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Neuroblastoma

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Prognosis and survival for neuroblastoma

Children with neuroblastoma and their parents may have questions about prognosis. A prognosis is the doctor’s best estimate of how cancer will affect a child and how it will respond to treatment. Prognosis and survival depend on many factors. Only a doctor familiar with a child’s medical history, the type, stage and characteristics of the cancer, treatments chosen and the response to treatment can put all of this information together with survival statistics to arrive at a prognosis.

Doctors use the following prognostic factors to assign neuroblastoma to risk groups that help them predict a prognosis and plan treatment.

Prognostic factors

A prognostic factor is an aspect of the cancer or a characteristic of the child that the doctor will consider when making a prognosis. A predictive factor influences how a cancer will respond to a certain treatment. Prognostic and predictive factors are often discussed together. They both play a part in deciding on a treatment plan and a prognosis.

The following are prognostic and predictive factors for neuroblastoma.

Age

Children younger than 12 months of age usually have a better outcome.

Stage

The earlier the stage at diagnosis, the better the prognosis. Surgery is the only treatment that most children with stage 1 neuroblastoma need. Children with stage 4 neuroblastoma need more intensive treatment with a combination of therapies.

Tumour histology

Tumour histology is what the cancer cells look like under the microscope and predicts how they behave. Doctors may use a classification system, such as the Shimada classification system, to determine if a tumour has a favourable or unfavourable histology.

Tumours with a favourable histology have more differentiated cancer cells, which means the cancer cells look and act more like normal cells. The cancer cells are also slower growing.

Tumours with an unfavourable histology often have less differentiated cancer cells, which means the cancer cells are very different from normal cells. The cancer cells are usually faster growing.

DNA ploidy

DNA ploidy, or DNA index, is the amount of DNA content in each cell. Healthy cells with a normal amount of DNA are classified as diploid and are given a DNA index of 1. Neuroblastoma cells that have more than the normal amount of DNA are classified as hyperdiploid and are given a DNA index greater than 1.

Diploid (DNA index = 1) tumours tend to be less responsive to treatment and often need more aggressive therapy. Hyperdiploid (DNA index > 1) tumours tend to respond better to treatment and usually have a better prognosis.

MYCN status

MYCN is a gene that regulates cell growth. A single copy of the gene (described as non-amplified) is normal and is linked with a less aggressive form of cancer. Many copies of the gene (described as amplified) are usually seen in more aggressive tumours.

Chromosome changes

Children with a greater number of chromosome changes often have a poorer prognosis. Chromosome changes are often linked with older age at diagnosis, advanced stage and higher risk of recurrence, or relapse.

Tumour cells missing parts of chromosomes 1 or 11 (called 1p or 11q deletions) are linked to a less favourable prognosis. Having an extra part of chromosome 17 (called 17q gain) is also linked to a less favourable prognosis.

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Risk groups

The Children’s Oncology Group (COG) further classifies neuroblastoma into risk groups based on most of the prognosis factors above.

Low-risk neuroblastoma
StageAgeMYCN statusTumour histologyDNA ploidy

1

0–21 years

any

any

any

2A or 2B

younger than 1 year

any

any

any

1–21 years

non-amplified

any

1–21 years

amplified

favourable

4S

younger than 1 year

non-amplified

favourable

hyperdiploid (DNA index > 1)

Intermediate-risk neuroblastoma
StageAgeMYCN statusTumour histologyDNA ploidy

3

younger than 1 year

non-amplified

any

any

1–21 years

non-amplified

favourable

4

younger than 1 year

non-amplified

any

any

4S

younger than 1 year

non-amplified

any

diploid (DNA index = 1)

younger than 1 year

non-amplified

unfavourable

any

High-risk neuroblastoma
StageAgeMYCN statusTumour histologyDNA ploidy

2A or 2B

1–21 years

amplified

unfavourable

3

younger than 1 year

amplified

any

any

1–21 years

non-amplified

unfavourable

1–21 years

amplified

any

4

younger than 1 year

amplified

any

any

1–21 years

any

any

4S

younger than 1 year

amplified

any

any

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