Tumors Flashcards
(144 cards)
1
Q
Indicate the WHO grade of the ff tumors:
- SEGA
- Diffuse astrocytoma
- Anaplastic astrocytoma
- Pineoblastoma
- Subependymoma
- Ependymoma
- Ganglioglioma
- Dysembryoplastic neuroepithelial tumor
A
- SEGA 1
- Diffuse astrocytoma 2
- Anaplastic astrocytoma 3
- Pineoblastoma 4
- Subependymoma 1
- Ependymoma 2
- Ganglioglioma 1
- Dysembryoplastic neuroepithelial tumor 2
2
Q
- PNET
- Atypical meningioma
- Hemangiopericytoma
- Craniopharyngioma
A
- PNET 4
- Atypical meningioma 2
- Hemangiopericytoma 2
- Craniopharyngioma 1
3
Q
What kind of edema is seen in:
- Lead encephalopathy
- Malignant hypertension
A
Vasogenic
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
5
Q
What are the 3 mechanisms of vasogenic edema?
A
- Loose tight endothelial junctions
- Active vesicular transport
- Protease induced protein fragments that may generate osmotic effect
6
Q
What kind of edema?
- SIADH
- Hepatic encephalopathy
- Osmotic disequilibrium syndrome of hemodialysis
A
Cytotoxic edema`
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
8
Q
What does cytotoxic edema look like on DWI compared to vasogenic edema
A
C: reduced diffusivity
V: elevated diffusivity
9
Q
T or F D5 NS may be given to patients with increased ICP
A
T
10
Q
What is the MOA of steroids for decreasing ICP?
A
Reduce endothelial cell permeability and shrink normal tissue
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
12
Q
What are signs of upward herniation?
A
Decerebrate posturing
Pupils: miosis –> anisocoria
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
14
Q
What % of GBM is multicentric?
A
3-6%
15
Q
Identify which of the following factors bode for a good prognosis in GBM patients:
- IDH1 and IDH2 mutations
- MGMT methylation
A
- Good
2. Good
16
Q
Which are associated with GBM from degeneration?
- p53 mutation
- EGFR amplification
- Younger age group
- IDH1 and IDH2 mutation
A
1, 3, 4
17
Q
What is the 1 year survival rate of GBM?
A
<20%
Only 10% live beyond 2 years
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)
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%
20
Q
What is the most common type of astrocytoma?
A
Well differentiated fibrillary astrocytes (Grade 2)
21
Q
What proportion of patients with astrocytoma present with seizures?
A
2/3
22
Q
T or F Early RT in low grade glioma increases PFS but not OS?
A
T
23
Q
T or F. There is scant enhancement of gliomatosis cerebri differentiating it from CNS lymphoma.
A
T
24
Q
What histologic features differentiate Oligodendroglioma?
A
Small round nucleus with a halo of unstained cytoplasm
Microscopic calcifications
25
What does a 1p19q deletion imply for oligodendroglioma?
1p responsiveness to PCV
| 19q associated with longer survival
26
What regimen is used for chemo of oligdendroglioma?
Procarbazine, Cyclophosphamide, Vincristine PCV or
| Temozolomide
27
What type of ependymoma is exclusively located in the filum terminale?
Myxopapillomatous
28
What is the most common glioma of the cord?
Ependymoma
29
What is the most common cerebral site of ependymoma?
4th ventricle-- 70% of arise here
30
What is the most common acquired genetic defect of meningiomas?
Truncating mutations in the NF2 gene (merlin) on chromosome 22
31
Where do meningiomas originate?
Arachnoid meningothelial cells REMEMBER THAT THESE CELLS ARE ALSO WITHIN THE CHOROID PLEXUS therefore intraventricular meningiomas can also exist
32
What is the most common form of meningioma?
Meningothelial or syncitial form
33
What tumors calcify?
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)
34
What are the malignant cells in primary CNS lymphoma?
Diffuse large cell type. B lymphocytes.
35
What percentage of primary CNS lymphoma would have ocular involvement?
10-20% BUT 2/3 of patients with ocular lymphoma would have cerebral involvement
36
Meningeal and cranial nerve lymphoma with similar histologic characteristics to primary CNS lymphoma that are actually complications of chronic lymphatic leukemia is called?
Richter transformation
37
T or F. Primary CNS lymphoma usually presents with infiltrating NECROTIC, HEMORRHAGIC periventricular masses.
F. NON necrotic NON hemorrhagic
38
T or F Lymphoma is associated with EBV in immunocompromised patients
T
39
What is the median survival of primary cns lymphoma patients on methotrexate with radiation?
4 years. Give methotrexate 3.5g per m2
40
What kinds of cancers have a tendency to send mets to the posterior fossa?
