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Ph RadOnc Residency PBRROE2-3 Review 2021 > Central Nervous System Tumors in Children > Flashcards

Central Nervous System Tumors in Children Flashcards

1
Q

The etiology of pediatric CNS tumors remains largely unknown. Only 2% to
5% can be ascribed to a known genetic predisposition.

Enumerate the conditions with genetic predisposition to develop CNS tumors in children.

A

NF1
NF2
nevoid basal cell (Gorlin’s) syndrome

familial adenomatous polyposis

Li Fraumeni syndrome.

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2
Q

In pediatric CSI, what should be the lower limit of the spine CTV?

A

The lower limit of the CTV for the spine field should be determined
using MRI.

The lower border of the spinal CTV should include the lower border
of the thecal sac, which can be as high as L5 or as low as S3

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3
Q

Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:

Field matching over cervical spine/risk of over- or underdosage

A

Angle brain fields

use half-beam block for brain fields

use couch rotation or match line wedge

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4
Q

Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:

Inhomogeneity along spinal axis

A

Use compensator, MLC

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5
Q

Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:

Irradiation of normal tissues - mandible teeth

A

Neck extension

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6
Q

Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:

Irradiation of normal tissues - thyroid

A

Care with level of junction

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7
Q

Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:

Irradiation of normal tissues - heart

A

Use lower junction

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8
Q

Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:

Irradiation of normal tissues - GI tract

A

Care with width of spine field

Use protons, IMRT, electrons

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9
Q

Give a possible solution to improve the outcomes encountered with the following scenarios/problem in CSI:

Irradiation of normal tissues - gonads

A

Care with lower limit and width of spine field

Use protons, IMRT, electrons

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10
Q

Central Nervous System Tumors in Children

LGA’s account to a third to half of all pediatric CNS tumors.

TRUE or FALSE?

A

True

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11
Q

Central Nervous System Tumors in Children

Most pediatric CNS tumors are supratentorial.

TRUE or FALSE?

A

False

Infratentorial

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12
Q

Central Nervous System Tumors in Children

What is the most common type of astrocytoma in children?

A

Pilocytic astroctyoma

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13
Q

Central Nervous System Tumors in Children

Describe the histology/pathologic picture of pilocytic astrocytoma.

A

well circumscribed with an associated cystic component.

biphasic pattern with a varying proportion of compacted bipolar cells with Rosenthal fibers and loose-textured multipolar cells with microcysts and granular bodies.

Rare mitoses, occasional hyperchromatic nuclei, microvascular proliferation, and even infiltration of meninges.

KIAA1549-BRAF fusions have been identified in 75% of patients
with PA using an RT-PCR assay from FFPE tissue.

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14
Q

Central Nervous System Tumors in Children

What variant of pilocytic astrocytoma, first described in infants and children with
chiasmatic/hypothalamic tumors, appears to be associated with more aggressive
behavior that may include leptomeningeal seeding?

A

pilomyxoid astrocytoma

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15
Q

Central Nervous System Tumors in Children

Where are the two most common locations of pilocytic astrocytoma, wherein they account for almost all LGAs in those areas?

A

cerebellum

anterior optic pathway

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16
Q

Central Nervous System Tumors in Children

Where are the two most common locations of diffuse astrocytoma?

A

cerebral hemispheres

pons

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17
Q

Central Nervous System Tumors in Children

Describe the histology/pathologic picture of diffuse astrocytoma.

A

well-differentiated fibrillary or gemistocytic neoplastic astrocytes on a background of loosely structured, often microcystic, tumor matrix.

moderately increased cellularity.

nuclear atypia is a diagnostic criterion
(mitotic activity, necrosis, and proliferation are absent)

low Ki-67 and MIB-1 labeling indices.

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18
Q

Central Nervous System Tumors in Children

What is the syndrome commonly manifested by young children with chiasmatic/hypothalamic tumors that present with the ff:

emaciation with loss of subcutaneous fat despite normal or increased appetite, alert appearance, increased vigor and euphoria, pallor without anemia, and nystagmoid movements of the eyes

A

diencephalic syndrome

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19
Q

Central Nervous System Tumors in Children

Neuroimaging findings in pilocytic astrocytoma

A

well circumscribed with an associated cystic component.

