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Together is a new resource for anyone affected by pediatric cancer - patients and their parents, family members, and friends.
Learn MoreNeuroblastoma is a cancer that grows from cells in the nervous system. It forms when healthy nervous system cells do not develop as they should. Instead, cancer cells called "neuroblasts" become stuck in an early stage of development. They do not become healthy, normal cells. The cancer cells begin to grow, and this causes a tumor to form.
Patients with neuroblastoma may have only one tumor. Or neuroblastoma cells may spread to other parts of the body. Neuroblastoma is usually found in children younger than 5. It is the most common solid tumor found outside the brain in children.
Neuroblastoma often starts in the abdomen, either in the adrenal gland or in other nerve cells. It can also form in the neck, chest, or pelvis.
Symptoms depend on the location of the tumor (or tumors if the cancer has spread). The symptoms may include a lump, pain, loss of appetite, tiredness, and irritability.
About 700 children are diagnosed with neuroblastoma in the U.S. each year.
Neuroblastoma often forms in nerve tissue of the adrenal glands, which are on top of the kidneys.
Signs and symptoms depend on where the cancer is located. They may include:
Other symptoms may include:
Neuroblastoma has often spread to other parts of the body before it is diagnosed.
Some neuroblastoma patients develop Horner syndrome, or nerve damage around the eye. Symptoms may include:
| Tumor Location |
Symptoms |
|---|---|
| Eye |
Bulging eye, dark circles, bruising, blindness |
| Neck |
Lump or swelling, Horner syndrome |
| Abdomen |
Lump, loss of appetite, vomiting, constipation, pain |
| Pelvis |
Change in toilet behavior; bowel or bladder problems |
| Spine |
Weakness, paralysis |
| Bone | Pain |
| Bone marrow | Pain, fatigue, burning |
| Chest | Horner syndrome |
Neuroblastoma is most common in young children. It happens slightly more often in males than females.
A small number of patients (1–2%) have hereditary neuroblastoma. This can be passed down in families. It is often caused by a change in the ALK or PHOX2B genes. But other gene mutations have also been linked to hereditary neuroblastoma.
Doctors use several types of tests when diagnosing neuroblastoma. Tests may include:
Doctors will study the tumor for features that are important to diagnose and treat neuroblastoma. Some tumors are aggressive and require intense therapy. Doctors can predict how a tumor might respond to treatment by how the cells look and whether there are certain gene changes in the tumor.
Neuroblastoma is staged using the International Neuroblastoma Risk Group Staging System (INRGSS). Staging is based on these factors:
| Stage | Spread of disease |
|---|---|
| Stage L1 Localized |
The tumor has not spread from where it began. It does not affect important nearby body organs. It is limited to one body area such as the neck, chest, abdomen, or pelvis. |
| Stage L2 Localized |
The tumor has not spread to parts of the body that are far away from the tumor. But tests show the tumor is acting more aggressive to nearby areas and organs. These are " image-defined risk factors." The tumor also may have spread nearby. |
| Stage M Metastatic |
The tumor has spread to distant parts of the body. This includes all metastatic disease except Stage MS tumors. |
| Stage MS Metastatic |
For children under 18 months old: Cancer spread is limited to the skin, liver, or less than 10% of bone marrow. |
Doctors use risk groups to classify neuroblastoma and plan treatments. There are 3 risk groups:
High-risk neuroblastoma means that the cancer is hard to remove and more likely to come back. Children with high-risk neuroblastoma need intense therapy.
Risk groups are based on factors that include:
Learn more about Risk of Relapse Groups from the Children’s Oncology Group.
Treatment depends on the assigned risk group. Treatment options may include:
Very young, low-risk patients may be followed with observation instead of active treatment. Sometimes neuroblastoma goes away on its own (regression). But this is rare. Patients are watched closely for tumor progression.
Patients with neuroblastoma may be offered treatment as part of a clinical trial. Neuroblastoma treatments are planned based on risk groups:
| Risk category | % of new patients | Main treatments | Prognosis |
|---|---|---|---|
Low Risk |
40% |
|
About 98% survival |
Intermediate Risk |
15% |
|
About 95% survival |
High Risk |
45–50% |
|
Less than 60% survival |
(1/4) A CT scan shows neuroblastoma in a patient's chest before treatment.
(2/4) After one course of chemo, the tumor got smaller.
(3/4) After a second course of chemo, the tumor is even smaller.
(4/4) The scan shows the patient's chest after surgery to remove the tumor.
Neuroblastoma survival depends on several factors:
Your child's doctor is the best source of information on your child's specific case.
Patients need follow-up care to watch for recurrence after treatment ends. The medical team will suggest specific tests and their schedule.
In patients who do not have high-risk disease, the chance of relapse is 5–15%. In high-risk patients, the risk of relapse is about 50%. Relapse is most common in the first 2 years after treatment. Relapse is rare when there is no sign of cancer 5 years after completing treatment.
Survivors treated with chemotherapy or radiation should be monitored for long-term and late effects of therapy. Problems due to treatment could include hearing loss, heart problems, thyroid problems, decreased growth, decreased bone density, and kidney damage.
About 25% of survivors have serious chronic health conditions 25 years after diagnosis, according to the Childhood Cancer Survivor Study.
Regular medical checkups by a primary health care provider are important to watch for health problems that can develop years after therapy.
Your child's care team should give you a survivorship care plan after treatment has ended. This report will include needed tests and tips for a healthful lifestyle.
—
Reviewed: November 2023
