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Neurology - Physiology > TUMORS NEW > Flashcards

TUMORS NEW Flashcards

1
Q

OLD CLASSIFICATION OF CNS TUMORS

A
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2
Q

OLD CLASSIFICATION OF CNS TUMORS

A
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3
Q

OLD CLASSIFICATION OF CNS TUMORS

A
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4
Q

OLD CLASSIFICATION OF CNS TUMORS

A
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5
Q

The glioblastomas are largely defined by the features of

________ and _______of nonneural elements such as

vascular proliferation and are set apart from anaplastic

astrocytomas on the basis not only of their histology but

also by a later age of onset than astrocytoma and a more

rapid course.

A

necrosis and anaplasia

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6
Q

For many years, thinking about the pathogenesis ofprimary CNS tumors was dominated by the _________________of Bailey and Cushing, which was based on the

assumed embryology of nerve and glia cells.

A

histogenetic theory

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7
Q

One prominent theory was that most tumors arise from neoplastic transformation of mature adult cells ________

A

dedifef rentiation

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8
Q

In fact, the cells of origin of the major types of brain

tumors have not been unequivocally identified or, in many

cases, they appear to arise from_____________

that reside in the brain

A

pluripotential stem cells

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9
Q

Medulloblastomas, polar spongioblastomas,

optic nerve gliomas, and pinealomas occur mainly

before the age of ________years, and meningiomas and glioblastomas are most frequent in the _______decade of life.

A

20

sixth

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10
Q

The _________associated with neurofibromatosis and

tuberous sclerosis and the cerebellar hemangioblastomas

of _________are the best examples of a genetic

determinant

A

gliomas

von Hippel-Lindau

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11
Q

The rare familial disorders of multiple endocrine

neoplasia and multiple hamartomas are associatedwith an increased incidence of ________ and ________respectively

A

anterior pituitary tumors

and meningiomas,

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12
Q

Because of this capacity to transform the cellular genome, the virus product is called an________

A

oncogene;

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13
Q

Because of this capacity to transform the cellular genome, the virus product is called an_________

A

oncogene;

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14
Q

More recently, single nucleotide polymorphisms

have been identified that in combination predispose to

certain childhood tumors such as _________, or to

the more aggressive forms of various tumors

A

neuroblastoma

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15
Q

over 50 percent of astrocytomas have deletions within this gene

A

tumor suppressor gene p53 on chromosome 17p;

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16
Q

mutations in the genes that code for __________are common in gliomas and oligodendrogliomas

and their presence relates to less tumor progression.

A

isocitrate dehydrogenase(IDH1

and 2)

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17
Q

oligodendrogliomas that have combined deletions in

___________respond well to chemotherapy

and this property increases survival

A

chromosomes 1p and 19q

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18
Q

various changes such as the amplification of the

__________oncogene has been associated with an aggressive clinical course and poor outcome in neuroblastoma and

medulloblastoma.

A

MYCN

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19
Q

approximately 50 percent of gliomas there is an overexpression or a mutant form of________ and ____________ suggesting a role for these in the progression of certain tumor types.

A

epidermal growth factor receptor (EGFR) and of plateletderived transforming growth factor receptor (PDGFR),

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20
Q

These high-grade gliomas account for approximately 20

percent of all intracranial tumors and for more than 80

percent of gliomas of the cerebral hemispheres in adults

A

GBM and Anaplastic Astrocytoma

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21
Q

The peak incidence is in middle adult life (mean age for

the occurrence of glioblastoma is approximately ______years, and ________years for anaplastic astrocytoma

A

60

46

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22
Q

In GBM,

Extraneural metastases, involving ___________, are very

rare; usually they occur only after a craniotomy

A

bone and lymph nodes

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23
Q

Approximately _______percent of glioblastomas

occupy more than one lobe of a hemisphere; between

_______percent show multicentric foci of growth and

thereby simulate metastatic cancer.

A

50

3 and 6

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24
Q

Gross appearance of GBM

A

The tumor has a variegated appearance, being a

mottled gray, red, orange, or brown, depending on the

degree of necrosis and presence of hemorrhage, recent or

old.

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25
Q

The characteristic histologic findings of glioblastomaare ___________, ____________ and ______________; identifiable astrocytes with fibrils in

combination with primitive forms in many cases; tumor

giant cells and cells in mitosis; hyperplasia of endothelial

cells of small vessels; and necrosis, hemorrhage, and

thrombosis of vessels.

