Neuroblastoma

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Stages of neuroblastoma

Staging describes or classifies a cancer based on how much cancer there is in the body and where it is when first diagnosed. This is often called the extent of cancer. Information from tests is used to find out the size of the tumour, which parts of the organ have cancer, whether the cancer has spread from where it first started and where the cancer has spread. The healthcare team uses the stage to plan treatment and estimate your child’s outcome (prognosis).

Doctors use 2 systems to stage neuroblastoma:

  • International Neuroblastoma Staging System
  • International Neuroblastoma Risk Group Staging System

When describing the stage, doctors may use the words local, regional or distant. Local means that the cancer is only in the area where it first started to grow and has not spread to other parts of the body. Regional means close to the area where it started to grow or around it. Distant means in a part of the body farther from the area where it first started to grow.

Generally, the higher the stage number, the larger the cancer is or the more the cancer has spread. Talk to your child’s doctor if you have questions about staging.

Find out more about staging cancer.

International Neuroblastoma Staging System (INSS)

The most common staging system for neuroblastoma is the International Neuroblastoma Staging System (INSS). The INSS is based on the results of surgery to remove the tumour. Each stage is given a number from 1 to 4.

Stage 1

The tumour is only in the area where it started and is on only the right side or the left side of the body. It can be completely removed with surgery.

Stage 2A

The tumour is only in the area where it started and is on only one side of the body, but it can’t be completely removed with surgery.

Stage 2B

The cancer is on only one side of the body and it may be completely removed with surgery.

The cancer has spread to nearby lymph nodes on the same side of the body as the tumour (called ipsilateral lymph nodes).

Stage 3

The cancer can’t be completely removed with surgery and one of the following applies:

  • The tumour has grown across the midline of the body (defined as the spine) and the cancer may also have spread to nearby lymph nodes.
  • The main tumour is on one side of the body, but the cancer has spread to nearby lymph nodes on the other side of the body.
  • The tumour is growing on the midline of the body and the cancer has spread to lymph nodes or tissues on both sides of the body.

Stage 4

The cancer has spread to other parts of the body (called distant metastasis), such as to distant lymph nodes, bone, bone marrow, liver, skin or other organs. This is also called metastatic neuroblastoma. Stage 4 doesn’t include tumours that are stage 4S.

Stage 4S (special neuroblastoma)

This stage is only used for children younger than 1 year old.

The main tumour is only in the area where it started and is classified as stage 1, 2A or 2B. But the cancer has spread to the liver, skin or bone marrow. If it has spread to the bone marrow, less than 10% of the cells in the bone marrow are cancer cells.

International Neuroblastoma Risk Group Staging System (INRGSS)

The International Neuroblastoma Risk Group Staging System (INRGSS) is based on the results of imaging tests and sometimes other tests done before surgery. If image-defined risk factors (IDRFs) can be seen on the imaging tests, it may mean that the tumour will be harder to remove. IDRFs are when the cancer looks like it has spread to nearby important structures. The INRGSS divides neuroblastoma into 4 stages.

Stage L1

The tumour is only in one area of the body where it started (such as the neck, chest, abdomen or pelvis) and hasn’t grown into any vital structures listed as an IDRF.

Stage L2

The tumour has spread to a nearby area and there are 1 or more IDRFs.

Stage M

The cancer has spread to distant parts of the body (called distant metastasis) but doesn’t include tumours that are stage MS.

Stage MS

Stage MS is only used for children younger than 18 months with metastatic cancer (cancer that has spread to distant parts of the body).

The cancer has only spread to the skin, liver or bone marrow. If it has spread to the bone marrow, less than 10% of the cells in the bone marrow are cancer cells. An MIBG scan doesn’t show cancer in the bone or bone marrow.

Recurrent neuroblastoma

Recurrent neuroblastoma means that the cancer has come back after it has been treated. If it comes back in the same place that the cancer first started, it’s called a local recurrence. If it comes back in tissues or lymph nodes close to where it first started, it’s called a regional recurrence. It can also recur in another part of the body. This is called distant metastasis or distant recurrence.

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