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🧠📖💯 > Neoplasms > Flashcards

Neoplasms Flashcards

1
Q

Most common initial manifestation of primary and metastatic brain tumors

A

First onset seizure in adulthood

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6
Q

[WHO grading]

Low levels of proliferative activity.
Infiltrative, often recur.
Some tend to progress to higher grades.

A

Grade II

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7
Q

[WHO grading]

Low proliferative potential
Possibility of cure after surgical resection alone

A

Grade I

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8
Q

[WHO grading]

Clear histological evidence of malignancy (nuclear atypia, brisk mitotic activity)
Treated w/ RT and/or chemotherapy in most cases

A

Grade III

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9
Q

[WHO grading]

Cytologically-malignant, mitotically active
Necrosis-prone
Rapid disease evolution and fatal outcome

A

Grade IV

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10
Q

4 viruses associated w/ NS tumors (Adams)

A

EBV HHH

EBV
Hep B
HPV
HTLV

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11
Q

Type of edema in CNS tumors

A

Vasogenic edema

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12
Q

Which type of edema is confined to the white matter?

A

Vasogenic edema

*loose structural organization in WM

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13
Q

Type of edema in acute hepatic encephalopathy

A

Cytotoxic edema

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14
Q

Type of edema in hypoxic-ischemic injury

A

Cytotoxic edema

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15
Q

Type of edema seen in lead encephalopathy

A

Vasogenic edema

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16
Q

What are the 3 types of cerebral edema?

A

Vasogenic
Cytotoxic
Interstitial

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17
Q

What type of IV fluid should be used in cerebral edema

A

Normal saline > LR

Avoid D5NS

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18
Q

What is the typical location of Medulloblastoma

A) Adults
B) Children

A

A) Adults - Cerebellar Vermis, posterior part

B) Children - 4th ventricle, neuroepithelial roof

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19
Q

Characteristics (3) of headaches in CNS tumors?

A

Nocturnal occurence
Presence on first awakening
Deep, nonpulsatile quality

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20
Q

Where would the headaches present, depending on the tumor location?

Supratentorial
Posterior fossa
Elevated ICP

A

Supratentorial - HA on side of the tumor

Posterior fossa - IPSIlateral retroauricular/occipitonuchal

Elevated ICP - bifrontal or bioccipital

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21
Q

Most common initial manifestation of primary and metastatic neoplasm (CNS)

A

Seizure

20-50% of all pts w/ cerebral tumors

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22
Q

Which is NOT true regarding the epidemiology of Glioblastoma

A) Accounts for 20% of all intracranial tumors and 80% of gliomas of the cerebral hemispheres in adults
B) Mean age for GBM is 60yo, AA is 46 yo
C) Incidence is higher in men
D) Almost all high-grade gliomas occur sporadically
E) None of the above

A

E) None of the above

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23
Q

Location of Gliomas

A

Deep white matter
50% - more than one lobe
3-6% - multicentric foci (like metastatic CA)

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24
Q

Imaging characteristics of Glioblastoma in terms of

Homogeneity
Center
Rim
Surroundings

A

Nonhomogenous
Center - hypointense, nonenhancing
Rim - irregular rim of contrast enhancement
Surroundings - nonenhancing edema, small nodular contrast enhancing lesions adjacent to but distinct from the primary lesion

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25
Q

Gross appearance of GBM?

A

Variegated appearance

Mottled gray, red, orange, or brown

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26
Q

Survival rates for untreated GBM

1 year survival - ____%
Beyond 2 years - ____%

A

1 year survival < 20%

Beyond 2 years < 10%

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27
Q

Survival rates for untreated GBM after 1.5 years depending on age:

> 60 yo
< 40 yo

A

> 60 yo - <10%

< 40 yo - 2/3

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28
Q

50% of astrocytomas have deletions w/ this gene

A 
Gene p53 (tumor suppressor)
Chromosome 17p
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29
Q

This gene is associated with progression to more malignant grade of astrocytoma or to a GBM

A

Epidermal Growth Factor Gene

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30
Q

Mutation here is associated with slower tumor progression. Common in gliomas and oligodendrogliomas

A

IDH 1 and 2 mutation

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31
Q

What is the implication of MGMT methylation status?

