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OMS - 2 Neuro Final > Brain Tumor DSA 2 > Flashcards

Brain Tumor DSA 2 Flashcards

1
Q
A

Meningioma

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2
Q

MC CNS tumor

A

meningioma

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3
Q

Most common symptoms of meningioma

A

seizures

focal neurologic deficits

(they do not herniate)

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4
Q

Patients with meningiomas of the spinal cord have an increased risk of developing _______.

A

bilateral schwannomas of the 8th cranial nerve

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5
Q

most common location for meningiomas

A

parasagittal area over cerebral convexities

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6
Q
A

meningothelial or syncytial meningioma

(epithelial appearance w/indistinct borders; pic for reference to other meningioma histology)

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7
Q
A

Transitional meningioma

(note: whorls of tumor cells some epithelial, some fibroblastic appearing)

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8
Q

_______% of meningiomas are anaplastic

A

1-3

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9
Q

______% of meningiomas are atypical

A

25

(low-grade; increased mitotic index, brain invasion, increased recurrence rate)

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10
Q
A

medulloblastoma

(always arise in the cerebellum)

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11
Q

medulloblastoma tumors arise from ______

A

primitive neuroectodermal cells derived from the external granular cell layer of the cerebellum

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12
Q

medulloblastomas are rapidly growing tumors that can lead to ______.

A

hydrocephalus by compressing the 4th ventricle (red arrow)

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13
Q

All medulloblastomas are WHO grade _______.

A

grade IV

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14
Q

Name 2 subcategories of medulloblastoma based on molecular subtypes

A
  1. Wnt activation of signaling pathway involved in normal cerebellar developmen
  2. Sonic hedgehog activation (SHH)
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15
Q

Dx? Why?

A
  • classic medulloblastoma
  • “small blue cells” (high N/C ratio)

(highly cellular sheet of primitive appearing cells)

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16
Q

Black arrows

A

Homer-wright rosettes

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17
Q

yellow arrow

A

homer-wright rosette: tumor cells surround an anuclear area of cytoplasmic processes from tumor cells

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18
Q

Dx? Why?

A
  • medulloblastoma
  • nests & cords of small round to elongated hyperchromatic tumor cells w/high N/C ratio
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19
Q

Medulloblastomas are undifferentiated tumors that lack _____ or _____ markers.

A
  • neuronal
  • glial
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20
Q

Medulloblastomas are _____ positive.

A

Ki-67: a marker of cellular proliferation

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21
Q

This is a _______ variant of a medulloblastoma.

A

desmoplastic/nodular

(numerous reticulin fibers; dark staining = Ki-67)

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22
Q

Tumor cells are arranged here in thin cords, where would you see this pattern form?

A

periphery of tumor; where it invades the subarachnoid space

(medulloblastoma)

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23
Q

Why do medulloblatoma patients receive radiation to the entire neuraxis?

A

it disseminates to the subarachnoid space and possibly down to the cauda equina (“drop metastases”)

(pic: periphery of tumor that invades the pia and disseminates)

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24
Q

Medulloblastoma survival rate? If it has metastasized?

A
  • 70%
  • 60%
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25
Q

Medulloblastoma WNT-subtype survival rate at 5 years

A

90%

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26
Q
A

Primary CNS lymphoma

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27
Q

Primary lymphoma tumors in the CNS are located in the ______.

A

deep within the cerebral hemispheres (near the lateral ventricles

(often bilateral)

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28
Q

Secondary CNS lymphomas are located ______

A

meninges or CSF

(rarely involve brain parenchyma)

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29
Q

MC CNS neoplasm is found in ______ patients

A

AIDS or immunocompromised

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30
Q

Dx? Why?

A
  • primary CNS lymphoma
  • round & dark blue cells

(no cohesion which is seen in carcinoma)

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31
Q

Primary CNS tumors are _______ (marker) positive.

A

CD20

(vast majority of primary CNS tumors are B-cell lymphomas)

32
Q

Primary lymphoma patients who are immunosuppressed have markers for ______ infection. In non-immunosuppressed patients, they have _______ (mutation).

A
  • EBV
  • PDL1 amplification

(EBV → immunosuppression → lymphoma)

33
Q

PDL1 gene function

A

encodes checkpoint proteins that inhibit T-cell responses

(PDL1 over-expression → decreases T cell response → B-cell lymphoma → primary CNS lymphoma)

34
Q

The yellow arrows show tumor cells that are arranged _______.

A

around blood vessels

(primary CNS lymphoma)

35
Q

________ (histologic characteristic) is highly indicative of primary CNS lymphoma

A

Angiocentric growth pattern (around blood vessels - yellow arrow)

36
Q

Prognosis

A

poor - primary CNS lymphomas are aggressive tumors

37
Q

Dx? Why?

A
  • Germinoma: germ cell tumor
  • Sheets of germ cells w/abundant clear cytoplasm
38
Q

What must be excluded before a diagnosis of primary germ cell tumor?

A

non-CNS primary germ cell tumor (metastasis is common)

39
Q

Dx? Why?

