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Neuroblastoma

 

 

Risk Categories

 

Patients are stratified into different prognostic categories or risk groups.

Therapy design is based on the patient’s risk status.

Risk categories depend on:

  • Pathology
    • histology
    • N-MYC status
    • DNA ploidy
  • Diagnostic imaging results

 

Neuroblastoma Risk Categories:

INSS Stage

Age

MYCN
Status

Shimada
Histology

DNA
Ploidy

Risk Group

1

0- 21 yrs

Any

Any

Any

low

2A/2B

<1 y
≥ 1-21 y
≥ 1-21 y
≥ 1-21 y

Any
Non-Amp
Amp
Amp

Any
Any
Fav
Unfav

Any
-
-
-

Low
Low
Low
High

3

<1yr
<1yr
≥ 1-21 y
≥ 1-21 y
≥ 1-21 y

Non-Amp
Amp
Non-Amp
Non-Amp
Amp

Any
Any
Fav
Unfav
Any

Any
Any
-
-
-

Intermediate
High
Intermediate
High
High

4

<1y
<1y
≥ 1-21 y

Non-Amp
Amp
Any

Any
Any
Any

Any
Any
-

Intermediate
High
High

4S

<1 y
<1 y
<1 y
<1 y

Non-Amp
Non-amp
Non-amp
Amp

Fav
Any
Unfav
Any

> 1
= 1
Any
Any

Low
Intermediate
Intermediate
High

The risk group classification determines the treatment for each individual patient.

More than half the children diagnosed with neuroblastoma will have high risk disease with a poor prognosis.

Recent studies suggest that toddlers aged 12-18 months with:

  • Stage 4 disease with N-MYC non-amplified, hyperdiploid, favorable histology have an event free survival of 90%
  • Stage 3 disease with N-MYC non-amplified, unfavorable histology have an event free survival of 100%

For this reason, this cohort of patients will now be treated according to the intermediate risk protocol.

Age and stage are major prognostic factors in neuroblastoma.

 

Simplified overview of Neuroblastoma Risk Categories:

Risk Category

Definition

Low risk

Age less than 1 year ( now extended to 420 days)

 

Stage 1 (with local resection), Stage 2A partially resected, Stage 4.

 

N-myc - non amplified

 

Hyperdiploid, near triploid DNA ploid

 

High TRKA expression

 

Lack the 1p loss of heterozygosity or 14q heterozygosity

 

Intermediate Risk

Age over 1 year

 

Stage 2B, Stage 3, Stage 4

 

N-myc non amplified

 

Near diploid and near tetraploid DNA ploid

 

TRKA variable

 

1p and 14q loss of heterozygosity variably present

 

High Risk

Age over 1 year

 

Stage 3 and Stage 4

 

N-myc ampified

 

Near diploid and tetraploid DNA ploid

 

TRKA expression low

 

1p and 14q loss of heterozygosity variably present

 

 

 

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