Neoplasms Flashcards
1
Q
Most common initial manifestation of primary and metastatic brain tumors
A
First onset seizure in adulthood
6
Q
[WHO grading]
Low levels of proliferative activity.
Infiltrative, often recur.
Some tend to progress to higher grades.
A
Grade II
7
Q
[WHO grading]
Low proliferative potential
Possibility of cure after surgical resection alone
A
Grade I
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
9
Q
[WHO grading]
Cytologically-malignant, mitotically active
Necrosis-prone
Rapid disease evolution and fatal outcome
A
Grade IV
10
Q
4 viruses associated w/ NS tumors (Adams)
A
EBV HHH
EBV
Hep B
HPV
HTLV
11
Q
Type of edema in CNS tumors
A
Vasogenic edema
12
Q
Which type of edema is confined to the white matter?
A
Vasogenic edema
*loose structural organization in WM
13
Q
Type of edema in acute hepatic encephalopathy
A
Cytotoxic edema
14
Q
Type of edema in hypoxic-ischemic injury
A
Cytotoxic edema
15
Q
Type of edema seen in lead encephalopathy
A
Vasogenic edema
16
Q
What are the 3 types of cerebral edema?
A
Vasogenic
Cytotoxic
Interstitial
17
Q
What type of IV fluid should be used in cerebral edema
A
Normal saline > LR
Avoid D5NS
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
19
Q
Characteristics (3) of headaches in CNS tumors?
A
Nocturnal occurence
Presence on first awakening
Deep, nonpulsatile quality
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
21
Q
Most common initial manifestation of primary and metastatic neoplasm (CNS)
A
Seizure
20-50% of all pts w/ cerebral tumors
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
23
Q
Location of Gliomas
A
Deep white matter
50% - more than one lobe
3-6% - multicentric foci (like metastatic CA)
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
25
Q
Gross appearance of GBM?
A
Variegated appearance
Mottled gray, red, orange, or brown
26
Q
Survival rates for untreated GBM
1 year survival - ____%
Beyond 2 years - ____%
A
1 year survival < 20%
Beyond 2 years < 10%
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
28
Q
50% of astrocytomas have deletions w/ this gene
A
Gene p53 (tumor suppressor) Chromosome 17p
29
This gene is associated with progression to more malignant grade of astrocytoma or to a GBM
Epidermal Growth Factor Gene
30
Mutation here is associated with slower tumor progression. Common in gliomas and oligodendrogliomas
IDH 1 and 2 mutation
31
What is the implication of MGMT methylation status?
Response to temozolomide
32
Effect of cranial irradiation on prognosis of AA and GBM?
Increased survival by 5 months
33
What is the MOA of TMZ
Alklylation of guanine at the O6 position -> DNA replication inhibition
34
What does MGMT do?
MGMT removes alkyl groups from guanine at the O6 position -> counteracts TMZ
35
True or false: MGMT promoter gene methylation is associated with prolonged survival
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
36
In GBM patients, what can you give in response to upregulation of EGFR?
Tyrosine kinase inhibitors (erlotinib, gefitinib)
37
What is the effect of TMZ on:
A) 2 year survival
B) Median survival (w/ RT)
A) 2 year survival inc from 10% -> 27%
B) Median survival TMZ + RT = 14.6 mos
*vs 12.1 mos for RT alone
38
How do you give TMZ?
6 weeks of 75mg/m2 + RT daily
4 weeks OFF
6 cycles of 5days Q28 days
39
What are the complications of TMZ?
PCP pneumonia
| Thrombocyto and leukopenia
40
Among GBM patients, what is the survival rate for:
A) Surgery + RT
B) W/o tx
Surgery + RT = 12 mos
w/o tx = 7-9 mos
41
Almost all glioblastomas recur where?
Within 2 cm of their original site
42
What is the usual location of anaplastic astrocytoma in
Adults
Children
Adults - cerebrum
Children - cerebellum, hypothalamus, optic nerve/chiasm, pons [CHOP]
43
Clinical symptom present in 2/3 of Low and intermediate-grade astrocytomas
Focal or generalized seizures
*60-75% have recurrent seizures
44
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.
