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Brain Power: Adam and Victor's Neurology > Tumors > Flashcards

Tumors Flashcards

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1
Q

Indicate the WHO grade of the ff tumors:

  1. SEGA
  2. Diffuse astrocytoma
  3. Anaplastic astrocytoma
  4. Pineoblastoma
  5. Subependymoma
  6. Ependymoma
  7. Ganglioglioma
  8. Dysembryoplastic neuroepithelial tumor
A
  1. SEGA 1
  2. Diffuse astrocytoma 2
  3. Anaplastic astrocytoma 3
  4. Pineoblastoma 4
  5. Subependymoma 1
  6. Ependymoma 2
  7. Ganglioglioma 1
  8. Dysembryoplastic neuroepithelial tumor 2
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2
Q
  1. PNET
  2. Atypical meningioma
  3. Hemangiopericytoma
  4. Craniopharyngioma
A
  1. PNET 4
  2. Atypical meningioma 2
  3. Hemangiopericytoma 2
  4. Craniopharyngioma 1
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3
Q

What kind of edema is seen in:

  1. Lead encephalopathy
  2. Malignant hypertension
A

Vasogenic

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4
Q

T or F
The looser structure of white matter makes it more vulnerable to the effects of fluid under pressure such as in vasogenic edema

A

T

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5
Q

What are the 3 mechanisms of vasogenic edema?

A
  1. Loose tight endothelial junctions
  2. Active vesicular transport
  3. Protease induced protein fragments that may generate osmotic effect
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6
Q

What kind of edema?

  1. SIADH
  2. Hepatic encephalopathy
  3. Osmotic disequilibrium syndrome of hemodialysis
A

Cytotoxic edema`

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7
Q

What is the cellular mechanism behind cytotoxic edema?

A

Failure of the ATP dependent sodium pump within cells. Sodium accumulates within cells and water follows

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8
Q

What does cytotoxic edema look like on DWI compared to vasogenic edema

A

C: reduced diffusivity
V: elevated diffusivity

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9
Q

T or F D5 NS may be given to patients with increased ICP

A

T

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10
Q

What is the MOA of steroids for decreasing ICP?

A

Reduce endothelial cell permeability and shrink normal tissue

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11
Q

What herniation is associated with: tonic extension and arching of the neck and back and extension and internal rotation of the limbs with respiratory disturbances, cardiac irregularity and loss of consciousness?

A

Cerebellar herniation

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12
Q

What are signs of upward herniation?

A

Decerebrate posturing

Pupils: miosis –> anisocoria

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13
Q

What are the characteristics of headache that make it likely to be from a tumor?

A

Nocturnal or on first awakening
Vomiting occuring at the peak of the head pain
Deep NONpulsatile quality

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14
Q

What % of GBM is multicentric?

A

3-6%

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15
Q

Identify which of the following factors bode for a good prognosis in GBM patients:

  1. IDH1 and IDH2 mutations
  2. MGMT methylation
A
  1. Good

2. Good

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16
Q

Which are associated with GBM from degeneration?

  1. p53 mutation
  2. EGFR amplification
  3. Younger age group
  4. IDH1 and IDH2 mutation
A

1, 3, 4

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17
Q

What is the 1 year survival rate of GBM?

A

<20%

Only 10% live beyond 2 years

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18
Q

How to give TMZ for GBM using the Stupp protocol?

A

Radiotherapy plus continuous daily temozolomide (75 mg per square meter of body-surface area per day, 7 days per week from the first to the last day of radiotherapy), followed by six cycles of adjuvant temozolomide (150 to 200 mg per square meter for 5 days during each 28-day cycle)

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19
Q

What is the median survival on patients on RT + TMZ?

A

14.6 months compared to 12.1 months on radiation alone compared to 7-9 months without ANY treatment

IN ADDITION THE 2 YEAR SURVIVAL RATE WAS MORE THAN DOUBLED FROM 10.4 TO 26.5%

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20
Q

What is the most common type of astrocytoma?

A

Well differentiated fibrillary astrocytes (Grade 2)

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21
Q

What proportion of patients with astrocytoma present with seizures?

A

2/3

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22
Q

T or F Early RT in low grade glioma increases PFS but not OS?

A

T

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23
Q

T or F. There is scant enhancement of gliomatosis cerebri differentiating it from CNS lymphoma.

