Primary Tumors

Question 1

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.

Answer 1

Glioblastoma Multiforme and
Anaplastic Astrocytoma

Although predominantly cerebral in location,
they may also arise in the brainstem,
cerebellum, or spinal cord.

Question 2

Most arise in the deep white matter as a
heterogenous mass and quickly infiltrate the
brain extensively

Answer 2

Glioblastoma Multiforme and
Anaplastic Astrocytoma

Question 3

T or F
50% of glioblastomas occupy more than one
lobe of a hemisphere

Answer 3

True

Question 4

T or F
3-6% show multicentric foci of growth and
thereby simulate metastatic cancer

Answer 4

True

Question 5

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.

Answer 5

Glioblastoma
Multiforme and
Anaplastic
Astrocytoma

Question 6

Contrast-enhanced T1 weighted MRI illustrates a large irregularly enhancing tumor with internal necrosis deep within the left cerebral hemisphere.

Answer 6

Glioblastoma multiforme

Question 7

The diagnosis for glioblastoma must be confirmed by

Answer 7

stereotactic biopsy or
by a craniotomy

that aims to remove as much tumor as is
feasible at the same time

Question 8

T or F
Full or partial resection of the tumor (“debulking”) seems to
prolong survival

Answer 8

True

Question 9

T or F
Except for palliation, little can be done to alter the course of
glioblastoma

Answer 9

True

Question 10

constitute between 25 and 30 percent
of cerebral gliomas, may occur anywhere in the brain or
spinal cord

Answer 10

lower-grade astrocytomas (grade II in the WHO classification)

Question 11

Favored sites of occurrence of lower-grade astrocytomas (grade II in the WHO
classification)

Answer 11

cerebrum, cerebellum,
hypothalamus, optic nerve and chiasm, and pons

Question 12

Arise mainly in adults in
their third and fourth decades or earlier

Answer 12

Astrocytomas of the cerebral hemispheres

Question 13

Astrocytomas in other parts of the nervous system that
are more frequent in children and
adolescents were located in

Answer 13

posterior fossa and optic nerves

Question 14

In about two-thirds of patients with
astrocytoma, the first symptom is a

Answer 14

Focal or
generalized seizure

60 and 75
percent of patients have recurrent seizures in
the course of their illness

Question 15

Late
occurrences astrocytoma

Answer 15

Headaches and signs of increased
intracranial pressure

Question 16

T2 weighted MRI shows an infiltrating tumor with minimal mass effect and mild edema. The degree of contrast enhancement is variable but most often less that glioblastoma

Answer 16

Astrocytoma (of the left frontal lobe)

Question 17

MRI demonstrates the large cystic component of the tumor which appears hypo intense compared to the solid tissue component

Answer 17

Cystic astrocytoma of the right cerebellum

Question 18

-Treatment-

is of
singular importance in delaying or preventing a
recurrence

Answer 18

Resection of the tumor nodule

Excision of part of a cerebral astrocytoma can improve
survival in a good functional state for many years.

Rate of survival 5 years after
successful surgery has been greater than 90 percent

Question 19

T or F
The outcome is less assured when the tumor also
involves the brainstem and cannot be safely resected

Answer 19

True

Question 20

In this variant of high-grade glioma there is a diffuse
infiltration of neoplastic glial cells

involving much of
one or both cerebral hemispheres

sparing of
neuronal elements but without a discrete tumor
mass

Answer 20

Gliomatosis Cerebri

the tumor is impossible
to classify (or to grade) using the conventional brain
tumor schemes

Question 21

CT and MRI
reveal small
ventricles and
one or more
large confluent
areas of signal
change

T2 weighted MRI shows a large confluent area of involvement in the frontal lobes with effacement of overlying cortical sulci.

There was slight enhancement along the margins of the lesions after gadolinium infusion.

The patient was mentally slow but had no other neurologic signs

Answer 21

Gliomatosis cerebri

Question 22

suggest it may be a promising agent for this tumor (gliomatosis cerebri)
but it will be difficult to conduct a randomized trial
given its rarity

Answer 22

temozolomide

Question 23

The addition of chemotherapy may confer a
marginal further benefit when survival at 1 year is
considered. What tumor is being referred to?

Answer 23

Gliomatosis cerebri

Question 24

It is derived from oligodendrocytes or their
precursor cells may occur at any age,
most often in the third and fourth decades, with an earlier peak at 6 to 12 years

May be
recognized at surgery by its pink-gray
color and multilobular form, its relative
avascularity and firmness (slightly
tougher than the surrounding brain), and its
tendency to encapsulate and form
calcium and small cysts

Answer 24

Oligodendroglioma

Question 25

The most common sites of oligodendroglioma are the? often deep in the white matter, with one or more streaks of calcium but little or no surrounding edema

Answer 25

frontal and temporal lobes (40 to 70%)

Question 26

The first symptom in oligodendroglioma in more than half of patients

Answer 26

focal or generalized seizure seizures often persist for many years before other symptoms develop. Approximately 15 percent of patients have early symptoms and signs of increased intracranial pressure; an even smaller number have focal cerebral signs (hemiparesis)

Question 28

Much less frequent symptoms in oligodendroglioma

Answer 28

unilateral extrapyramidal rigidity cerebellar ataxia Parinaud syndrome intratumoral hemorrhage meningeal oligodendrogliosis (cranialspinal nerve palsies, hydrocephalus, lymphocytes, and tumor cells in CSF).