A

Response to temozolomide

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32
Q

Effect of cranial irradiation on prognosis of AA and GBM?

A

Increased survival by 5 months

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33
Q

What is the MOA of TMZ

A

Alklylation of guanine at the O6 position -> DNA replication inhibition

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34
Q

What does MGMT do?

A

MGMT removes alkyl groups from guanine at the O6 position -> counteracts TMZ

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35
Q

True or false: MGMT promoter gene methylation is associated with prolonged survival

A

True

MGMT gene promoter methylation -> x MGMT gene -> dec MGMT -> dec REMOVAL of alkyl groups from guanine -> more alkyl in guanine O6 = more inhibition of DNA replication -> inc TMZ efficacy -> prolonged survival

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36
Q

In GBM patients, what can you give in response to upregulation of EGFR?

A

Tyrosine kinase inhibitors (erlotinib, gefitinib)

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37
Q

What is the effect of TMZ on:

A) 2 year survival
B) Median survival (w/ RT)

A

A) 2 year survival inc from 10% -> 27%
B) Median survival TMZ + RT = 14.6 mos
*vs 12.1 mos for RT alone

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38
Q

How do you give TMZ?

A

6 weeks of 75mg/m2 + RT daily

4 weeks OFF

6 cycles of 5days Q28 days

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39
Q

What are the complications of TMZ?

A

PCP pneumonia

Thrombocyto and leukopenia

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40
Q

Among GBM patients, what is the survival rate for:

A) Surgery + RT
B) W/o tx

A

Surgery + RT = 12 mos

w/o tx = 7-9 mos

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41
Q

Almost all glioblastomas recur where?

A

Within 2 cm of their original site

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42
Q

What is the usual location of anaplastic astrocytoma in

Adults
Children

A

Adults - cerebrum

Children - cerebellum, hypothalamus, optic nerve/chiasm, pons [CHOP]

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43
Q

Clinical symptom present in 2/3 of Low and intermediate-grade astrocytomas

A

Focal or generalized seizures

*60-75% have recurrent seizures

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44
Q

This is a tumor crossing and thickening the corpus callosum, involving one or more large confluent areas of signal change w/ scant contrast enhancement. Presents with frontal lobe behavioral syndrome, pseudobulbar palsy.

A

Gliomatosis Cerebri

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45
Q

Age at presentation of oligodendroglioma (bimodal)

A

6-12 years

30s-40s

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46
Q

Oligodendroglioma is more common in

Men/Women

A

Men

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47
Q

Clinical implications of the ff genetic changes in Oligodendrogliomas:

A) Loss of alleles on chromosome 1p
B) Loss of chromosome 19q

A

x alleles on chromosome 1p = high degree of responsiveness to PCV chemotherapy

X chromosome 19q = longer survival

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48
Q

Common locations of Oligodendroglioimas

A

Frontal and temporal lobes (40-70%)

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49
Q

Most common clinical presentation of Oligodendrogliomas

A

Focal or generalized seizures

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50
Q

Imaging findings in Oligodendrogliomas (3)

A

CCC

Cortical surface heterogenous mass w/ well-defined borders
Intratumoral Calcification
Contrast enhancement

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51
Q

Aspects of oligodendrogliomas that show good response to chemo
(PCV or TMZ)

A

1p/19q codeletion
IDH mutations
Anaplastic

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52
Q

Most common glioma of the spinal cord

A

Ependymoma

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53
Q

Most common cerebral site of Ependymomas

A

4th ventricle

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54
Q

Typical location of myxopapillary ependymomas

A

Filum terminale

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55
Q

Genetic abnormality associated with ependymomas

A

RELA-fusion positive in supratentorial tumors of children

Chromosome 11

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56
Q

Which of the following is NOT true regarding the epidemiology of meningiomas

A. Comprises 1/3 of all primary intracranial tumors
B. More common in men
C. Presents in the 6th-7th decade of life
D. Risk factors include prior radiation therapy and trauma

A

B. More common in women

Estrogen and progesterone receptors suspected (most sources say PROGESTERONE more likely to be implicated)
Tendency to enlarge during pregnancy
Associated w/ breast CA

