Neuro-oncology Flashcards

1
Q

Patient presents with opsoclonus myoclonus syndrome (spontaneous rapid, irregular and high-amplitude conjugate eye movements that occur in all direction). What are the possible diagnosis?

A

Neuroblastoma

Paraneoplastic
Breast Cancer (Anti-Ri/ANNA-2), Ovarian and Small Cell Lung Cancer
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2
Q

What is the management for neuroblastoma?

A

ACTH

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3
Q

What are examples of diffuse astrocytoma?

A
fibrillary
gemistocytic
protoplasmic
small cell
giant cell
epithelioid
granular cell
glioblastoma with oligodendroglioma component
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4
Q

What type of diffuse astrocytoma presents with elongated hyperchromatic nuclei, scant cytoplasm, and the presence of a fibrillary background?

A. Gemistocytic Astrocytoma
B. Fibrillary Astrocytoma
C. Proteoplasmic Astrocytoma
D. Pleomorphic Xanthoastrocytoma

A

B. Fibrillary Astrocytoma

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5
Q

What type of diffuse astrocytoma presents with rounded cells with prominent eosinophilic cytoplasm?

A. Gemistocytic Astrocytoma
B. Fibrillary Astrocytoma
C. Proteoplasmic Astrocytoma
D. Pleomorphic Xanthoastrocytoma

A

A. Gemistocytic Astrocytoma

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6
Q

What type of diffuse astrocytoma presents with cells with oval-shaped nuclei and wispy cobweb-like processes?

A. Gemistocytic Astrocytoma
B. Fibrillary Astrocytoma
C. Proteoplasmic Astrocytoma
D. Pleomorphic Xanthoastrocytoma

A

C. Proteoplasmic Astrocytoma

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7
Q

What is the most common intracranial brain tumor?

A

Meningioma

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8
Q

Buzzwords

Perinecrotic Pseudopalisading

A

Glioblastoma

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9
Q

What are the histopathologic findings in Glioblastoma?

A

nuclear atypia
mitoses
endothelial hyperplasia and necrosis
Perinecrotic Pseudopalisading

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10
Q

Buzzwords

Cells with “fried egg” appearance

A

oligodendrogliomas

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11
Q

Buzzwords

Perivascular pseudorosettes

A

Ependymoma

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12
Q

Buzzwords

Homer-Wright rosettes

A

Medulloblastoma

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13
Q

Buzzwords

Rosenthal fibers

A
  • Alexander’s disease
  • Pilocytic astrocytoma
  • Pleomorphic xanthoastrocytoma
  • Chronic reactive gliosis.
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14
Q

This is the most common glioma in children. Most commonly seen in the cerebellum. Imaging findings would show cystic lesions with gadolinium enhancing mural nodule. However, in the hypothalamus and optic nerve they are solid looking

A

Pilocytic Astrocytoma WHO Grade I

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15
Q

Which type of Pilocytic Astrocytoma most commonly associated with Neurofibromatosis Type I?

A

Optic Nerve Glioma

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16
Q
What is the most common location of pleomorhic xanthoastrocytoma (PXA)?
A. Frontal Lobes
B. Temporal Lobes
C. Brainstem (pons)
D. Cerebellum
A

B. Temporal Lobes

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17
Q

What are the histopathologic findings of pleomorphic xanthoastrocytoma?

A

• pleomorphic astrocytes arranged in fascicles, with intercellular reticulin deposition, mesenchymal-like cells, and multinucleated giant cells.

  • There are lipidized astrocytes
  • Eosinophilic granular bodies are typically seen, and Rosenthal fibers may be seen in the periphery of the lesion.
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18
Q

What are the histopathologic findings of subependymal giant cell astrocytoma?

A
  1. glioneuronal appearance, and the cells are packed in fascicles and around blood vessels, giving the appearance of perivascular pseudorosettes
  2. Immunoreactivity for both GFAP and neuronal markers
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19
Q

In oligodendroglioma, there is the deletion of 1p/19q. What does this imply?

A

Deletion of 1p/19q is associated with prolonged survival and better response to treatment.

Chromosome 1p
• predictive of high degree of responsiveness to PCV chemotherapy regimen

Chromosome 19q
(+) longer survival
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20
Q
What is the most common location of oligodendroglioma?
A. Frontal Lobes
B. Temporal Lobes
C. Brainstem (pons)
D. Cerebellum
A

A. Frontal Lobes

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21
Q

Is oligodendroglioma, GFAP positive or negative?

