What Is Neuroblastoma?

Neuroblastoma is a cancer that develops from nerve cells found in several areas of the body. Neuroblastoma most commonly arises in and around the adrenal glands, which have similar origins to nerve cells and sit atop the kidneys. However, neuroblastoma can also develop in other areas of the abdomen and in the chest, neck and pelvis, where groups of nerve cells exist.

Neuroblastoma most commonly affects children age 5 or younger, though it may rarely occur in older children.

Some forms of neuroblastoma go away on their own, while others may require multiple treatments. Your child’s neuroblastoma treatment options will depend on several factors.

Signs and symptoms of neuroblastoma vary depending on what part of the body is affected.

Neuroblastoma in the abdomen — the most common form — may cause signs and symptoms such as:
  • Abdominal pain
  • A mass under the skin that isn’t tender when touched
  • Changes in bowel habits, such as diarrhea
  • Swelling in the legs
Neuroblastoma in the chest –  may cause signs and symptoms such as:
  • Wheezing
  • Chest pain
  • Changes to the eyes, including drooping eyelids and unequal pupil size
Other signs and symptoms that may indicate neuroblastoma include:
  • Lumps of tissue under the skin
  • Eyeballs that seem to protrude from the sockets (proptosis)
  • Dark circles, similar to bruises, around the eyes
  • Back pain
  • Fever
  • Unexplained weight loss
  • Bone pain

Causes

In general, cancer begins with a genetic mutation that allows normal, healthy cells to continue growing without responding to the signals to stop, which normal cells do. Cancer cells grow and multiply out of control. The accumulating abnormal cells form a mass (tumor). Cancer cells may invade nearby tissues and can break off from an initial tumor to spread elsewhere in the body (metastasize).

Neuroblastoma begins in neuroblasts — immature nerve cells that a fetus makes as part of its development process. As the fetus matures, neuroblasts eventually turn into nerve cells and fibers and the cells that make up the adrenal glands. The majority of neuroblasts mature by birth, though studies have found a small number of immature neuroblasts in newborns. In most cases, these neuroblasts will mature or disappear. Others, however, form a tumor — a neuroblastoma.

It isn’t clear what causes the initial genetic mutation that leads to neuroblastoma. However, because beuroblastoma usually affects very young children, researchers believe the mutation occurs during pregnancy or soon after birth.

Risk factors

The only factor known to increase the risk of neuroblastoma is a family history of the cancer. Yet, familial neuroblastoma is thought to comprise a very small number of neuroblastoma cases. In most cases of neuroblastoma, a cause is never identified.

Complications of neuroblastoma may include:
  • Spread of the cancer (metastasis). Neuroblastoma may spread (metastasize) to other parts of the body, such as the lymph nodes, bone marrow, liver, skin and bones.
  • Spinal cord compression. Tumors may grow and press on the spinal cord, causing spinal cord compression. Spinal cord compression may cause pain and paralysis.
  • Signs and symptoms caused by tumor secretions. Neuroblastomas may secrete certain chemicals that irritate other normal tissues, causing signs and symptoms called paraneoplastic syndromes. One paraneoplastic syndrome that occurs rarely in people with neuroblastoma causes rapid eye movements and difficulty with coordination. Another rare syndrome causes abdominal swelling and diarrhea.
Tests and procedures used to diagnose neuroblastoma include:
  • Physical exam. Your child’s doctor conducts a physical exam to check out any signs and symptoms. The doctor will ask you questions about your child’s habits and behaviors.
  • Urine and blood tests in children. These may indicate the cause of any signs and symptoms your child is experiencing. Urine tests may be used to check for high levels of certain chemicals that result from the neuroblastoma cells producing excess catecholamines.
  • Imaging tests. Imaging tests may reveal a mass that can indicate a tumor. Imaging tests may include X-ray, ultrasound, computerized tomography (CT) scan and magnetic resonance imaging (MRI), among others.
  • Removing a sample of tissue for testing. If a mass is found, your child’s doctor may want to remove a sample of the tissue for laboratory testing (biopsy). Specialized tests on the tissue sample can reveal what types of cells are involved in the tumor and specific genetic characteristics of the cancer cells. This information helps your child’s doctor devise an individualized treatment plan.
  • Removing a sample of bone marrow for testing. Your child may also undergo bone marrow biopsy and bone marrow aspiration procedures to see if neuroblastoma has spread to the bone marrow — the spongy material inside the largest bones where blood cells are formed. In order to remove bone marrow for testing, the surgeon inserts a needle into your child’s hipbone or lower back and draws out the marrow.

Staging

Once Neuroblastoma is diagnosed, your child’s doctor may order further testing to determine the extent of the cancer and whether it has spread to distant organs — a process called staging. Imaging tests used to stage cancer include X-rays, bone scans, and CT and MRI scans, among others.

Using the information from those procedures, your child’s doctor assigns a stage to the neuroblastoma. Stages of neuroblastoma include:
  • Stage I. Neuroblastoma at this stage is localized, meaning it’s confined to one area, and may be completely removed with surgery. Lymph nodes connected to the tumor may have signs of cancer, but other lymph nodes don’t have cancer.
  • Stage IIA. Neuroblastoma at this stage is localized, but may not be as easily removed through surgery.
  • Stage IIB. Neuroblastoma at this stage is localized and may or may not be easily removed through surgery. Both the lymph nodes connected to the tumor and the lymph nodes nearby contain cancer cells.
  • Stage III. Neuroblastoma at this stage is considered advanced, and it isn’t possible to remove the tumor through surgery. The tumor may be a larger size at this stage. Lymph nodes may or may not contain cancer cells.
  • Stage IV. Neuroblastoma at this stage is considered advanced and has spread (metastasized) to other parts of the body.

Stage IVS. This stage is a special category of neuroblastoma that doesn’t behave like other forms of neuroblastoma, though it isn’t clear why. Stage IVS neuroblastoma applies only to children younger than 1 year old. Stage IVS indicates that neuroblastoma has spread to another part of the body — most commonly the skin, liver or limited bone marrow involvement. Despite the extent of neuroblastoma, babies with this stage have a good chance of recovery. Neuroblastoma at this stage sometimes goes away on its own and often doesn’t require any treatment.

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