A

hypercellularity with pleomorphism of cells

and nuclear atypia

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26
Q

This __________distinguishes

glioblastoma from the anaplastic astrocytomas,

which show frequent mitoses and atypical cytogenic

features but no grossly necrotic or hemorrhagic areas

A

variegated appearance

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27
Q

In GBM,

The vasculature and fibroblasts

can undergo a sarcomatous transformation giving the

tumor a mixed appearance that is termed______

A

gliosarcoma

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28
Q

The natural history of untreated glioblastoma is well

characterized. Fewer than _______percent of patients survive for 1 year after the onset of symptoms and only about ________percent live beyond 2 years

A

20

10

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29
Q

In GBM , Age is an important prognostic factor; fewer than_________ percent of patients older than age 60 years survive for 18 months, in comparison to two-thirds of patients younger than age ________years

A

10

40

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30
Q

Survival with anaplastic astrocytoma is somewhat better,

typically________

A

3 to 5 years

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31
Q

In pts with GBM who have Sz,

Serious skin reactions (erythema multiforme

and Stevens-Johnson syndrome) may occur in patients

receiving _______at the same time as cranial radiation

A

phenytoin

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32
Q

In GBM,

Cranial irradiation to a total dose of

__________increases survival by 5 months on average

A

6,000 cGy

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33
Q

In pts with GBM,

It had been considered for several decades that the

addition of chemotherapeutic nitrosourea agents such

as ______ and _________increase

survival slightly

A

carmustine (BCNU) or lomustine (CCNU)

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34
Q

The methylating agent ________given in the

form of an orally administered prodrug, has lower toxicity

and has been shown in several trials to produce slightly

superior vs other chemo agents in treating GBM

A

temozolomide,

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35
Q

In a large trial conducted by Stupp and colleagues, the median survival was 14.6 months with radiation and temozolomide, compared to 12.1 months with radiation alone, but 2-year survival was more than doubled from____________

A

10 to 27 percent

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36
Q

How to administer TMZ

A

The

drug is administered daily (75 mg/m2) concurrently with

radiotherapy and, after a hiatus of 4 weeks, given for 5 d

every 28 d for 6 cycles.

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37
Q

Main complications of TMZ

A

Its main complications are thrombocytopenia

or leukopenia in 5 to 10 percent of patients, and

rare cases of Pneumocystis carinii pneumonia

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38
Q

In GBM, Hegi and colleagues found a relationship between the epigenetic silencing of the promoter of this gene _________ and the response to temozolomide.

A

(“methylation status”)

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39
Q

In GBM

there may be an interaction

between MGMT methylation and mutations in other

genes such as_________

A

IDH1

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40
Q

In the Tx of GBM,

Tyrosine kinase inhibitors (_______ and ______) have

been developed in response to the upregulation of EGFR

A

erlotinib, gefitinib

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41
Q

Almost all glioblastomas recur within ________of their original site and _______ percent develop additional lesions at distant locations.

A

2 cm

10

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42
Q

In pts with recurrent GBM,

Reoperation is sometimes undertaken for local recurrences. The most aggressive approach-a second surgery and chemotherapy-can prove effective and has been generally used in patients younger than age __________years

whose original operation was many months earlier.

A

40

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43
Q

A promising approach for recurrent malignant

gliomas is the use of drugs that target the tumor’s

vasculature. Antiangiogenic agents such as_____

a VEGF inhibitor, sometimes given in combination with

chemotherapy, may delay progression and greatly reduce

cerebral edema, therefore diminishing the requirement

for corticosteroids, but also do not________

A

bevacizumab,

prolong survival

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44
Q

A recent but preliminary provocative observation from retrospective series has been that patients with glioblastoma receiving _______, ostensibly for concurrent CMV infections, had better survival than those who did not

receive the drug

A

valganciclovir

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45
Q

The lower-grade astrocytomas (grade II in the WHO classification),which constitute between ________percent

of cerebral gliomas, may occur anywhere in the brain or

spinal cord

A

25 and 30

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46
Q

Favored sites for anaplastic astrocytoma

A

cerebrum, cerebellum, hypothalamus, optic nerve

and chiasm, and pons

47
Q

Astrocytomas of the _______arise mainly in adults in their third and fourth decades or earlier;

astrocytomas in other parts of the nervous system, particularly the______ and _____, are more frequent in children and adolescents.