There is usually little edema or mass effect.
The solid component enhances brightly and uniformly with contrast material.

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20
Q

Central Nervous System Tumors in Children

Diffuse or fibrillary astrocytomas are usually not well seen on
nonenhanced CT or MRI and usually show little enhancement with contrast
material.

What sequences usually best demonstrate the extent of disease?

A

T2-weighted
or
FLAIR

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21
Q

Central Nervous System Tumors in Children

For pilocytic astrocytomas and diffuse or fibrillary LGAs, postoperative adjuvant therapy is not indicated after complete resection.

TRUE or FALSE?

A

TRUE

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22
Q

Central Nervous System Tumors in Children

For pilocytic astrocytomas and diffuse or fibrillary LGAs, postoperative adjuvant therapy observation can be an option.

How frequent is surveillance MRI performed?

A

every 6 months for 3 years.

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23
Q

Central Nervous System Tumors in Children

For pilocytic astrocytomas and diffuse or fibrillary LGAs, postoperative adjuvant therapy observation can be an option.

What are the options at the time of progression?

A

Second surgical resection.

if not resectable RT (or chemotherapy: for children ≤10 and children of all ages with NF1)

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24
Q

Central Nervous System Tumors in Children

What are the indications for RT in LGA?

A

following incomplete resection in situations when tumor progression would compromise neurologic function (e.g., “threat to vision”)

patients with progressive and/or symptomatic disease that is unresectable.

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25
Q

Central Nervous System Tumors in Children

What is the GTV in LGA?

A

all disease seen on MRI just prior to RT

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26
Q

Central Nervous System Tumors in Children

What is the CTV for fibrillary astrocytoma?

A

GTV as seen on T2 weighted or FLAIR

+ 1 to 1.5 cm margins

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27
Q

Central Nervous System Tumors in Children

What is the CTV for pilocytic astrocytoma?

A

GTV as seen on T1-W with contrast + 0 to 1 cm.

GTV=CTV is acceptable.

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28
Q

Central Nervous System Tumors in Children

What is the standard RT total dose?

A

50 to 54 Gy

Evidence for a dose–response correlation in LGAs in children is scant. Although
the European and North American studies that randomized adult patients with
LGAs between low dose (45 and 50.4 Gy, respectively) and high dose (59.4 and
64.8 Gy, respectively) radiotherapy failed to demonstrate any advantage for the
higher dose, it may be unwise to extrapolate that in children doses of 45 to 50
Gy are as effective as the higher doses of 54 to 55 Gy that until now have
constituted standard practice. There are biologic differences between LGAs in
children and in adults. As well, children who have progressed on chemotherapy
may have tumors that are less sensitive. For now, the recommendation for
children with LGAs would be a “standard” dose of 50 to 54 Gy depending on the
age of the child and the location of the tumor and its relationship to critical
normal structures such as the optic chiasm.

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29
Q

Central Nervous System Tumors in Children

Is SRS an option for LGA?

A

yes.
may be considered for pilocytic but not for diffuse.

10 to 20 Gy to the periphery

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30
Q

Central Nervous System Tumors in Children

What are the histopathologic features of anaplastic astrocytoma?

A

nuclear atypia
increased cellularity
significant proliferative activity.

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31
Q

Central Nervous System Tumors in Children

What are the histopathologic features of glioblastoma multiforme?

A 
mitotic activity
nuclear atypia
microvascular proliferation
necrosis
cellular pleomorphism
vascular thrombosis
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32
Q

Central Nervous System Tumors in Children

Where is the most common locations of HGA in children?

A

cerebral hemispheres (2/3)

remainder equally divided between deep midline structures and cerebellum.

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33
Q

Central Nervous System Tumors in Children

What is the standard of care for HGAs?

A

maximal safe resection + PORT

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34
Q

Central Nervous System Tumors in Children

GTV and CTV in HGAs

A

GTV = tumor plus any T2-weighted MRI abnormality.