A

T

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24
Q

What histologic features differentiate Oligodendroglioma?

A

Small round nucleus with a halo of unstained cytoplasm

Microscopic calcifications

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25
Q

What does a 1p19q deletion imply for oligodendroglioma?

A

1p responsiveness to PCV

19q associated with longer survival

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26
Q

What regimen is used for chemo of oligdendroglioma?

A

Procarbazine, Cyclophosphamide, Vincristine PCV or

Temozolomide

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27
Q

What type of ependymoma is exclusively located in the filum terminale?

A

Myxopapillomatous

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28
Q

What is the most common glioma of the cord?

A

Ependymoma

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29
Q

What is the most common cerebral site of ependymoma?

A

4th ventricle– 70% of arise here

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30
Q

What is the most common acquired genetic defect of meningiomas?

A

Truncating mutations in the NF2 gene (merlin) on chromosome 22

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31
Q

Where do meningiomas originate?

A

Arachnoid meningothelial cells REMEMBER THAT THESE CELLS ARE ALSO WITHIN THE CHOROID PLEXUS therefore intraventricular meningiomas can also exist

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32
Q

What is the most common form of meningioma?

A

Meningothelial or syncitial form

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33
Q

What tumors calcify?

A

craniopharyngioma (adenomatous variety shows stippled and peripheral, in papillary variant calcification is rare).
meningioma
primitive neuroectodermal tumour (PNET)
chordoma
central neurocytoma (punctuate calcification)
ependymoma (coarse calcification)
subependymoma
ganglioglioma
intracranial dermoid
pineoblastoma (exploded calcification)
pineocytoma (exploded calcification)
pineal germinoma
atypical teratoid /rhabdoid tumour
intracranial teratoma (clump like calcification)
oligodendroglioma (central or peripheral ribbon like calcification)

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34
Q

What are the malignant cells in primary CNS lymphoma?

A

Diffuse large cell type. B lymphocytes.

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35
Q

What percentage of primary CNS lymphoma would have ocular involvement?

A

10-20% BUT 2/3 of patients with ocular lymphoma would have cerebral involvement

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36
Q

Meningeal and cranial nerve lymphoma with similar histologic characteristics to primary CNS lymphoma that are actually complications of chronic lymphatic leukemia is called?

A

Richter transformation

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37
Q

T or F. Primary CNS lymphoma usually presents with infiltrating NECROTIC, HEMORRHAGIC periventricular masses.

A

F. NON necrotic NON hemorrhagic

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38
Q

T or F Lymphoma is associated with EBV in immunocompromised patients

A

T

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39
Q

What is the median survival of primary cns lymphoma patients on methotrexate with radiation?

A

4 years. Give methotrexate 3.5g per m2

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40
Q

What kinds of cancers have a tendency to send mets to the posterior fossa?

A

Pelvis and colon

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41
Q

What are the common tumors that send mets to the skull and dura?

A

Breast: systemic circulation
Prostate: Batsons
Multiple myeloma

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42
Q

What are the most common tumors to send mets to the brain parenchyma?

A 
Lung
Breast
Melanoma
Colon and Rectum
Kidneys
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43
Q

T or F. Mets from the prostate, esophagus, oropharynx commonly metastasize to the brain.

A

F

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44
Q

What tumors ARE PRONE to send mets to the brain?

A

Melanoma 75%
Testicular 55%
Bronchial CA 35% (of which 40% are Small cell CA)

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45
Q

What intracranial mets are often hemorrhagic?

A

Melanoma
Chorioepithelioma
Lung, thyroid, kidney

BUT LUNG CA IS STILL THE MOST COMMON METASTATIC TUMOR TO BLEED ON ACCOUNT ITS RELATIVE FREQUENCY

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46
Q

How to give RT to brain mets?

A

WBRT over 2 weeks 10 doses of 300 cGy each

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47
Q

What are the prerequisites to excision of a metastatic tumor?

A
  1. Single
  2. Growth of primary controlled
  3. Systemic mets controlled
  4. Accessible location in non-eloquent area
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48
Q

What is the average period of survival in patients with brain metastases?

A

6 months

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49
Q

What tumor may have carcinomatous meningitis as its first presentation?

A

Gastric

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50
Q

What are the 3 cardinal symptoms of carcinomatous meningitis?

