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Neuroblastoma > Background and recommendations > Flashcards

Background and recommendations Flashcards

1
Q

What are the 3 neuroblastic histologies? How to they differ?

A
  1. Neuroblastoma
  2. Ganglioneuroblastoma
  3. Ganglioneuroma

They differ based on the degree of cellular maturation

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2
Q

What is the median age of NB?

A

17 months

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3
Q

What are genetic syndromes associated with neuroblastoma?

A
  1. NF
  2. Hirschsprungs disease
  3. Fetal hyantoin syndrome
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4
Q

What histologic markers distinguish NB from other small round blue cell tumors?

A
  1. Neuron-specific enolase
  2. Synaptophysin
  3. Neurofilament
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5
Q

What percentage of patients with NB have positive urine catecholamines.

A

90%

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6
Q

What percentage of patients present with metastatic disease?

A

75%

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7
Q

What imaging is needed for work up?

A

Abdominal US
CT scan of the chest/abdomen/pelvis
MRI of abdomen/liver/spine
I-131 MIBG scan

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8
Q

What tissue diagnosis is needed at work up?

A

BM bx

Resection

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9
Q

INSS Stage 1?

A 
Unilateral, localized tumor s/p R0 or R1 resection 
LN negative (separate from primary specimen)
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10
Q

INSS Stage 2?

A

2A: Unilateral, R2 resection, LN negative (separate from primary specimen)
2B: Unilateral, with involved ipsilateral nodes

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11
Q

INSS Stage 3?

A

Unresectable localized tumor extending past midline

or unilateral tumor with contralateral nodes

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12
Q

INSS Stage 4

A

Distant disease except that listed for 4S

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13
Q

INSS Stage 4S

A

Distant disease to the skin, liver, and or < 1 year old.

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14
Q

What are the most important factors for patients with NB?

A

Age

Stage at diagnosis

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15
Q

What are the factors that classify patients into different risk groups?

A
  1. Stage, INSS
  2. Age
  3. N-myc status
  4. DNA ploidy
  5. Shimada classification

SANDS

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16
Q

What factor makes a patient with 4S high risk?

A

N-myc amplification

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17
Q

Dose constraint for contralateral kidney?

A

Entire kidney < 15 Gy

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18
Q

Dose constraint for liver?

A

V15 < 66%

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19
Q

Dose constraint for the lung?

A

V15 < 66%

20
Q

Should elective nodal RT be used with neuroblastoma?

A

No

21
Q

What is the treatment paradigm for low risk patients (non infants)?

A

Surgery alone and chemo for peristent or recurrent disease

22
Q

What is the treatment paradigm for patients with intermediate risk disease?

A

Surgery followed by short or long course chemo and second look surgery if needed

23
Q

Most recent protocol?

A

ANBL 0532

24
Q

What is the treatment paradigm for high risk disease?

A
  1. Chemo
  2. Resection
  3. High dose chemo
  4. Stem cell transplant
  5. Consolidation RT
  6. Oral cis-retinoic acid
25
Q

What is the treatment paradigm for low risk stage 4S?

A
  1. Bx
  2. Supportive care
  3. Chemo and.or RT reserved for symptomatic disease or rapid progression
26
Q

What is the treatment paradigm for infants with localized disease, non n-myc amplified?

A

Observation

27
Q

What are the targets for patients with high risk disease?

A
  1. Local tumor: Post-chemo, preop tumor bed+2 cm to 21.6 Gy at 1.8 Gy/fx if GTR. If STR, give 21.6 Gy to prep GTV and boost residual to 14.4 Gy.

Cover full width of vertebral body

28
Q

What is first line treatment for NB related spinal cord compression?

A

Chemo. Unresponsive disease can be treated with RT or surgery.

29
Q

If RT is needed for symptomatic cord compression, what dose do you give?

A

If child < 3, 9 Gy at 1.8 Gy/fx

If 3 or older, 21.6 Gy at 1.8 Gy/fx

30
Q

What dose do you give patients with massive splenomegaly?

A

4.5 Gy at 1.5 Gy/fx

31
Q

What are the standard chemo drugs?

A
  1. Cytoxan
  2. Doxorubicin
  3. Etoposide
  4. Carboplatin
  5. Ifosfamide
32
Q

Dose constraint for contralateral kidney?

A

V15<100%

33
Q

Dose constraint for liver?

A

V15<66%

34
Q

Dose constraint for liver?

A

V15<66%

35
Q

What is the treatment paradigm for unfavorable 4S disease (intermediate risk)?

A

Chemo x 8 cycles

36
Q

History elements needed at work up?

A

History: Abdominal mass, fever, malaise, pain, weight loss. Periorbital ecchymosis, scalp, nodules, bone pain, diarrhea

37
Q

How often does NB metastasize to lungs?

A

Rarely

38
Q

What are the common sites of mets for NB?

A 
Bone: 50%
LNs: 35%
BM 
liver 
skin 
orbits
39
Q

What are the most common sites of bony disease?

A
  1. Skull and orbit often causing proptosis, ecchymosis, scalp mass
40
Q

What histologic signs are seen with NB?

A

Small round blue cells

Homer-wright pseudorosettes

41
Q

What is the 3 year OS for patients with High risk NB?

A

<30%

42
Q

What is the 3 year OS for patients with Intermediate risk NB?

A

75-98%

43
Q

What is the 3 year OS for patients with Intermediate risk NB?

A

95-100%

44
Q

What is the origin of NB?

A

Primitive neural crest cells of the spinal ganglion, dorsal spine nerve roots, and adrenal medulla

45
Q

What are late effects of RT?

A 
Growth disturbance
Infertility 
Bladder and pulmonary effects 
SBO
Cardiac effects

Neuroblastoma (1 decks)

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