CTV = GTV + 1.5 cm

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35
Q

Central Nervous System Tumors in Children

RT dose (HGA)

A

59 to 60 Gy

may consider volume reduction to CTV1cm after 50 to 54 Gy

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36
Q

Central Nervous System Tumors in Children

Children younger than age 3 with HGAs fare better than older children

TRUE or FALSE?

A

True

In contrast to most other tumor types, children
younger than age 3 with HGAs fare better than older children, with overall
survival at 3 to 5 years in the 33% to 50% range in the North American and
United Kingdom Children’s Cancer Study Group/International Paediatric
Oncology Society (UKCCSG/SIOP) baby studies.

The prognosis may be
even better for children younger than age 1.

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37
Q

Central Nervous System Tumors in Children

p53 overexpression and a high MIB-1 labeling index appears to be a favorable prognostic factor.

TRUE or FALSE?

A

False

p53 overexpression and a
high MIB-1 labeling index appear to identify patients with a particularly adverse
prognosis

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38
Q

Central Nervous System Tumors in Children

What are the three types of optic pathway gliomas?

A

anterior tumors

  • confined to optic nerve
  • optic chiasm +/- nerve

posterior tumors
-chiasmatic/hypothalamic

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39
Q

Central Nervous System Tumors in Children

One third of optic pathway gliomas are associated with what genetic condition, where bilateral involvement of the optic nerves is pathognomonic?

A

NF-1

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40
Q

Central Nervous System Tumors in Children

What is the usual presentation of a patient with optic nerve glioma without NF-1?

A

proptosis, long-standing

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41
Q

Central Nervous System Tumors in Children

MRI findings in optic nerve gliomas?

A

small, well-circumscribed, with bright enhancement typical for pilocytic astroctyoma.

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42
Q

Central Nervous System Tumors in Children

in optic nerve gliomas “and” optic chiasm gliomas, biopsy is necessary to make a diagnosis.

TRUE or FALSE?

A

False.

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43
Q

Central Nervous System Tumors in Children

What is the preferred treatment for optic nerve glioma patients predicted to not have functional vision?

A

surgical resection

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44
Q

Central Nervous System Tumors in Children

What is the preferred treatment for optic nerve glioma patients with functional vision for patients with NF-1?

A

chemotherapy

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45
Q

Central Nervous System Tumors in Children

What is the preferred treatment for optic nerve glioma patients with functional vision for infants and patients up to age 5?

A

chemotherapy

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46
Q

Central Nervous System Tumors in Children

What is the preferred treatment for optic nerve glioma patients with functional vision for older patients?

A

chemotherapy.

RT may be considered.

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47
Q

Central Nervous System Tumors in Children

MRI findings in optic chiasm gliomas?

A

small and well circumscribed and enhance uniformly and brightly with contrast
material, suggestive of pilocytic histology

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48
Q

Central Nervous System Tumors in Children

What is the appropriate initial step in the management of chiasmatic gliomas in patients with NF-1?

A

surveillance

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49
Q

Central Nervous System Tumors in Children

What is the role of RT in the management of chiasmatic gliomas?

A

reserved for
salvage after chemotherapy

definitive treatment of older children without
NF-1

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50
Q

Central Nervous System Tumors in Children

What is the most common optic pathway glioma?

A

posterior tumors
-chiasmatic/hypothalamic

(70%)

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51
Q

Central Nervous System Tumors in Children

What is the role of RT in the management of posterior optic pathway gliomas?

A

progressive disease on chemotherapy
for children younger than 10

progressive disease at diagnosis or after
surgery for older patients

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52
Q

Central Nervous System Tumors in Children

RT dose, posterior optic pathway gliomas

younger children ≤5 years

A

45 to 50 Gy

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53
Q

Central Nervous System Tumors in Children

RT dose, posterior optic pathway gliomas

older than 5 years

A

50 to 54 Gy

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54
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

