Neuroblastoma is the most common extracranial solid tumor in childhood. Neuroblastoma arises from immature nerve tissue and it is often present before a child is born. However, children are typically not diagnosed with neuroblastoma until the tumor begins to grow and subsequently starts to compress organs and tissues that surround the tumor.

The majority of neuroblastomas arise in the abdomen usually within the adrenal gland that sits on top of the kidney, but can also be found in the neck, chest, and/or pelvis.

Neuroblastoma cancer cells can spread (metastasize) to other areas of the body, most commonly the lymph nodes, bones, liver and bone marrow. Approximately 40 percent of patients present with localized disease that requires minimal therapy, but the majority of patients (60 percent) present with aggressive metastatic disease.

What are the signs and symptoms of neuroblastoma?

The signs and symptoms of neuroblastoma are different in each child depending on where the tumor is located, the size of the tumor and whether or not it has metastasized. Common symptoms of neuroblastoma may include:

  • Swollen or larger abdomen
  • Painless, bluish lumps under the skin (in infants)
  • Trouble breathing
  • High blood pressure and increased heart rate
  • Swelling and bruising around the eyes
  • Uncontrolled eye movement or bulging eyes
  • Changes in urination or bowel movements
  • Pain, limping, paralysis or weakness
  • Diarrhea
  • Fever

How is neuroblastoma diagnosed?

A child will need to undergo a series of tests in order for our doctors to make a diagnosis. Some of these tests may include:

  • Blood and urine tests 
  • Neurological exam: A series of questions or tests that check a child's brain, spinal cord and nerve function.
  • CT or MRI scans: These scans look at the location of the tumor, the size of the tumor and how much the tumor has spread.
  • Bone scans: These pictures or X-rays are used to detect bone abnormalities from tumor metastases.
  • 123I-MIBG (metaiodobenzylguanidine) scan: A small amount of radioactive iodine (123I) is attached to MIBG and then injected into the child's bloodstream. The scan, like a CT, lights up in the areas that have neuroblastoma in the body because the majority (90 percent) of neuroblastoma’s specifically take up MIBG so that the tumor and all metastastic sites are seen on the radiographic picture.
  • Bone marrow aspiration and/or biopsy: A small amount of bone marrow is taken from the child through aspiration (a small amount is suctioned through a hollow needle) and/or by a biopsy (small piece of solid bone marrow tissue is cut out). This is usually done from the hip bone under anesthesia.

How is neuroblastoma staged?

Staging looks at whether or not the cancer has spread and, if it has spread, how far.

  • Stage 1: The tumor has not spread to other areas of the body and is completely resectable (removable by surgery). Lymph nodes on the same side of the tumor do not have tumor cells.
  • Stage 2: The tumor has not spread to other areas of the body, but may or may not be completely resectable. Lymph nodes on the same side of the tumor may have tumor cells.
  • Stage 3: The tumor has spread to nearby lymph nodes and is not completely resectable.
  • Stage 4: The tumor has metastasized (spread) to distant lymph nodes, bone marrow, liver, skin, and/or other organs.

How are treatment and prognosis determined?

Neuroblastoma’s are considered low, intermediate, or high risk based on the stage of the child's neuroblastoma as well as other genetic characteristics that are tested on tumor tissue at diagnosis (from biopsy or resected tumor). This risk classification stratifies patients to specific treatment regimens.

How is neuroblastoma treated?

Every child treated at the Aflac Cancer Center will receive an individualized treatment plan. Treatment may include some of the following:

  • Surgery (to remove all or part of the tumor)
  • Chemotherapy
  • Radiation
  • Biologic therapy with retinoids and an antibody (CH14;18) that target neuroblastoma cells
  • Targeted therapy
  • Peripheral blood stem cell transplant

We recommend continuous follow-up care for children with neuroblastoma even following completion of treatment. Chemotherapy and/or radiation side effects, as well as recurrence of the disease, can occur in survivors of neuroblastoma. Researchers are continuously working to discover methods to improve the treatment for children with neuroblastoma and decrease side effects.

Refractory or Recurrent Neuroblastoma

For children with neuroblastoma, especially those with high-risk disease, the cancer may not respond (refractory) or may come back (recurrent or relapsed) during or after treatment.

The Aflac Cancer Center is now offering 131I-MIBG therapy as a treatment option for patients with refractory or recurrent neuroblastoma. There are currently only a small number of pediatric centers in the country that offer MIBG therapy.

Related Resources

National Cancer Institute: General Information About Neuroblastoma
National Cancer Institute: Treatment Option Overview
American Society of Clinical Oncology
American Cancer Society: What are the key statistics about neuroblastoma?
American Cancer Society: What are the risk factors for neuroblastoma?
American Cancer Society: How is neuroblastoma staged?
American Cancer Society: What is neuroblastoma?