Tumors of the Nervous System part 2 Flashcards

1
Q

What age group do Embryonal tumors usually affect?

A

KIDS

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2
Q

What is the most common type of Embryonal tumor in kids?

A

Medulloblastoma

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3
Q

What grade is a Medulloblastoma and where does it usually sit anatomically?

A

Grade 4

= Sits midline cerebellum and occludes CSF flow

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4
Q

There are 4 unique subtypes of Medulloblastomas based on molecular changes. List them and denote which has the best/worst prognosis.

A
  1. WNT chr.6/beta catenin = best prognosis
  2. SHH
  3. Group 3 MYC and 17(I17Q) = worst prognosis
  4. Group 4 MYCN and I17Q
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5
Q

There are 4 unique subtypes of Medulloblastomas based on molecular changes. List them and denote which has the best/worst prognosis.

A
  1. WNT chr.6/beta catenin = best prognosis
  2. SHH
  3. Group 3 MYC 17(I17Q) = worst prognosis
  4. Group 4 MYCN I17Q
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6
Q

Where can Medulloblastomas spread?

A

Drop metz

= Disseminates via CSF to cauda equine

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7
Q

What is a good feature about treating Medulloblastomas?

A

Exquisitely Radiosensitive!

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8
Q

Medulloblastomas involve anaplastic cells, abundant mitoses and what type of Rosettes?

A

Homer-Wright Rosettes

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9
Q

Another type of Embyronal tumor is an Atypical Teratoid/Rhabdoid Tumor (AT/RT). What is its grade, location and chromosome mutation?

A

Grade 4
Posterior fossa
Chromosome 22

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10
Q

What cells are present and what color is the cytoplasm with an Atypical Teratoid/Rhabdoid Tumor (AT/RT)?

A

Rhabdoid cells with eosinophilic cytoplasm

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11
Q

What 2 molecules are present with an Atypical Teratoid/Rhabdoid Tumor?

A

EMA

Vimentin

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12
Q

What patients often get Primary CNS Lymphomas?

A

AIDs patients

– Latent EBV infection

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13
Q

What cell origin are Primary CNS Lymphomas?

A

B cell origin (CD20)

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14
Q

Where do Primary CNS Lymphomas accumulate and what word describes their cells?

A

Accumulate around vessels

– hooping

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15
Q

Where do Germ Cell Tumors occur?

A

Along MIDLINE

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16
Q

What causes a Meningioma?

A

Radiation induced

17
Q

How do Meningiomas look and what do they do anatomically?

A

Bosselated (large bumps) dural mass –> compress brain and penetrate bone

18
Q

What tumor is a dural mass that compresses brain and penetrates bone often from radiation?

A

Meningioma

19
Q

What gene mutation is often associated with a Meningioma?

A

NF2 – higher grade with multiple tumors

20
Q

What histo manifestations do Meningiomas have? (2)

A
  1. Calcified Psammoma bodies

2. Whorled clusters of monotonous cells

21
Q

What histo manifestations do Meningiomas have? (2)

A
  1. Calcified Psammoma bodies

2. Whorled clusters of monotonous cells

22
Q

What receptor do Meningiomas express which causes them to grow rapidly during pregnancy?

A

Progesterone receptors

23
Q

Metastatic Brain Tumors are usually from what 4 sources?

A

Lung
Breast
Melanoma
Choriocarcinoma

24
Q

Where do Metastatic Brain Tumors go?

A

Meninges or intraparenchymal (gray-white junction)

25
Q

What can be symptoms of a Metastatic Brain Tumor?

A

Hemorrhage

Meningeal Carcinomatosis = neuropathies from nerve compression

26
Q

What are 3 common Familial Tumor Syndromes?

A

Von Hippel Lindau

Neurofibromatosis 1 and 2

27
Q

The Familial Tumor Syndromes in this deck are inherited how?

A

Autosomal Dominant

28
Q

What 4 things will present with Von Hippel Lindau Syndrome?

A

Hemangioblastoma of CNS
Renal cell carcinoma
Polycythemia
Pheochromocytoma

29
Q

What 4 things will present with Von Hippel Lindau Syndrome?

A

Hemangioblastoma of CNS
Polycythemia
Renal Cell Carcinoma
Pheochromocytoma

30
Q

Neurofibromatosis 1 mutation

A

NF1 - 17Q11 = Neurofibromin

31
Q

What 3 things will present with Neurofibromatosis 1?

A

Neurofibromas
Eye lesions
Cafe au lait spots

32
Q

What 3 things will present with Neurofibromatosis 1?

A

Neurofibromas
Eye lesions
Cafe au lait spots

33
Q

Neurofibromatosis 2 mutation

A

NF2 - 22Q12 = Merlin

34
Q

What will present with Neurofibromatosis 2?

A

Bilateral Schwannomas => CN8 affected

35
Q

What will present with Neurofibromatosis 2?

A

Bilateral Schwannomas => CN8