MRI findings in focal tumors

A

well circumscribed

without evidence of infiltration and edema

may be cystic

uniform bright enhancement with contrast material, which correlates with pilocytic histology

absence of peritumoral hypodensity

55
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

Most common location of focal tumors

A

midbrain and medulla

56
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

treatment of choice for focal tumors

A

surgery

57
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

role of RT in focal tumors

A

same as in other LGAs

58
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

Most common location of dorsal exophytic tumors

A

floor of the fourth ventricle

59
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

MRI findings in dorsal exophytic tumors

A

sharply delineated

hypointense on T1W
hyperintense on T2
enhance uniformly

60
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

treatment of choice for dorsal exophytic tumors

A

surgery

maximal safe resection

61
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

treatment of choice for dorsal exophytic tumors with residual after surgery

A

observation.

routine RT is not indicated.

62
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

role of RT in dorsal exophytic tumors

A

high-grade lesion

progressive disease (<9 months postop)

inoperable disease

63
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

treatment of choice for dorsal exophytic tumors with later recurrence postop

A

second surgery

64
Q

Central Nervous System Tumors in Children
DIPG

DIPG accounts for the majority of brainstem gliomas.

TRUE or FALSE?

A

True

70% to 80%

65
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

CT and MR findings in DIPGs

A

On CT, DIPGs are isodense or hypodense, with little enhancement after contrast
injection, similar to diffuse fibrillary astrocytomas at other sites. They are best
seen on T2-weighted or FLAIR MRI. The presence of ring enhancement is
suggestive of high-grade histology.

66
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

Treatment of choice for DIPG?

A

RT

67
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

Conventional RT dose for DIPG?

A

54/30

68
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

GTV and CTV for DIPG?

A

GTV = T2W or FLAIR abnormality

CTV 1-1.5 cm

69
Q

Central Nervous System Tumors in Children
Brainstem Gliomas

HFRT dose for DIPG?

A

39/3/13

45/3/15

70
Q

Central Nervous System Tumors in Children
Astrocytoma of the Spinal Cord

CTV?

A 
solid portion (including intratumoral cysts)
margin of 1 to 1.5 cm

(at least 1.5 for HGAs)

71
Q

Central Nervous System Tumors in Children
Astrocytoma of the Spinal Cord

RT dose

A

50.4/1.8/28

same for both LGA and HGAs, unlike in adults where HGAs are treated to 54

72
Q

Central Nervous System Tumors in Children
Ependymoma

Most common location of myxopapillary ependymoma?

A

filum terminale

73
Q

Central Nervous System Tumors in Children
Ependymoma

Most common presentation of myxopapillary ependymoma?

A

back pain

74
Q

Central Nervous System Tumors in Children
Ependymoma

Leptomeningeal seeding is common despite low-grade histology of myopapillary ependymoma.

TRUE or FALSE?

A

True

75
Q

Central Nervous System Tumors in Children
Ependymoma

RT volume (CTV) for PORT in myxopapillary ependymoma

A

macroscopic residual + cc margin 1.5 cm (or one vertebral body)

76
Q

Central Nervous System Tumors in Children
Ependymoma

RT dose for PORT in myxopapillary ependymoma

A

50.4 Gy

77
Q

Central Nervous System Tumors in Children
Ependymoma

Treatment for leptomeningeal seeding (myxopapillary)

A

CSI curative intent + boost to primary site

78
Q

Central Nervous System Tumors in Children
Ependymoma

Most common site for ependymoma (WHO grade II)

A

ependymal lining of the fourth ventricle

79
Q

Central Nervous System Tumors in Children
Ependymoma

Complete resection is more frequently possible in patients with infratentorial ependymomas than in spinal or supratentorial variants.

TRUE or FALSE?

A

False

80
Q

Central Nervous System Tumors in Children
Ependymoma

PORT is standard of care in WHO grade II ependymomas.

When can you withhold RT?

A

(a) patients with ependymoma of the spinal cord who have undergone complete resection for whom disease-free survival in contemporary series approaches 100%

and (b) selected patients
with supratentorial ependymoma, such as those with intraventricular tumors or
with extraventricular tumors that are solid and located in noneloquent areas and
can be resected with a wider margin.

