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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?

20
Q

Should elective nodal RT be used with neuroblastoma?

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?

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
What is the treatment paradigm for low risk stage 4S?
1. Bx 2. Supportive care 3. Chemo and.or RT reserved for symptomatic disease or rapid progression
26
What is the treatment paradigm for infants with localized disease, non n-myc amplified?
Observation
27
What are the targets for patients with high risk disease?
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
What is first line treatment for NB related spinal cord compression?
Chemo. Unresponsive disease can be treated with RT or surgery.
29
If RT is needed for symptomatic cord compression, what dose do you give?
If child < 3, 9 Gy at 1.8 Gy/fx If 3 or older, 21.6 Gy at 1.8 Gy/fx
30
What dose do you give patients with massive splenomegaly?
4.5 Gy at 1.5 Gy/fx
31
What are the standard chemo drugs?
1. Cytoxan 2. Doxorubicin 3. Etoposide 4. Carboplatin 5. Ifosfamide
32
Dose constraint for contralateral kidney?
V15<100%
33
Dose constraint for liver?
V15<66%
34
Dose constraint for liver?
V15<66%
35
What is the treatment paradigm for unfavorable 4S disease (intermediate risk)?
Chemo x 8 cycles
36
History elements needed at work up?
History: Abdominal mass, fever, malaise, pain, weight loss. Periorbital ecchymosis, scalp, nodules, bone pain, diarrhea
37
How often does NB metastasize to lungs?
Rarely
38
What are the common sites of mets for NB?
``` Bone: 50% LNs: 35% BM liver skin orbits ```
39
What are the most common sites of bony disease?
1. Skull and orbit often causing proptosis, ecchymosis, scalp mass
40
What histologic signs are seen with NB?
Small round blue cells | Homer-wright pseudorosettes
41
What is the 3 year OS for patients with High risk NB?
<30%
42
What is the 3 year OS for patients with Intermediate risk NB?
75-98%
43
What is the 3 year OS for patients with Intermediate risk NB?
95-100%
44
What is the origin of NB?
Primitive neural crest cells of the spinal ganglion, dorsal spine nerve roots, and adrenal medulla
45
What are late effects of RT?
``` Growth disturbance Infertility Bladder and pulmonary effects SBO Cardiac effects ```

Neuroblastoma (1 decks)

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