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57
Q

Most frequent genetic abnormality associated with meningiomas

A

Merlin NF2 Gene on Chromosome 22q

  • truncating (inactivating) mutations
  • freq in fibroblastic, transitional types
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58
Q

Cells of origin for meningiomas

A

Arachnoidal (meningothelial) cells forming the archnoid villi

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59
Q

Classic histologic finding in meningiomas

A

Psammoma bodies

Laminated calcific concretions

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60
Q

Most common type of meningioma

A

Meningothelial (syncitial) form

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61
Q

Neuroimaging findings for meningiomas

A

Tumor blush
Smooth contour
Dural tail
Homogenous enhancement

BCDE
Blush, Contour, Dural, Enhancement

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62
Q

True or false. CSF protein may be elevated in Meningiomas

A

True

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63
Q

Usual age at presentation of primary CNS lymphoma?

> Normal population
AIDS

A

Normal pop: 5th-7th decades of life

Pts w/ AIDS: 3rd-4th decade of life

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64
Q

Re: usual locations of PCNSL:

60%: _____
10-20%: _____

A

60%: cerebral hemispheres

10-20%: Retinal, Uveal, Vitreous involvement (RUV your eyes)

65
Q

Which is NOT true of PCNSL’s histologic picture?

A) Highly cellular
B) Angiocentric pattern
C) (+) Necrosis
D) None of the above

A

C) (+) Necrosis

No tendency to necrosis in PCNSL

66
Q

Which is NOT true of the imaging findings in PCNSL?

A) Several dense lesions
B) (+) Diffusion restriction
C) Non-necrotic
D) Hemorrhagic

A

D) Hemorrhagic

PCNSL: Non-hemorrhagic

67
Q

True or false. CSF lymphocytic and mononuclear pleocytosis are found more frequently in PCNSL than in gliomas AND metastatic tumors

A

True

68
Q

True or false. In PCNSL, CSF cytology is more sensitive than Immunoglobulin heavy chain gene (IGH)

A

False

IGH is more sensitive than cytology of spinal fluid

69
Q

Choice of chemotherapeutic agents for PCNSL?

A

Methotrexate-based regimens

70
Q

Prognosis for PCNSL?

A

Survival rates at best near 50%

71
Q

What is the distribution of intracranial metastatic CA in terms of their location in the brain?

80%: ____
20%: ____

A

80%: cerebral hemispheres

20%: posterior fossa

72
Q

Skull and dural mets are associated with (3):

A

Breast CA
Prostate CA
Multiple Myeloma

73
Q

What are the most common sources of brain mets?

  1. ___
  2. ___
  3. ___
A
  1. Lung CA
  2. Breast CA
  3. Melanoma
  • GI (rectum, kidney) > renal
74
Q

Neoplasms with the highest tendency to metastasize to the brain

  1. ___
  2. ___
  3. ___
A
  1. Melanomas (75%)
  2. Testicular tumor
  3. Bronchial CA

Metastasize To Brain

75
Q

Metastatic tumors most likely to be

A) Single (4)
B) Multiple (2)

A

Single - Kidney, breast, thyroid, Lung adenoCA

Multiple: Small cell CA, melanomas

76
Q

Metastatic tumors likely to be hemorrhagic

A

Lung CA (MC bec of sheer number, but small proportion oly)

Mets that bleed: MahinaCoag LRT
Melanoma, Chorioepithelioma (more likely)
Lung, Renal, Thyroid (less likely)

77
Q

In stroke-like events in CA patients, consider ____.

A

Bacterial thrombotic (marantic) endocarditis

78
Q

Stereotactic RT is arbitrarily limited to __ mets

A

4

79
Q

When is surgical extirpation indicated for single metastatic brain lesions?

A

Growth of primary tumor & systemic mets is under control
Mets are accessible to surgeon
Not located in a strategic motor or language area

80
Q

In terms of prognosis, what is the life expectancy for those w/ brain mets who’ve undergone therapy?

