Stages of Neuroblastoma

After neuroblastoma has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body.

The process used to find out the extent or spread of cancer is called staging. The information gathered from the staging process helps determine the stage of the disease. For neuroblastoma, the stage of disease affects whether the cancer is low risk, intermediate risk, or high risk. It also affects the treatment plan. The results of some tests and procedures used to diagnose neuroblastoma may be used for staging. See the General Information section for a description of these tests and procedures.

The International Neuroblastoma Risk Group Staging System (INRGSS) is used to determine the stage of neuroblastoma.

Stage L1

In stage L1, the cancer is in only one area, and there are no image-defined risk factors (IDRFs). IDRFs are found on MRI or CT scans done during diagnosis. IDRFs are used to determine the risk of surgery and the chance of completely removing the tumor.

Stage L2

In stage L2, the cancer is in one area, has not spread beyond nearby tissue, and there are one or more IDRFs.

Stage M

In stage M, neuroblastoma has spread to areas far from the tumor. This does not include stage MS.

Stage MS

In stage MS, children younger than 18 months have cancer that has spread to the skin, liver, or bone marrow.

Treatment of neuroblastoma is based on risk groups.

For many types of cancer, stages are used to plan treatment. For neuroblastoma, treatment depends on the patient's risk group. The risk group is determined by the following factors:

• the stage of the cancer
• the child's age at diagnosis
• the International Neuroblastoma Pathologic Classification (INPC) (tumor histology)
• whether the tumors are diploid or hyperdiploid (DNA index)
• whether the cancer gene MYCN is found in the tumor cells
• how much of the cancer could be removed by surgery

There are three risk groups: low risk, intermediate risk, and high risk.

• Low-risk and intermediate-risk neuroblastoma have a good chance of being cured.
• High-risk neuroblastoma may be hard to cure.

For more information on the risk group classification, see the Revised International Neuroblastoma Response Criteria (INRC) section in Neuroblastoma Treatment (Health Professional Version).

Sometimes neuroblastoma does not respond to treatment or comes back after treatment.

Refractory neuroblastoma is a tumor that does not respond to treatment.

Recurrent neuroblastoma is cancer that has recurred (come back) after it has been treated. The tumor may come back in the site where it began or in the central nervous system.