Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Ph RadOnc Residency PBRROE2-3 Review 2021 > Neuroblastoma > Flashcards

Neuroblastoma Flashcards

1
Q

Pediatric Maligancies

BONUS:

What is the most common extracranial tumor of childhood?

A

Neuroblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Neuroblastoma

Infant screening led to a decrease in the mortality rates when compared to non-screened control population.

TRUE or FALSE?

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Neuroblastoma

What urinary metabolites can be quantitatively assayed by HPLC and normalized to urinary creatinine to detect for neuroblastoma?

A 
homovanillic acid (HVA)
vanillylmandelicacid (VMA)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Neuroblastoma

What urinary metabolite of epinephrine can be qualitatively to detect for neuroblastoma?

A

vanillylmandelicacid (VMA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Neuroblastoma

NB may arise from any site in the sympathetic nervous system.
What are the most common sites of origin?

A

adrenal medulla (30-40%)

paraspinal ganglia in the abdomen or pelvis (25%)

thoracic (15%)

head and neck (5%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Neuroblastoma

What are the most common sites of metastases?

A

lymph nodes,
bone
bone marrow
skin

(lung and cns are rare sites)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Neuroblastoma

NB may spontaneously regress to a mature ______?

A

ganglioneuroma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Neuroblastoma

What is the most common presenting symptom?

A

pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Neuroblastoma

What “sign” is the term used to describe the skin metastases due to its tinge?

A

blueberry muffin sign

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Neuroblastoma

What syndrome is an unusual presentation of localized NB that is manifested by truncal ataxia and cerebellar encephalopathy, which indicates a favorable prognosis but may have lasting neurologic sequelae after successful therapy?

A

opsoclonus–myoclonus syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Neuroblastoma

I. Pathologic evaluation of bone marrow is also a requirement for
staging of neuroblastoma.

II. Characteristically, neuroblastoma in bone marrow
appears in clumps and pseudorosettes.

III. The absence of pseudorosettes
eliminates the possibility of neuroblastoma.

I and II are true?
II and III are true?
only II is true?
all are true?

A

I and II only.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Neuroblastoma

A ratio of VMA to HVA >1.5 is associated with an unfavorable prognosis in patients with metastatic neuroblastoma.

TRUE or FALSE?

A

False.

Favorable.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Neuroblastoma

Poor scintigraphic response on __________(nuclear scan procedure) after induction chemotherapy has
been shown to predict for a poor event-free survival in patients undergoing high-dose
chemotherapy with stem cell rescue.

A

123I-MIBG scans

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Neuroblastoma

The expression of somatostatin receptors by neuroblastoma tissues is a favorable
prognostic factor

TRUE or FALSE?

A

True.

Like other neural crest–derived
neoplasms, neuroblastoma can express somatostatin receptors.

The long-acting
somatostatin analog octreotide labeled with 123I has been used to image
neuroblastoma with a sensitivity comparable to that of 131I-MIBG.

The
expression of somatostatin receptors by neuroblastoma tissues is a favorable
prognostic factor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Neuroblastoma

In imaging/staging of NB, what modality often overstages stage I tumors?

A

MRI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Neuroblastoma

In imaging/staging of NB, what modality often understage stage II tumors?

A

CT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Neuroblastoma

The most commonly used staging system is the International Neuroblastoma Staging System.
This system was established to stage patients before any treatment and is based on clinical criterial and image-defined risk factors.

TRUE or FALSE?

A

False.

The INSS uses clinical, radiographic, and surgical findings.

(INRG is the one described in the question)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Neuroblastoma

Identify the INSS stage:

Localized tumor with incomplete gross excision;

representative ipsilateral nonadherent lymph nodes negative for tumor microscopically

A

INSS Stage 2A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Neuroblastoma

Identify the INSS stage:

Localized tumor with complete gross excision, without microscopic residual disease;

representative ipsilateral lymph nodes negative for tumor microscopically
(nodes attached to and removed with the primary tumor may be positive)

A

INSS Stage 1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Neuroblastoma

Identify the INSS stage:

Localized tumor with or without complete gross excision, with
ipsilateral nonadherent lymph nodes positive for tumor;

enlarged contralateral lymph
nodes must be negative microscopically

A

INSS Stage 2B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Neuroblastoma

Identify the INSS stage:

Unresectable unilateral tumor infiltrating across the midline,
with or without regional lymph node involvement;

or localized unilateral tumor with contralateral regional lymph node involvement;

or midline tumor with bilateral
extension by infiltration (unresectable) or by lymph node involvement

A

INSS Stage 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Neuroblastoma

Identify the INSS stage:

Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organs

A

INSS Stage 4 or 4S

23
Q

Neuroblastoma

Identify the INSS stage:

Localized primary tumor
as defined for stage 1,
2A, or 2B with
dissemination limited
to skin, liver, and/or
bone marrow (limited
to infants <1 year of
age)
A

INSS Stage 4S

24
Q

Neuroblastoma

Identify the INRG stage:

Metastatic disease in children younger than 18 months with metastasis confined to the skin, liver, and/or bone marrow

A

INRG Stage MS

25
Q

Neuroblastoma

Identify the INRG stage:

Distant metastatic disease

A

INRG Stage M or MS

26
Q

Neuroblastoma

Identify the INRG stage:

Locoregional tumor with
presence of one or more image-defined factors

A

INRG Stage L2

27
Q

Neuroblastoma

Identify the INRG stage:

Localized tumor not involving
vital structures as defined by the list of image-defined risk factors and confined to one body compartment

A

INRG Stage L1

28
Q

Neuroblastoma

Bonus:
Identify the Evans and D’Angio stage:

Tumor extending in continuity
beyond the midline; regional lymph nodes may beinvolved bilaterally.