Pelvis and colon
41
What are the common tumors that send mets to the skull and dura?
Breast: systemic circulation
Prostate: Batsons
Multiple myeloma
42
What are the most common tumors to send mets to the brain parenchyma?
```
Lung
Breast
Melanoma
Colon and Rectum
Kidneys
```
43
T or F. Mets from the prostate, esophagus, oropharynx commonly metastasize to the brain.
F
44
What tumors ARE PRONE to send mets to the brain?
Melanoma 75%
Testicular 55%
Bronchial CA 35% (of which 40% are Small cell CA)
45
What intracranial mets are often hemorrhagic?
Melanoma
Chorioepithelioma
Lung, thyroid, kidney
BUT LUNG CA IS STILL THE MOST COMMON METASTATIC TUMOR TO BLEED ON ACCOUNT ITS RELATIVE FREQUENCY
46
How to give RT to brain mets?
WBRT over 2 weeks 10 doses of 300 cGy each
47
What are the prerequisites to excision of a metastatic tumor?
1. Single
2. Growth of primary controlled
3. Systemic mets controlled
4. Accessible location in non-eloquent area
48
What is the average period of survival in patients with brain metastases?
6 months
49
What tumor may have carcinomatous meningitis as its first presentation?
Gastric
50
What are the 3 cardinal symptoms of carcinomatous meningitis?
1. Polyradiculopathy
2. Cranial nerve palsies
3. Confusional state
51
What cases of carcinomatous meningitis respond relatively well to RT + MTX?
breast and lymphoma
52
What proportion of patients with leukemia will involve the CNS?
1/3
53
How to differentiate tumor mets from necrotizing leukoencephalopathy?
mets enhance!
54
What is the most common neurologic complication of all types of lymphoma?
Extradural compression of the spinal cord
55
Hodgkin lymphoma meningeal involvement would show what on LP?
Eosinophilic pleocytosis
56
What cranial nerve is involved with meningeal dissemination of NHL?
CN 8
57
Differentiate the location of medulloblastoma in children and in adults.
Children: cerebellar vermis
Adults: Lateral cerebellum and cerebrum
58
What is the 5 year survival rate in medulloblastoma with surgery radiation and chemo?
80%
59
What is the most common solid tumor of childhood?
Neuroblastoma
60
In cases of cerebellar foraminal herniation due to a tumor the occiput of the head moves where?
away from the side of the tumor... head tilt
61
What are the locations of choroid plexus papilloma?
| 50:10:40 ratio
Lateral:3rd:4th ventricles
62
What hematologic abnormality is associated with Hemangioblastoma?
Polycythemia on account of elaboration of erythropoietic factor
63
What comprises VHL?
```
Hemangioblastoma
Retinal angioma
Hepatic or pancreatic cyst
Pheochromocytoma
Renal cell carcinoma
```
64
What are the 4 types of pineal region tumors and which one is most prevalent?
1. Germinoma MOST COMMON 50%
2. Non-germinomaous germ cell tumor
3. Pinealoma
4. Glioma
65
What serum levels are elevated with a germ cell tumor of the pineal region?
Beta HCG: Choriocarcinoma
| AFP: Endodermal sinus tumors (Yolk sac tumor) and teratoma
66
What cerebellar tumor has the tiger stripe appearance due to layers of dysmorphic cerebellar cells?
Dysplastic gangliocytoma of Lhermitte duclos disease
67
What does dysembryoplastic neuroepithelioma tumor look like on imaging?
nodule or small cyst that is juxtacortical or intracortical
68
What other nerves are involved by schwannoma?
besides 8, 5 and spinal nerve roots
69
T or F. Acoustic neuroma always arises from the acoustic portion of the CN 8
F. Vestibular
70
What is the most common early symptom of acoustic neuroma?
Hearing loss
71
What test is most sensitive to the presence of acoustic neuroma?
BAER
72
Craniopharyngioma originates from?
Adenohypophysial diverticulum AKA Rathke's pouch
73
What is the origin of glomus jugulare tumors?
Non chromaffin paraganglioma cells
74
```
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
```
Glomus jugulare tumor
75
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?
Paranglioma or Carotid body tumor
MAY BE AN ETIOLOGY OF TIA
76
Chromophone and acidophil cells produce what hormones? (Pit Ad)
Prolactin, GH, TSH
| PGT!
77
What are the most common hormones secreted by a Pit Ad?
PGAT
| Prolactin 60-70%, GH 10-15%, ACTH, TSH
78
T or F
Pit Ad: Affects upper visual fields first
Craniopharyngioma: Affects inferior fields first
T
79
What are the manifestations of hypothalamic compression?