A

6 months

81
Q

Characteristic presentation of carcinomatous and lymphomatous meningitis

A

CN neuropathy + bilateral limb weakness, asymmetrical

82
Q

CSF picture of carcinomatous and lymphomatous meningitis

A

Pressure: increased
CSF protein: elevated
CSF sugar: decreased
Lymphocytic pleocytosis (up to 100)

83
Q

Treatment of Carcinomatous meningitis

A

RT + intraventricular methotrexate

84
Q

Median duration of survival after diagnosis of carcinomatous meningitis

A

6 months

85
Q

Which of the following is NOT true of NS involvement in Leukemia?

A) More common in acute than in chronic leukemia
B) More common in myelocytic than in lymphocytic leukemia
C) More common in children with ALL who relapse
D) Primarily a pial disease
E) Similar CSF picture to meningeal carcinomatosis

A

B) More common in myelocytic than in lymphocytic leukemia

NS involvement more common in lymphocytic leukemia

86
Q

Solid green-colored mass of myelogenous leukemic cells that infiltrate the dura

A

Chloroma

87
Q

Most common neurologic complication of all types of lymphoma

A

Extradural compression of the spinal cord

88
Q

Which of the following is NOT true of Neurolymphomatosis?
A) This is the appearance of a NHL in the meninges, adjacent roots, peripheral nerves
B) (+) Eosinophilic pleocytosis
C) CN palsies are common, w/ predilection for CN VIII
D) Cauda equina involvement is rare

A

D) Cauda equina involvement is rare

*Cauda equina is involved eventually in most cases

89
Q

Castleman disease is precipitated by constituent ____ infection

A

HHV-8

90
Q

Which clinical symptom appears in the late stages of Medulloblastoma?

A

Decerebrate attacks / cerebellar fits

91
Q

Which of the following is NOT true of Medulloblastoma?
A) Grade IV WHO tumor
B) Desmoplastic variant is associated with Gorlin syndrome (a/w Sonic hedgehog ligand)
C) N-MYC amplification a/w a better prognosis
D) Aberrations in the copy number of chromosomes 6q and 17q noted
E) Possible association w/ JC virus

A

C) N-MYC amplification a/w a better prognosis

N-MYC = poorer prognosis

92
Q

Histologic findings in medulloblastoma

A

Pseurorosettes

93
Q

Treatment of medulloblastoma

A

Maximal resection
Chemotherapy
RT of entire neuraxis

*5y survival 80%

94
Q

True or false. Desmoplastic features of medulloblastoma are a/w better prognosis

A

True

95
Q

Most common solid tumor of childhood

A

Neuroblastoma

*arises in the adrenal medulla

96
Q

What clinical syndrome (symptoms) occur in neuroblastoma?

A

Polymyoclonus w/ opsoclonus and ataxia - parang na…blast…
(Paraneoplastic complication)

97
Q

Genetic abnormality in Retinoblastoma

A

Abnormal protein encoded by a growth suppressor or antioncogenic gene (Rb)

98
Q

70% of ependymomas originate where?

A) Lateral ventricles
B) 3rd ventricle
C) 4th ventricle
D) None of the above

A

C) 4th ventricle

e4
e4t??

99
Q

Which of the following is TRUE regarding papillomas of the choroid plexus?

A) Located in the 3rd ventricle
B) Cellular element: columnar epithelium
C) Oncogene T antigen of SV40 virus is involved
D) Good prognosis

A

Choroid Plexus Papilloma

True: C) Oncogene T antigen of SV40 virus is involved

  • Located in the lateral and 4th ventricles
  • Cellular element: cuboidal epithelium
  • Poor prognosis
100
Q

Hemangiomas of the cerebellum
Retinal angioma
Hepatic/pancreatic cysts
Polycythemia

A

Hemangioblastoma of the Cerebellum

101
Q

True or false. Sleep is often affected in pineal gland tumors

A

False (Adams)

102
Q

Most common pineal region tumor

A

Germinoma (50%)

103
Q

Tiger stripe appearance. PTEN gene.

A

Lhermitte-Duclos Disease

Dysplastic Gangliocytoma of the Cerebellum

104
Q

Dysembryoplastic neuroepithelioma tumor has a proclivity for the ___ lobe.