81
Q

Central Nervous System Tumors in Children
Ependymoma

GTV/CTV for WHO grade II ependymoma?

A

The GTV is a composite of the
tumor bed, including any extension caudal to the foramen magnum and taking
into account any anatomic shifts because of surgery plus any residual tumor

CTV 1 cm (SJCRH)
CTV 0.5 (COG)

82
Q

Central Nervous System Tumors in Children
Ependymoma

RT dose for WHO grade II ependymoma?

A

54 to 59.4

54 if limited by OARs

83
Q

Central Nervous System Tumors in Children
Ependymoma

What is the histology of anaplastic ependymomas?

A

By definition, an anaplastic ependymoma is a malignant glioma of ependymal
differentiation that is characterized by high mitotic activity, often accompanied
by microvascular proliferation and pseudopalisading necrosis. There are no
histopathologic features that can reliably differentiate anaplastic ependymomas
from the more slowly growing and more favorable ependymomas

84
Q

Central Nervous System Tumors in Children
Ependymoma

GTV/CTV for anaplastic ependymoma?

A

tumor bed and any
macroscopic residual disease with a margin for the CTV of 1 cm

dose similar to WHO grade II

CSI was abandoned as standard of care

85
Q

Central Nervous System Tumors in Children
Ependymoma

What is a hallmark finging of ependymoma on pathologic specimens?

A

Rosette formation

from adult CNS chapter

86
Q

Central Nervous System Tumors in Children
Ependymoma

boost volume for ependymoma of posterior fossa

A

(from adult CNS chapter)

Historically, for posterior fossa tumors, the entire posterior fossa has been irradiated.
However, Paulino has shown that the pattern of failure to be “local,” i.e., within the tumor bed itself.

In 9 patients who received RT to the tumor bed + a 2 cm margin, the two failures in this group were within the tumor bed (i.e., there were no failures within the PF outside the tumor bed).

87
Q

Central Nervous System Tumors in Children
Ependymoma

In what variant can a neuraxis spread be treated focally rather than CSI?

A

Myxopapillary

88
Q

Central Nervous System Tumors in Children
Ependymoma

When do you include the entire thecal sac in the treatment volume?

A

when the cauda is involved

taken from spinal chapter

89
Q

Central Nervous System Tumors in Children
Choroid Plexus Tumors

What accounts for the majority of CP tumors in children?

A

Papilloma (CPP) (WHO I)

90
Q

Central Nervous System Tumors in Children
Choroid Plexus Tumors

If a CPP tumor has high mitotic activity, what type of tumor is suggested?

A

atypical (WHO II)

91
Q

Central Nervous System Tumors in Children
Choroid Plexus Tumors

Histology of CPP

A

delicate fibrovascular
connective tissue fronds covered by a single layer of uniform cuboidal columnar
epithelial cells with round or oval basally situated monomorphic nuclei.

low mitotic activity

92
Q

Central Nervous System Tumors in Children
Choroid Plexus Tumors

If a CP tumor shows frequent mitosis (>5%/10 HPF), and has increased cellular density, nuclear pleomorphism, blurring of the papillary
pattern with poorly structured sheets of tumor cells, and necrotic areas,

what is the classification?

A

carcinoma (CPC) (WHO III)

93
Q

Central Nervous System Tumors in Children
Choroid Plexus Tumors

Most common location?

A

lateral ventricles

causing CSF flow obstruction.

94
Q

Central Nervous System Tumors in Children
Choroid Plexus Tumors

neuroimaging findings

A

usually hyperdense,

contrast-enhancing masses

95
Q

Central Nervous System Tumors in Children
Neuronal and Mixed Neuronal-Glial Tumors

What are the two histologies where post-op RT may be indicated?

A

ganglioglioma and central neurocytoma

96
Q

Central Nervous System Tumors in Children
Neuronal and Mixed Neuronal-Glial Tumors

RT dose for typical central neuroctyoma?

A

50 Gy

97
Q

Central Nervous System Tumors in Children
Neuronal and Mixed Neuronal-Glial Tumors

RT dose for atypical central neuroctyoma?