A

cerebral hemispheres

posterior fossa and optic nerves

48
Q

Histologic classification of astrocytoma

_______or fibrillary;

_______(enlarged cells distended with hyaline and eosinophilic material);

_______ (elongated, bipolar cells);

and mixed astrocytomaoligodendroglioma

types.

A

protoplasmic

gemistocytic

pilocytic

49
Q

In astrocytoma, The most common type is

composed of well-differentiated fibrillary astrocytes. The

tumor cells contain ______________,

which is a useful diagnostic marker in biopsy specimens

A

glial fibrillary acidic protein (GFAP)

50
Q

Cerebral astrocytoma is a slowly growing tumor of

infiltrative character with a tendency in some cases to

form_______ or _________

A

large cavities or pseudocysts

51
Q

It may not be possible on clinical or imaging grounds

to distinguish low-grade gliomas from a number of raretumors in childhood such as the______

A

dysembryoplastic neuroepithelioma (DNET)

52
Q

In about two-thirds of patients with astrocytoma, the first symptom is a _________, and between 60 and 75 percent of patients have recurrent seizures in the course of their illness

A

focal or generalized seizure

53
Q

___________types are sharply demarcated, with smooth borders and little edema. On Tl-weighted MRl, they are isointense or hypointense; on T2 sequences, hyperintense, and there tends to be marked enhancement of the nodular solid portion of the tumor after gadolinium infusion

A

Pilocytic

54
Q

Treatment of ow grade astrocytoma

A

removing accessible tumors when they are discovered,

and another, of observing the patient by sequential

imaging to determine if the tumor has transformed into

a more aggressive mode

55
Q

Most important aspect in treating cerebral astocytoma to prevent recurrency

A

resection

of the tumor nodule is of singular importance in

delaying or preventing a recurrence

56
Q

Natural Hx of LGG

A

The natural history of the low-grade gliomas is to

grow slowly and eventually undergo malignant transformation

57
Q

In LGG, An extensive randomized trial of early radiotherapy in adults demonstrated that median progression-free survival was extended to __________ years by early treatment in comparison to 3.4 years for

observation only and radiation treatment that was initiated

when signs of progression occurred, but that overall

survival was unaffected, averaging just over________ years in

both groups

A

5.3

7

58
Q

In this variant of high-grade glioma there is a diffuse

infiltration of neoplastic glial cells, involving much of one

or both cerebral hemispheres with sparing of neuronal

elements but without a discrete tumor mass

A

G l i o m atos i s C e re b ri

59
Q

If there is a syndrome that can be associated early on with

gliomatosis, in our experience it has been a nondescript

_____________sometimes mistaken

for depression or a subacute dementia, or pseudobulbar

palsy may be the first manifestation.

A

frontal lobe behavioral syndrome

60
Q

What differentiates PCNSL from Gliomatosis on MRI?

A

Contrast enhancement is scant, differentiating the tumor from cerebral lymphoma, which otherwise may have a similar appearance

61
Q

Peak age of ODG

A

most often in the third

and fourth decades, with an earlier peak at 6 to 12 years.

It is relatively infrequent,

62
Q

Gross appearance of ODG

A

pink-gray color and multilobular form, its relative avascularity and firmness (slightly tougher than surrounding brain), and its tendency to encapsulate and form calcium and small cysts.

63
Q

Most oligodendrogliomas, however, are grossly indistinguishable from other gliomas, and a proportion-up to half in some series-are mixed oligoastroet;tomas, suggesting that the precursor cell is________

A

pluripotential

64
Q

In ODG

By extending to the pial surface

or ependymal wall, the tumor may metastasize distantly

in ventricular and subarachnoid spaces, accounting for

________percent of the series of gliomas with meningeal dissemination

A

11

65
Q

Imaging of ODG

A

The appearance on imaging studies is variable, but

the most typical is a hypodense (on CT) or T2 hyperintense

(on MRI) heterogenous mass near the cortical surface with

relatively well-defined borders

66
Q

In ODG

loss of certain

alleles on chromosome ______has been predictive of a high degree of responsiveness to the below-described PCV chemotherapy regimen, and a similar loss on chromosome _____________ has been associated with longer survival

A

1p

19q

67
Q

__________has been the conventional treatment for oligodendroglioma

A

Surgical excision followed by radiation

therapy

68
Q

T or F

Well-differentiated oligodendrogliomas should

probably not receive radiation if seizures are well controlled

and there are no neurologic deficits

A

T

69
Q

In ODG

loss of regions on ________, median survival was over 10 years.

