Neuroblastoma

Neuroblastoma is a type of pediatric cancer that develops in the nervous system of babies and young children. Neuroblastoma grows in immature nerve tissue (neuroblasts).

It usually affects neuroblasts in the adrenal glands (small organs that sit on top of the kidneys). The adrenal glands make hormones that control automatic body functions, such as digestion, blood pressure, breathing and heart rate. Neuroblastoma can also develop in nerve tissue in the spinal cord, abdomen, chest or neck. It can spread to other parts of the body.

The outlook for kids with neuroblastoma depends on the tumor’s location, the child’s age and the cancer stage. The stage describes how advanced the disease is.

How common is neuroblastoma?

Neuroblastoma is rare, but it’s the most common cancer in babies. Every year in the United States, about 800 children are diagnosed with neuroblastoma.

Neuroblastoma almost always develops before age 5. It can occur in babies before they are born. Neuroblastoma is very rare in children over age 10.

What are the stages of neuroblastoma?

Healthcare providers classify neuroblastoma in children based on how advanced the cancer is and how fast it’s growing. They also consider whether it has spread (metastasized) to other parts of the body.

Using this information, providers determine the cancer’s risk level. Then they choose the most appropriate treatments. The stage of neuroblastoma used to be determined by how much neuroblastoma was found in the body after surgery. Now, the International Neuroblastoma Risk Group Staging System (INRGSS) is used. The stage of neuroblastoma is determined by how much tumor spread is seen on initial imaging studies (such as CT scan or MRI, as discussed below), called “image-defined risk factors.” The INRG stages of neuroblastoma are:

• Stage L1: This is the stage with the lowest risk. L1 tumors are confined to one body compartment and have not spread. Also, the tumor does not involve vital structures of the body (no image-defined risk factors are present).
• Stage L2: In this stage, the tumor is confined to one body compartment, but cancer cells can spread to regional lymph nodes, for instance. Also, there is involvement of vital structures of the body, such as tumor wrapping around large blood vessels (i.e., at least one image-defined risk factor is present).
• Stage M: In this stage, the cancer cells have spread to more than one body compartment – called “distant metastatic disease.” This stage carries the highest risk.
• Stage MS: This is a “special” category of neuroblastoma, affecting children younger than 18 months of age. In this stage, the cancer cells have spread (or metastasized) to either the skin, liver or bone marrow only. Children with stage MS neuroblastoma generally have an excellent prognosis. Stage MS neuroblastoma is usually considered low-risk disease.