What is neuroblastoma?

Neuroblastoma is cancer that starts in young nerve cells called neuroblasts.

In a healthy child, these cells become the nerves that control functions such as the heartbeat and blood pressure, and the way your child's body reacts to stress. A part of the nervous system called the sympathetic nervous system controls these functions.

In neuroblastoma, the neuroblasts never mature. Instead, they divide and grow into tumors.

Most often these tumors start in the nerves of the adrenal glands. These glands sit on top of the kidneys and release hormones that control the sympathetic nervous system.

But these tumors can begin in any part of the body. The chest, neck, pelvis and spinal cord are other common places for them.

As the disease goes on, it can spread (metastasize) to other areas of the body. It usually spreads to the lymph nodes (part of the immune system), bones, bone marrow (where blood cells are made), liver or skin.

For most children, the neuroblastoma has spread to at least one of these areas by the time doctors diagnose the disease.

Neuroblastoma in Children

Cancer in children is rare, and neuroblastoma is one of the less common types.

It is the most common solid tumor in children younger than 1 year of age. But it accounts for less than 10% of all childhood cancers.

Most children with this disease get it before age 5. In some kids, the disease starts before birth.

In most cases - no matter what the child's age - parents and doctors don't know the disease is there until tumors grow large enough to be felt.

Doctors do not know what causes the changes in cells that lead to this disease. It's not clear whether certain factors increase a child's risk.

Neuroblastoma Stages

Staging refers to the way doctors classify cancer based on where it is in the body. The cancer stage, along with other factors, helps doctors choose a course of treatment.

Children who have neuroblastoma are considered to be at one of these stages:

Stage 1 neuroblastoma

Applies to children who have a tumor in only one place, and surgeons can remove all of it that they can see.

Stage 2 neuroblastoma

Divided into 2A and 2B:

  • 2A applies to children who have a tumor in only one place, and surgeons cannot remove all of it that they can see.
  • 2B applies to children who have a tumor in only one place (doctors may or may not be able to remove all of it) and cancer cells in nearby lymph nodes.

Stage 3 neuroblastoma

Applies to children who have any one of these:

  • A tumor that doctors cannot remove all the way and that has spread to the other side of the body and maybe to nearby lymph nodes
  • A tumor that doctors cannot remove all the way, that is in the middle of the body and that has spread to both sides of the body (in lymph nodes or other structures)
  • A tumor in one place on one side of the body and also lymph nodes on the other side of the body

Stage 4 neuroblastoma

Divided into 4 and 4S:

  • Stage 4 applies to children whose tumor has spread to distant lymph nodes or other parts of the body.
  • Stage 4S applies when the child is younger than 1 year of age and the cancer has spread to the skin, liver or bone marrow, but not to bone. In stage 4S, the primary tumor must be small, doctors may or may not be able to remove it all and cancer cells may or may not be in nearby lymph nodes .

Additional risk ratings

Doctors further group childhood neuroblastoma as low risk, intermediate risk and high risk.

The risk group is based on the child's age when doctors diagnose the disease, the stage of the disease and special features of the cancer cells. Your child's doctor can explain your child's risk level.

Neuroblastoma at Seattle Children's

Our doctors have extensive experience treating children with neuroblastoma.

Dr. Julie R. Park is a national leader in caring for children with neuroblastoma and in studying the disease. She led the most recent national Children's Oncology Group (COG) trial for high-risk neuroblastoma. She is the chair of the COG neuroblastoma committee, with a goal to improve treatment of high-risk neuroblastoma.

Park is an expert in a newer type of radiation therapy, I-131-MIBG. Children with high-risk neuroblastoma that has come back after treatment (recurrent neuroblastoma) or that does not respond well to initial treatment (refractory) may be able to have this treatment. This is a way to get radiation inside your child's body and into their cancer cells via the bloodstream. Learn more.

She also works with national groups to find and develop new treatment options, such as new chemotherapy medicines for children at all stages of disease, including those whose cancer comes back after treatment.

Park is now leading a Phase 1 clinical trial testing T-cell therapy in children and adolescents with recurrent or refractory neuroblastoma. This trial is known as Engineered Neuroblastoma Cellular Immunotherapy (ENCIT)-01. It is based on promising results from previous trials using immunotherapy for recurrent leukemia. Learn more.

Park takes part in research by the Children's Oncology Group (COG) and the New Approaches to Neuroblastoma Therapy (NANT) consortium. COG is an international organization of childhood cancer specialists who conduct studies on many forms of childhood cancer. Seattle Children's is the only NANT site in the Pacific Northwest. NANT aims to develop and test new therapies that will be targeted specifically to neuroblastoma cells and improve outcomes with fewer side effects.

Our dedication to researching this disease means that our patients have the chance to get the very latest treatment options being studied – options not offered at all treatment centers. If your child has neuroblastoma, the team at Seattle Children's will talk with you about any studies with treatments that may help.

Neuroblastoma survival rates

Doctors who treat people with cancer use five-year survival rates as one way to measure treatment success. The five-year survival rate means the percentage of patients with the disease who are alive five years after their disease was diagnosed.

For all forms of childhood cancer combined, our five-year survival rate at Seattle Children's is higher than the national average. Our survival rate is 82%. The national average is 76.4%.

For neuroblastoma, our survival rate is nearly 5 points higher than the national average. More than 90% of children with low-risk disease survive. About 70% to 90% of those with intermediate-risk disease survive. Children in the high-risk group have a 30% chance of survival.

Read more childhood cancer statistics .

Read more about research and cancer programs and services at Seattle Children's.