A

Temporal lobe

105
Q

Subependymal Giant Cell Astrocytoma

Associated with?
Arise where?
Treatment?

A

A/w: Tuberous Sclerosis
Arise near: foramen of Monro
Tx: Everolimus (mTOR complex inhibitor)

106
Q

Presents with ball-valve obstruction (HA modified by posture)
Cysts present clinically during adulthood
Derived from the paraphysis
Cysts always situated in the anterior portion
Cysts filled w/ gelatinous mtl w/ mucopolysaccharides

A

Colloid cyst of the 3rd ventricle

107
Q

Arachnoid Cyst

Clinical presentation in children?
Location?
Treatment?

A

Clinical: macrocrania, u/l transillumination
Location: sylvian fissure, temporal pole
Tx: marsupialization

108
Q

Syndromes (3) associated with Vestibular Schwannomas and differences

A

A. Type 1 Neurofibromatosis / Von Recklinghausen
Adults; Unilateral CN VIII

B. Type 2 NF
< 21 yo; Bilateral acoustic neuromas

C. Familial Schwannomatosis
Multiple schwannomas w/o vestibular tumors
Chromosome 22 | SMARCb1 gene

109
Q

For pts w/ vestibular schwannomas who undergo surgical excision, hearing can be preserved if lesion is ___ cm

A

< 2.5 cm

110
Q

Apart from surgical excision, what are the other treatment options for VS?

A

RT - older pts w/ few sx

Chemo (Bevacizumab) - pts w/ NF2

111
Q

Most common location of Craniopharyngiomas

A

Above the sella turcica

112
Q

On gross pathology, the cysts of craniopharyngiomas contain what? (3)

A

Dark albuminous fluid
Cholesterol crystals
Calcium deposits

113
Q

Clinical presentation of craniopharyngiomas in children

A

Visual loss + Diabetes insipidus

114
Q

Glomus jugulare tumor is most commonly found among what population of patients geographic location?

A

Patients living at high altitudes

115
Q

Which of the following is NOT part of the clinical presentation for Glomus Jugulare tumors?

A) Self-audible bruit
B) Partial deafness, facial palsy, dysphagia
C) Unilateral tongue atrophy
D) Vascular polyp, EAM & Palpable mass below, anterior to the mastoid eminence
E) TIAs

A

E) TIAs

116
Q

Associations with Carotid Body tumor (3)
1 geographic location of pts
2 syndromes

A

Patients living at high altitudes

NF1
VHL

117
Q

Which of the following is NOT part of the clinical presentation of Carotid Body Tumor?

A) Painless mass below the angle of the jaw
B) Vascular polyp, EAM
C) Syndrome of the retroparotid space
D) TIAs
E) Sleep apnea
A

B) Vascular polyp, EAM (found in glomus jugulare tumors)

118
Q

What type of pituitary adenomas is most common in both men and women? (60-70%)

A

Prolactin-secreting tumors

119
Q

Pituitary adenomas are most often composed of _____ cells

A

Chromophobe

120
Q

Products of chromophobe/acidophils

A

Prolactin
GH
TSH

121
Q

Products of Basophils

A

ACTH
LH
FSH
B-lipotropin

  • ALFBet
  • Baso/Basic Alphabet
122
Q

Natural clinicoanatomic progression of pituitary tumors

A

1) Compress pituitary gland
2) Extend up and out of sella
3) Compress optic chiasm
4) Extend to -> cavernous sinus, 3rd V, temporal, post. Fossa

123
Q

Which is NOT true of prolactinomas?
A) Manifests during childbearing years
B) Longer duration of amenorrhea, higher prolactin serum level = larger prolactinoma
C) Larger tumor in females
D) Patients do NOT respond (prolactin does not increase) to administration of Chlorpromazine or TRH

A

C) Larger tumor in females

124
Q

Clinical presentation. Overproduction of GH:

Before puberty
After puberty

A

Before puberty: gigantism

After puberty: acromegaly

125
Q

GRH antagonist

A

Pegvisomant

126
Q

Early on, in pituitary tumors, the lower/upper parts of the visual fields are affected

A

Upper parts of the visual field

127
Q

This symptom occurs in 50% of pts with macroadenomas

A

Headache

128
Q

What is the empty sella syndrome?

