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aNeuro Exam 2- Lindsey's > Neoplasms I-III > Flashcards

Neoplasms I-III Flashcards

1
Q

Dx?

  • variety of appearances
  • inactivation of neurofibromatosis gene Merlin on ch22
  • less favorable prognosis
  • meningeal-based tumor
A

hemangiopericytoma

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2
Q

What is a WHO grade II brain lesion?

A
  • infiltrative in nature
  • often recur
  • tend to progress to higher grade of malignancy
  • tx depends on size, type, location, and clinical features
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3
Q

Tell me everything there is to know about anaplastic ependymoma.

A
  • more mitotically active
  • childhood (4th ventricle)
  • recurs
  • WHO grade III
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4
Q

What is Li-Fraumeni Syndrome?

A
  • germ line mutation in p53
  • malignant gliomas
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5
Q

Tell me everything there is to know about mixed anaplastic oligoastrocytoma.

A
  • admixed tumor cell population
  • increased mitotic activity
  • microvascular prolif
  • NO necrosis
  • LOH 1p, 19q = better prognosis
  • WHO grade III
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6
Q

Dx?

  • auto dominant
  • intra- and extracranial Schwann cell tumors
  • optic gliomas
  • astrocytomas
  • meningiomas
  • 17q11.2 encodes neurofibromin protein that normally inhibits RAS
A

Neurofibromatosis Type 1 (NF1)

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7
Q

Dx?

  • usu temporal lobe
  • cystic
  • well demarcated
  • more calcified
  • jumbled, abnormal neurons in low grade glial bakground
A

gangliogliomas

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8
Q

Tell me everything there is to know about Pilocytic Astrocytomas.

A
  • common in childhood
  • affects cerebellar hemispheres, optic nerve and chiasm, and hypothalamic region
  • well circumscribed, non-infiltrative
  • cured by surgical excision alone
  • very little tendency to go malignant
  • cystic looking, piloid astrocytes, can be calcified, contains mucin and microcysts
  • B-raf activation common (good)
  • WHO grade I
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9
Q

What kinds of cells give rise to gliomas?

A

neuroextoderm cells such as glia

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10
Q

Dx?

  • arises in white matter
  • cerebral hemisphere
  • 30-50yo pts
  • infiltrative growth pattern
  • surgery for debulkment
  • mild hypercellularity
  • mild nuclear pleomorphism
  • irregular astrocyte distribution
A

diffuse astrocytoma (WHO grade II)

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11
Q

Dx?

  • bilateral vestibular schwannomas
  • multiple meningiomas
  • systemic schwannomas
  • systemic liomas
  • ependymomas of spinal cord
  • chromosome 22q12 encodes Merlin protein that regulates cell receptor signaling
A

Neurofibromatosis Type 2 (NF2)

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12
Q

What is a WHO grade IV brain lesion?

A
  • cytologically malignant
  • mitotically active
  • necrosis-prone
  • rapid evolution
  • fatal outcome
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13
Q

What kinds of cells give rise to meningiomas?

A

meningeal/mesenchymal cells

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14
Q

Tell me everything there is to know about diffuse astrocytoma.

A
  • arises in white matter
  • cerebral hemisphere
  • 30-50yo pts
  • infiltrative growth pattern
  • surgery for debulkment
  • mild hypercellularity
  • mild nuclear pleomorphism
  • irregular astrocyte distribution
  • WHO grade II
How well did you know this?
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15
Q

Dx?

  • germ line mutation in p53
  • malignant gliomas
A

Li-Fraumeni Syndrome

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16
Q

Tell me everything there is to know about anaplastic oligodendroglioma.

A
  • (+) enhancement
  • round, uniform nuclei w/ scant cytoplasm = “fried egg” appearance
  • calcification
  • microvascular proliferation
  • more mitotic activity
  • (+) MIB-1 labeling
  • LOH 1p, 19q = good, without = bad
  • WHO grade III
How well did you know this?
1
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2
3
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5
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17
Q

Dx?

