Neuroblastoma

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Neuroblastoma

Neuroblastoma (NEW roh blast OH muh) is a rare cancer in children. It is a tumor that develops when some of the nerve cells (neuroblasts) in a fetus form abnormally. The abnormal neuroblasts then grow out of control and form a tumor. Generally, the tumor starts in the adrenal glands just above the kidneys (Picture 2). It can also begin in or spread (metastasize) to other parts of the body.

Neuroblastoma is the second most common solid tumor in children. It is the most common type of cancer in children younger than age 1.  It is often diagnosed before age 5.

For most, the exact cause of neuroblastoma is unknown. A very small percentage of cases are passed down in families (inherited).  It is slightly more common in boys.

The chances of cure are greatest when the diagnosis is made early and in infancy.

Signs and symptoms

Neuroblastoma is difficult to detect in the early stages of the disease. Often there are no symptoms or the symptoms are very much like other ordinary childhood problems. The parent may first notice a hard lump in the child's abdomen or it may be found by the doctor during a routine check-up.  Often the tumor is not found

until it begins to press on body organs. The symptoms will vary based on where the tumor is located (Picture 2 on page 2).

In the abdomen:

  • Swollen stomach
  • poor appetite
  • weight loss
  • constipation
  • persistent diarrhea
  • pain

In the urinary bladder or kidneys:

  • difficulty urinating
  • urinating often

In the chest:

  • difficulty breathing

In the neck:

  • drooping eyelid
  • unequal pupils
  • lack of sweating on one side of the face
  • enlarged lymph node

In the spinal cord:

  • numbness
  • weakness
  • problems moving a body part
  • limping or trouble walking

Other general symptoms:

  • general aches and pain
  • fever
  • bleeding
  • bruising
  • high blood pressure
  • rapid heart rate
  • pale complexion

Diagnosis

Your child may need to have several tests done to diagnose neuroblastoma. Some of these same tests will be used to assess how well the tumor is responding to treatment. The doctor will choose one or more of the following:

  1. Biopsy.  Usually, the only way to be sure that a child has neuroblastoma is to remove a part of the tumor and examine it. This is called a biopsy. The piece of tumor is examined under a microscope by a pathologist.
  2. Urine test. Urine is tested to measure the amounts of vanillylmandelic acid (VMA) and homovanillic acid (HVA). VMA and HVA are chemicals made by most neuroblastoma tumors.
  3. Bone marrow aspiration.  Some of the cells inside the bone will be removed with a needle and tested.  This test helps to find out if tumor cells have spread to the bone. If tumor cells are found, the diagnosis may be made without needing to do a biopsy of the tumor. (See Helping Hand HH-III-16, Bone Marrow Aspiration Test.)
  4. Imaging tests.  These tests help to locate the tumor and find out if it has spread.
    • MRI (magnetic resonance imaging). A large magnet and radio waves are used to view the organs inside the body. An MRI can give the exact size and location of the tumor. (See Helping Hand HH-III-69, MRI Magnetic Resonance Imaging.) 
    • CT scan (computed tomography). Special X-ray equipment is used to create detailed pictures of organs, bones, tissues and blood vessels. Like a slice of bread, a CT gives a picture of a cross-section of a specific part of the body. (See Helping Hand HH-III- 19, CT Scan.)
    • MIBG scan (meta-iodobenzyl-guanidine). A radioactive substance is used to see where cells have spread.
    • PET scan (positron emission tomography). A radioactive substance, called a tracer, is used to see how the organs inside the body are working.
  5. Molecular studies. The genetic make-up of the tumor cells is studied to help decide treatment and the likelihood (known medically as the risk) of cure.

Stages of neuroblastoma

A system of stages is used to describe how far the tumor has spread. The stage, along with the age of your child, helps the doctor choose the best treatment. There are five stages of neuroblastoma.

Stage I

The tumor is still in the organ or place where it started. It is called a “localized primary tumor”.

Stage II

The tumor is still in the place where it started and there are cancer cells in the lymph nodes on the same side of the body as the tumor. The cancer has not crossed the “midline” to the other side of the body (Picture 2).

Stage III

The tumor extends beyond the midline. Lymph nodes on both sides of the body are affected.  It has not spread to distant parts of the body.

Stage IV

The cancer has spread (metastasized) to other parts of the body, especially the bones or soft tissues (for example, the tissue behind the eye).

Stage IV-S

Occurs only in children younger than 1 year of age who have a localized primary tumor on one side of the body with spread limited to one or more places (liver, skin or bone marrow) and no signs of spread to the bones.


Classifying potential for cure

Staging plus age and at times, the results of genetic testing (molecular studies) are used to predict how likely it is that the child can be cured of cancer.

Children fall into 3 groups for predicting potential for cure. Health care providers refer to low, intermediate and high risk groups. Generally, younger children (under 18 months) in the early stages of disease are in the "low risk" group. This means that these children have a better chance of being cured. It is more difficult to cure neuroblastoma in older children who also have metastasis (high risk group).

Treatment

Once the neuroblastoma is staged and the potential for cure (risk group) is decided, the doctor will discuss treatment with you. If the cancer has spread, a combination of treatments is used.

  • Surgery. The most effective treatment for neuroblastoma is removing the entire tumor. Tumors in Stage I and II can often be removed. In these stages, the tumor has not spread to both sides of the body.
  • Chemotherapy  or “chemo.”  Powerful medicines are given by mouth or injected into the blood directly. Chemo aims to kill the cancer or stop the cancer cells from growing. Chemo may be used instead of surgery when the cancer is only in the spinal cord. (See Helping Hand HH-V-65 Chemotherapy.)
  • Radiation. High energy X-rays target the cancer cells and kill them. (See Helping Hand HH-II-34, Radiation Therapy.)
  • Stem cell transplant or bone marrow transplant. Unique blood cells are taken from a donor and given to your child. These special cells travel through the blood to the bone marrow. They then grow and divide to make mature, healthy blood cells.  The new blood cells help to replace the blood cells lost during chemo and radiation. (See Helping Hand HH-I-164, Bone Marrow Transplant (BMT).)
  • MIBG (meta-iodobenzyl-guanidine) therapy. A higher dose of MIBG is used in treatment than when it is used to scan for neuroblastoma. This therapy destroys tumors while sparing normal, healthy tissue.
  • Immunotherapy. Certain medicines are given to trigger your child’s own immune system to recognize and kill cancer cells.

Follow-Up Care

Your child’s progress will be evaluated regularly. He or she will have follow-up visits with a doctor who specializes in hematology (blood illnesses) and oncology (cancer). You can expect to have many tests repeated to find out if the tumor has gone away or is growing smaller, larger, or spreading.

It is difficult to hear that your child has cancer.  Try to stay informed. Do not be afraid to ask the health providers questions.  We are here to help you.

HH-I-128 Neuroblastoma (PDF) HH-I-128 6/90, Revised 10/16 Copyright 1990 Nationwide Children’s Hospital

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