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Once the healthcare team diagnoses neuroblastoma, the next step may be to give the cancer a stage. The stage tells the healthcare team about how big the cancer is, whether it involves important structures such as nerves and blood vessels, and whether it has spread to other parts of the body. The stage is one piece of information that's needed to decide on a treatment plan.

The stages of neuroblastoma have changed over the years. The stages currently follow a system called the International Neuroblastoma Risk Group Staging System. The current staging system uses imaging tests to decide on the stage. An older staging system required results from surgery. As most children with neuroblastoma start treatment with chemotherapy and many may not have surgery, the older staging system isn't used as much.

The stages of neuroblastoma are L1, L2, M and MS.

• Stage L1 means the neuroblastoma is growing in one area. Healthcare professionals sometimes call this localized neuroblastoma. The cancer doesn't involve any structures that would make it hard to remove the cancer completely with surgery. In an older staging system, this was called stage 1.
• Stage L2 means the neuroblastoma is growing in one area. However, the cancer involves structures that might make it hard to remove all the cancer with surgery. Healthcare professionals sometimes call this regional neuroblastoma. In an older staging system, this was called stage 2 and stage 3.
• Stage M means the neuroblastoma has spread to other parts of the body. Healthcare professionals sometimes call this metastatic neuroblastoma. In an older staging system, stage M was called stage 4.
• Stage MS applies to children younger than 18 months. It means the neuroblastoma has spread to the skin, liver or bone marrow. In an older staging system, stage MS was called stage 4S.

The stage is one thing healthcare professionals consider when thinking about what treatments to use for neuroblastoma. Staging can tell where the cancer is and how far it has spread. But it doesn't tell the healthcare team about how aggressive the cancer is or whether it's likely to respond to treatment. That's where risk groups can help.

Neuroblastoma risk groups consider many other factors in order to decide whether a cancer is low risk, intermediate risk or high risk. The risk group helps the healthcare team understand whether there is a low, intermediate or high risk that the cancer will grow quickly and whether it might come back after treatment.

To choose the risk group, the healthcare team uses:

• Cancer stage. Stage M neuroblastoma, formerly called stage 4, has spread to other parts of the body. It has the greatest risk of coming back after treatment.
• The child's age. In general, children younger than 18 months tend to have a better prognosis than older children.
• Histology. Histology means how the cancer cells look and behave when viewed with a microscope. If healthcare professionals in the lab find concerning results, they might say the neuroblastoma has "unfavorable histology." This increases the risk that the cancer may come back after treatment.
• MYCN gene. Tests in the lab also can show if the cancer cells have an extra copy of a gene called MYCN that tends to make the cancer grow more quickly. Neuroblastomas with extra copies of MYCN have a higher risk of coming back after treatment.

The risk groups for neuroblastoma are low, intermediate and high.

• Low-risk neuroblastoma has a low risk of coming back after treatment. It often can be removed completely with surgery. Sometimes it doesn't need treatment, for example, for babies with cancer that affects only the adrenal gland.
• Intermediate-risk neuroblastoma needs stronger treatment to keep it from coming back. Treatment usually involves chemotherapy to help shrink the cancer. This may make it more likely that the cancer can be removed completely.
• High-risk neuroblastoma has the greatest risk of coming back, so it needs the strongest treatments. Treatments often involve chemotherapy, surgery, bone marrow transplant, radiation therapy, immunotherapy and other medicines.

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