Gliomatosis Cerebri
45
Age at presentation of oligodendroglioma (bimodal)
6-12 years
30s-40s
46
Oligodendroglioma is more common in
Men/Women
Men
47
Clinical implications of the ff genetic changes in Oligodendrogliomas:
A) Loss of alleles on chromosome 1p
B) Loss of chromosome 19q
x alleles on chromosome 1p = high degree of responsiveness to PCV chemotherapy
X chromosome 19q = longer survival
48
Common locations of Oligodendroglioimas
Frontal and temporal lobes (40-70%)
49
Most common clinical presentation of Oligodendrogliomas
Focal or generalized seizures
50
Imaging findings in Oligodendrogliomas (3)
CCC
Cortical surface heterogenous mass w/ well-defined borders
Intratumoral Calcification
Contrast enhancement
51
Aspects of oligodendrogliomas that show good response to chemo
(PCV or TMZ)
1p/19q codeletion
IDH mutations
Anaplastic
52
Most common glioma of the spinal cord
Ependymoma
53
Most common cerebral site of Ependymomas
4th ventricle
54
Typical location of myxopapillary ependymomas
Filum terminale
55
Genetic abnormality associated with ependymomas
RELA-fusion positive in supratentorial tumors of children
| Chromosome 11
56
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
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
57
Most frequent genetic abnormality associated with meningiomas
Merlin NF2 Gene on Chromosome 22q
- truncating (inactivating) mutations
- freq in fibroblastic, transitional types
58
Cells of origin for meningiomas
Arachnoidal (meningothelial) cells forming the archnoid villi
59
Classic histologic finding in meningiomas
Psammoma bodies
| Laminated calcific concretions
60
Most common type of meningioma
Meningothelial (syncitial) form
61
Neuroimaging findings for meningiomas
Tumor blush
Smooth contour
Dural tail
Homogenous enhancement
BCDE
Blush, Contour, Dural, Enhancement
62
True or false. CSF protein may be elevated in Meningiomas
True
63
Usual age at presentation of primary CNS lymphoma?
> Normal population
> AIDS
Normal pop: 5th-7th decades of life
Pts w/ AIDS: 3rd-4th decade of life
64
Re: usual locations of PCNSL:
60%: _____
10-20%: _____
60%: cerebral hemispheres
10-20%: Retinal, Uveal, Vitreous involvement (RUV your eyes)
65
Which is NOT true of PCNSL’s histologic picture?
A) Highly cellular
B) Angiocentric pattern
C) (+) Necrosis
D) None of the above
C) (+) Necrosis
No tendency to necrosis in PCNSL
66
Which is NOT true of the imaging findings in PCNSL?
A) Several dense lesions
B) (+) Diffusion restriction
C) Non-necrotic
D) Hemorrhagic
D) Hemorrhagic
PCNSL: Non-hemorrhagic
67
True or false. CSF lymphocytic and mononuclear pleocytosis are found more frequently in PCNSL than in gliomas AND metastatic tumors
True
68
True or false. In PCNSL, CSF cytology is more sensitive than Immunoglobulin heavy chain gene (IGH)
False
IGH is more sensitive than cytology of spinal fluid
69
Choice of chemotherapeutic agents for PCNSL?
Methotrexate-based regimens
70
Prognosis for PCNSL?
Survival rates at best near 50%
71
What is the distribution of intracranial metastatic CA in terms of their location in the brain?
80%: ____
20%: ____
80%: cerebral hemispheres
| 20%: posterior fossa
72
Skull and dural mets are associated with (3):
Breast CA
Prostate CA
Multiple Myeloma
73
What are the most common sources of brain mets?
1. ___
2. ___
3. ___
1. Lung CA
2. Breast CA
3. Melanoma
* GI (rectum, kidney) > renal
74
Neoplasms with the highest tendency to metastasize to the brain
1. ___
2. ___
3. ___
1. Melanomas (75%)
2. Testicular tumor
3. Bronchial CA
Metastasize To Brain
75
Metastatic tumors most likely to be
A) Single (4)
B) Multiple (2)
Single - Kidney, breast, thyroid, Lung adenoCA
Multiple: Small cell CA, melanomas
76
Metastatic tumors likely to be hemorrhagic
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
In stroke-like events in CA patients, consider ____.
