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CNS Patho > Sheet 8 > Flashcards

Sheet 8 Flashcards

1
Q

What are the 3 types of gliomas?

A

1) Astrocytomas
2) Oligodendrogliomas
3) Ependymoma

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2
Q

Oligodendrogliomas account for -% of gliomas.

A

5-15%

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3
Q

Oligodendrogliomas are diagnosed at which ages?

A

40-50

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4
Q

Where are oligodendrogliomas located?

A

Mostly in the cerebral hemispheres, mainly in the frontal or temporal lobes, white matter.

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5
Q

What is diagnostic for oligodendrogliomas?

A

The presence of IDH mutation AND 1p & 19q co-deletion

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6
Q

How do we treat oligodendrogliomas?

A

With a combination of surgery, chemotherapy, and radiotherapy

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7
Q

What is the average survival rate of a grade 2 oligodendrogliomas?

A

10-20 years (because of infiltration)

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8
Q

What is the average survival rate of a grade 3 oligodendrogliomas?

A

5-10 years (because of anaplastic features)

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9
Q

Which grades of oligodendrogliomas don’t exist?

A

1 and 4

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10
Q

Which has a better prognosis(!):

Grade 3 astrocytoma or grade 3 oligodendroglioma?

A

Grade 3 oligodendroglioma

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11
Q

What are the characteristics of grade 2 oligodendrogliomas?

A

1) Infiltrative tumors
2) +/- cysts
3) Focal hemorrhage
4) Calcification

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12
Q

If we see calcifications with a diffuse infiltrating tumor in the radiograph, which tumor is it most likely to be?

A

Oligodendroglioma

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13
Q

What are the histological characteristics of grade 2 oligodendrogliomas?

A

1) Sheets of regular cells with spherical nuclei containing finely granular chromatin
2) The nuclei are surrounded by a clear perinuclear halo of cytoplasm (Fried-egg appearance).
3) Delicate network of anastomosing capillaries “chicken-wire”.
4) Calcification in 90% of tumors.
5) Low mitotic activity
6) No spontaneous necrosis.
7) No microvascular proliferation.

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14
Q

What are the histological characteristics of grade 3 oligodendrogliomas?

A

1) Increased cellularity
2) Easily identifiable mitotic figures
3) Microvascular proliferation, ± necrosis

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15
Q

Where do ependymomas arise?

A

Next to the ependyma- lined ventricular system, including the central canal of the spinal cord.

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16
Q

Where are ependymomas located?

A

1) Posterior fossa (60%)
2) Supratentorial (30%)
3) Spinal (10%)

17
Q

At which age do ependymomas appear?

A

1) In the first 2 decades of life; near the 4th ventricle (posterior fossa)
2) In adults, the spinal cord and supratentorial ependymomas occur with almost equal frequency

18
Q

Which clinical outcome is better for completely resected ependymomas:
Supratentorial & spinal
ependymomas or posterior fossa ependymomas?

A

Supratentorial & spinal

ependymomas

19
Q

What is the morphology of ependymomas?

A

Composed of uniform small cells with round to oval nuclei and granular chromatin in a fibrillary matrix and characterized by:

1) Rosette formation
2) Low cell density and a low mitotic count
3) Cilia and microvilli are seen on ultrastructural examination

20
Q

What are the 2 types of rosettes?

A

1) Ependymal rosettes: diagnostic hallmark of ependymoma (25%)
2) Perivascular pseudorosettes: not specific for ependymoma.

21
Q

What are rosettes?

A

Tumor cells arranged like a

circle around a central structure

22
Q

What are ependymal rosettes?

A

Tumor cells arranged around central canal or lumen that resemble the embryologic ependymal canal, with long, delicate processes extending into a lumen.

23
Q

What are perivascular pseudorosettes?

A

Composed of tumor cells radially arranged around vessels with an intervening anucleated zone containing thin ependymal processes.

24
Q

What is the morphology of anaplastic ependymomas?

A

1) Increased cell density
2) High mitotic rates
3) Necrosis
4) Microvascular proliferation
5) Less evident ependymal
differentiation

25
Q

What are the neuronal and glioneuronal tumors?

A

1) Central neurocytoma
2) Ganglioglioma
3) Dysembryoplastic neuroepithelial tumor (DNT or DNET)

26
Q

What is the shared feature of all neuronal and glioneuronal tumors?

A

All of them show neuronal
differentiation = when you stain them with immune stains for neurons, they are going to be positive (synaptophysin positive and neurofilament positive)

27
Q

What is the most common presentation of neuronal and glioneuronal tumors?

A

Seizures

28
Q

Which are more frequent:

Neuronal tumors or Gliomas?

A

Gliomas

29
Q

What are neuronal tumors composed of?

A

Cells with neuronal characteristics and express neuronal markers, such as synaptophysin and neurofilaments.

30
Q

What is a Central neurocytoma, WHO grade 2?

A 
Low-grade neuronal tumor within and adjacent to the
lateral ventricle(s) and/or the third ventricle affecting young adults (NO glial component).
31
Q

What is a Ganglioglioma, WHO grade 1?

A

Well differentiated glioneuronal tumor affecting children and young adults. composed of a mixture of neoplastic ganglion and glial cells, most commonly in
the temporal lobe.

32
Q

What is a Dysembryoplastic neuroepithelial tumor (DNT), WHO grade 1?

A

Low-grade (well differentiated) glioneuronal tumor affecting the cerebral cortex of children and young adults most commonly in the superficial temporal lobe.

33
Q

What are the 3 oncogenic pathways of medullablastomas?

A

1) WNT pathway activation
2) Hedgehog pathway activation
3) MYC overexpression

34
Q

What is the most common CNS neoplasm in immunosuppressed individuals?

A

Primary CNS Lymphoma

35
Q

What is the most common subtype of Primary CNS Lymphoma?

A

Diffuse large B-cell lymphomas

36
Q

How does Primary CNS Lymphoma present as?

A

1) Multiple tumor nodules within the brain parenchyma
2) Well-defined
3) Not as discrete as metastases

37
Q

What is the most common primary CNS germ cell tumor?

A

Germinoma, testicular seminoma

38
Q

Where are primary brain germ cell tumors found?

A

Along the midline, pineal and suprasellar regions

CNS Patho (8 decks)

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