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Flashcards in CNS Neoplasms Deck (58)
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1
Q

The majority of Primary (Intrinsic) CNS tumors arise from cells of which lineage?

A

Neuroectodermal lineage

2
Q

Are meningeal tumors more common in adults or children?

A

Adults

3
Q

What are the 5 most common primary tumor sites to metastasize to the CNS?

A
  1. Lung
  2. Breast
  3. Melanoma
  4. Kidney
  5. GI tract
4
Q

What grade is Pilocytic Astrocytoma?

A

WHO Grade I

5
Q

What grade is Diffuse Astrocytoma?

A

WHO Grade II

6
Q

What grade is Glioblastoma?

A

WHO Grade IV

7
Q

What grade is Oligodendroglioma?

A

WHO Grade II

8
Q

What grade is Anaplastic Oligodendroglioma?

A

WHO Grade III

9
Q

What grade is Anaplastic Astrocytoma?

A

WHO Grade III

10
Q

What grade is Choroid Plexus Papilloma?

A

WHO Grade I

11
Q

What grade is Ependymoma?

A

WHO Grade II

12
Q

What grade is Anaplastic Ependymoma?

A

WHO Grade III

13
Q

What grade is Medulloblastoma?

A

WHO Grade IV

14
Q

Are meningiomas and schwannomas well-demarcated or infiltrative?

A

Well-demarcated

15
Q

What is the most common CNS neoplasm in children?

A

Pilocytic Astrocytoma (Grade I)

16
Q

What are the usual sites of pilocytic astrocytoma?

A

Cerebellum, optic pathway, hypothalamus, also thalamus, spinal cord, temporal lobe

17
Q

Is pilocytic astrocytoma well-circumscribed or infiltrative?

A

Well-circumscribed; often forms cysts

18
Q

What is the genetic underpinning of pilocytic astrocytoma?

A

BRAF:KIAA fusion –> leads to a more indolent tumor because of senescence (this fusion = good prognosis!)

19
Q

What is the mean age range for diagnosis of diffuse astrocytoma?

A

30s-40s

20
Q

What is the common location of diffuse astrocytoma?

A

Cerebral hemispheres; rarely posterior fossa

21
Q

Is diffuse astrocytoma infiltrative or well-demarcated?

A

Infiltrative - usually not fully excised

22
Q

Is a diffuse astrocytoma tumor homogenous or heterogeneous in its astrocytic cell types?

A

Heterogeneous - with a predominance of fibrillary astrocytes

23
Q

What is the mean age for diagnosis of anaplastic astrocytoma?

A

45 years

24
Q

What is the common location of anaplastic astrocytoma?

A

Cerebral hemispheres

25
Q

What is prognostic for anaplastic astrocytoma?

A

Mitotic activity

26
Q

What is the genetic underpinning of diffuse astrocytomas?

A

IDHI mutation + no LOH 1p,19q + p53 mutation

27
Q

What age group is most common for diagnosis of oligodendroglioma?

A

Young adults

28
Q

What is the common location of oligodendrogliomas?

A

Cerebral white matter - but quickly infiltrates overlying cortex

29
Q

How does oligodendroglioma usually present clinically?

A

Seizures

30
Q

What is the histological appearance of oligodendrogliomas?

A

“Chicken wire” vascular pattern and “fried egg” appearance (round nuclei and little cytoplasm)

31
Q

What is the genetic underpinning of oligodendrogliomas?

A

LOH 1p,19q

32
Q

What is the mitotic rate of oligodendrogliomas?

A

Minimal or absent

33
Q

What is the mean age of onset for diagnosis of anaplastic oligodendroglioma?

A

48 years

34
Q

What is the histological difference between oligodendrogliomas and anaplastic oligodendrogliomas?

A

Anaplastic oligodendrogliomas has increased cellularity, nuclear atypia, and mitoses.

35
Q

Does the LOH 1p,19q of oligodendrogliomas and anaplastic oligodendrogliomas lead to a better or worse prognosis? Why?

A

Better prognosis!! More sensitive to chemotherapy and radiotherapy.

36
Q

What is the mean age for diagnosis of glioblastoma?

A

50s-60s

37
Q

What is the common location for glioblastoma?

A

Cerebral hemispheres

38
Q

What are the characteristics of glioblastoma?

A

Infiltrative, hemorrhagic, vascular proliferation, necrotic

39
Q

What is the median survival for glioblastoma?

A

Just a few years :(

40
Q

Can glioblastoma be cured by surgical resection?

A

No. Appears well-demarcated but in fact is not.

41
Q

What age group do ependymomas affect?

A

Children; especially when 4th ventricle is involved

42
Q

Which tumors are often calcified?

A

Pilocytic astrocytomas, gangliogliomas, oligodendrogliomas, & ependymomas.

43
Q

When adults have ependymomas, where are they located most often?

A

Spinal cord

44
Q

What is a defining feature of “ependymal” differentiation?

A

Perivascular pseudorosettes

45
Q

Choroid plexus papillomas (in children) are most commonly located where?

A

Lateral ventricles

46
Q

Choroid plexus papillomas (in adults) are most commonly located where?

A

4th ventricle

47
Q

What is the most common malignant brain tumor in children?

A

Medulloblastoma

48
Q

What is the peak age range for medulloblastoma?

A

3-8 years old

49
Q

What is the survival rate of medulloblastoma?

A

~50%

50
Q

How do patients with medulloblastoma present?

A

Increased ICP, headache, vomiting, pailledema, gait disturbances, nystamus, dysmetria

51
Q

Does medulloblastoma metastasize past point of origin?

A

Yes! Exception to the general trend that CNS tumor infiltrate only the CNS. It spreads through CSF pathways.

52
Q

Mutations in the SHH gene can lead to what type of CNS tumor?

A

Medulloblastoma

53
Q

Meningiomas are mostly which WHO grade?

A

I, treatable

54
Q

Are meningiomas and mesenchymal tumors more common in childhood or adulthood?

A

Adulthood

55
Q

Which cranial nerves use Schwann cells to make their myelin?

A

Cranial nerves 3-12

56
Q

Are Schwannomas (aka neurofibromas) mostly benign or malignant?

A

Benign

57
Q

What is the most common site for medulloblastoma?

A

Cerebellar vermis

58
Q

Are metastases to the brain usually infiltrative or well-demarcated?

A

Well-demarcated