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Neuro 2-Unit 2 > Ch 28-CNS (tumors) > Flashcards

Ch 28-CNS (tumors) Flashcards

1
Q

This is the most common group of primary brain tumors and includes astrocytomas, oligodendrogliomas, and ependymomas

A

Gliomas

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2
Q

___ account for about 80% of adult primary brain tumors and are usually found in the cerebral hemispheres. Pts present with seizures, headaches, and focal neurologic deficits related to anatomic site of involvement

A

Infiltrating astrocytomas

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3
Q

Which age group is most likely to develop an infiltrating astrocytoma?

A

Adults

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4
Q

The majority of primary glioblastomas (classic subtype) have mutations of ___ gene, deletions of Chromosome ___, and amplification of ___ oncogene

A

PTEN

Chr 10

EGFR

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5
Q

The proneural type is associated with secondary glioblastoma and characterized by mutations of ___, and point mutations in the ___ genes

A

TP53

IDH1 and IDH2

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6
Q

Among the higher grade astrocytomas (III and IV), presence of the mutant form of ___ is associated with a significantly better outcome than in tumors with the wild-type form

A

IDH1

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7
Q

___ has a similar histologic appearance to anaplastic astrocytoma but has necrosis and vascular/endothelial cell proliferation. The necrosis occurs in a serpentine pattern and has pseudo-pallisading tumor cells in necrotic regions

A

Glioblastoma

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8
Q

What is the prognosis for pts with glioblastoma?

A

VERY POOR

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9
Q

What is the grading of a pilocytic astrocytoma, who is more likely to be diagnosed, and where are they usually located?

A

Grade 1, typically occur in children and young adults, usually located in cerebellum

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10
Q

These inhibitors may play a role in the tx of pilocytic astrocytomas

A

BRAF

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11
Q

This type of tumor is often cystic with bipolar cells that are GFAP-positive. Rosenthal fibers and eosinophilic granular boides are characteristic findings

A

Pilocytic astrocytoma

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12
Q

Where do pleomorphic xanthoastrocytomas most often occur, who is most likely to be diagnosed, and what is the grade?

A

Most often occur in temporal lobe of children and young adults, and is a low grade tumor (II/IV)

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13
Q

This astrocytoma occurs most often in the first 2 decades of life and make up 10-20% of all brain tumors in this age group

A

Brainstem glioma

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14
Q

This brainstem glioma is the most common, has an aggressive course and short survival

A

Intrinsic pontine glioma

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15
Q

This brainstem glioma anatomic pattern is often exophytic and a less aggressive course

A

Cervicomedullary junction

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16
Q

This brainstem glioma anatomic pattern has an even more benin course which may arise in the tectum of the midbrain, pons, or medulla

A

Dorsally exophytic glioma

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17
Q

This tumor constitues 5-15% of glioma and most common in 4th and 5th decades. Pts may have had several years of neurologic complaints, often including seizures.

A

Oligodendrogliomas

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18
Q

This glioma presents often with seizures, lesions found mostly in cerebral hemispheres with a predilection for white matter.

A

Oligodendroglioma

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19
Q

What is the most common genetic alteration (mutation) in oligodendroglioma and how does it affect the prognosis?

A

IDH1 and IDH1, which occur in up to 90% of oligodendrogliomas and portend a BETTER PROGNOSIS

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20
Q

This co-deletion is seen in up to 80% of cases of oligodendrogliomas and this deletion shows long-lasting responses to chemotherapy and radiation

A

Co-deletion of Chr 1p and 19q

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21
Q

A fried-egg appearance morphologic pattern is associated with ___

A

Oligodendroglioma

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22
Q

what is the prognosis of oligodendroglioma?

A

Better prognosis than those with astrocytomas but those with anaplastic oligodendrogliomas have an overall worse prognosis

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23
Q

What age group is likely to have an ependymoma, where does it normally occur?

A

First 2 decades of life, typically occur near the 4th ventricle

Constitue 5-10% of primary brain tumors in this age group

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24
Q

This gene is commonly mutated in ependymomas in the SC but not at other sites

A

NF2 gene on Chr 22

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25
Q

In ependymomas, aberrations of large regions of chromosomes or whole chromosomes tend to have what prognosis?

