CNS tumors- Norton Flashcards

1
Q

where do most brain tumors occur in children

A

posterior fossa

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2
Q

where do most brain tumors occur in adults

A

supratentorial

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3
Q

what can be found on the PE for a primary brain tumor

A
fundoscopic exam: papilledema 
CN3 and 6 nerve palsy 
increase ICP 
headache
hemiparesis 
aphasia 
seizures
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4
Q

tumors in the ventricles present with what clinical features

A

hydrocephalus

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5
Q

what tool is used to diagnose brain tumor

A

MRI with contrast

BBB does not allow contrast but the new vessels in tumor do

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6
Q

what is the only risk factor for brain tumors

A

ionizing radiation

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7
Q

what are the glioma tumors impact what cells and create their own tumors

A

astrocytes
oligodendrocytes
Ependymal cells

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8
Q

what are the two types of Astocytoma

A

infiltrating

noninfiltrating

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9
Q

what is the most common genetic mutation for astrocytoma infiltrating

A

p53

over expression of platelet derived growth factor

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10
Q

what is the histologic grading for astrocytomas I -IV

A

I: well-differentiated- pilocytic astrocytoma

II: diffuse growth of well-differentiated astrocytes-diffuse or fibrillary astrocytoma

III: anaplastic features (pleomorphism, increased mitosis) - anaplastic astrocytoma

IV: undifferentiated w/necrosis - gliobastoma

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11
Q

name a type of astrocytoma infiltrating that occurs in younger adults

A

diffuse astrocytoma

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12
Q

what is the histology for diffuse astrocytoma

A
  • increased number of glial cells
  • variable nuclear pleomorphism
  • GFAP-positive astrocytic processes give a fibrillary background
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13
Q

how long do patients survive with diffuse astrocytoma

A

5 years

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14
Q

Name a astrocytoma infiltrating tumor that has regions of more densely cellular with greater nuclear pleomorphism, mitotic figures

A

anaplastic astrocytoma

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15
Q

what tool is used to diagnose diffuse and anaplastic astrocytoma infiltrating

A

MRI

brain biopsy

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16
Q

treatment options for diffuse and anaplastic astrocytoma infiltrating

A

surgical resection

radiation

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17
Q

survival rate for diffuse and anaplastic astrocytoma infiltrating

A

2 years

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18
Q

name the astrocytoma infiltrating tumor that is high grade end of spectrum of astrocytomas, most atypical and mitotically active

A

gliobastoma

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19
Q

what is the treatment for gliobastoma

A

surgery
radiation
chemotherapy

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20
Q

glioblastoma usually occurs in who and what is the prongosis

A

5th - 7th decade (40s-60s)

always fatal

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21
Q

what does the histology for glioblastoma look like

A

densely cellular, vascular proliferation, necrosis and pseudopalisading (ring shape)

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22
Q

Name a non-infiltrating astrocytoma

A

pilocytic

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23
Q

who usually gets pilocytic astrocytomas and what is the prognosis

A

children and young adults

lived for 40 years

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24
Q

where does pilocytic astrocytoma usually occur in the brain? what grows

A

cerebellum
third ventricle
cystic with mural nodule in wall of cyst

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25
Q

what does pilocytic astrocytoma look like under the microscope

A

cells with hair-like processes

Rosenthal eosinophilic fibers

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26
Q

Name a type of tumore that occurs in the 30s - 40s that is slow growing, may be diagnostic after years of neurologic complaints, and often seen in cerebral hemisphere

A

Oligodendroglioma

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27
Q

what does oligodendroglioma look like under the microscope

A

sheets of regular cells with spherical nuclei

surrounded by clear halo of cytoplasm

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28
Q

what is the genetics of oligodendroglioma

A

deletions of chromosome 1p and 19q

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29
Q

what is the treatment and survival of oligodendroglioma

A

surgery radiation and chemotherapy

5-10 years

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30
Q

Ependymoma usually occurs in who and where in the brain for adults and young people

A

children and young adults
ependymal-lined ventricles
children/young adults: 4th ventricle
adults: spinal cour

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31
Q

ependymoma presents with what?

A

hydrocephalus

32
Q

what is seen under the microscope for ependymoma cells

A

small dark cells with perivascular pseudorosettes

benign appearing

33
Q

what is the molecular genetics for ependymoma tumors with spinal cord lesions and supratentoral ependymomas

A

spinal cord: mutation in NF2 gene on chromosome 22

supratentorial: alterations in chromosome 9

34
Q

prognosis for ependymoma

A

5 years after surgery

better for supratentorial and spinal then posterior fossa

35
Q

what is myxopapillary pendymoma

A

in filum terminale
may extend into subarachnoid space
surround roots fo cauda equina

36
Q

Choroid plexus papillomas is most common in who and location of tumur in adults and childres

