival than astrocytomas (5-10 yrs for grade II)

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13
Q

What does an oligodendroglioma look like on imaging?

A

well defined hypodense/hypointense mass, may see calcification

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14
Q

What does oligodendroglioma histology look like?

A

round nuclei, freid egg cell and calcifications

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15
Q

What are key features of ependymomas?

A

children and young adults
occurs along ventricular system, usually posterior fossa (4th ventricle)
hydrocephalus, occasonially seizures
avg survival 4 yrs
may invade brain parenchyma
may disseminate through subarachnoid space

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16
Q

What does ependymoma look like on imaging?

A

well circumscribed mass

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17
Q

What is seen on histology for ependymoma?

A

true rosettes-columnar cells arranged around central lumen
perivascular pseudorosettes
anaplastic ependymoma-increased mitoses & vascular proliferation

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18
Q

What are key features of choroid plexus papillomas?

A

1st 2 decades
4th ventricle, lateral ventricle, 3rd ventricle, cerebello-pontine angle
presents with hydrocephalus
can progress to choroid plexus carcinoma (usually in children)
good prog unless progresses to carcinoma

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19
Q

How can you distinguish a choroid plexus papilloma from a choroid plexus carcinoma/

A

papillomas do not invade adjacent parenchyma but carcinomas do

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20
Q

What are the key features of colloid cysts?

A

usually attached to roof of 3rd ventricle
intermittent obstruction of foramen of monro (positional)
positional headache, thin walled cyst lined by cuboid/columnar epithelium

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21
Q

What are the eky features of ganglioglioma?

A

first 3 decades
long standing history of seizures is common
usually supretentorial and in temporal lobe
surgery usually curative

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22
Q

What does a ganglioglioma look like on imaging?

A

solid or cycstic well circumcribed mass, mural nodule, calcification

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23
Q

What does histology of a ganglioglioma look like?

A

atypical ganglion cells (neurons) and neoplastic glial component

24
Q

What are the key clinical features of medulloblastoma?

A

primitive neuroectodermal neoplasm of posterior fossa
first decade
cerebellar dysfunction
incrased intracranial pressure
solid, well defined homogenous mass, tendency to spread through subarachnoid space and for drop metastases

25
Q

What does a medulloblastoma look like on imaging?

A

well defined, contrast enhancing mass, may see leptomeningeal spread

26
Q

What does medulloblastoma look like on histology?

A

homer wright rosettes
synaptophysin immunoreactivity
highly cellular and composed of undifferentiated cells

27
Q

What are the key features of primary CNS lymphomas?

A 
40-60 yo typically
immunocompirmised more common, EBV assoc
non specific symptoms, referable to mass lesion
usually Bcell
poor prognosis
usually supratentorial
28
Q

What are key features of meningiomas?

A

most common extraparenchymal neoplasm of CNS
middle to late adult life
usually women
sx due to enlarging mass

29
Q

How do meningiomas look on imaging?

A

dural based, vascular, contrast enhancing, well defined

30
Q

What are 2 associations with meningiomas?

A

radiation tx

NF2

31
Q

Describe the 3 grades of meningiomas

A

1(typical): excellent 5 yr survival
2 (atypical): high mitotic index or architectural loss, hypercellularity, macronucleoli, small cell differentiation or brain invasion
3 (anaplastic): excessive mitotic activity or loss of meningothelial differentiation

32
Q

What does meningioma histology look like?

A

sheets of tumor cells with indistinct borders
whorls
round to oval nuclei, dispersed chromatin, wispy eosinophilic cytoplasm
psammomma bodies
necrosis, brain invasion

33
Q

What are common clinical signs/sx of metastatic turmos to the CNS?

A

headaches
focal neurologic signs
altered mental status

34
Q

How mets to the CNS usually look on imaging?

A

distinct, contrast enhancing mass with surrounding edema, usually multiple

35
Q

Where to mets to the CNS usually come from?

A

lung or breast

sometimes kidney, GI, skin (malignant melanoma)

36
Q

Hwat are key features of peripheral nerve sheath tumors (schwannomas)?