A
  1. Polyradiculopathy
  2. Cranial nerve palsies
  3. Confusional state
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51
Q

What cases of carcinomatous meningitis respond relatively well to RT + MTX?

A

breast and lymphoma

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52
Q

What proportion of patients with leukemia will involve the CNS?

A

1/3

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53
Q

How to differentiate tumor mets from necrotizing leukoencephalopathy?

A

mets enhance!

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54
Q

What is the most common neurologic complication of all types of lymphoma?

A

Extradural compression of the spinal cord

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55
Q

Hodgkin lymphoma meningeal involvement would show what on LP?

A

Eosinophilic pleocytosis

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56
Q

What cranial nerve is involved with meningeal dissemination of NHL?

A

CN 8

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57
Q

Differentiate the location of medulloblastoma in children and in adults.

A

Children: cerebellar vermis
Adults: Lateral cerebellum and cerebrum

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58
Q

What is the 5 year survival rate in medulloblastoma with surgery radiation and chemo?

A

80%

59
Q

What is the most common solid tumor of childhood?

A

Neuroblastoma

60
Q

In cases of cerebellar foraminal herniation due to a tumor the occiput of the head moves where?

A

away from the side of the tumor… head tilt

61
Q

What are the locations of choroid plexus papilloma?

50:10:40 ratio

A

Lateral:3rd:4th ventricles

62
Q

What hematologic abnormality is associated with Hemangioblastoma?

A

Polycythemia on account of elaboration of erythropoietic factor

63
Q

What comprises VHL?

A 
Hemangioblastoma
Retinal angioma
Hepatic or pancreatic cyst
Pheochromocytoma
Renal cell carcinoma
64
Q

What are the 4 types of pineal region tumors and which one is most prevalent?

A
  1. Germinoma MOST COMMON 50%
  2. Non-germinomaous germ cell tumor
  3. Pinealoma
  4. Glioma
65
Q

What serum levels are elevated with a germ cell tumor of the pineal region?

A

Beta HCG: Choriocarcinoma

AFP: Endodermal sinus tumors (Yolk sac tumor) and teratoma

66
Q

What cerebellar tumor has the tiger stripe appearance due to layers of dysmorphic cerebellar cells?

A

Dysplastic gangliocytoma of Lhermitte duclos disease

67
Q

What does dysembryoplastic neuroepithelioma tumor look like on imaging?

A

nodule or small cyst that is juxtacortical or intracortical

68
Q

What other nerves are involved by schwannoma?

A

besides 8, 5 and spinal nerve roots

69
Q

T or F. Acoustic neuroma always arises from the acoustic portion of the CN 8

A

F. Vestibular

70
Q

What is the most common early symptom of acoustic neuroma?

A

Hearing loss

71
Q

What test is most sensitive to the presence of acoustic neuroma?

A

BAER

72
Q

Craniopharyngioma originates from?

A

Adenohypophysial diverticulum AKA Rathke’s pouch

73
Q

What is the origin of glomus jugulare tumors?

A

Non chromaffin paraganglioma cells

74
Q 
The ff are manifestations of what kind of tumor
Unilateral atrophy of the tongue
Vascular polyp at the EAM
Deafness
Facial palsy
Dysphagia
Self audible bruit
A

Glomus jugulare tumor

75
Q

The syndrome of the retroparotid space (sympathetic, 9, 10, 11, 12) palsy with PAINLESS MASS AT THE SIDE OF THE NECK BELOW THE ANGLE OF THE JAW is seen in what tumor?

A

Paranglioma or Carotid body tumor

MAY BE AN ETIOLOGY OF TIA

76
Q

Chromophone and acidophil cells produce what hormones? (Pit Ad)

A

Prolactin, GH, TSH

PGT!

77
Q

What are the most common hormones secreted by a Pit Ad?

A

PGAT

Prolactin 60-70%, GH 10-15%, ACTH, TSH

78
Q

T or F
Pit Ad: Affects upper visual fields first
Craniopharyngioma: Affects inferior fields first

A

T

79
Q

What are the manifestations of hypothalamic compression?

A

DI
Temperature dysregulation (Anterior part is for cooling)
Sleep abnormalities (Destruction of lateral part results in narcolepsy)
Apetite (Medial part is satiety center)

80
Q

What are the manifestations of a prolactinoma in men and women?