A

GFAP negative except menigemistocystic or gliofibrillary oligodendrocytes are present (GFAP Positive)

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22
Q

Oligodendroglioma’s borders are well-defined and non-infiltrating. True or False?

A

False. Oligodendrogliomas are diffuse glioma which are infiltrating with ill-defined borders.

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23
Q

What are the histopathologic findings in pilocytic astrocytoma?

A
  1. Presence of biphasic pattern with compact fibrillar and loose microcytic areas
  2. Presence of Rosenthal fibers located in the compact region. There is also eosinophilic granular bodies seen.
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24
Q

Buzzwords

Eosinophilic Granular Bodies

A
  • Pleomorphic xanthoastrocytoma
  • Pilocytic Astrocytoma
  • Gangliogliomas
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25
Q

What are the histopathologic findings in oligodendroglioma?

A

hypercellular tumor with uniformly rounded nuclei and clear pericellular haloes, giving it a “fried egg” appearance. There are also capillaries with a “chicken wire” appearance

Clear halos is caused by a delayed fixation artifact

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26
Q

What are the histopathologic findings in ependymoma?

A
  1. arranged in sheets of spindled cells with round nuclei and small nucleoli
  2. perivascular pseudorosettes, in which the cells surround blood vessels
  3. True ependymal Rosettes (Flexner Wintersteiner) - cells surround clear space
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27
Q

Tumor that is reactive with synaptophysin, a marker of neuronal differentiation and which detects neuronal vesicle proteins.

A. Oligodendroglioma
B. Neurocytoma
C. Ependymoma
D. Meningioma

A

B. Neurocytoma

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28
Q

Where is the most common location of colloid cyst?

A

Anterior 3rd Ventricle

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29
Q

What is the histopathologic finding of colloid cyst?

A

hin-walled lining and contain thick and cloudy gelatinous fluid. Microscopically, there is a single layer of columnar ciliated or goblet cells.

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30
Q

Where is the most common location of dermoid cyst?

A

cerebellar vermis

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31
Q

In HIV and immunocompromised patients, primary CNS Lymphoma is frequently associated with Epstein–Barr virus (EBV). True or False?

A

True

PCNSL can also occur in immunocompetent hosts; however, this is rare, and there is a lower association with EBV.

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32
Q

Buzzwords

Verocay Bodies

A

Vestibular Schwannoma

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33
Q

What are the common locations of these cysts?

Epidermoid

A

Epidermoid- CP-Angle

34
Q

Among intracranial tumors, which 2 have female predominance?

A

Meningioma

35
Q

Match the disease with associated CNS tumor.

Neurofibromatosis 2

A- Meningioma
B- Lhermitte Duclos
C- Medulloblastoma
D- SEGA
E- Hemangioblastoma
A

A- Meningioma

36
Q

Match the disease with associated CNS tumor.

Tuberous Sclerosis

A- Meningioma
B- Lhermitte Duclos
C- Medulloblastoma
D- SEGA
E- Hemangioblastoma
A

D- SEGA

37
Q

Match the disease with associated CNS tumor.

Von Hippel Lindau

A- Meningioma
B- Lhermitte Duclos
C- Medulloblastoma
D- SEGA
E- Hemangioblastoma
A

E- Hemangioblastoma

38
Q

Most serious and most common primary malignant tumor

A

Glial Cell Origin

39
Q

Meningioma is a disease of the dura and arachnoid. Which is a pial disease?
A. CNS Lymphoma
B. CNS Leukemia
C. Sarcoidosis

A

B. CNS Leukemia

40
Q

What are the 4 malignancies with tendency to metastasize to CNS?

A

Lung
Breast
Melanoma
Renal Cell CA

41
Q
A patient presents with solitary metastatic brain lesion. You decide to screen for the following primary malignancy EXCEPT:
A. Breast ca
B. Melanoma
C. Thyroid ca
D. Renal cell ca
A

B. Melanoma

*Remember paired organs present as solitary tumors

42
Q

Identify 5 malignancies that can present as intracranial hemorrhage:

A
Lung
Melanoma
Thyroid
Renal Cell CA
Chorioepithelioma
43
Q

What are the common locations of these cysts?

Arachnoid

A

Arachnoid- Sylvian Fissure

44
Q

Among intracranial tumors, which 2 have female predominance?

