6/15- Neuro-oncology II Flashcards

1
Q

What is the most common primitive neuroectodermal tumor of the brain?

A

Medulloblastoma

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2
Q

What are the major gliomas?

A
  • Astrocytomas
  • Oligodendroglioma
  • Ependymoma
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3
Q

What is the importance of the 1p/19q co-deletion?

A

Prognostic indicator in oligodendrogliomas

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4
Q

Breakdown of different types of neural tumors (flowchart)

A
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5
Q

What tumors are grade IV?

A
  • PNET: medulloblastoma
  • Glioblsatoma multiforme
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6
Q

What is the grade I tumor primarily focused on in this lecture?

A
  • Juvenile pilocytic astrocytomas
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7
Q

What demographic is most commonly afflicted with medulloblastomas?

2nd most common?

A
  • Common childhood brain tumor
  • Second peak in young adults
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8
Q

What is this? Symptoms?

A

Medulloblastoma

  • Tends to arise in posterior fossa
  • In scan, can see midline hyperdensity
  • In gross specimen, can see tumor draping over pons arising mostly from the ventricles
  • Would initially see midline (vermal) ataxia, followed by limbs
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9
Q

The word “anaplastic” identifies the tumor as what?

A

Grade III

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10
Q

Which of the “big 4” tumors commonly arise in the ventricles?

A
  • Ependymoma
  • Medulloblastoma
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11
Q

What is this? Features?

A

“Small blue cell tumor” (Medulloblastoma)

  • Hypercellularity (too many cells)
  • Little/no cytoplasm
  • Very irregular nuclei (in size and shape); “nuclear pleomorphism” or “nuclear atypia”
  • Tumors can start to show some features indicating that they’re trying to differentiate

(Homer-Wright rosette- central space within circle-forming cell indicating early differentiation of this primitive neuroectodermal tissue. Indicates that this is a medulloblastoma; bottom-left picture)

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12
Q

What is this?

A

Tumor cells that have broken loose and gotten into CSF, “drop metastases

  • Medulloblastoma in CSF (CSF cytology)
  • Can see that they’re even trying to forma Homer-Wright rosette here (upper)
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13
Q

What is this?

A

Glioblastoma multiforme crossing the midline via the corpus callosum

  • No distinct borders; terribly infiltrative
  • Fast-growing
  • These tumors love to cross at the corpus callosum
  • Pattern of growth known as “butterfly glioma
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14
Q

What is this?

A

Glioblastoma multiforme

  • Terribly fast growing!
  • Additional tumor, edema
  • Dark areas are probably necrosis within tumor (growing so fast that they outstrip blood supply)
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15
Q

What is this? Symptoms?

A

Diffuse intrinsic glioma of the brainstem (one of the big 4 childhood tumors)

  • Maybe some cerebellar ataxia
  • Probably will spread to pick off cranial nerves
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16
Q

What is one of the most common gliomas in adulthood?

A

Glioblastoma multiforme

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17
Q

T/F: Glioblastomas can occur in the posterior fossa

A

True; see past picture of DIG in the brainstem

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18
Q

What is this?

A

Glioblastoma multiforme histopathology

  • Hypercellularity

2 key histological features to diagnose glioma as glioblastoma (grade IV):

  • Vascular proliferation- subset of tumor cells have mutated, allowing them to stimulate new vessel growth; very unregulated, no tight junctions (will leak contrast) (top L darker circles and top R)
  • Necrosis- outstripping blood supply (bottom L, bottom R); cells lining up at area of necrosis
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19
Q

What is this?

A

Glioblastoma multiforme

  • Can’t really tell what these are other than just big, bad tumor cells (“monster cells”)
  • Cell on the left is multinucleate
  • To prove it’s a glioma, need to stain with GFAP
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20
Q

What is this?

A

(“Monstrocellular”) Glioblastoma multiforme

  • GFAP imunopositive
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21
Q

What is this?

A

Glioblastoma multiforme with extreme cellular pleomorphism

  • High potential for mutation (radiation/chemo resistance)
22
Q

What is this? Expected symptoms?

A

Anaplastic astrocytoma (found on histology)

  • Squished ventricle
  • Abnormality down in temporal lobe
  • No distinct border; infiltrative
  • Like glioblastoma, but no vascularization and no necrosis

Symptoms:

  • Loss of hearing
  • Memory disturbances
  • Partial complex seizure (often associated with olfactory aura; clouding of consciousness)
23
Q

What is this?

A

Anaplastic astrocytoma

  • Hypercellularity (normal in top left)
24
Q

What is this?

A

Anaplastic astrocytoma

  • Hypercellular
  • Can see 5 mitoses (mitotically active)
  • No vascularity (see normal BV in bottom right), or necrosis
  • May progress to glioblastoma (since mutationally active)
25
Q

Common treatment for glioblastoma (and anaplastic astrocytoma)?