DI
Temperature dysregulation (Anterior part is for cooling)
Sleep abnormalities (Destruction of lateral part results in narcolepsy)
Apetite (Medial part is satiety center)
80
What are the manifestations of a prolactinoma in men and women?
Women: Amenorrhea galactorrhea
Men: Impotence
81
What growth hormone receptor antagonist is used for acromegaly? How about the somatostatin analogue?
Pegvisomant-- Receptor antagonist
| Octreotide-- Somatostatin analgoue
82
T or F. Pituitary tissue is normally enhancing hence small tumors appear as relatively hypoenhancing nodules
T
83
What pituitary hormones increase with administration of TRH (Thyrotropin releasing hormone)?
Prolactin, GH and TSH
PGT as well secreted by chromophobes and acidophils
84
What is the main defect resulting in an empty sella syndrome?
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
What is the MOA by which bromocriptine reduces prolactin secretion?
Dopamine agonist
Cabergoline is also a DA
86
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
T
THERE SHOULD BE NO THREAT TO VISION AT THE START OF THERAPY
87
What structures are affected by sphenoid wing meningioma?
Cavernous sinus
Orbit
Temporal bone
May present with foster kennedy syndrome
88
What are the 2 common locations of a chordoma?
Clivus + Sacrococcygeal region
89
What cranial nerves can be involved with a chordoma at the clivus?
CN 2 - 12
90
What characteristic pain is associated with chordoma?
Neck pain radiating to vertex on neck flexion
The tumor may destroy the clivus and bulge into the nasopharynx
91
What are ddx for base of the skull tumors?
1. Osteomas
2. Chordomas
3. Epidermoids
4. Teratomas
5. Nasopharyngeal CA
6. Meningioma
92
The bobble head doll syndrome is associated with what tumor?
Suprasellar arachdoid cyst
Due to a thickened arachnoid (membrane of lillequist)
Choroid plexus papilloma
93
What are ddx for a foramen magnum tumor?
1. Meningioma
2. Schwannoma
3. Neurofibroma
4. Dermoid
94
Identify the site of lesion of the following eponyms
1. Rochon-Duvigneau
2. Jacod Rollet
3. Foix- Jefferson
4. Gradenigo-Lannois
5. Jacod
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
Identify the site of lesion of the following eponyms
6. Vernet
7. Collet Sicard
8. Villaret
9. Garcin
6. Vernet- Jugular foramen (9,10,11)
7. Collet Sicard- Anterior occipital condyles (9,10,11,12)
8. Villaret- Retroparotid space (9,10,11,12,sympathetic)
9. Garcin- Half of the base of the skull (all 12)
96
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)
1. Anti-yo (Ovarian) Cerebellar degeneration
2. Anti-Hu (Small cell lung CA) Encephalomyelitis, Neuropathy sensory, Chorea
3. Anti-NMDA (Ovarian teratoma) Encephalomyelitis
97
Identify the following neurologic disorder brought about by the following autoantibodies
4. Anti-recoverin (Small cell lung CA)
5. Anti-volatage gated calcium channel (Small cell lung CA, Hodgkin)
6. Anti CRMP-5 (Lung)
7. Anti Ri/ ANNA 2 (Breast, small cell CA)
4. Anti-recoverin (Small cell lung CA) Retinal degeneration
5. Anti-volatage gated calcium channel (Small cell lung CA, Hodgkin) Lambert eaton myasthenic syndrome
6. Anti CRMP-5 Optic neuropathy
7. Opsoclonus myoclonus ataxia
98
What are the MRI findings in both limbic encephalitis and anti nmda encephalitis?
T2 hyperintensities
99
What type of radiation injury?
1. More than 3 months post radiation
2. Fibrinoid necrosis with microthrombosis
3. Enlargement of tumor mass
1. early/late delayed
2. late delayed
3. early delayed
100
How does PET dfx between tumor progression and radiation necrosis
Cerebral blood volume is deceased in radiation necrosis and increased in tumor progresssion
101
What autoantibodies cause stiff person syndrome?
Antiamyphiphysin, Anti-CAspr2, Anti-GAD
102
What neurologic disorders can be caused by anti CRMP5?
Optic neuropathy, Chorea, Encephalomyelitis
103
What neurologic disorders are caused by anti Hu?
Encephalomyelitis including the brainstem and limbic
Subacute sensory neuropathy and neuronopathy
Chorea
104
What syndrome can be caused by anti VGKC antitbodies?
Limbic encephalitis and lambert eaton
105
What is the target of the anti-NMDA antibody?
The NR1 receptor
106
What did Denny Brown describe in 1948?
Sensory neuronopathy and neuropathy usually caused by anti-Hu
107
Where do anti Yo antibodies bind to to destroy purkinje cells?