A

54 Gy

98
Q

Central Nervous System Tumors in Children
Pineocytoma

Some will have symptoms of upper mesencephalic tegmental dysfunction consisting of:

limitation of upward gaze,
lid retraction,
retraction nystagmus, and pupils that react more poorly to light than to accommodation.

What is this syndrome?

A

Parinaud syndrome

99
Q

Central Nervous System Tumors in Children
Pineocytoma

RT volume (GTV/CTV) and dose?

A

The target volume is local, consisting of macroscopic residual disease with a margin for the CTV of 1 cm, and the dose, 50 to 55 Gy over 6 weeks.

100
Q

Central Nervous System Tumors in Children
Pineblastoma

What is the most common presentation?

A

enlarging head circumference

signs of short duration increased ICP.

101
Q

Central Nervous System Tumors in Children
Pineblastoma

Complete surgical resection is difficult.
What is the adjuvant treatment?

A

CSI and chemotherapy

102
Q

Central Nervous System Tumors in Children

What is the second most common type of CNS tumor in the pediatric age group?

A

Medulloblastoma

103
Q

Central Nervous System Tumors in Children

Desmoplastic MB is associated
with a _____ prognosis, particularly in very young children.

A

good

104
Q

Central Nervous System Tumors in Children

What are the four molecular subgroups of medulloblastoma?

A

WNT-activated

SHH (TP53 mutant/wild tyep)

non WNT/non-SHH (3)

non WNT/non-SHH (4)

105
Q

Central Nervous System Tumors in Children

Which of the four subgroups of medulloblastoma has the worst prognosis?

A

group 3

106
Q

Central Nervous System Tumors in Children

Standard-risk MB?

A

<1.5 cm2 residual on MRI 48-72 hours post op.

no evidence of CSF dissemination

107
Q

Central Nervous System Tumors in Children

When to do lumbar puncture for MB?

A

prior to surgery or at least 2 weeks post-op to avoid artifacts

108
Q

Central Nervous System Tumors in Children

Standard of care for standard-risk MB

A

CSI 23.4 Gy with a boost to the tumor bed to a total dose of 54.0 Gy, followed by eight cycles of chemotherapy with vincristine, CCNU, and cisplatin.

109
Q

Central Nervous System Tumors in Children

What is the characteristic molecular findings in atypical teratoid rhabdoid tumor

A

deletion and/or mutation of the INI1 locus on chromosome 22

110
Q

Central Nervous System Tumors in Children

What are the most common locations of CNS germ cell tumors.

A

Pineal gland

suprasellar region

111
Q

Central Nervous System Tumors in Children

What CNS germ cell tumor is more commonly found in the pineal gland?

A

NGGCT

112
Q

Central Nervous System Tumors in Children

What CNS germ cell tumor is more commonly found in the suprasellar region?

A

germinoma

113
Q

Central Nervous System Tumors in Children

Describe the common presentation of pineal germ cell tumors.

A

*obstruction to csf flow

signs of increased ICP
hydrocephalus
parinaud syndrome

114
Q

Central Nervous System Tumors in Children

Describe the common presentation of suprasellar germ cell tumors.

A

long history of neuroendocrine deficits

later, increased ICP

115
Q

Central Nervous System Tumors in Children

Identify the most likely germ cell tumor involved (with just this data):

modest elevation of b-hcg <100 IU/mL)

A

pure germinomas

116
Q

Central Nervous System Tumors in Children

Identify the most likely germ cell tumor involved (with just this data):

extremely elevated b-hcg levels

A

choriocarcinoma

117
Q

Central Nervous System Tumors in Children

Identify the most likely germ cell tumor involved (with just this data):

elevated a-FP

A

yolk-sac tumor

118
Q

Central Nervous System Tumors in Children

Identify the most likely germ cell tumor involved (with just this data):

imaging: heterogeneous with cysts, areas of calcification, and sometimes fat,

A

teratoma

119
Q

Central Nervous System Tumors in Children

Identify the most likely germ cell tumor involved (with just this data):

contains areas of hemorrhage

A

choriocarcinoma

120
Q

Central Nervous System Tumors in Children

Standard of care for germinomas without leptomeningeal seeding.