A

common children: lateral ventricle

adults: 4th ventricle

37
Q

what does choroid plexus papillomas look like under microscope

A

normal choroid plexus

38
Q

how do people with choroid plexus papillomas present

A

hydrocephalus

39
Q

who usually gets colloid cyst of 3rd ventricle? location? what can this obstruct? what can it cause? prognosis ? clinical complaint

A
children
roof of ventricle
foramina of monro
hydrocephalus 
fatal
complain of positional headache
40
Q

What is the histology of colloid cyst of 3rd ventricle

A

spherical mass filled with mucinous material

-simple epithelial lining may contain ciliated or goblet cells

41
Q

Name two embryonal tumors

A

medulloblastoma

CNS PNET

42
Q

where does medulloblastoma occur in the brain and who usually gets it

A

cerebellum only, midline

children

43
Q

what does medulloblastoma look like under the microscope

A

small round blue cell tumor

rosettes or perivascular pseudorosettes

44
Q

what is the molecular genetics for medulloblastoma

A

loss from 17p

45
Q

medulloblastoma is sensitive to what and prognosis

A

radiosenstive

5 yr

46
Q

what brain tumor occurs in children in the cerebral hemisphere. resembles medulloblastoma in histology and genetically distinct from medulloblastoma

A

CNS supratentroial primitive neuroectodermal tumors CNS PNET

47
Q

What is the most common CNS neoplasm in immunosuppressed patients

A

primary brain lymphoma

48
Q

do primary brain lymphomas spread

A

not to lymph nodes or outside of brain

49
Q

most common sites for metastic tumors

A
Lung 
breast
skin 
kidney
GI 
lots of bad stuff kills gila
50
Q

this is actually not a true brain tumor, arises from meningothelial cells from external membranes covering the brain

A

meningioma

51
Q

how can the patient present in the clinical with Meningioma

A

headache or neurological symptoms

not usually found until autopsy

52
Q

multiple meningiomas are found in patients with what

A

neurofibromatosis type 2

53
Q

what is seen on an MRI for meningiomas

A

“dural tail”

54
Q

what does meningioma look like under the microscope

A

whorls of bland cells

psammoma bodies

55
Q

where does Craniopharyngioma occur

A

suprasellar asrising in pituitary stalk
arise from remnants of Rathke’s pouch
sold or mixed solid and cystic

56
Q

what is the age distribution for carniopharyngioma

A

5-14 yrs

50-75 yrs

57
Q

what is the histologic feature of craniopharyngioma in children

A

adamantinomatous
- stratified squamous epithelium in nests or chords
spongy reticulum
calcifications
lamellar keratin formation
cysts with thick brownish-yellow fluid content

58
Q

what is the histologic feature of craniopharyngioma in adults

A

squamous epithelium lined solid sheets and papillae

no keratin, calcifications or cysts

59
Q

what are clinical symptoms for craniopharyngioma

A

visual from pressure on optic chiasm
endocrine symptoms from compressed pituitary
headache

60
Q

what is the treatment for craniopharyngioma

A

surgery

radiation therapy

61
Q

what is commonly seen at cerebellopontine angle where attached vestibular branch of 8th nerve - also called acoustic neuroma

A

schwannoma

62
Q

how do patients with schwannoma present in the clinic

A

tinnitus and hearing loss

63
Q

Schwannoma is associated with what

A

neurofibromastosis type 2

64
Q

Schwannoma shows a mixture of what 2 growth patterns

A

Antoni A: moderate to high cellularity with nuclear palisading ; “ nuclear-free zones” called Verocay bodies

Antoni B: less cellular area, more myxoid

65
Q

Neurofibromas are benign spindle cell lesions which occur in what 2 forms

A

cutaneous or solitary

plexiform

66
Q

cutaneous or solitary neurofibroma occurs where

A

skin or peripheral nerve

never turns malignant

67
Q

plexiform neurofibromas occurs in what type of patients

A

with NF

potential malignant transformation

68
Q

what are clinical features of neurofibromastosis 1

A

gliomas of optic nerve
pigmented nodules of iris ( Lisch nodules)
Cafe au lait spots: brown spots on skin

69
Q

neurofibromastosis 1 is associated with what gene

A

NF1 gene tumor suppressor gene

chromosome 17

70
Q

which neurofibromastosis develop a range of tumors

A

neurofibromastosis 2

71
Q

neurofibromastosis 2 what chromosome association

A

chromosome 22

72
Q

what tumor has CNS hamartomas- occur as haphazardly arranged neurons which lack normal neural oragnziation “cortical tubers”

A

Tuberous sclerosis

73
Q

where do extracranial lesions occur for tuberous sclerosis

A

renal angiomyolipomas
pulmonary and cardiac myomas
skin rash

74
Q

Von-Hippel-Lindau develop what

A

capillary hemangioblastomas in cerebellum, retina, and spinal cord
- highly vascular tumor

75
Q

von-Hippel_lindau can also have cysts where else

A

pancreas liver and kidney