CNS Neoplasms

Question 1

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

Answer 1

Neuroectodermal lineage

Question 2

Are meningeal tumors more common in adults or children?

Answer 2

Adults

Question 3

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

Answer 3

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

Question 4

What grade is Pilocytic Astrocytoma?

Answer 4

WHO Grade I

Question 5

What grade is Diffuse Astrocytoma?

Answer 5

WHO Grade II

Question 6

What grade is Glioblastoma?

Answer 6

WHO Grade IV

Question 7

What grade is Oligodendroglioma?

Answer 7

WHO Grade II

Question 8

What grade is Anaplastic Oligodendroglioma?

Answer 8

WHO Grade III

Question 9

What grade is Anaplastic Astrocytoma?

Answer 9

WHO Grade III

Question 10

What grade is Choroid Plexus Papilloma?

Answer 10

WHO Grade I

Question 11

What grade is Ependymoma?

Answer 11

WHO Grade II

Question 12

What grade is Anaplastic Ependymoma?

Answer 12

WHO Grade III

Question 13

What grade is Medulloblastoma?

Answer 13

WHO Grade IV

Question 14

Are meningiomas and schwannomas well-demarcated or infiltrative?

Answer 14

Well-demarcated

Question 15

What is the most common CNS neoplasm in children?

Answer 15

Pilocytic Astrocytoma (Grade I)

Question 16

What are the usual sites of pilocytic astrocytoma?

Answer 16

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

Question 17

Is pilocytic astrocytoma well-circumscribed or infiltrative?

Answer 17

Well-circumscribed; often forms cysts

Question 18

What is the genetic underpinning of pilocytic astrocytoma?

Answer 18

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

Question 19

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

Answer 19

30s-40s

Question 20

What is the common location of diffuse astrocytoma?

Answer 20

Cerebral hemispheres; rarely posterior fossa

Question 21

Is diffuse astrocytoma infiltrative or well-demarcated?

Answer 21

Infiltrative - usually not fully excised

Question 22

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

Answer 22

Heterogeneous - with a predominance of fibrillary astrocytes

Question 23

What is the mean age for diagnosis of anaplastic astrocytoma?

Answer 23

45 years

Question 24

What is the common location of anaplastic astrocytoma?

Answer 24

Cerebral hemispheres

Question 25

What is prognostic for anaplastic astrocytoma?

Answer 25

Mitotic activity

Question 26

What is the genetic underpinning of diffuse astrocytomas?

Answer 26

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

Question 27

What age group is most common for diagnosis of oligodendroglioma?

Answer 27

Young adults

Question 28

What is the common location of oligodendrogliomas?

Answer 28

Cerebral white matter - but quickly infiltrates overlying cortex

Question 29

How does oligodendroglioma usually present clinically?

Answer 29

Seizures

Question 30

What is the histological appearance of oligodendrogliomas?

Answer 30

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

Question 31

What is the genetic underpinning of oligodendrogliomas?

Answer 31

LOH 1p,19q

Question 32

What is the mitotic rate of oligodendrogliomas?

Answer 32

Minimal or absent

Question 33

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

Answer 33

48 years

Question 34

What is the histological difference between oligodendrogliomas and anaplastic oligodendrogliomas?

Answer 34

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

Question 35

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

Answer 35

Better prognosis!! More sensitive to chemotherapy and radiotherapy.

Question 36

What is the mean age for diagnosis of glioblastoma?

Answer 36

50s-60s

Question 37

What is the common location for glioblastoma?

Answer 37

Cerebral hemispheres

Question 38

What are the characteristics of glioblastoma?

Answer 38

Infiltrative, hemorrhagic, vascular proliferation, necrotic

Question 39

What is the median survival for glioblastoma?

Answer 39

Just a few years :(

Question 40

Can glioblastoma be cured by surgical resection?

Answer 40

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

Question 41

What age group do ependymomas affect?

Answer 41

Children; especially when 4th ventricle is involved

Question 42

Which tumors are often calcified?

Answer 42

Pilocytic astrocytomas, gangliogliomas, oligodendrogliomas, & ependymomas.

Question 43

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

Answer 43

Spinal cord

Question 44

What is a defining feature of "ependymal" differentiation?

Answer 44

Perivascular pseudorosettes

Question 45

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

Answer 45

Lateral ventricles

Question 46

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

Answer 46

4th ventricle

Question 47

What is the most common malignant brain tumor in children?

Answer 47

Medulloblastoma

Question 48

What is the peak age range for medulloblastoma?

Answer 48

3-8 years old

Question 49

What is the survival rate of medulloblastoma?

Answer 49

~50%

Question 50

How do patients with medulloblastoma present?

Answer 50

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

Question 51

Does medulloblastoma metastasize past point of origin?

Answer 51

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

Question 52

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

Answer 52

Medulloblastoma

Question 53

Meningiomas are mostly which WHO grade?

Answer 53

I, treatable

Question 54

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

Answer 54

Adulthood

Question 55

Which cranial nerves use Schwann cells to make their myelin?

Answer 55

Cranial nerves 3-12

Question 56

Are Schwannomas (aka neurofibromas) mostly benign or malignant?

Answer 56

Benign

Question 57

What is the most common site for medulloblastoma?

Answer 57

Cerebellar vermis

Question 58

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

Answer 58

Well-demarcated