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

Neoplasms I-III Flashcards

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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
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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
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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
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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
Tell me everything there is to know about oligodendroglioma.
* 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
26
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
WHO grade II
27
Dx? * mutation in PTCH gene * upregulation of Shh pathways * medulloblastomas
Gorlin syndrome
28
Tell me everything there is to know about choroid plexus papillomas.
* 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
Dx? * medulloblastomas or glioblastomas * mutation of the APC
Turcot Syndrome
30
How do CNS neoplasms cause death?
* destroy vital brain areas * by mass effects (tumor + edema = herniation)
31
Tell me everything there is to know about glioblastoma.
* 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
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
schwannomas
33
Dx? * more mitotically active * childhood (4th ventricle) * recurs * WHO grade III
anaplastic ependymoma
34
What is von Hippel-Lindau (VHL) Syndrome?
* germ line mutation of VHL gene * downreguated hypoxia-inducible factor 1 (HIF-1) * overexpression of VEGF and EPO * polycythemia * hemangioblastomas
35
What tumor grade is the most aggressive?
IV (4)
36
Tell me everything there is to know about Neurofibromatosis Type 2 (NF2).
* bilateral vestibular schwannomas * multiple meningiomas * systemic schwannomas * systemic liomas * ependymomas of spinal cord * chromosome 22q12 encodes Merlin protein that regulates cell receptor signaling
37
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
choroid plexus papilloma
38
Dx? * women \> men * age 30+ (esp in 50s) * problem only when arises in surgically difficult location * solitary * arise from cell called "arachnoid cap"
meningiomas "mostly WHO grade I"
39
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
glioblastoma
40
Tell me everything there is to know about ependymoma.
* 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
Tell me everything there is to know about anaplastic astrocytoma.
* 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
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)
Pilocytic Astrocytoma
43
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
Tuberous Sclerosis
44
Tell me everything there is to know about Tuberous Sclerosis.
* 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
\_\_\_\_\_ metastases are typically sharply demarcated, treated with surgical excision, and are commonly found at the grey-white junction.
Intraparenchymal
46
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
medulloblastoma (WHO grade IV)
47
Pediatric pts develop about 2/3 of their brain tumors in the _____ rather than the _____ space.
infratentorial; supratentorial
48
What is the most common type of primary brain tumor?
astrocytomas
49
Tell me everything there is to know about Neurofibromatosis Type 1 (NF1).
* auto dominant * intra- and extracranial Schwann cell tumors * optic gliomas * astrocytomas * meningiomas * 17q11.2 encodes neurofibromin protein that inhibits RAS
50
Dx? * germ line mutation of VHL gene * downreguated hypoxia-inducible factor 1 (HIF-1) * overexpression of VEGF and EPO * polycythemia * hemangioblastomas
von Hippel-Lindau (VHL) Syndrome
51
Tell me everything there is to know about schwannomas.
* 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
Grade the lesion: * cytologically malignant * mitotically active * necrosis-prone * rapid evolution * fatal outcome
WHO grade IV
53
Tell me everything there is to know about meningiomas.
* 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
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
anaplastic astrocytoma (WHO grade III)
55
Tell me everything there is to know about medulloblastoma.
* 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
What is a WHO grade I brain lesion?
* tumors w/ low proliferative potential * possible cure by surgical resection alone
57
Grade the lesion: * tumors w/ low proliferative potential * possible cure by surgical resection alone
WHO grade I
58
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
mixed oligoastrocytoma (WHO grade II)
59
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
ependymoma (WHO grade II)
60
Tell me everything there is to know about Gangliogliomas.
* usu temporal lobe * cystic * well demarcated * more calcified * jumbled, abnormal neurons in low grade glial background * WHO grade I
61
What is Gorlin syndrome?
* mutation in PTCH gene * upregulation of Shh pathways * medulloblastomas
62
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
anaplastic oligodendroglioma (WHO grade III)
63
Name 2 different cell classes that CNS neoplasms can arise from and what class of tumors they become.
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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