A

Women: Amenorrhea galactorrhea
Men: Impotence

81
Q

What growth hormone receptor antagonist is used for acromegaly? How about the somatostatin analogue?

A

Pegvisomant– Receptor antagonist

Octreotide– Somatostatin analgoue

82
Q

T or F. Pituitary tissue is normally enhancing hence small tumors appear as relatively hypoenhancing nodules

A

T

83
Q

What pituitary hormones increase with administration of TRH (Thyrotropin releasing hormone)?

A

Prolactin, GH and TSH

PGT as well secreted by chromophobes and acidophils

84
Q

What is the main defect resulting in an empty sella syndrome?

A

Defect in the dural diaphragm (diaphragm sellae) that covers the rostral part of the pituitary gland just letting the infundibular stalk pass through:

  1. Raised ICP
  2. Post surgery
85
Q

What is the MOA by which bromocriptine reduces prolactin secretion?

A

Dopamine agonist

Cabergoline is also a DA

86
Q

T or F The advantage of gamma knife for pit ad is low recurrence rate, but the effect is attainable only after a few months

A

T

THERE SHOULD BE NO THREAT TO VISION AT THE START OF THERAPY

87
Q

What structures are affected by sphenoid wing meningioma?

A

Cavernous sinus
Orbit
Temporal bone

May present with foster kennedy syndrome

88
Q

What are the 2 common locations of a chordoma?

A

Clivus + Sacrococcygeal region

89
Q

What cranial nerves can be involved with a chordoma at the clivus?

A

CN 2 - 12

90
Q

What characteristic pain is associated with chordoma?

A

Neck pain radiating to vertex on neck flexion

The tumor may destroy the clivus and bulge into the nasopharynx

91
Q

What are ddx for base of the skull tumors?

A
  1. Osteomas
  2. Chordomas
  3. Epidermoids
  4. Teratomas
  5. Nasopharyngeal CA
  6. Meningioma
92
Q

The bobble head doll syndrome is associated with what tumor?

A

Suprasellar arachdoid cyst

Due to a thickened arachnoid (membrane of lillequist)

Choroid plexus papilloma

93
Q

What are ddx for a foramen magnum tumor?

A
  1. Meningioma
  2. Schwannoma
  3. Neurofibroma
  4. Dermoid
94
Q

Identify the site of lesion of the following eponyms

  1. Rochon-Duvigneau
  2. Jacod Rollet
  3. Foix- Jefferson
  4. Gradenigo-Lannois
  5. Jacod
A
  1. Rochon-Duvigneau- superior orbital fissure (3,4,V1,6)
  2. Jacod Rollet- apex of the orbit (2,3,4,V1,6)
  3. Foix- Jefferson- cavernous sinus (3,4,V1,V2,6)
  4. Gradenigo-Lannois- apex of the petrous temporal bone (5,6)
  5. Jacod- sphenoid and petrosal bones (3,4,6)
95
Q

Identify the site of lesion of the following eponyms

  1. Vernet
  2. Collet Sicard
  3. Villaret
  4. Garcin
A
  1. Vernet- Jugular foramen (9,10,11)
  2. Collet Sicard- Anterior occipital condyles (9,10,11,12)
  3. Villaret- Retroparotid space (9,10,11,12,sympathetic)
  4. Garcin- Half of the base of the skull (all 12)
96
Q

Identify the following neurologic disorder brought about by the following autoantibodies

  1. Anti-yo (1/3 small cell CA, 1/4 Ovarian, Hodgkin)
  2. Anti-Hu (Small cell lung CA, Hodgkin)
  3. Anti-NMDA (Ovarian teratoma)
A
  1. Anti-yo (Ovarian) Cerebellar degeneration
  2. Anti-Hu (Small cell lung CA) Encephalomyelitis, Neuropathy sensory, Chorea
  3. Anti-NMDA (Ovarian teratoma) Encephalomyelitis
97
Q

Identify the following neurologic disorder brought about by the following autoantibodies

  1. Anti-recoverin (Small cell lung CA)
  2. Anti-volatage gated calcium channel (Small cell lung CA, Hodgkin)
  3. Anti CRMP-5 (Lung)
  4. Anti Ri/ ANNA 2 (Breast, small cell CA)
A
  1. Anti-recoverin (Small cell lung CA) Retinal degeneration
  2. Anti-volatage gated calcium channel (Small cell lung CA, Hodgkin) Lambert eaton myasthenic syndrome
  3. Anti CRMP-5 Optic neuropathy
  4. Opsoclonus myoclonus ataxia
98
Q

What are the MRI findings in both limbic encephalitis and anti nmda encephalitis?

