What Is the Survival Rate for Neuroblastoma?

Neuroblastoma is rare cancer. It develops in nerve cells and forms neuroblasts. Commonly, neuroblastoma develops in the adrenal glands above the kidneys but can develop in other parts of the body, such as the neck, chest, and others.

It accounts for less than six percent of all pediatric cancers. Though it can occur at any age, nearly 90 percent of neuroblastomas are diagnosed in children younger than five years.

The exact cause of neuroblastoma is not known. It is believed to be related to the development of nerve cells in the body.

Neuroblasts are immature nerve cells found in the developing fetus and are generally present in the adrenal gland, neck, chest, and other parts of the body. In most cases, neuroblasts mature and turn into normal nerve cells. However, they may continue to grow and divide, leading to the formation of neuroblastoma.

There are no known ways to prevent neuroblastoma, and it is unclear why some people develop the disease while others do not.

A few factors may increase the risk of developing neuroblastoma include:

• Age: Neuroblastoma is mostly diagnosed in children younger than five years.
• Family history: Children with a family history of neuroblastoma could be at an increased risk of developing the disease.
• Genetic diseases: Certain genetic abnormalities may increase the risk of neuroblastoma.
• Gender: Boys are slightly more likely to develop neuroblastoma than girls.

It is crucial to note that having one or more of these risk factors does not necessarily mean a child will develop neuroblastoma. Most children with these risk factors do not develop the disease.

Symptoms of neuroblastoma can vary but may include:

• Abdominal pain or swelling
• A lump in the neck, chest, or abdomen
• Bone pain
• Unusual reflexes
• Fever
• Weight loss
• Fatigue
• Persistent cough

There are several different staging systems for neuroblastoma, but one commonly used system is the International Neuroblastoma Staging System (INSS).

The INSS divides neuroblastoma into four stages:

1. Stage I:
   • The tumor is localized and on one side of the body.
   • Cancer can be removed entirely with surgery.
   • Lymph nodes near the tumor could be unaffected (although nodes enclosed within the tumor may contain neuroblastoma cells).
2. Stage II:
   • Stage IIA:
     • Cancer is confined to the area where it originated.
     • The visible tumor could not be removed completely by surgery.
     • The nearby lymph nodes could be cancer free (although nodes within the tumor may contain neuroblastoma cells).
   • Stage IIB:
     • Cancer is confined to one side of the body.
     • Surgery may or may not completely remove it.
     • Neuroblastoma cells have been found in nearby lymph nodes outside the tumor, but cancer has not spread to lymph nodes on the opposite side of the body or elsewhere.
3. Stage III: Cancer has not spread to distant organs and one of the following applies:
   • Surgery cannot remove cancer completely because it has crossed the midline (defined as the spine) and spread to the opposite side of the body. It may or may not have spread to nearby lymph nodes.
   • Cancer has returned to the site where it began and spread to one side of the body. It has spread to relatively close lymph nodes on the opposite side of the body.
   • Cancer is in the center of the body and has spread to both sides (either directly or by spreading to nearby lymph nodes).
4. Stage IV: The tumor has spread to distant lymph nodes and other parts of the body, such as bones, liver, skin, and bone marrow, and cannot be removed entirely by surgery.
   • Stage 4S (special neuroblastoma):
     • The affected child is younger than one year.
     • Cancer has spread to one side of the body and could spread to lymph nodes on the same side of the body but not the other.
     • Cancer has spread to the liver, skin, and bone marrow.
     • About 10 percent of bone marrow cells are cancer cells, and imaging tests, such as the meta-iodobenzylguanidine scan (a nuclear medicine scan that utilizes iodine-123 meta-iodobenzylguanidine), do not detect cancer in the bone marrow.

Neuroblastoma stages can change over time because cancer may grow or spread. Regular follow-up care and monitoring are important to track the progress of the disease and adjust treatment as needed.

The healthcare provider will examine the child for any abnormalities, such as a mass or swelling, and signs of nerve damage.

The following tests may be used to diagnose neuroblastoma:

• Imaging tests: X-ray, CT, MRI, meta-iodobenzylguanidine, or positron emission tomography scans generate images of the body's internal parts, which helps detect potential abnormalities.
• Biopsy: A small tissue sample is removed from the affected area and examined under a microscope to confirm the diagnosis of neuroblastoma.
• Laboratory tests: Blood and urine tests check for certain substances that could be produced by neuroblastoma cells.

The diagnosis of neuroblastoma can be difficult because the symptoms of the disease can be similar to those of other conditions.

Treatment for neuroblastoma may include surgery, chemotherapy, radiation therapy, immunotherapy, or a combination of these approaches. The specific treatment plan will depend on the stage and location of the cancer, as well as the child’s age and overall health.

• Surgery
  • The primary treatment for neuroblastoma. 
  • The goal is to remove as much of the cancerous tissue as possible.
  • Surgery could be used to:
    • Remove the tumor: The tumor is small and can be completely removed without damaging surrounding tissue.
    • Debulking surgery (remove part of the tumor): The tumor is too large to be removed entirely; the surgeon may remove as much of the tumor as possible.
    • Remove lymph nodes: Lymph nodes contain cancer cells.
    • Remove affected organs: In some cases, an affected organ, such as the adrenal gland, is removed.
• Chemotherapy
  • Drugs kill cancer cells.
  • Administered orally or intravenously.
  • Often an important part of the treatment plan for neuroblastoma and can help improve the chances of a successful outcome.
  • Could be administered before surgery to shrink the size of the tumor or after surgery to kill any remaining cancer cells.
• Radiation therapy
  • High-energy beams kill cancer cells.
  • Could be used to shrink a tumor before surgery or kill any remaining cancer cells after surgery.
• Immunotherapy
  • Medications stimulate the immune system to fight cancer cells.
  • A relatively new treatment option for neuroblastoma and is typically used in combination with other treatments, such as chemotherapy and surgery.
  • Types of immunotherapies used to treat neuroblastoma are:
    • Monoclonal antibodies: Proteins are designed to target and attack specific cancer cells.
    • Checkpoint inhibitors: Block proteins on immune cells that cancer cells use to avoid detection.
    • Oncolytic viruses: Genetically modified viruses specifically attack cancer cells.
  • Usually, well-tolerated but can cause side effects, such as fatigue, nausea, and skin rash.
  • Studies on immunotherapy for neuroblastoma treatment are underway, and more research is needed to determine its effectiveness.
  • Discuss the risks and benefits of immunotherapy with a healthcare provider to determine if it is the appropriate treatment for the child.
• Stem cell transplant
  • Replaces damaged bone marrow with healthy stem cells.
  • Used after high-dose chemotherapy or radiation therapy to help the body recover.

Discuss all treatment options with a healthcare provider to understand the outcome chosen course of treatment.