A

An overall better prognosis

26
Q

Perivascular pseudorosettes in which tumor cells are arranged around vessels with an intervening zone and GFAP expression is found in most ___

A

Ependymomas

27
Q

___ often manifest with hydrocephalus secondary to progressive obstruction of the 4th ventricle; CSF dissemination is a common occur and portends a poor prognosis

A

Posterior fossa ependymomas

28
Q

___ tumors are far less common than glial tumors and most often seen in younger adults and often presents with seizures

A

Neuronal

29
Q

___ is the most common of the neuronal tumors of the CNS, has a mix of neuronal and glial cells and presents with seizures. Mostly the temporal lobe

A

Gangliomas

30
Q

___ is a malignant embryonal tumor that occurs predominantly in children and exclusively in the cerebellum. It is largely undifferentiated and grade IV

A

Medulloblastoma

31
Q

This medulloblastoma subtype tends to occur in older kids, can show monosomy of Chr 6 and nuclear expression of beta-catenin, has the best prognosis

A

WNT

32
Q

This medulloblastoma subtype occurs in infants or young adults, has MYCN amplification, intermediate prognosis

A

SHH

33
Q

This medulloblastoma subtype has MYC amplification and i17q, occurs in infants and children and has the worst prognosis

A

Group 3

34
Q

This medulloblastoma subtype has an i17q alteration without MYC amplification but can have it at times. The prognosis is intermediate

A

Group 4

35
Q

This alteration in medulloblastoma subtypes signals a poor prognosis in groups 3 and 4

A

i17q

36
Q

In children with medulloblatoma, the tumor is located ___, while in adults it is found ___

A

Children –> midline of cerebellum

Adults–> lateral locations

37
Q

Dissemination of this tumor through CSF is a common complication that can lead to drop metastases

A

Medulloblastoma

38
Q

What is prognosis for medulloblastoma?

A

Poor

Grade IV

39
Q

What is the prognosis of an atypical teratoid/rhabdoid tumor? Who is most likely to get it? Where do they typically occur

A

Highly malignant

Young children

Posterior fossa and supratentorial compartments in equal proportions

40
Q

What is the pathologic hallmark of an atypical teratoid/rhabdoid tumor?

A

Alterations in Chr 22 a hallmark of rhabdoid tumor (hSNF5/INI1)

41
Q

What is the clinical outcome/prognosis of atypical teratoid/rhabdoid tumorS?

A

Highly aggressive of the very young, most before age 5 die, within 1 year

42
Q

This is the most common CNS neoplasm of the immunosuppressed pts

A

Primary CNS lymphoma

43
Q

The vast majority of primary brain lymphomas are of __ cell origin

A

B

44
Q

In immunosuppressed setting, cells in nearly all primary brain lymphomas are latently infected by ___

A

EBV

45
Q

This type of tumor occurs along the midline, most commonly in the pineal and suprasellar regions; accounts for up to 10% of brain tumors in Japanese

A

Primary brain germ cell tumor

46
Q

___ is a predominantly benign tumor of adults and prior radiation to head and neck (decades earlier) is a risk factor

A

Meningioma

47
Q

The most common cytogenetic abnormality of a meningioma is loss of ___

A

Chr 22q12 –> NF2 gene –> Merlin

Of sporadic meningiomas, 50-60% harbor mutations in NF2 genes

48
Q

In meningiomas without NF2 mutations, the most common mutations occur in ___

A

TRAF7

49
Q

Higher grade meningiomas are often associated with:

A

NF2 mutations, loss of CHr 22, and chromosomal instability

50
Q

This meningioma is highly aggressive with appearance of a high-grade sarcoma

A

Atypical (malignant) meningiomas

Grade III/IV

51
Q

Who is more likely to have a meningioma?

A

Uncommon in children, show moderate (3:2) female predominance, although ratio is 10:1 for spinal meningiomas

52
Q

Meningiomas often express ___ receptors and may grow more rapidly during ___

A

Progesterone

Pregnancy

53
Q

Meningeal carcinomatosis most commonly associated with carcinoma of the ___

A

Lung and. Breast

54
Q

List the mutation in the following familial tumor syndromes:

Cowden syndrome: dysplastic gangliocytoma of cerebellum with mutation in ___

Li-Fraumeni syndrom: medulloblastoma caused by mutations in ___

Turcot syndrome: medulloblastoma or glioblastoma with mutations in ___

Gorlin syndrome: medulloblastom with mutation in ____

A

PTEN

TP53

APC

PTCH

55
Q

__ is autosomal dominant, 1 in 6000 births, characterized by hamartomas and benign neoplasms involving brain and other tissues

A

Tuberous sclerosis complex

56
Q

What are the most frequent clinical manifestations of tuberous sclerosis complex?

A

Seizures, autism, MR

57
Q

___ encodes protein hamartin

___ encodes tuberin

A

TSC1 on Chr 9q34

TSC2 at Chr 16p13.3

58
Q

__ is autosomal dominant; develop hemangioblastomas of CNS and cysts involving pancreas, liver, and kidneys and have propensity to develop renal cell carcinoma and pheochromocytoma

A

VHL disease

59
Q

Hemangioblastomas are most common in ___

A

Cerebellum and retina

60
Q

VHL is a tumor suppressor gene on Chr 3p25.3 and encodes the protein VHL and down-regulates ___

A

HIF-1

61
Q

Rosenthal fibers are within astrocytic processes and are characteristic of this glial tumor:

A

Pilocytic astrocytoma

Neuro 2-Unit 2 (4 decks)

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