Mutations in _________also appear to confer survival benefit. Mixed oligodendrogliomas and astrocytomas should generally be treated like astrocytomas, but temozolomide probably suffice to treat both components

A

1p

IDH1

70
Q

There is also a __________type of ependymoma,

localized exclusively in the filum terminale of the

spinal cord

A

myxopapillomatous

71
Q

this is the most common glioma of the spinal cord

A

ependymoma

72
Q

MC sites for IC ependyoma

A

The most common cerebral site is the fourth ventricle;

less often they occur in the lateral or third ventricles

73
Q

In ependymo,a, The

tumor cells tend to form rosettes with central lumens or,

more often, circular arrangements around blood vessels

A

pseudorosettes

74
Q

T or F,

the postoperative survival of ependymoma is

poor

A

T

75
Q

Tx of ependymoma

A

Surgical removal is supplemented by radiation therapy, particularly to address the high rate of seeding of the ventricles and spinal axis

76
Q

Meningiomas represent approximately 15 percent

of all primary intracranial tumors; they are more common

in women than in men (2: 1) and have their highest

incidence in the _______ and _______decades of life

A

sixth and seventh

77
Q

The most frequent acquired genetic defects of meningiomas are truncating (inactivating) mutations in the

_________on chromosome_______

A

neurofibromatosis 2 gene (merlin)

78
Q

Chromosome abberations associated with meningioma

A

1p, 6q, 9p, 10q, 14q, and 18q

79
Q

Meningiomas

also elaborate a variety of soluble proteins, some of which

__________are angiogenic and probably relate to both the

highly vascularized nature of these tumors and their

prominent surrounding edema

A

(VEGF)

80
Q

The cells of meningiomas are relatively uniform,

with round or elongated nuclei, visible cytoplasmic

membrane, and a characteristic tendency to encircle one

another, forming whorls and _________(laminated

calcific concretions).

A

psammoma bodies

81
Q

EM characteristics of Meningioma

A

formation of very complex interdigitations

between cells and the presence of desmosomes

82
Q

MC form of meningioma

A

Currently neuropathologists recognize

a meningothelial (syncytial) form as being the most common.

83
Q

Characteristic of Meningioma on Angiography

A

These changes

are reflected by homogeneous contrast enhancement and

by “tumor blush” on angiography

84
Q

Most difficult to remove Meningioma

A

Tumors that lie beneath the hypothalamus, along the medial part of the sphenmd bone and parasellar region, or anterior to the br􀄈tem 􀃌e the most difficult to remove surgically.

85
Q

In PCNSL

There is􀃐 a preponderance of men, with the peak incidence

m the _________decades of life, or in the third

and fourth decades in patients with______

A

fifth through seventh

AIDS.

86
Q

Cells of origin of PCNSL

A

lymphocytes and lymphoblasts

87
Q

In PCNSL,

The B lymphocyte or

lymphoblast is the tumor cell, whereas the_____

and _________are secondary interstitial reactions

A

fine reticulum

“microgliacytes”

88
Q

Location of PCNSL

with _______percent being 􀎆 the cerebral hemispheres;

they may be solitary or multifocal.

A periventricular localization is common.

Vitreous, uveal, and retinal (ocular) involvement occurs in__________ pe􀃕rc􀃖ent of cases;

A

60

10 to 20

89
Q

The pia and arachnoid may be infiltrated, and a

purely meningeal form of B-cell lymphoma that involves

peripheral and cranial nerves.

It presents

with painful, predominantly motor polyradiculopathies.

A

neurolymphomatosis

90
Q

PCNSL forms a pinkish gray; soft, ill-defined,

infiltrative mass in the brain, difficult at times to distinguish

from an_______

A

astrocytoma

91
Q

Several of our cases of meningeal and cranial nerve

lymphoma with similar histologic characteristics to primary

CNS lymphoma were complications of chronic lymphatic

leukemia, a type of so-called_________

A

Richter transformation

92
Q

In PCNSL,

The immunohistochemical demonstration

in CSF of monoclonal lymphocytes or an elevated betamicroglobulin points to____

A

leptomeningeal spread of the

tumor

93
Q

In PCNSL,

the median survival of patients

treated in this way has been _____months and less in those with AIDS and in individuals who are otherwise immunocompromised.