A

Stage III

Evans and D’Angio stages range from I to IV and IV-S

29
Q

Neuroblastoma

Bonus:
Identify the POG stage:

Complete or incomplete resection of primary, intracavitary nodes not adhered to primary histologically positive for tumor; liver histologically free of tumor

A

POG Stage C

POG stages range from A to D and DS

30
Q

Neuroblastoma

In the INSS staging, what is meant by “minimal” involvement of marrow?

A

<10% of total nucleated cells
identified as malignant on bone marrow biopsy or on marrow aspirate

or negative MIBG if performed

31
Q

Neuroblastoma

What are the three tumor types representing different degrees of differentiation?

A

Ganglioneuroma (most mature)

Ganglioneuroblastoma

Neuroblastoma

32
Q

Neuroblastoma

IHC staining characteristics of NB?

A

positive for:

  • neurofilaments
  • neuron-specific enolase (NSE)
  • synaptophysin
  • chromogranin A

negative for:

  • muscle
  • leukocyte antigens
33
Q

Neuroblastoma

What three histologic features along with the patient’s age at diagnosis divide the patients into favorable and unfavorable prognostic groups (Shimada classification)

A

stromal development (poor or rich)

neuroblastic differentiation

mitosis–karyorrhexis index of neuroblastic cells.

34
Q

Neuroblastoma

What are the two most important factors that influence outcome in neuroblastoma

A

age and stage at initial presentation

35
Q

Neuroblastoma

What gene amplification has been associated with the multidrug resistance gene, which may account for this tumor’s notorious resistance to therapy?

Allelic loss of the arm on which it resides is also associated with poor prognosis independent of age and stage.

A

MYCN (N-myc) on the short arm of chromosome 2.

36
Q

Neuroblastoma

What mutations have also been described in neuroblastoma and have been
identified as the predisposition gene for familial neuroblastoma?

A

Mutations in ALK (encoding anaplastic lymphoma kinase)

37
Q

Neuroblastoma

Ferritin levels considered unfavorable?

A

> 143 ng/mL

38
Q

Neuroblastoma

Neuron-specific enolase levels considered unfavorable?

A

> 100

39
Q

Neuroblastoma

Chromogranin levels considered unfavorable?

A

> 190 ng/mL

40
Q

Neuroblastoma

GD2 ganglioside levels considered favorable? unfavorable?

A

f: <103 pmol/mL
u: >568 pmol/mL

41
Q

Neuroblastoma

DNA index considered unfavorable?

A

1

>1.1 is considered favorable

42
Q

Neuroblastoma

For low-stage, resectable tumors (INSS 1-3 with negative nodes), gross resection alone confers an excellent prognosis. adjuvant treatment has not improved outcomes for completely resected tumors with favorable prognosis.

TRUE or FALSE?

A

True

43
Q

Neuroblastoma

Re: low-stage, resectable tumors (INSS 1-3 with negative nodes), favorable group.

ALL positive surgical margins or microscopic residual require more aggressive therapy.

TRUE or FALSE?

A

False

Positive
surgical margins or microscopic residual disease does not uniformly require
more aggressive therapy.

44
Q

Neuroblastoma

Re: low-risk disease

What subgroups may require adjuvant therapy?

A

those with N-myc amplification or low DNA index

45
Q

Neuroblastoma

What is the treatment for intermediate-risk patients with intraspinal extension of neuroblastoma and have severe neurologic compromise resulting from spinal cord compression.

A

primary chemotherapy

46
Q

Neuroblastoma

In the POG experience for intermediate-risk patients,
what RT doses were given?

A

24 Gy/1.5 (2 years below)

30 Gy/1.5 (older)

47
Q

Neuroblastoma

What are the 4 most active agents against intermediate-risk patients?

A

cyclophosphamide
doxorubicin
etoposide
carboplatin

48
Q

Neuroblastoma

What is the monoclonal antibody against tumor-associated disialoganglioside GD2, that has been shown to have activity against neuroblastoma?

A

ch14.18

49
Q

Neuroblastoma

What is the general management for patients with high-risk disease (according to COG guidelines)?

A

combine
induction chemotherapy, surgical resection,
autologous stem cell rescue, and radiation therapy to the primary site and select metastatic sites, 13-cis-retinoic acid and immunotherapy in an attempt to improve outcome

The current protocol utilizes a higher radiation total dose of 36 Gy for patients with gross residual disease at their primary sites.

50
Q

Neuroblastoma

For primary site RT, what is the CTV?

A

post-induction chemotherapy tumor bed and residual tumor with 1 to 1.5 cm margin.

51
Q

Neuroblastoma
For patients who had TBI as a preparative regimen for BMT were less likely to have relapse in prior sites of disease compared to those who did not have TBI.

TRUE or FALSE?

A

True

Li and colleagues

52
Q

Neuroblastoma

In general, what is the RT dose given to the PTV (primary site)

A

The CTV and PTV are typically treated to 21.6 Gy in 12 fractions.

53
Q

Neuroblastoma

Persistent activity in metastatic sites after induction therapy can be treated with radiotherapy as long as the patient can tolerate the treatment.

What is the dose?

A

The dose is 21.6 Gy in 12 fractions over 2.4 weeks and follows the same CTV and PTV guidelines for the primary site.

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

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions