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Clinical Neuroscience > Path- 2: Tumors > Flashcards

Path- 2: Tumors Flashcards

1
Q

50-75% of all tumors are either primary or secondary?

A

Primary

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2
Q

What are the 3 gliomas?

A

Astrocytomas, oligodendrogliomas, ependymomas

the 3 glia of the CNS lol

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3
Q

What comprises ~80% of adult primary tumors?

A

Fibrillary astrocytoma

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4
Q

Where are the fibrillary astrocytomas located in the CNS?

A

Cerebal hemispheres

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5
Q

When do fibrillary astrocytomas occur during oens life?

A

3rd or 4th decade of life

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6
Q

What are the clinical Sx of fibrillary astrocytomas?

A

szrs, headaches, no focal neurological deficits

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7
Q

True or False: Fibrillary astrocytomas have focal dense cellularity, proliferation of vascular elements, mitotic activity, and zones of necrosis.

A

FALSE

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8
Q

What is the life spans of pt’s with Glioblastomas following Dx?

A

8-10 months.

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9
Q

Does this occur in younger or older pt’s with a diagnosed glioblastoma: short, rapidly progressive, arising without preexisting low grade tumors.

A

Older

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10
Q

Does this occur in younger or older pt’s with a diagnosed glioblastoma: prveiously diagnosed low grade astrocytoma with p53 mutations.

A

Younger

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11
Q

What is the hallmark of GBM’s (grade IV glioblastomas)?

A

Necrosis

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12
Q

What age groups does pilocytic astrocytomas effect?

A

young adults and kids

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13
Q

Are pilocytic astrocytomas benign or malignant?

A

Relatively benign

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14
Q

Where in the brain are pilocytic astrocytomas?

A

CEREBELLUM

Sometimes can be in the floor of the 3rd vent and press on the ant optic pathway

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15
Q

What types of tests can you run to Dx a pilocytic astrocytoma?

A

B-crytallin and HSP’s, immunoreactive for alpha-1-antitrypsin

comes up as a bright-white cyst.

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16
Q

Where in the brain do Pleomorphic Xanthoastrocytoma’s occur?

A

Superficial temporal sides of the hemispheres

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17
Q

Pt’s have what main Sx with PX’s?

A

Seizures

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18
Q

Whgat age group does PX’x occur?

A

Kids and young adults

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19
Q

What is the composition of the tumors in PX’s?

A

mix of pleomorphic cells, raning from fibrillary to giant multinucleated cells with intracellilar lipid vacuoles (xanthomas)

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20
Q

What is the prognosis of PX’s?

A

may progress to grade III or Grade (IV)

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21
Q

What pt’s have brainstem gliomas?

A

first 2 decades of life

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22
Q

What are intial pontine gliomas?

A

most common pediatric, aggressive, short survival

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23
Q

What are exophylic tumors?

A

Happen at the cervicomedullary jxn, less aggressive than initial pontine gliomas

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24
Q

Are tectal gliomas more or less benign that exophylic tumors?

A

More benign

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25
Q

In adults, what types of brainstem gliomas can occur?

A

Rare intrinsic pontine gliomas

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26
Q

Define: 5-15% of gliomas, happen in young and middle aged adults, in cerebral hemispheres white matter.

A

Oligodendrogliomas

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27
Q

What is the characteristics of oligodendrogliomas that could differentiate from say fibrillary astrocytomas (which also happen in the hemispheres)?

A

Oligodendrogliomas represent foci of intratumoral calcification

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28
Q

What do you see on cross-sections of the brain of oligodendrogliomas?

A

Chicken wire-like blood vessels and fried egg-like cells with clear cytoplasm and well defined borders with H&E stain

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29
Q

What is the mutation to cause oligodendrogliomas?

A

loss of chromosome 1p and 19q

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30
Q

What is the prognosis of oligodendrogliomas?

A

better than astrocytomas, survival of 5-10 years

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31
Q

Define: these are tumors that arise next to CSF-lined structures.

A

Ependymomas

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32
Q

If the ependymoma occurs with people in their first 2 decades of life, where do they usually occur in the CNS?

A

near the 4th vent

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33
Q

If the ependymoma occurs with people in adulthood, where do they usually occur in the CNS?

A

Spinal cord

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34
Q

What is the clinical Sx/prognosis of ependymomas?

A

psoterior fossa ependymomas occur with hydrocephalus secodnary to obstruction, often with a poor prognosis (~4 years)

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35
Q

What shows on histology of ependymomas?

A

Rosettes or perivascular pseudorosettes

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36
Q

Define: non-neoplasic lesion, occurs in young adutls, attahced to roof of 3rd vent –> noncommunicating hydrocephalus, headache.

A

Colloid cyst

37
Q

Where do central neurocytomas occur?

A

Intraventicular (usually lateral)

38
Q

What ages affect central neurocytomas?

A

Young adults (25-30)

39
Q

Sx of central neurocytomas?

A

nausea, vomiting, headache due to obstructive hydrocephalus

40
Q

Tx of central neurocytomas?

A

Resection (these are just benign balls of shit)

41
Q

What are gangliogliomas?

A

Mixture of glial and ganglial cells, most common CNS tumor, contain mature appearing ganglion cells

42
Q

Sx of gangliogliomas?