A
  • Von Hippel-Lindau disease
  • many small blood vessels w/RBCs, stromal cells w/pale pink cytoplasm & vacuoles.
40
Q

This is due to a germline mutation in the _____ gene on chromosome ____.

A
  • VHL
  • 3p
41
Q

Von Hippel-Lindau disease causes _______ (tumor)

A

hemangioblastoma

42
Q

In addition to hemangioblastomas (pictured), Von Hippel-Lindau disease also causes _______(2 diz)

A
  1. renal cell carcinoma (clear cell type)
  2. pheochromocytoma
43
Q

VHL gene function

A

encodes tumor suppressor protein pVHL

(MC result : hemangioblastoma - pictured)

44
Q

What happens when pVHL activity is lost?

A

pVHL is a component of ubiquitin ligase lost → HIF1 (hypoxia induced factor) is overactive → VEGF & erythropoietin → excessive blood vessel growth

45
Q

Stromal cells (pink cells w/abundant clear cytoplasm ww/vacuoles) are the neoplastic element of hemangioblastomas. They express _______ (3).

A
  1. erythropoetin
  2. VEGF
  3. inhibin
46
Q

Neoplastic stromal cells seen Von Hippel-Lindau hemangiomas are derived from _______.

A

early mesenchymal progenitor cells

(capable of differentiating into endothelial and hemopoietic cells)

47
Q

Hemangioblastomas are _______ (grade) and _______ (slow/fast) growing tumors.

A
  • WHO grade I
  • slow-growing
48
Q

_______ are exquisitely radiosensitive brain tumors.

A

Medulloblastomas

49
Q

Schwannomas are typically found in _______ (location)

A

Antoni A: cellular (yellow)

Antoni B: myxoid (red)

50
Q

Dx? Why?

A
  • Schwannoma
  • Verocay bodies
51
Q

Verocay bodies

A

tumor cells are palisading around anuclear consisting of cytoplasmic processes from tumor cells.

(Characteristic of schwannoma)

52
Q

define schwannosis

A

schwann cells in the spinal cord

53
Q

meningioangiomatosis

A

proliferation of meningeal cells and blood vessels that grow into the brain

54
Q

Glial hamartia

A

microscopic nodular collections of glial cells at abnormal locations (i.e. superficial and deep layers of cerebral cortex)

55
Q
A

neurofibroma: benign nerve sheath tumor

(more heterogeneous than schwannomas)

56
Q

Neurofibromas composition (4)

A
  1. perineural-like cells
  2. fibroblasts
  3. mast cells
  4. CD34+
57
Q

Neurofibromas are usually benign, but may become malignant in _______ (diz).

A

Neurofibromatosis type 1 (NF1)

58
Q

Neurofibromas arise from ______ (2).

A
  1. peripheral nerves (including spinal nerve roots)
  2. skin
59
Q

3 growth patterns of neurofibromas

A
  1. superficial cutaneous
  2. diffuse
  3. plexiform
60
Q

What is the main distinction between neurofibromas and schwannomas?

A

neurofibromas are dispersed throughout the nerve axons

(schwannomas do not)

61
Q

Why are schwannomas easier to excise than neurofibromas, sparing nerve function?

A

they do not course through the nerve and axons

62
Q

What are the main histologic differences between neurofibromas and schwannomas?

A

neurofibromas: wavy, spindle shaped cells embedded in collagenous stroma & are more homogenous

63
Q

Neurofibromatosis Type 1 affects one in ______ people

A

3,000

64
Q

In addition to neurofibromas, patients with NF1 may also develop _______ (2)

A
  1. optic nerve tumors
  2. pheochromocytomas
65
Q

NF1 patients have ______ (3 symptoms).

A
  1. seizures
  2. nodules of iris (Lisch nodules)
  3. cafe au lait spots
66
Q

NF1 gene function

A

encodes tumor suppressor protein neurofibromin

67
Q

Neurofibromin is a GTPase activating protein (GAP) that binds ______→______.

A

RAS proteins → returning alpha subunit to inactive state›

68
Q
A

plexiform neurofibroma

69
Q

What makes a plexiform neurofibroma unique?

A

involves the individual fascicles of a nerve and extend along the course of the nerve

70
Q

Plexiform neurofibromas may be single lesions or multiple. Single lesions are associated with ______.

A

neurofibromatosis type 1

71
Q
A

Plexiform neurofibroma

(note individual nerve fascicles are expanded by tumor having a myxoid stroma)

72
Q

In plexiform neurofibromas, there is a homozygous alteration of ______ in the schwann cell component.

A

NF1 gene

73
Q

Dx? Why?

A
  • Malignant peripheral nerve sheath tumor (MPNSTs)
  • Spindle-shaped cells
74
Q

Malignant Peripheral Nerve Sheath tumors are typically malignant forms of _______.

A

plexiform neurofibromas

(usually start in large peripheral nerves of chest, abdomen, pelvis, neck or limb girdle)

75
Q

_______% of malignant peripheral nerve sheath tumors arise in patients with NF1.

A

50

76
Q

triton tumor

A

malignant peripheral nerve sheath tumor that exhibits rhabdomyoblastic differentiation

OMS - 2 Neuro Final (36 decks)

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