A

Nontumorous enlargement of the sella 2/2 defect in the dural diaphragm

129
Q

Treatment for prolactinoma

A

Bromocriptine 0.5-1.25mg/day

130
Q

Treatment for acromegaly

A

Ocreotide (somatostatin analogue)

131
Q

Risk factors for necrosis or hemorrhage of pituitary tumor

A 
Anticoagulation
Pituitary function testing
Radiation
Bromocriptine
Head trauma
132
Q

Treatment of Pituitary Apoplexy

A

Hydrocortisone to treat acute addisonian state

If no improvement after 2 days, resect

133
Q

Most common cause of ischemic necrosis of the pituitary

A

Sheehan Syndrome

134
Q

Symptoms of unilateral exophthalmos, anosmia, foster-kennedy syndrome, uncinate fits

A) Meningioma of the olfactory groove
B) Meningioma of the sphenoid ridge
C) Meningioma of the tuberculum sella

A

B) Meningioma of the sphenoid ridge

135
Q

Bitemporal hemianopia w/ normal sellar size
Asymmetrical visual deficits
No hypothalamic-pituitary deficits

A) Meningioma of the olfactory groove
B) Meningioma of the sphenoid ridge
C) Meningioma of the tuberculum sella

A

C) Meningioma of the tuberculum sella

136
Q

Anosmia, optic atrophy, Witzelsucht, abulia, confusion

A) Meningioma of the olfactory groove
B) Meningioma of the sphenoid ridge
C) Meningioma of the tuberculum sella

A

A) Meningioma of the olfactory groove

137
Q

Usual age of presentation of brainstem gliomas

A

7 yo

138
Q

Chordomas are soft, jelly-like, gray-pink growths arising from where?

A

Primitive notochord (remnants)

139
Q

Chordomas are most often located in

A

Within the clivus (dorsum sellae-foramen magnum)

Sacrococcygeal region

140
Q

Most common symptom (?) of paraneoplastic d/o

A

Sensory neuropathy (54%)

141
Q

Anti-NMDA Encephalitis is associated with which tumors? (2)

A

Ovarian Teratoma

Small cell lung CA

142
Q

Antibodies associated with paraneoplastic sensory neuropathy

A

Anti-Hu > Anti CRMP5

143
Q

Not true of paraneoplastic sensory neuropathy’s clinical presentation

A) Neuropathy and encephalomyelitis often occur together
B) Widespread distal distribution
C) Autonomic dysfunction
D) Ataxia, pseudoathetoid movements of the hands

A

B) Widespread distal distribution

Proximal. Hence apparent as sensory ganglionitis and radiculitis

144
Q

Most characteristic of the paraneoplastic syndromes

A

Paraneoplastic cerebellar degeneration

145
Q

Most commonly associated tumor w/ paraneoplastic cerebellar degeneration

A

Lung CA - small cell

146
Q

What are the associated tumors in Opsoclonus-Myoclonus-Ataxia Syndrome?

A) in children
B) in adults

A

In children - neuroblastoma

IN adults - breast CA, small cell lung CA

147
Q

Antibodies in Opsoclonus-Myoclonus-Ataxia Syndrome

A

Anti-Ri ANNA 2

148
Q

Antibody associated with rapidly progressive amyotrophy and fasciculations w/ or w/o brisk reflexes

A

Anti-Hu

149
Q

Ophelia syndrome is due to antibodies against _____

A

mGluR5 (metabotropic glutamate receptor)

150
Q

Paraneoplastic optic neuropathy is associated with which antibody?

A

CRMP-5 antibody

151
Q

Most common underlying malignancy for paraneoplastic retinopathy

A

Small cell CA of the lung

Lesion: antibodies against recoverin

152
Q

Most common paraneoplastic neurologic syndrome

A

Lambert-Eaton Syndrome

153
Q

CAs (3) associated with Isaac Syndrome / Paraneoplastic Neuromyotonia

A

Lung CA
Lymphoma
Thymoma

🧠📖💯 (20 decks)

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