A

Seizures

43
Q

Where in the brain are gangliogliomas?

A

In temporal (often with cyst component)

44
Q

What ages does Dysembryoplastic neuroepithelial tumors effect?

A

Kids

think “embryo” –> kids

45
Q

What are the Sx and prognosis of Dysembryoplastic neuroepithelial tumors?

A

rare, low grade, good prognosis, happen in superficial temporal lobe

46
Q

What is the most common poorly defined differentiated neoplasm?

A

Medullablastoma

47
Q

Which ages doe smedullablastomas occur in?

A

Kids (~20% of brain tumors)

48
Q

What are the clinical features of medullablastomas?

A

highly malignant, VERY RADIOSENSTITIVE, loss of 17p –> poor prognosis (5 yr survival)

49
Q

Which ages does atypical rhabdoid/teratoid tumors affect?

A

kids < 5 yrs

50
Q

What is the mutation to cause rhabdoid/teratoid tumors?

A

chromosme 22 alterations

51
Q

What are the clinical features of rhabdoid/teratoid tumors?

A

Death within a year

52
Q

Which ages get primary CNS lymphomas?

A

Old people.

53
Q

What types of pts get primary CNS lymphomas?

A

immunosupressed people

54
Q

Where do primary CNS lymphomas come from?

A

from B-cells

55
Q

What is the prognosis of primary CNS lymphomas?

A

poor because it’s very aggressive.

56
Q

What are the clinical features of pirmary CNS lymphomas?

A

perivascular localization, may involve neuroparenchyma if systemic

57
Q

What are the 2 age groups that get craniopharyngiomas?

A

10-14 years and the second peak is in the 7th decade

58
Q

Sx of craniopharyngiomas?

A

headaches and increased ICP (from obstructive hydrocephalus), visual problems

59
Q

Clinical presentation of craniopharyngiomas?

A

80-87% are calcified

60
Q

Tx of craniopharyngiomas?

A

CUT IT OUT

61
Q

Why are there bilateral temporal hemianopnia with craniopharyngiomas?

A

COmpression of the optic chiasm

62
Q

Who gets meningiomas?

A

Adults

63
Q

What are the clinical feastures of meningiomas?

A

benign, attached to dura, slow growing

64
Q

What are the freuqent sites of metastatic tumors?

A

Meningies (present as mass lesions)

65
Q

Define: these happen when central and peripheral immune responses to tumor antigens cross-react in the CNS or PNS.

A

Paraneoplastic syndromes

66
Q

What are the clinical features of encephalomyelitis?

A
  • eye movement disorders
    - limbic system
    - cerebellar degeneration - Lambert-Eaton
    - sensory neuropathy
67
Q

Where can peripheral nerve sheath tumors arise?

A

in both brain and PNS

68
Q

What are the 3 characteristics of Peripheral nerve sheath tumors?

A

Schwann cells (express S-100 Ag), perineural cells, fibroblasts

69
Q

What are the clinical featueres of schwannomas?

A

benign, arise from neural crest cells, space occupying lesions

70
Q

What is the mutation to cause schwannomas?

A

mutations of chromosome 22, loss of merlin

71
Q

What does Merlin do?

A

restricts actions of cell surface growth factor EGFR

72
Q

Where do msot schwannomas occur?

A

VIII (cerebellopontine angle) –> tinnitus and hearing loss (acoustic neuroma)

73
Q

What is the inheritance of von Hipple Lindau disease?

A

AD

74
Q

Pt’s with vHLD develop hemangioblastomas in which 2 locations?

A

Cerebellum and retina

75
Q

vHL pt’s have an increased risk of developing what 2 other tumors?

A

Renal cell carcinoma and pheochromocytomas

76
Q

What is the inheritance of tuberous sclerosis?

A

AD

77
Q

What are the clinical features of tuberous sclerosis?

A

Harmatomous + benign neoplasms of the brain and other tissues. Can have cortical tuberous and subependymal giant cell astrocutomas in the Cx of the brain.

78
Q

What happens if the tubers in the brain grow and invade the ventricular system?

A

Seizures

79
Q

What are the clinical findings of tuberous sclerosis?

A

tubers/potatoes on CT of the brain, may also form in heart, liver and kidneys. skin and fingerenails may show ungual fibromas

80
Q

What is the inheritance of neurofibromatosis type 1 (NF1)?

A

AD

81
Q

Incidence of NF1?

A

1/3000

82
Q

What tumors form in NF1?

A

plexiform and solitary neurofibromas (plexiform is more malignant), gliomas of the optic nerve, Lisch nodules

83
Q

If there is a mutation of the NF1 gene, what is activated to cause proliferation of the cancer cells?

A

RAS (normally inhibited by NF1 on chromosome 17)

84
Q

Where are Lisch nodules?

A

They’re yellow elevations on the iris

85
Q

What is the inheritance of NF2?

A

AD

86
Q

What are the tumors assocated with NF2?

A

VIII schwannomas, multiple meningiomas and ependymomas of the spinal cord

87
Q

Incidence of NF2?

A

1/40,000

88
Q

Is there any malignant transformation of NF2?

A

No

89
Q

What are the Sx of NF2?

A

heaing loss, facial weakness, dizziness, ataxia, cataracts at an early age

Clinical Neuroscience (12 decks)

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