C-myc protein
108
In the pediatric age group what cancer is opsoclonus-myoclonus-ataxia syndrome related to?
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
T or F there is a paraneoplastic kind of Devic syndrome
T
110
What is the most common paraneoplastic neurologic syndrome?
Lambert Eaton syndrome
111
What is SMART?
Stroke like migraine attacks after radiation therapy
112
What are the cancers most commonly associated with PN disorders?
1. Small cell lung CA
2. AdenoCA of lung
3. AndenoCA of ovary
4. Hodgkin lymphoma
113
Anti-Caspr2 is associated with what PN disorders?
Limbic and brainstem encephalitis
| Stiff person syndrome and neuromyotonia
114
What antibody is responsible for retinal degeneration?
Antirecoverin (anti CAR) seen in small cell lung, thymoma, renal cell and melanoma
115
Besides sensory loss what are the other symptoms attached to paraneoplastic sensory neuronopathy?
1. Lancinating pains initially
2. Disabling ataxia
3. Pseudoathetoid movements of the outstretched hands
4. Autonomic dysfunctions
116
Besides small cell lung CA what other 2 CA account for paraneoplastic cerebellar degeneration? 25 and 15% respectively.
Ovarian CA and Hodgkin disease
117
What percentage of patients with a clinical picture of paraneoplastic cerebellar degeneration will have anti-Yo?
1/2
118
What is another name for anti-Yo antibodies? Where do they bind?
Anti-Purkinje cell antibodies. C-myc protein
119
Anti-Ma is usually associated with tumors of what region?
Testicular
120
What syndrome is associated with Anti mGluR5?
Ophelia syndrome: Memory loss in a patient Hodgkin lymphoma
121
What is an extraordinaty distorder of continuous muscle fiber activity, insomnia and hallucinosis?
Choree fibrillaire
| Anti VGKC
122
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
1. Acute: during the latter part of the radiation cycle or soon after
2. Early delayed
3. Late delayed
123
What percentage of childhood brain tumors does medulloblastoma account for?
20%
124
What two tumor syndromes feature medulloblastoma?
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
How do medulloblastomas look on MRI?
HIGH INTENSITY IN BOTH T1 AND T2
With heterogenous enhancement
Located on the vermis extedning into the 4th ventricle
126
What is the 5 year survival rate in medulloblastomas that have surgery, radiation of the entire neuraxis and chemotherapy in a timely manner?
80%
127
What blood abnormality is associated with VHL diseae?
Polycythemia because of the elaboraiton of EPO by the hemangioblastoma.
128
Gangliocytoma is associated with a germ line mutation of what gene? What is the prognosis of this tumor?
PTEN
LACK OF GROWTH POTENTIAL AND FAVORABLE PROGNOSIS
129
What is a common cause of a mass in the third ventricle that causes a ball valve headache?
Colloid paraphysial cyst
130
Which is due to excessive secretion of pituitary ACTH? Cushing disease or syndrome?
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
What are the ddx for bitemporal hemianopia with a normal sized sella?
1. Saccular aneurysm of the distal carotid artery
2. Meningioma
3. Craniopharyngioma
132
T or F: Chordomas do NOT metastasize
T
133
What is the mean age for the occurrence of GBM? How about anaplastic astrocytoma? How about Oligodendroglioma?
60 YO
46 YO
20-30s YO
134
What two pathologic features differentiate GBM from anaplastic astrocytoma?
Necrosis and hemorrhage
135
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
Amplication of the EGFR gene
136
What does an IDH1 and IDH2 mutation imply for GBM?
The tumor at hand degenerated from a more benign tumor. These have a better prognosis than those that start off as GBM.
137
A methylated MGMT promoter gene makes a tumor more or less responsive to chemo?
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
What percentage of GBM patients survive beyond 2 years without treatment?
10.4% VS 26.5
139
How will planned delay in the administration of radiation in young astrocytoma patients affect the overall survival of the patient?
No effect. OS is still 7 years.
BUT the PFS is lowered from 5.3 to 3.4
140
What tumors have:
1. Homer Wright Rosettes
2. Flexner-wintersteiner rosettes
3. True ependymal rosettes
4. Perivascular pseudorosette
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
Which tumors tend to be multiple when sending mets to the brain?
Small cell CAs and melanomas
TEND TO BE SINGLE: Kidney, Breast, Thyroid, AdenoCA of the lung
142
What is the arbitrary limit for doing focused RT vs WBRT?
4 mets if more do WBRT
143
What condition may come up when ALL patients with CNS relapse are treated with RT, IT and IV MTX are used?
Necrotizing leukoencephalopathy
144
What are the relative volumes of the:
1. Brain
2. CSF
3. Blood
1. Brain: 1200-1400ml
2. CSF: 70-140
3. Blood: 150