A

T2 hyperintensities

99
Q

What type of radiation injury?

  1. More than 3 months post radiation
  2. Fibrinoid necrosis with microthrombosis
  3. Enlargement of tumor mass
A
  1. early/late delayed
  2. late delayed
  3. early delayed
100
Q

How does PET dfx between tumor progression and radiation necrosis

A

Cerebral blood volume is deceased in radiation necrosis and increased in tumor progresssion

101
Q

What autoantibodies cause stiff person syndrome?

A

Antiamyphiphysin, Anti-CAspr2, Anti-GAD

102
Q

What neurologic disorders can be caused by anti CRMP5?

A

Optic neuropathy, Chorea, Encephalomyelitis

103
Q

What neurologic disorders are caused by anti Hu?

A

Encephalomyelitis including the brainstem and limbic
Subacute sensory neuropathy and neuronopathy
Chorea

104
Q

What syndrome can be caused by anti VGKC antitbodies?

A

Limbic encephalitis and lambert eaton

105
Q

What is the target of the anti-NMDA antibody?

A

The NR1 receptor

106
Q

What did Denny Brown describe in 1948?

A

Sensory neuronopathy and neuropathy usually caused by anti-Hu

107
Q

Where do anti Yo antibodies bind to to destroy purkinje cells?

A

C-myc protein

108
Q

In the pediatric age group what cancer is opsoclonus-myoclonus-ataxia syndrome related to?

A

Neuroblastoma but in adults breast and small cell CA
Anti-Ri in breast ca
When neuroblastoma is the cause ACTH and Steroids can be tried!

109
Q

T or F there is a paraneoplastic kind of Devic syndrome

A

T

110
Q

What is the most common paraneoplastic neurologic syndrome?

A

Lambert Eaton syndrome

111
Q

What is SMART?

A

Stroke like migraine attacks after radiation therapy

112
Q

What are the cancers most commonly associated with PN disorders?

A
  1. Small cell lung CA
  2. AdenoCA of lung
  3. AndenoCA of ovary
  4. Hodgkin lymphoma
113
Q

Anti-Caspr2 is associated with what PN disorders?

A

Limbic and brainstem encephalitis

Stiff person syndrome and neuromyotonia

114
Q

What antibody is responsible for retinal degeneration?

A

Antirecoverin (anti CAR) seen in small cell lung, thymoma, renal cell and melanoma

115
Q

Besides sensory loss what are the other symptoms attached to paraneoplastic sensory neuronopathy?

A
  1. Lancinating pains initially
  2. Disabling ataxia
  3. Pseudoathetoid movements of the outstretched hands
  4. Autonomic dysfunctions
116
Q

Besides small cell lung CA what other 2 CA account for paraneoplastic cerebellar degeneration? 25 and 15% respectively.

A

Ovarian CA and Hodgkin disease

117
Q

What percentage of patients with a clinical picture of paraneoplastic cerebellar degeneration will have anti-Yo?

A

1/2

118
Q

What is another name for anti-Yo antibodies? Where do they bind?

A

Anti-Purkinje cell antibodies. C-myc protein

119
Q

Anti-Ma is usually associated with tumors of what region?

A

Testicular

120
Q

What syndrome is associated with Anti mGluR5?

A

Ophelia syndrome: Memory loss in a patient Hodgkin lymphoma

121
Q

What is an extraordinaty distorder of continuous muscle fiber activity, insomnia and hallucinosis?

A

Choree fibrillaire

Anti VGKC

122
Q

What type radiation injury based on histopath?

  1. Brain edema only
  2. Extensive demyelination and loss of oligodendrocytes beyond the confines of the tumor
  3. Necrosis of the white matter. Diffuse vascular changes on account of fibrinoid necrosis and widespread microthrombosis
A
  1. Acute: during the latter part of the radiation cycle or soon after
  2. Early delayed
  3. Late delayed
123
Q

What percentage of childhood brain tumors does medulloblastoma account for?