A
  • Debulk via surgery
  • Radiation therapy
  • Tenozolidimide
26
Q

What is this?

A

Anaplastic astrocytoma

27
Q

What is this?

A

Low grade astrocytoma (normal top left)

  • Slight hypercellularity
  • Variation and nuclear morphology (subtly nuclear atypy)
  • If you start to see mitotic activity, then it moves up to anaplasty
  • Low grade astroyctoma may progress into anaplastic and then possibly GBM (glioblastoma multiforme)
  • May have partial complex seizures
28
Q

Survival times (with treatment) for glioblastoma multiforme vs. anaplastic astrocytoma?

A

Glioblastoma multiforme: 12 mo survival on treatment

Anaplastic astrocytoma: 24-36 mo on treatment

29
Q

Treatment for low grade astrocytoma?

A

We don’t really know; don’t want to treat with low therapy with high chance for resistance development

30
Q

What is this?

A

Optic nerve glioma (pilocytic astorcytomas (grade I))

  • Can see faint line of dura
  • Tumor has spread into subarachnoid space
31
Q

What is this?

A

Bilateral optic nerve glioma

  • Pilocytic astroytomas (grade I)
  • Will take vision but typically not kill you (unless in hypothalamus)
32
Q

What is this?

A

Pilocytic astrocytoma

  • Hypercelluarity
  • Thick process (pear-like) -> “pylocytic”
  • Reddish, amorphous, cork-screw like collections (Rosenthal fiber)
33
Q

Characteristics of pilocytic astrocytoma?

A
  • Well circumscribed
  • Rarely infiltrative
34
Q

What is this?

A

Infiltrating glioma

  • Intraparenchymal (intra-axial) contrast enhancing mass without distinct borders
  • Astrocytoma is likely (must differentiate from oligodendroglioma) ?
35
Q

What is this?

A

Oligodendrolgioma, grade II (analogous to astrocytomas grade II)

  • “Fried egg” cells
36
Q

What is seen in the molecular biology of oligodendroglioma?

Features tied in with outcome?

A

Loss of heterozygosity 19q and 1p

  • Recent studies indicate that a clinically significant subset of oligodendroglial tumors show co-deletion on chromsomes 1p and 19q
  • Response to therapy is much better in pts with co-deletion 1p/19q
37
Q

What is the difference is seen in the mean survival of pts with different 1p/19q characteristics?

A
  • Not co-deleted: 12 mo (1 yr)
  • Co-deleted: 124 mo (10 yrs)
38
Q

Treatment for oligodendroglioma?

A

Combination chemo-therapy with regimens such as PCF or single drug temozolamide with/without radiation (XRT)

(PCV: procarbazine; CCNU and vincristine)

39
Q

What is this?

A

Ependymom

  • Intraventricular or in association with central canal of SC
  • 4th ventricular tumor on L picture (obstructive symptoms)
40
Q

What is a key difference in medulloblastomas and epndymomas that both can arise in 4th ventricles?

A
  • Medulloblastoma is way faster-growing
  • Ependymoma tumors will try to create little ventricles
41
Q

Which tumors are typically more easily removed (surgically resectable)?

A
  • Pilocytic astrocytoma
  • Ependymoma
42
Q

What is this?

A

Ependymoma in 4th ventricle

43
Q

What is a risk of both medulloblastomas and ependymomas?

A

Dissemination through CSF (since in contact); more commonly happens with medulloblastomas

44
Q

What is this?

A

Epnedymoma disseminated through ventricular system

45
Q

What is this?

A

Ventricular tubule with a lumen (tiny ventricle)

  • Ependymoma (?)
46
Q

What is this?

A

Perivascular paucinuclear zone with surrounding cells

  • Very commonly seen in ependymomas
47
Q

What is seen ultrastructurally in ependymomas?

A
  • Tight junctions
  • Cilia
48
Q

What is this?

Grade?

Origin?

Symptoms?

A

Colloid cyst of the 3rd ventricle (grape-like structure)

  • Grade I
  • Arises from ependyma (modified- ependyma)
  • Positional hydrocephalus may result in sudden death (but typically, doesn’t cause too much trouble)
49
Q

What is this?

A

Colloid cyst of the 3rd ventricle

50
Q

What is this?

A

Central neurocytoma

  • Intraventricular mature neuronal tumor
  • (“cytoma” typically means more mature)
51
Q

What is this?

A

Central neurocytoma

  • Clear halo cells-oligo-like
  • Neuronal features by EM and immunohistochemistry
52
Q

4 main childhood tumors?

A
  • Medulloblastoma
  • ?
  • Ependymoma
  • Pontine glioma (diffuse intrinsic glioma of pons)