  • admixed tumor cell population
  • increased mitotic activity
  • microvascular prolif
  • NO necrosis
  • LOH 1p, 19q = better prognosis
A

mixed anaplastic oligoastrocytoma (WHO grade III)

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18
Q

What is a WHO grade III brain lesion?

A
  • lesions w/ evidence of malignancy
  • nuclear atypica
  • brisk mitotic activity
  • tx = radiation or chemo
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19
Q

What is Turcot Syndrome?

A
  • medulloblastomas or glioblastomas
  • mutation of the APC
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20
Q

Grade the lesion:

  • evidence of malignancy
  • nuclear atypica
  • brisk mitotic activity
  • tx = radiation or chemo
A

WHO grade III

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21
Q

Dx?

  • adults
  • cerebral hemisphere white matter
  • presents with seizures
  • calcified
  • “chicken wire” vascular pattern
  • round, monotonous nuclei + little to no cytoplasm = “fried egg” appearance
A

oligodendroglioma (WHO grade II)

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22
Q

What are the 5 most common 1a sites that metastasize to the CNS?

A
  1. lung
  2. breast
  3. melanoma
  4. kidney
  5. GI
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23
Q

Tell me everything there is to know about mixed oligoastrocytoma.

A
  • astrocytes and oligodendrocytes admixed in the same tumor
  • GFAP (+) on stain
  • minimal to absent mitotic activity
  • necrosis and microvascular prolif negative
  • cell cycle variable but NO G0
  • WHO grade II
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Tell me everything there is to know about hemangiopericytomas.

A
  • variety of appearances
  • inactivation of neurofibromatosis gene Merlin on ch22
  • less favorable prognosis
  • meningeal-based tumor
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Tell me everything there is to know about oligodendroglioma.

A
  • adults
  • cerebral hemisphere white matter
  • presents with seizures
  • calcified
  • “chicken wire” vascular pattern
  • round, monotonous nuclei + little to no cytoplasm = “fried egg” appearance
  • WHO grade II
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Grade the lesion:

  • infiltrative in nature
  • often recur
  • tend to progress to higher grade of malignancy
  • tx depends on size, type, location, and clinical features
A

WHO grade II

27
Q

Dx?

  • mutation in PTCH gene
  • upregulation of Shh pathways
  • medulloblastomas
A

Gorlin syndrome

28
Q

Tell me everything there is to know about choroid plexus papillomas.

A
  • always intraventricular locations
  • papillary formations
  • children =lateral ventricles
  • adults = 4th ventricle
  • low mitotic rate, mild nuclear atypia
  • easily curable with surgical excision
  • produces hydrocephalus by blocking CSF flow
  • WHO grade I
29
Q

Dx?

  • medulloblastomas or glioblastomas
  • mutation of the APC
A

Turcot Syndrome

30
Q

How do CNS neoplasms cause death?

A
  • destroy vital brain areas
  • by mass effects (tumor + edema = herniation)
31
Q

Tell me everything there is to know about glioblastoma.

A
  • most malignant
  • majority are de novo (primary)
  • pts in 50s-60s
  • infiltrative
  • hemorrhagic
  • necrotic
  • multifocal origin
  • evolve over time
  • not surgically curable but done to debulk
  • irradiation (temozolomide)
  • median survival less than 1 year
  • IDH1 and IDH2 mutations = better
32
Q

Dx?

  • peripheral or cranial nerve sheath tumors
  • mostly benign but can become neurofibrosarcomas
  • esp 8th cranial nerve –> acoustic neuromas proximal
  • spinal nerves –> dumbbell shaped tumor
A

schwannomas

33
Q

Dx?

  • more mitotically active
  • childhood (4th ventricle)
  • recurs
  • WHO grade III
A

anaplastic ependymoma

34
Q

What is von Hippel-Lindau (VHL) Syndrome?

A
  • germ line mutation of VHL gene
  • downreguated hypoxia-inducible factor 1 (HIF-1)
  • overexpression of VEGF and EPO
  • polycythemia
  • hemangioblastomas
35
Q

What tumor grade is the most aggressive?

A

IV (4)

36
Q

Tell me everything there is to know about Neurofibromatosis Type 2 (NF2).