A

10 to 18

94
Q

Tx of PCNSL

A

Glass and colleagues (1994) recommended a treatment

regimen consisting of several cycles of intravenous

methotrexate (3.5 g/m2) and citrovorum, administered at

2- to 3-week intervals and at times continued indefinitely

if the treatment is tolerated

95
Q

T OR F

IN PCNSL

Cranial irradiation is not

typically part of the initial treatment

A

T

96
Q

Ocular lymphoma

is eradicated only by_____

A

radiation therapy

97
Q

Cancers of the ______ and ______are exceptional in

that they have a somewhat higher tendency than

this to spread to the posterior fossa

A

pelvis and colon

98
Q

Metastases to the skull and dura occur with any tumor that metastasizes to bone, but they are particularly common with carcinoma of the ______,_______ and ______

A

breast and prostate and in the special case of multiple myeloma

99
Q

MC brain mets tumors

A

Lung, Breast, MM, colon/rectum/kidney

100
Q

The metastatic tumors most likely to be single come from

1.

2.

3.

4.

A

kidney, breast, thyroid, and adenocarcinoma of the lung

101
Q

____ and ________more often tend to

be multiple,

A

Small cell carcinomas and melanomas

102
Q

Metastases from________ and _________

are often hemorrhagic, but some from the lung, thyroid,

and kidney exhibit this characteristic as well

A

melanoma and chorioepithelioma

103
Q

The relative frequency of _____makes it the most

common metastatic tumor to bleed, even though only a

small proportion does so

A

lung cancer

104
Q

CT, with and without contrast, will detect practically all sizable (_______) metastases though MRI is more sensitive especially for cerebellar and subcentimeter lesions, and will expose associated leptomeningeal

disease.

A

1 cm

105
Q

In the Tx of Brain Mets

One randomized trial comparing stereotactic radiotherapy

alone, or combined with whole-brain radiationfor 1 to 4 metastases, found ______

A

no difference in survival

but there was a reduction in the frequency of recurrence

at other sites in the brain when whole-brain treatment

was added

106
Q

In Brain Mets

Prophylactic radiation prolonged survival by

_______and reduced the later emergence of metastasis

to the brain.

A

1.5 months

107
Q

In patients with a single parenchymatous metastasis

(shown to be solitary by gadolinium-enhanced MRI),

surgical extirpation may be undertaken provided that:

A
  1. growth of the primary tumor and its systemic metastases

is under good control and the

  1. metastasis is accessible to the surgeon and
  2. not located in a strategic motor or

language area of the brain

108
Q

The average period of survival

in cases of brain metastases, even with therapy, is about

_______, but it varies widely and is dominated by the

extent of other systemic metastases.

Between__________ percent of patients live for a year and ________percent for 2 years

A

6 months

15 and 30

5 to 10

109
Q

What are the radiosensitive tumors?

A

lymphoma,

testicular carcinoma, choriocarcinoma, some breast cancers

110
Q

What is this?

Widespread dissemination o f tumor cells throughout

the meninges and ventricles, a special form of metastatic

cancer, is the pattern in approximately 5 percent of cases

of adenocarcinoma of breast, lung, and gastrointestinal

tract, melanoma, childhood leukemia, and systemic lymphoma.

A

Ca rci n o m ato u s M e n i n g it i s

111
Q

Also known is a rare ____________of the meninges that acts in a similar way to carcinomatous

meningitis but has the striking feature of bloody CSF

(1,000 to 10,000 red blood cells per mm3) .

A

primary malignant melanoma

112
Q

Tx of Carcinomatous meningitis

A

This consists of radiation therapy to the symptomatic areas (cranium, posterior fossa, or spine) followed in selected cases by the intraventricular administration of methotrexate

113
Q

How to give IT MTX in Carcinomatous Meningitis

A

The

methotrexate is administered into the lateral ventricle via

an Ommaya reservoir (12 mg diluted in preservative-free

saline) or into the lumbar subarachnoid space through a

lumbar puncture needle (12 to 15 mg)

114
Q
A

Neurology - Physiology (44 decks)

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