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 heartbeat and blood pressure, and the way your child's body reacts to stress.
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. This part of the nervous system controls heartbeat, blood pressure and reactions to stress. However, 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
(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 at Seattle Children's
Seattle Children’s has deep experience treating children with neuroblastoma. We also are leaders in finding new, better treatments with fewer side effects. We are the only center in the Pacific Northwest to belong to the NANT (New Approaches to Neuroblastoma Therapy) group. We are leading national clinical trials to study how to improve the outcome for children with neuroblastoma.
According to the American Cancer Society, more than 95% of children with low-risk neuroblastoma survive. About 90% of those with intermediate-risk disease survive. Children in the high-risk group have a 40% to 50% chance of survival.
Read more childhood cancer statistics.
The experts you need are here
Dr. Julie R. Park is a national leader in caring for children with neuroblastoma and developing new treatments for children at all stages of the disease. Her clinical research focuses on finding therapies to improve the treatment for children with high-risk neuroblastoma.
Dr. Park led the most recent national Children’s Oncology Group (COG) trial for high-risk neuroblastoma and chairs the COG neuroblastoma committee. COG is an international organization of childhood cancer specialists that conducts studies on many forms of childhood cancer.
Our research expands your child’s treatment options
Our dedication to researching neuroblastoma means that our patients have the chance to get the very latest treatment options being studied. These options are not offered at all treatment centers.
Dr. Park is now leading a phase 1 clinical trial testing T-cell therapy in children and teens with neuroblastoma that does not respond well to early treatment (refractory) or that comes back (recurrent).
This trial is known as Engineered Neuroblastoma Cellular Immunotherapy (ENCIT)-01. This therapy works by reprogramming the body’s infection-fighting T cells to find and destroy cancer cells with few side effects. It is based on promising results from previous trials using immunotherapy for recurrent leukemia. Learn more.
Dr. Park also developed a program at Seattle Children’s that provides a newer type of radiation therapy, I-131-MIBG, for children with neuroblastoma. Children with high-risk recurrent or refractory neuroblastoma 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.
If your child has neuroblastoma, the team at Seattle Children’s will talk with you about any studies with treatments that may help.
Read more about research and cancer programs and services at Seattle Children’s.
Neuroblastoma in Children
Cancer in children is rare, and neuroblastoma accounts for less than 10% of all childhood cancers. But it is the most common solid tumor in children younger than 1 year of age. In children of all ages, it is the most common solid tumor arising outside of the brain.
Most children with this disease get it before age 5. In some kids, the disease starts before birth.
In most cases, parents and doctors don’t know the disease is there until tumors grow large enough to be felt or to cause symptoms.
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.
Staging refers to the way doctors classify cancer based on where it is in the body. The cancer stage, along with other factors like risk group, helps doctors choose a course of treatment.
There are two ways to determine the stage of neuroblastoma in children. They are called the International Neuroblastoma Staging System (INSS) and the International Neuroblastoma Risk Group Staging System (INRGSS). INRGSS is newer and somewhat simpler.
International Neuroblastoma Staging System (INSS)
Stage 1 neuroblastoma
- Applies to children who have a tumor in only one place. Surgeons can remove all of it that they can see.
Stage 2 neuroblastoma is divided into 2A and 2B
- 2A applies to children who have a tumor in only one place. Surgeons cannot remove all of it that they can see.
- 2B applies to children who have a tumor in only one place and cancer cells in nearby lymph nodes. Doctors may or may not be able to remove all of the tumor.
Stage 3 neuroblastoma
Stage 3 applies to children who have any one of these:
- A tumor that doctors cannot remove all the way. The tumor has spread to the other side of the body and maybe to nearby lymph nodes.
- A tumor that doctors cannot remove all the way and that is in the middle of the body. It 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. Cancer also is found in lymph nodes on the other side of the body.
Stage 4 neuroblastoma is 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. The primary tumor must be small. Doctors may or may not be able to remove it all. Cancer cells may or may not be in nearby lymph nodes.
International Neuroblastoma Risk Group Staging System (INRGSS)
L 1 neuroblastoma
- Applies to children who have a tumor in only one place. The tumor has not invaded surrounding organs, blood vessels or nerves.
L 2 neuroblastoma
- Applies to children who have a tumor in only one part of the body. The tumor has spread to nearby lymph nodes, or it is invading surrounding organs, blood vessels or nerves.
- Applies to children whose tumor has spread to distant lymph nodes or other parts of the body. M stage excludes children who are considered MS stage.
- Applies when the child is younger than 18 months of age and the cancer has spread to the skin, liver or bone marrow, but not to bone.
Additional risk ratings
Doctors also group childhood neuroblastoma as non-high risk (low risk and 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.
Contact the Cancer and Blood Disorders Center at 206-987-2106 for a referral, a second opinion or more information.