Question 29

The appearance on imaging studies is variable, but the most typical

Answer 29

Most typical is a hypodense (on CT) or T2 hyperintense (on MRI) heterogenous mass near the cortical surface with relatively well-defined borders

Question 30

has been the conventional treatment for oligodendroglioma.

Answer 30

Surgical excision followed by radiation therapy

Question 31

T or F Well-differentiated oligodendrogliomas should probably not receive radiation if seizures are well controlled and there are no neurologic deficits

Answer 31

True

Question 32

The better-tolerated and preferred treatment for oligodendroglioma

Answer 32

TEMOZOLAMIDE

Question 33

Most common glioma of the spinal cord, cells have both glial and epithelial. Approximately 6 percent of all intracranial gliomas , the percentage being slightly higher in children (8 percent)

Answer 33

Ependymoma

Question 34

most common cerebral site of ependymoma

Answer 34

Fourth ventricle

Question 35

T orF In a follow-up study of 101 cases in Norway, where ependymomas made up 1.2 percent of all primary intracranial tumors (and 32 percent of intraspinal tumors), the postoperative survival was poor

Answer 35

True

Question 37

Treatment for ependymoma, particularly to address the high rate of seeding of the ventricles and spinal axis

Answer 37

Surgical removal, supplemented by radiation therapy

Question 38

antineoplastic drugs are often used in combination with radiation therapy in this type

Answer 38

anaplastic ependymomas (grade 3)

Question 39

Represent approximately 15 percent of all primary intracranial tumors. More common in women than in men (2:1) highest incidence in the sixth and seventh decades of life

Answer 39

Meningiomas

Question 40

The most frequent acquired genetic defects of meningiomas

Answer 40

truncating (inactivating) mutations in the neurofibromatosis 2 gene (merlin) on chromosome 22q.

Question 41

elaborate a variety of soluble proteins, some of which (VEGF) are angiogenic and probably relate to both the highly vascularized nature of these tumors and their prominent surrounding edema

Answer 41

Meningiomas

Question 42

uniform, with round or elongated nuclei, visible cytoplasmic membrane, and a characteristic tendency to encircle one another, forming whorls and psammoma bodies (laminated calcific concretions).

Answer 42

cells of meningiomas

Question 43

notable electron microscopic characteristic is the formation of very complex interdigitations between cells and the presence of

Answer 43

desmosomes

Question 44

Tor F Ninety percent of meningiomas supratentorial, and the majority of infratentorial meningiomas occur at the cerebellopontine angle

Answer 44

True occur at sites of dural folds, most commonly the frontoparietal parasagittal convexities, falx, tentorium cerebelli, sphenoid wings, olfactory groove, and tuberculum sellae

Question 45

In meningioma, a few tumors show malignant qualities. What are these?

Answer 45

a high mitotic index, nuclear atypia, marked nuclear and cellular pleomorphism, and invasiveness of brain

Question 46

T or F In meningioma, by invading adjacent bone, they may become impossible to remove totally.

Answer 46

True

Question 47

T or F Carefully planned radiation therapy is beneficial in cases that are inoperable and when the tumor is incompletely removed or shows malignant characteristics

Answer 47

True

Question 48

Smaller tumors at the base of the skull can be obliterated or reduced in size by

Answer 48

focused radiation probably with comparable or less risk than posed by surgery.

Question 49

T or F Conventional chemotherapy and hormonal therapy, are probably ineffective

Answer 49

True

Question 50

This tumor has assumed increasing significance in the last few decades because of its increased incidence in patients with AIDS and other immunosuppressed states.

Answer 50

Primary Central Nervous System Lymphoma There is a preponderance of men, with the peak incidence in the fifth through seventh decades of life, or in the third and fourth decades in patients with AIDS.

Question 52

may arise in any part of the cerebrum, cerebellum, or brainstem, with 60 percent being in the cerebral hemispheres; they may be solitary or multifocal

Answer 52

Primary CNS lymphoma

Question 53

T or F Vitreous, uveal, and retinal (ocular) involvement occurs in 10 to 20 percent of cases

Answer 53

True

Question 54

T or F Surgical resection is ineffective except in rare instances

Answer 54

True

Question 55

produces a partial or, transiently, a complete response, but the tumor recurs in greater than 90 percent of patients

Answer 55

Treatment with cranial irradiation and corticosteroids The median survival of patients treated in this way has been 10 to 18 months and less in those with AIDS and in individuals

Question 57

In immunocompetent patients, this has increased median survival to more than 3 years and apparent cure is not unknown.

Answer 57

addition of intrathecal methotrexate and intravenous cytosine arabinoside However, this combined treatment is associated with a high risk of a leukoencephalopathy and causes considerable systemic side effects

Question 58

Treatment regimen consisting of several cycles ( primary Central lymphoma)

Answer 58

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

Question 59

T or F More recently, methotrexate plus either cytarabine or a combination of rituximab and temozolomide has been favored

Answer 59

True

Question 60

not typically part of the initial treatment in primary Central lymphoma

Answer 60

Cranial irradiation

Question 61

Ocular lymphoma is eradicated only by

Answer 61

radiation therapy Corticosteroids are added at any point to control neurologic symptoms