CNS Tumors Flashcards

1
Q

In gliomas, we see amplification of

A

EGFR

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2
Q

In an astrocytoma, we see loss of tumor suppressor genes

A

TP53 and ATRX

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3
Q

In a glioma, we see the loss of the tumor suppressor gene

-better prognosis

A

IDH

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4
Q

An example of epigenetic gene silence is seen in a glioblastoma with

A

MGMT hypermethylation

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5
Q

An example of a tumor caused by persistent activation of gene mutation product is seen in gliomas with

A

EGFRvIII

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6
Q

An example of a tumor caused by chromosomal abnormality is seen in an oligodendroglioma with the co-deletion of

A

1p19q

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7
Q

Radiation causes an increase in the incidence of

A

Meningiomas

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8
Q

May induce mesenchymal and glial tumors

A

Nitroso compounds

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9
Q

Mostly arise in the cerebellum and hypothalamus

-seen most commonly in children and young adults

A

Pilocytic Astrocytoma

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10
Q

Has a bihasic histological pattern and rosenthal fibers on histological staining

A

Pilocytic Astrocytoma

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11
Q

Pilocytic astrocytomas are characterized as being

A

GFAP positive

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12
Q

Tumor most frequently seen in cerebral hemispheres

  • usually a poorly demarcated lesion
  • commonly in children and young adults
A

Diffure Astrocytoma

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13
Q

Histologically, shows atypical cells infiltrating in a diffuse fashion and large cells with eosinophilic cytoplasm (gemistocytic astrocytoma)

A

Diffuse astrocytoma

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14
Q

The most mlignant glioma

-most frequently in middle aged adults

A

Glioblastoma

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15
Q

Glioblastomas are most commonly in the

A

Frontal and temporal lobes

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16
Q

A poorly defined mass with heterogenous appearance

A

Glioblastoma

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17
Q

Characterised by increased cellularity, nuclear atypia, mitosis, vascular endothelial hyperplasia, and necrosis

A

Glioblastoma

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18
Q

Cause of glioblastoma with better chemosensitivity

A

MGMT gene promoter hypermethylation

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19
Q

Insidious and slow growing tumors mostly in cerebral hemispheres

-Usually in middle aged adults

A

Oligodendroglioma

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20
Q

The types of oligodenrogliomas that are chemosensitive solid tumors are due to a co-deletion of

A

1p and 19q

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21
Q

More circumscribed than astrocytoma

A

Oligodendroglioma

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22
Q

Tumor cells are uniform with “fried egg” appearance

A

Oligodendroglioma

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23
Q

An oligodendroglioma is classified as grade II or III (Anaplastic) if there is marked

A

Mitosis, vascular endothelial proliferation, and necrosis

24
Q

Some oligodendrogliomas have an astrocytic component. These are called

A

Oligoastrocytomas

25
Q

Oligodendrogliomas are positive for

A

Oligo-2

26
Q

Tumor frequently in ventricles causing hydrocephalus

-predominantly in children and adolescents

A

Ependymoma

27
Q

An ependymoma in the spinal cord and filum terminale

A

Myxopapillary Ependymoma

28
Q

Characterized as being exophytic and well demarcated

A

Ependymoma

29
Q

Grade III ependymoma characterized by more mitoses, vascular endothelial hyperplasia and necrosis

A

Anaplastic Ependymoma

30
Q

Ependymomas are positive for

A

GFAP, MAP2, and EMA

31
Q

Greyish, friable mass, commonly in the ventricles

-Mostly in adults

A

Central neurocytoma

32
Q

Composed of round uniform cells with scanty cytoplasm

-WHO grade II tumor

A

Central Neurocytoma

33
Q

A central neurocytoma is positive for

A

Synaptophysin and chromogranin

34
Q

Associated with cortical malformation

-Presents with a history of chronic epilepsy

A

Ganglioglioma

35
Q

Cystic tumor with calcified mural node

-Common in children

A

Ganglioglioma

36
Q

Typically a Grade I tumor and is rarely a higher grade

A

Ganglioma

37
Q

Most frequent malignant tumor in children

  • Tumor of cerebellum
  • solid and ill-defined
A

Medulloblastoma

38
Q

Highly cellular, small oval or round cells with neuronal, glial and other differentiations and neuronal rosette formation

A

Medulloblastoma

39
Q

Medulloblastoma is characterized as a grade

A

IV tumor

40
Q

A curcumscribed mass attached to the dura

-most common in adult women

A

Meningioma

41
Q

Basic histology: pseudointranuclear inclusion, whorl, psammomatous calcification

A

Meningioma

42
Q

Meningiomas are positive with

A

EMA and PR

43
Q

Tumor of the cerebellum associated with von Hippel Lindau disease

-occur in young to middle aged adults

A

Hemangioblastoma

44
Q

Histological characteristics: Numerous delicate capillaries set in a background of clear foamy cells

A

Hemangioblastoma

45
Q

A hemangioblastoma is positive with

A

Inhibin, CD31, and CD34

46
Q

Suprasella solid and cystic mass

-most frequently seen in children and adolescents

A

Craniopharyngioma

47
Q

Histological characteristics: Squamoid cells and keratin with loose connective tissue stroma

  • CKs positive
  • Grade I tumor
A

Craniopharyngioma

48
Q

Most common secretory tumor

  • Suprasella
  • Most common in adults
  • More common in females
A

Pituitary adenoma

49
Q

What percentage of pituitary adenomas are non-secretory?

A

20%

50
Q

Histological characteristics: Sheets of uniform cells with round nuclei with delicate chromatin

-Positive in synaptophysin, chromogranin, NSE, and hormone (if secretory)

A

Pituitary adenoma

51
Q

Extracranial malignancy often demarcated from brain tissue

-morphologically similar to primary extracranial tumor

A

Metastatic neoplasm

52
Q

Associated with a gene mutation

-neoplasm and non-neoplasm

A

Tumor predisposition syndromes (Phakomatoses)

53
Q

Characteried by neurofibromin negatively regulating the Ras onco-protein

A

Neurofibromatosis Type I (NF1) (von Recklinghausen’s disease)

54
Q

The mechanism is via Merlin which involves cellular proliferation

A

Neurofibromatosis Type II (NF2)

55
Q

Mechanism is via VHL protein which activates Hypoxia Inducible Factor (HIF)

A

Von Hippel Lindau Syndrome

56
Q

Mechanism is through Hamartin or Tuberin which negatively regulates mTOR protein in a kinase signaling pathway involved in regulation of energy, metabolism and mRNA translation as a tumor suppressor

A

Tuberous Sclerosis Complex (TSC)