Bacterial thrombotic (marantic) endocarditis
78
Stereotactic RT is arbitrarily limited to __ mets
4
79
When is surgical extirpation indicated for single metastatic brain lesions?
Growth of primary tumor & systemic mets is under control
Mets are accessible to surgeon
Not located in a strategic motor or language area
80
In terms of prognosis, what is the life expectancy for those w/ brain mets who’ve undergone therapy?
6 months
81
Characteristic presentation of carcinomatous and lymphomatous meningitis
CN neuropathy + bilateral limb weakness, asymmetrical
82
CSF picture of carcinomatous and lymphomatous meningitis
Pressure: increased
CSF protein: elevated
CSF sugar: decreased
Lymphocytic pleocytosis (up to 100)
83
Treatment of Carcinomatous meningitis
RT + intraventricular methotrexate
84
Median duration of survival after diagnosis of carcinomatous meningitis
6 months
85
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
B) More common in myelocytic than in lymphocytic leukemia
NS involvement more common in lymphocytic leukemia
86
Solid green-colored mass of myelogenous leukemic cells that infiltrate the dura
Chloroma
87
Most common neurologic complication of all types of lymphoma
Extradural compression of the spinal cord
88
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
D) Cauda equina involvement is rare
*Cauda equina is involved eventually in most cases
89
Castleman disease is precipitated by constituent ____ infection
HHV-8
90
Which clinical symptom appears in the late stages of Medulloblastoma?
Decerebrate attacks / cerebellar fits
91
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
C) N-MYC amplification a/w a better prognosis
N-MYC = poorer prognosis
92
Histologic findings in medulloblastoma
Pseurorosettes
93
Treatment of medulloblastoma
Maximal resection
Chemotherapy
RT of entire neuraxis
*5y survival 80%
94
True or false. Desmoplastic features of medulloblastoma are a/w better prognosis
True
95
Most common solid tumor of childhood
Neuroblastoma
| *arises in the adrenal medulla
96
What clinical syndrome (symptoms) occur in neuroblastoma?
Polymyoclonus w/ opsoclonus and ataxia - parang na...blast...
(Paraneoplastic complication)
97
Genetic abnormality in Retinoblastoma
Abnormal protein encoded by a growth suppressor or antioncogenic gene (Rb)
98
70% of ependymomas originate where?
A) Lateral ventricles
B) 3rd ventricle
C) 4th ventricle
D) None of the above
C) 4th ventricle
e4
e4t??
99
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
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
Hemangiomas of the cerebellum
Retinal angioma
Hepatic/pancreatic cysts
Polycythemia
Hemangioblastoma of the Cerebellum
101
True or false. Sleep is often affected in pineal gland tumors
False (Adams)
102
Most common pineal region tumor
Germinoma (50%)
103
Tiger stripe appearance. PTEN gene.
Lhermitte-Duclos Disease
| Dysplastic Gangliocytoma of the Cerebellum
104
Dysembryoplastic neuroepithelioma tumor has a proclivity for the ___ lobe.
Temporal lobe
105
Subependymal Giant Cell Astrocytoma
Associated with?
Arise where?
Treatment?
A/w: Tuberous Sclerosis
Arise near: foramen of Monro
Tx: Everolimus (mTOR complex inhibitor)
106
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
Colloid cyst of the 3rd ventricle
107
Arachnoid Cyst
Clinical presentation in children?
Location?
Treatment?
Clinical: macrocrania, u/l transillumination
Location: sylvian fissure, temporal pole
Tx: marsupialization
108
Syndromes (3) associated with Vestibular Schwannomas and differences
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
For pts w/ vestibular schwannomas who undergo surgical excision, hearing can be preserved if lesion is ___ cm
< 2.5 cm
110
Apart from surgical excision, what are the other treatment options for VS?