A

20%

124
Q

What two tumor syndromes feature medulloblastoma?

A

Turcot also has intestinal polyps as well

Gorlin aka Nevoid basal cell carcinoma syndrome has a lot of skin lesions, syndactyly, jaw cysts, pitting of soles

125
Q

How do medulloblastomas look on MRI?

A

HIGH INTENSITY IN BOTH T1 AND T2

With heterogenous enhancement

Located on the vermis extedning into the 4th ventricle

126
Q

What is the 5 year survival rate in medulloblastomas that have surgery, radiation of the entire neuraxis and chemotherapy in a timely manner?

A

80%

127
Q

What blood abnormality is associated with VHL diseae?

A

Polycythemia because of the elaboraiton of EPO by the hemangioblastoma.

128
Q

Gangliocytoma is associated with a germ line mutation of what gene? What is the prognosis of this tumor?

A

PTEN

LACK OF GROWTH POTENTIAL AND FAVORABLE PROGNOSIS

129
Q

What is a common cause of a mass in the third ventricle that causes a ball valve headache?

A

Colloid paraphysial cyst

130
Q

Which is due to excessive secretion of pituitary ACTH? Cushing disease or syndrome?

A

Disease

Syndrome refers to the effects of cortisol excess from any one of several sources: exogenous steroids, adenoma of the adrenals, bronchial CA producing ACTH

131
Q

What are the ddx for bitemporal hemianopia with a normal sized sella?

A
  1. Saccular aneurysm of the distal carotid artery
  2. Meningioma
  3. Craniopharyngioma
132
Q

T or F: Chordomas do NOT metastasize

A

T

133
Q

What is the mean age for the occurrence of GBM? How about anaplastic astrocytoma? How about Oligodendroglioma?

A

60 YO
46 YO
20-30s YO

134
Q

What two pathologic features differentiate GBM from anaplastic astrocytoma?

A

Necrosis and hemorrhage

135
Q

Which is mutation is associated with with GBMs that begin as GBMs usually in the older patients? VS those that degenerate from a more benign tumor

A

Amplication of the EGFR gene

136
Q

What does an IDH1 and IDH2 mutation imply for GBM?

A

The tumor at hand degenerated from a more benign tumor. These have a better prognosis than those that start off as GBM.

137
Q

A methylated MGMT promoter gene makes a tumor more or less responsive to chemo?

A

MGMT is DNA repair enzyme– if it is methylated it is silenced. As a result, tumor is unable to repair itself making if more susceptible to chemo therapy.

138
Q

What percentage of GBM patients survive beyond 2 years without treatment?

A

10.4% VS 26.5

139
Q

How will planned delay in the administration of radiation in young astrocytoma patients affect the overall survival of the patient?

A

No effect. OS is still 7 years.

BUT the PFS is lowered from 5.3 to 3.4

140
Q

What tumors have:

  1. Homer Wright Rosettes
  2. Flexner-wintersteiner rosettes
  3. True ependymal rosettes
  4. Perivascular pseudorosette
A
  1. Homer Wright Rosettes: Neuroblastoma, Medulloblastoma, PNET, Pinealoblastoma
  2. Flexner-wintersteiner rosettes: Retinoblastoma, pineoblastoma, medulloepithelioma
  3. True ependymal rosettes: Ependymoma
  4. Perivascular pseudorosette: Ependymoma, medulloblastoma, PNET, centraly neurocytoma, GBM, pilomyxoid astrocytoma
141
Q

Which tumors tend to be multiple when sending mets to the brain?

A

Small cell CAs and melanomas

TEND TO BE SINGLE: Kidney, Breast, Thyroid, AdenoCA of the lung

142
Q

What is the arbitrary limit for doing focused RT vs WBRT?

A

4 mets if more do WBRT

143
Q

What condition may come up when ALL patients with CNS relapse are treated with RT, IT and IV MTX are used?

A

Necrotizing leukoencephalopathy

144
Q

What are the relative volumes of the:

  1. Brain
  2. CSF
  3. Blood
A
  1. Brain: 1200-1400ml
  2. CSF: 70-140
  3. Blood: 150

Brain Power: Adam and Victor's Neurology (19 decks)

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