A

whole brain (whole ventricular RT) + margin with concurrent platinum based chemotherapy.

121
Q

Central Nervous System Tumors in Children

RT dose for germinomas without leptomeningeal seeding.

A

There is less controversy now with regard to the radiotherapy dose and dosefractionation
schedule for germinoma. Results are excellent with a CSI dose as
low as 21 Gy even in patients with leptomeningeal spread. The total dose to the
primary site has typically been 40 to 45 Gy but probably can be safely reduced
to 30 Gy or even to 24 Gy in patients treated with a combined
chemotherapy–radiotherapy regimen who have had a complete response to
chemotherapy. Finally, because germinoma is a very radiosensitive tumor, a
fraction size of 1.5 Gy can be used, which, in theory, further reduces the risk of
injury to normal structures.

122
Q

Central Nervous System Tumors in Children

RT dose for NGGCT.

A

The usual dose to the whole ventricle volume or CSI is 30 to 36 Gy and to the primary site, 54 Gy. Patients who have less than a complete response to chemotherapy fare poorly.

123
Q

(from in-service bank)

  1. Considered WHO 1 brain tumors except

a. pilocytic astrocytoma
b. subependymoma
c. myxopapillary ependymoma
d. classic ependymoma

A

D

124
Q

(from in-service bank)

  1. Are non-germinomatous germ cell tumors, except

a. choriocarcinoma
b. endodermal sinus tumor
c. embryonal carcinoma
d. hemangiopericytoma

A

D

125
Q

(from in-service bank)

  1. These age group will have the worst neurocognitive toxicity in children who undergo radiation therapy to the CNS

a. <3
b. 4-7
c. 7-10
d. >10

A

A

126
Q

(from in-service bank)

  1. Post-op MRI if the brain is best done in the ff

a. 1-2 weeks post-op
b. within 48 Hours post-op
c. anytime
d. 6 mos post-op

A

B

127
Q

(from in-service bank)

  1. True or False: Glioblastoma multiforme is more common than anaplastic astrocytoma in children.
A

False

128
Q

(from in-service bank)

  1. These tumors usually have poorly defined margins with central necrosis and vasogenic edema

a. Low grade astrocytoma
b. Diffuse Intrinsic Pontine Glioma
c. High Grade Astrocytoma
d. Craniopharyngioma

A

C

129
Q

(from in-service bank)

  1. Which marker is always elevated in NGGCT ___ “but never in pure germinoma”
A

aFP

130
Q

(from in-service bank)

  1. One criteria for HIGH Risk Medulloblastoma is the size of residual disease after surgery and that is > _____ cm2.
A

1.5

131
Q

(from in-service bank)

  1. Best describes the prognosis of Low grade gliomas EXCEPT

a. OS in the 80-90%
b. Adjuvant RT is necessary for all even after GTR.
c. With RT, 5 yr OS is 67-100%
d. GTR is the primary Tx,

A

B

132
Q

(from in-service bank)

  1. True about craniopharyngiomas EXCEPT

a. Best treated with 54Gy to tumor
b. Usually associated with solid and cystic components
c. PFS of gross total resection and limited resection plus RT are the same.
d. Chemotherapy is generally given post-RT.

A

D

133
Q

(from in-service bank)

  1. CCG (Childrens Cancer Group) introduced these protocols in the treatment of medulloblastoma. EXCEPT

a. 23.4 Gy CSI with chemo with 54Gy PF boost in standard risk MB
b. Use of vincristine, CCNU, prednisone as chemotherapy agents
c. Chemotherapy provides survival benefit for T3-T4/M1-3 disease
d. None of the above

A

D

134
Q

(from in-service bank)

  1. Typical target volume for this tumor is the ff: CTV1: GTC/edema + 2cm (on T2), CTV2 GTV+1 cm (on T1)

a. Low grade astrocytoma
b. NGGCT
c. Medulloblastoma
d. High grade astrocytoma

A

D

Ph RadOnc Residency PBRROE2-3 Review 2021 (57 decks)

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