A
  • bilateral vestibular schwannomas
  • multiple meningiomas
  • systemic schwannomas
  • systemic liomas
  • ependymomas of spinal cord
  • chromosome 22q12 encodes Merlin protein that regulates cell receptor signaling
37
Q

Dx?

  • always intraventricular locations
  • papillary formations
  • children =lateral ventricles
  • adults = 4th ventricle
  • low mitotic rate, mild nuclear atypia
  • easily curable with surgical excision
  • produces hydrocephalus by blocking CSF flow
A

choroid plexus papilloma

38
Q

Dx?

  • women > men
  • age 30+ (esp in 50s)
  • problem only when arises in surgically difficult location
  • solitary
  • arise from cell called “arachnoid cap”
A

meningiomas “mostly WHO grade I”

39
Q

Dx?

  • most malignant
  • majority are de novo (primary)
  • pts in 50s-60s
  • infiltrative
  • hemorrhagic
  • necrotic
  • multifocal origin
  • evolve over time
  • not surgically curable but done to debulk
  • irradiation (temozolomide)
  • median survival less than 1 year
  • IDH1 and IDH2 mutations = better
A

glioblastoma

40
Q

Tell me everything there is to know about ependymoma.

A
  • from ependymal cells lining the ventricles
  • found in intraventricular locations
  • pts 0-20yo –> usually 4th ventricle
  • adult pts –> spinal cord
  • presents w/ obstructive hydrocephalus
  • calcified
  • demarcated in spinal cord –> excision
  • brainstem location –> no excision (location dictates prognosis)
  • perivascular
  • pseudorosettes
  • WHO grade II
41
Q

Tell me everything there is to know about anaplastic astrocytoma.

A
  • increased mitotic rate
  • MIB-1 nuclear labeling (+)
  • no necrosis or microvascular prolif
  • crowded cells
  • nuclear hyperchromatism and size/shape variation
  • prognosis = dependent upon growth rate
  • WHO grade III
42
Q

Dx?

  • common in childhood
  • affects cerebellar hemispheres, optic nerve and chiasm, and hypothalamic region
  • well circumscribed, non-infiltrative
  • cured by surgical excision alone
  • very little tendency to go malignant
  • cystic looking, piloid astrocytes, can be calcified, contains mucin and microcysts
  • B-raf activation common (good)
A

Pilocytic Astrocytoma

43
Q

Dx?

  • auto dominant
  • caused by mutation in TSC1 and TSC2 genes that inhibit mTOR (cell size and anabolic growth regulator)
  • hamartomas and benign neoplasms
  • sub-ependymal giant cell astrocytomas
A

Tuberous Sclerosis

44
Q

Tell me everything there is to know about Tuberous Sclerosis.

A
  • auto dominant
  • caused by mutation in TSC1 and TSC2 genes that inhibit mTOR (cell size and anabolic growth regulator)
  • hamartomas and benign neoplasms
  • sub-ependymal giant cell astrocytomas
45
Q

_____ metastases are typically sharply demarcated, treated with surgical excision, and are commonly found at the grey-white junction.

A

Intraparenchymal

46
Q

Dx?

  • malignant embryonal tumor of the cerebellum
  • occurs in children (3-8yo, esp males)
  • spreads via CSF pathways
  • most common type of PNET
  • presents with increased ICP signs (HA, vomiting, papilledema) and gait, nystagmus, and dysmetria problems
  • responsive to aggressive chemo and radiation IF no CSF spread
  • patternless sheets of small embryological cells w/ scant cytoplasm (small blue cells)
  • Homer Wright rosettes
  • problem with Shh pathway
A

medulloblastoma (WHO grade IV)

47
Q

Pediatric pts develop about 2/3 of their brain tumors in the _____ rather than the _____ space.

A

infratentorial; supratentorial

48
Q

What is the most common type of primary brain tumor?

A

astrocytomas

49
Q

Tell me everything there is to know about Neurofibromatosis Type 1 (NF1).