A

Meningioma
Glioma of Optic Chiasm

  • Everthing else, male predominance
  • Schwannoma male = female
45
Q

When do the MOST SERIOUS RT-related injury occur?
A. ACUTE - when there are signs of increased ICP
B. EARLY DELAYED- when there is increased tumor mass on MRI
C. LATE DELAYED- when there is necrosis of white matter

A

C. LATE DELAYED- when there is necrosis of white matter

*In Pedia, LAte Delayed effects include PANHYPOPITUITARISM which causes growth retardation

46
Q

Your patient who underwent RT developed severe headache and aphasia. Repeat MRI showed gyriform enhancement and focal narrowing of blood vessels. What is your Diagnosis? Treatment?

A

SMART

Steroids

47
Q
Which autoantibody is not associated with Lung ca?
A. Anti-Ri
B. Anti-NMDA
C. Anti-GAD
D. Anti-CRMP5
A

B. Anti-NMDA

Anti-NMDA (ovarian CA)

48
Q

Your patient underwent transphenoidal excision of pituitary tumor. Unfortunately, like 15% of GH secreting tumors and prolactinomas, the tumor recurred after 1 year. You offered RT. An alternative primary treatment is a stereotactic radiosurgery. Give 2 conditions where this may be applicable.

A

Vision not threateneed

No urgent need for surgery

Advantage: Tumor recurrence is rare

Disadvantage: Obtained Several Months

49
Q

If you’re only allowed to do 3 hormone tests for a pituitary adenoma, which hormones will most likely yield an abnormal result?

A

Prolactin, GH, TSH

*chromophobe or acidophils, most common tumor of pituitary adenoma

vs Basophilic: ACTH, B, lipotopin, LH, FSH
*Frequency: Chromo > acido >basophil (5:4:1)

50
Q

What is the most sensitive test to check for presence of Acoustic Schwannoma?

A

BAER

51
Q

According to Adams, ependymomas occur twice as frequently as oligodendrogliomas. True or false?

A

False

GBM 80%
Low grade glioma 25-30%
Oligodendroglioma 5-7%
Ependymoma 6%

52
Q

Match the disease with associated CNS tumor.

Cowden Syndrome
Neurofibromatosis 2
Tuberous Sclerosis
Von Hippel Lindau
Gorlin and Turcot syndrome
A- Meningioma
B- Lhermitte Duclos
C- Medulloblastoma
D- SEGA
E- Hemangioblastoma
A
Cowden Syndrome = B
Neurofibromatosis 2 = A
Tuberous Sclerosis = D
Von Hippel Lindau = E
Gorlin and Turcot syndrome = C
53
Q

If you were a choroid plexus papilloma, where would you most likely be found?
A. Lateral vent
B. 3rd vent
C. 4th vent

A

A. Lateral vent

Ependymoma 4th vent is most common cerebral site

54
Q
Cranial nerve palsy suggestive of lymphoma?
A. CN 5
B. CN 6
C. CN 7
D. CN 8
A

D. CN 8

55
Q

What is the gene defect associated with Meningioma?

A

Merlin Gene (Neurofibromatosis 2) chromosome 22q

56
Q
What is the most common initial manifestation of primary and metastatic neoplasm?
A. Nocturnal/ first awakening headache
B. First seizure
C. Vomiting and Dizziness
D. Mental Asthenia
A

B. First seizure

57
Q
A cancer patient will want which type of mutation for better prognosis?
A. Deletion on chromosome 17p
B. MYC N oncogene amplification
C. IDH 1 and 2 mutation
D. Chrom 1p,19q duplication
E. C&D
A

C. IDH 1 and 2 mutation

A- Deletion on Chromosome 17p will inactivate Tumor Suppressor Gene
B-MYC N oncogene amplification will result in aggressive clinical course
C. IDH 1 and 2 mutation will cause less tumor progression
D. Chromosome 1p, 19q DELETION (not duplication) will offer good response to chemotherapy

58
Q
  1. True/False regarding causes of edema:
    VASOGENIC: tumor growth
    INTERSTITIAL: Hypoxic-ischemic
    CYTOTOXIC: obstructive hydrocephalus
A

VASOGENIC: tumor growth =TRUE
INTERSTITIAL: Hypoxic-ischemic =FALSE
CYTOTOXIC: obstructive hydrocephalus =FALSE

59
Q

When you see this finding on one eye (picture of papilledema) plus optic atrophy on the other eye, what is your diagnosis?