A 
benign tumor composed of schwann cells
4th to 6th decades
peripheral nerves, usaully in head and neck (ie vestibulocochlear)
spinal tumors-radicular pain
hearing loss, facial numbness
well definted contrast enhancing mass
associated w/NF2
37
Q

What does schwannoma histology look like?

A

antoni A: compact spindle cells

antoni B: loose spongy areas, small cells with round nuclei

38
Q

What is a neurofibroma?

A

benign tumor of schwann cells, fibroblasts and perineural cells
associated with NF1
cutaneous neorfibroma or peripheral nerve forms

39
Q

What does neurofibroma histology look like?

A

hypocellular, elongated spindle cells with wavy nuclei

diffusely infiltrate adjacent nerve and soft tissue

40
Q

What are key features of plexiform neurofibroma?

A

almost exclusively in NF1
transformation of mutiple fascicles of nerves into neurofibroma with preservation of anatomic configuration
typically affects larger nerves or a plexus
high likelihood of malignant transformation

41
Q

What are key features of malignant peripheral nerve sheath tumors?

A 
usually in extremities
in CNS assoc w/CN V
strong assoc w NF1
high grade, agressive
infiltrative non-encapsulated fleshy masses
42
Q

What does malignant peripheral nerve sheath tumor histology look like?

A

highly cellular, moderate to marked nuclear pleomorphism

high mitotic rate

43
Q

What are key features of neurofibromatosis type 1? (von Recklinghausen disease)

A

AD
1/3000
neurfibromas, cafe au lai spots, lisch nodules (pigmented hamartomas in iris), optic glioma, osseous lesions, axillary freckling, family hx

44
Q

Where is the neurofibromatosis 1 gene found?

A

chromosome 17

gene product: neurofibromin

45
Q

What are key features of NF2?

A 
AD
1/40,000-50,000
gene on chromosome 22
gene product is merlin
bilateral vestibular schwannomas, fam hx, meningiomas, schwannomas, gliomas, neurofibromas, lens opacity, cerebral calcifications
46
Q

What chromosome is the VHL gene found on?

A

3

47
Q

What are key features of von hippl lindau disease?

A

hemangioblastomas of CNS and retina
renal cell carcinoma
pheochromocytoma
visceral cysts

48
Q

What do hemangioblastomas look like on imaging?

A

well-defined, contrast enhancing cystic mass with mural nodule

49
Q

What do hemangioblastomas look like on histology?

A

numerous vessels interspersed wit hstromas cells

stromal cells have abundant foamy cytoplasm (contains fat)

50
Q

What are key features fo tuberous sclerosis?

A

AD
casued by mutations in TSC1 (chr 9)-hamartin &
TSC2 (chr 16)-tuberin
cortical hamartomas
subcortical glioneuronal hamrtomas
subependymal giant cell astrocytomas
cutaneous angiofibromas, subungual fibromas, cardiac rhabdomyomas, intestinal polyps, etc

51
Q

What do tubers look like on histology?

A

neuronas haphazardly arranged in cortex

often have glial as well as neuronal features

52
Q

What are key features of subependymal giant cell astrocytoma?

A

cause obstructive hydrocephalus
exophytic solid well-defined mass arising in the wall of lateral ventricle
large pleomrophic multinucleated tumor cells w/eosinophilic cytoplasm
may be of astrocytic or glioneuronal origin
no malignant transformation, local invasion reported

53
Q

What malignancies most commonly cause paraneoplastic syndromes?

A 
small cell carcinoma
gynecologic malignancies
hodgkin's and non-hodgkins lymphoma
testicular cancer
neuroblastoma
54
Q

What are the 2 main categories or paraneoplastic syndromes?

A
  1. related to ectopic hormone production

2. neurologic syndromes (rare)

55
Q

Describe subacute cerebellar ataxia

A

a paraneoplastic neurologic syndrome
progressive ataxia, dysarthria, nystagmus, vertigo, diplopia, titubation
associated w/ ovarian and breast cancer
ab to purkinje cells

56
Q

Describe Lambert Eaton Myasthenic syndrome

A

a paraneoplastic neurologic syndrome
muscle weakness, esp in the legs that improves with testing on exam (extraocular muscle are spared)
Ab to P/Q type voltage gated calcium channels, leads to decreased ACh release
most commonly assoc w/SCLC

Neuro (14 decks)

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