A
  • auto dominant
  • intra- and extracranial Schwann cell tumors
  • optic gliomas
  • astrocytomas
  • meningiomas
  • 17q11.2 encodes neurofibromin protein that inhibits RAS
50
Q

Dx?

  • germ line mutation of VHL gene
  • downreguated hypoxia-inducible factor 1 (HIF-1)
  • overexpression of VEGF and EPO
  • polycythemia
  • hemangioblastomas
A

von Hippel-Lindau (VHL) Syndrome

51
Q

Tell me everything there is to know about schwannomas.

A
  • peripheral or cranial nerve sheath tumors
  • mostly benign but can become neurofibrosarcomas
  • esp 8th cranial nerve –> acoustic neuromas
  • proximal spinal nerves –> dumbbell shaped tumors
52
Q

Grade the lesion:

  • cytologically malignant
  • mitotically active
  • necrosis-prone
  • rapid evolution
  • fatal outcome
A

WHO grade IV

53
Q

Tell me everything there is to know about meningiomas.

A
  • women > men
  • age 30+ (esp in 50s)
  • problem only when arises in surgically difficult location
  • solitary
  • arise from cell called “arachnoid cap”
  • mostly WHO grade I
54
Q

Dx?

  • increased mitotic rate
  • MIB-1 nuclear labeling (+)
  • no necrosis or microvascular prolif
  • crowded cells
  • nuclear hyperchromatism and size/shape variation
  • prognosis = depends on growth rate
A

anaplastic astrocytoma (WHO grade III)

55
Q

Tell me everything there is to know about medulloblastoma.

A
  • malignant embryonal tumor of the cerebellum
  • occurs in children (3-8yo, esp males)
  • spreads via CSF pathways
  • most common type of PNET
  • presents with increased ICP signs (HA, vomiting, papilledema) and gait, nystagmus, and dysmetria problems
  • responsive to aggressive chemo and radiation IF no CSF spread
  • patternless sheets of small embryological cells w/ scant cytoplasm (small blue cells)
  • Homer Wright rosettes
  • problem with Shh pathway
  • WHO grade IV
56
Q

What is a WHO grade I brain lesion?

A
  • tumors w/ low proliferative potential
  • possible cure by surgical resection alone
57
Q

Grade the lesion:

  • tumors w/ low proliferative potential
  • possible cure by surgical resection alone
A

WHO grade I

58
Q

Dx?

  • astrocytes and oligodendrocytes admixed in the same tumor
  • GFAP (+) on stain
  • minimal to absent mitotic activity
  • necrosis and microvascular prolif negative
  • cell cycle variable but NO G0
A

mixed oligoastrocytoma (WHO grade II)

59
Q

Dx?

  • from ependymal cells lining the ventricles
  • found in intraventricular locations
  • pts 0-20yo –> usually 4th ventricle
  • adult pts –> spinal cord
  • presents w/ obstructive hydrocephalus
  • calcified
  • well demarcated in spinal cord –> excision
  • brainstem location –> no excision (location dictates prognosis)
  • perivascular pseudorosettes
A

ependymoma (WHO grade II)

60
Q

Tell me everything there is to know about Gangliogliomas.

A
  • usu temporal lobe
  • cystic
  • well demarcated
  • more calcified
  • jumbled, abnormal neurons in low grade glial background
  • WHO grade I
61
Q

What is Gorlin syndrome?

A
  • mutation in PTCH gene
  • upregulation of Shh pathways
  • medulloblastomas
62
Q

Dx?

  • (+) enhancement
  • round, uniform nuclei w/ scant cytoplasm = “fried egg” appearance
  • calcification
  • microvascular proliferation
  • more mitotic activity
  • (+) MIB-1 labeling
  • LOH 1p, 19q = good, without = bad
A

anaplastic oligodendroglioma (WHO grade III)

63
Q

Name 2 different cell classes that CNS neoplasms can arise from and what class of tumors they become.

A
  1. cells within the neural tube –> primary/intrinsic brain tumors
  2. cells from supporting structures —> extrinsic brain tumors

aNeuro Exam 2- Lindsey's (23 decks)

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