A

Foster-Kennedy Syndrome

60
Q

Where is the most common location of ependymoma?

A. Cerebellar Vermis
B. Frontal Lobe
C. Parietal Lobe
D. 4th Ventricle

A

D. 4th Ventricle

61
Q

Where is the most common location of oligodendroglioma?

A. Cerebellar Vermis
B. Frontal Lobe
C. Parietal Lobe
D. 4th Ventricle

A

B. Frontal Lobe

62
Q

Where is the most common location of medulloblastoma?

A. Cerebellar Vermis
B. Frontal Lobe
C. Parietal Lobe
D. 4th Ventricle

A

A. Cerebellar Vermis

63
Q

What other tumors may show Homer-Wright Rosettes?

A

Medulloblatoma
Pineoblastoma
PNET
Olfactory Neuroblastoma

64
Q

What is the pathophysiology of papilledema in brain tumors?

A. Due to Elevation of ICP and perioptic pressure
B. Impairs axonal transport in the optic nerve
C. Impairs venous drainage from optic nerve head and retina
D. All of the above

A

D. All of the above

65
Q

Why is white matter more involved than grey matter in during edema formation?

A

White matter is affected due to its loose structural organization –> less resistance to fluid under pressure

66
Q

Tumor growth

What kind of edema?

A. Vasogenic
B. Cytotoxic
C. Interstitial

A

A. Vasogenic

67
Q

Lead encephalopathy

What kind of edema?

A. Vasogenic
B. Cytotoxic
C. Interstitial

A

A. Vasogenic

68
Q

Malignant hypertension
What kind of edema?

A. Vasogenic
B. Cytotoxic
C. Interstitial

A

A. Vasogenic

69
Q

Osmotic dysequilibrium syndrome of hemodialysis
What kind of edema?

A. Vasogenic
B. Cytotoxic
C. Interstitial

A

B. Cytotoxic

70
Q

Inappropriate secretion of antidiuretic hormone
What kind of edema?

A. Vasogenic
B. Cytotoxic
C. Interstitial

A

B. Cytotoxic

71
Q

Hypoxic-ischemic injury
What kind of edema?

A. Vasogenic
B. Cytotoxic
C. Interstitial

A

B. Cytotoxic

72
Q

Acute hepatic encephalopathy
What kind of edema?

A. Vasogenic
B. Cytotoxic
C. Interstitial

A

B. Cytotoxic

73
Q

Acute hypoosmolality of plasma
What kind of edema?

A. Vasogenic
B. Cytotoxic
C. Interstitial

A

B. Cytotoxic

74
Q

What is the pathophysiology of vasogenic edema?

A. Defect in the gap endothelial cell junctions
B. Defect in the tight endothelial cell junctions
C. Shift of water from extracellular to intracellular compartment
D. Failure of ATP dependent sodium pump within cells (Na-K-ATPase Pump)
E. Ependymal lining is disrupted

A

B. Defect in the tight endothelial cell junctions

75
Q

What is the pathophysiology of interstital edema?

A. Defect in the gap endothelial cell junctions
B. Defect in the tight endothelial cell junctions
C. Shift of water from extracellular to intracellular compartment
D. Failure of ATP dependent sodium pump within cells (Na-K-ATPase Pump)
E. Ependymal lining is disrupted

A

E. Ependymal lining is disrupted

76
Q

What is the pathophysiology of cytotoxic edema?

A. Defect in the gap endothelial cell junctions
B. Defect in the tight endothelial cell junctions
C. Shift of water from intracellular to extracellular compartment
D. Failure of ATP dependent sodium pump within cells (Na-K-ATPase Pump)
E. Ependymal lining is disrupted

A

D. Failure of ATP dependent sodium pump within cells (Na-K-ATPase Pump)

77
Q

How to differentiate cyctotoxic from vasogenic edema based on imaging?

A
DWI
Vasogenic Edema:
Increased diffusivity
Cytotoxic Edema
Decreased diffusivity
78
Q

What is the effect of CO2 in the brain?

A. Vasodilation
B. Vasoconstriction

A

A. Vasodilation

79
Q

Most common solid tumor of childhood.

A. Neuroblastoma
B. Ependymoma
C. Medulloblastoma
D. Metastatic Carcinoma

A

A. Neuroblastoma

80
Q

N-MYCN amplification

good or poor prognosis?

A

Poor prognosis

81
Q

WNT mutation

good or poor prognosis?

A

Good prognosis