RT - older pts w/ few sx
Chemo (Bevacizumab) - pts w/ NF2
111
Most common location of Craniopharyngiomas
Above the sella turcica
112
On gross pathology, the cysts of craniopharyngiomas contain what? (3)
Dark albuminous fluid
Cholesterol crystals
Calcium deposits
113
Clinical presentation of craniopharyngiomas in children
Visual loss + Diabetes insipidus
114
Glomus jugulare tumor is most commonly found among what population of patients *geographic location*?
Patients living at high altitudes
115
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
E) TIAs
116
Associations with Carotid Body tumor (3)
1 geographic location of pts
2 syndromes
Patients living at high altitudes
NF1
VHL
117
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
```
B) Vascular polyp, EAM (found in glomus jugulare tumors)
118
What type of pituitary adenomas is most common in both men and women? (60-70%)
Prolactin-secreting tumors
119
Pituitary adenomas are most often composed of _____ cells
Chromophobe
120
Products of chromophobe/acidophils
Prolactin
GH
TSH
121
Products of Basophils
ACTH
LH
FSH
B-lipotropin
* ALFBet
* Baso/Basic Alphabet
122
Natural clinicoanatomic progression of pituitary tumors
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
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
C) Larger tumor in females
124
Clinical presentation. Overproduction of GH:
Before puberty
After puberty
Before puberty: gigantism
After puberty: acromegaly
125
GRH antagonist
Pegvisomant
126
Early on, in pituitary tumors, the lower/upper parts of the visual fields are affected
Upper parts of the visual field
127
This symptom occurs in 50% of pts with macroadenomas
Headache
128
What is the empty sella syndrome?
Nontumorous enlargement of the sella 2/2 defect in the dural diaphragm
129
Treatment for prolactinoma
Bromocriptine 0.5-1.25mg/day
130
Treatment for acromegaly
Ocreotide (somatostatin analogue)
131
Risk factors for necrosis or hemorrhage of pituitary tumor
```
Anticoagulation
Pituitary function testing
Radiation
Bromocriptine
Head trauma
```
132
Treatment of Pituitary Apoplexy
Hydrocortisone to treat acute addisonian state
| If no improvement after 2 days, resect
133
Most common cause of ischemic necrosis of the pituitary
Sheehan Syndrome
134
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
B) Meningioma of the sphenoid ridge
135
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
C) Meningioma of the tuberculum sella
136
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) Meningioma of the olfactory groove
137
Usual age of presentation of brainstem gliomas
7 yo
138
Chordomas are soft, jelly-like, gray-pink growths arising from where?
Primitive notochord (remnants)
139
Chordomas are most often located in
Within the clivus (dorsum sellae-foramen magnum)
| Sacrococcygeal region
140
Most common symptom (?) of paraneoplastic d/o
Sensory neuropathy (54%)
141
Anti-NMDA Encephalitis is associated with which tumors? (2)
Ovarian Teratoma
| Small cell lung CA
142
Antibodies associated with paraneoplastic sensory neuropathy
Anti-Hu > Anti CRMP5
143
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
B) Widespread distal distribution
Proximal. Hence apparent as sensory ganglionitis and radiculitis
144
Most characteristic of the paraneoplastic syndromes
Paraneoplastic cerebellar degeneration
145
Most commonly associated tumor w/ paraneoplastic cerebellar degeneration
Lung CA - small cell
146
What are the associated tumors in Opsoclonus-Myoclonus-Ataxia Syndrome?
A) in children
B) in adults
In children - neuroblastoma
IN adults - breast CA, small cell lung CA
147
Antibodies in Opsoclonus-Myoclonus-Ataxia Syndrome
Anti-Ri ANNA 2
148
Antibody associated with rapidly progressive amyotrophy and fasciculations w/ or w/o brisk reflexes
Anti-Hu
149
Ophelia syndrome is due to antibodies against _____
mGluR5 (metabotropic glutamate receptor)
150
Paraneoplastic optic neuropathy is associated with which antibody?
CRMP-5 antibody
151
Most common underlying malignancy for paraneoplastic retinopathy
Small cell CA of the lung
Lesion: antibodies against recoverin
152
Most common paraneoplastic neurologic syndrome
Lambert-Eaton Syndrome
153
CAs (3) associated with Isaac Syndrome / Paraneoplastic Neuromyotonia
Lung CA
Lymphoma
Thymoma
