The Facts, my Lovlies

Neuroblastoma is a form of cancer, found primarily in children, that attacks the developing nerve cells. In Neuroblastoma, cancerous tumors form from cells called neural crest cells. These cells are similar to those found in the developing nervous systems of an embryo or a fetus. The human body has two glands known as adrenal glands, one above each kidney. These glands are triangular in shape and are responsible for secreting hormones that control the body’s involuntary responses such as blood pressure and heart rate. Neuroblastoma can start in the bundle of nerves in the adrenals, as well as in the nerve groups in other parts of the body responsible for the body’s involuntary responses. There are two types of tumors that can be found in these nerve tissues. The first is GanglioNeuroblastoma, which consists of the cancerous nerve tissue mentioned above. These are the tumors of Neuroblastoma. The second is a tumor that resembles Neuroblastoma, but consists of mature nerve cells. These tumors are referred to as Ganglioneuromas and are not cancer.

Stages:

The accepted method for determining the severity of a case of Neuroblastoma is known as the International Neuroblastoma Staging System, or the INSS. The disease can be ranked on the INSS scale of 1 to 4, with 1 being the earliest stage of the disease and 4 being the most advanced. The following is a description of the symptoms and progression of the disease as seen in each stage:

When doctors look for the location of lymph nodes on different sides of the body, they are referring to the midline, which is shown by the thick black line running down the middle of the body in the diagram below. If the disease is found on both sides (right and left) of the black line, it is said to be on “both sides” of the body.

Stage 1: There is only one tumor. This tumor exists only in the area where the disease originated and can be completely removed by surgery. All lymph nodes are found to be free of the disease. Stage 2A: The tumor is not easily removed, but the disease is confined to one side of the body. (Either the tan or white side, as shown in the diagram) All lymph nodes still remain free from the disease. Stage 2B: The tumor has only affected one side of the body, but it may or may not be removed easily. In Stage 2B the lymph nodes on the opposite side of the body (the “contralateral” side) are not found to show signs of the disease, but those lymph nodes on the side of the body where the disease originated (the “ipsilateral” side) have been affected.

• Stage 3: There are three ways in which a patient can demonstrate signs of Stage 3 Neuroblastoma.
  • The tumor has spread so that it can be found on both sides of the body.
  • The tumor remains on one side of the body, but lymph nodes on the other side of the body have been affected.
  • The tumor is found on both sides of the body and the lymph nodes on both sides have been affected.
• Stage 4: This is the most advanced stage for patients over the age of 1. Here, the disease has spread to include affected lymph nodes, bone, bone marrow, and other organs.
• Stage 4S: Stage 4S is a classification for a “special” case of Neuroblastoma. This stage only applies to infants who demonstrate a tumor on one side of the body, as seen in Stages 1 and 2, but also have affected liver, skin, and/or bone marrow.

Who gets it?

• Neuroblastoma is almost always found in children.
• Nearly 90% of the cases of Neuroblastoma are diagnosed before the age of 6.
• Of all childhood cancers, Neuroblastoma accounts for approximately 7% of the cases diagnosed.
• The Neuroblastoma Children’s Cancer Society reports that 500 to 1,000 children are diagnosed each year in the United States.
• Slightly more cases of Neuroblastoma are seen in boys than in girls.

Symptoms:

The symptoms of Neuroblastoma depend on the age of the child and the origin of the disease. The majority of the symptoms are a result of the effect of the tumors on the body as opposed to the disease itself.

The general symptoms associated with Neuroblastoma include:

• Diarrhea
• Fever
• Anemia (weakness or tiredness due to a low number of red blood cells)
• Hypertension (high blood pressure)
• Loss of appetite

These symptoms occur often in children and can resemble a cold or flu. If your child is exhibiting any of these symptoms, it does not mean that he or she has Neuroblastoma. It is best to see your doctor anytime your child is feeling ill. Only a doctor can determine if a child has Neuroblastoma.

Some symptoms depend on the location of the tumor. When a tumor exists inside the body, it can put pressure on different nerves and organs, leading to a variety of symptoms.

• A tumor in the chest area can cause a child to have trouble breathing and can make swallowing difficult.
• A tumor in the stomach area can cause a child’s belly to swell and can cause constipation or urinary problems.
• Tumors along the spinal cord can cause a child to develop a limp or to have difficulty walking.
• Children with Neuroblastoma can also exhibit jerky motions in their muscles (known as “myoclonus“) and rotational movements of the eyes (called “opsoclonus“).

How is it detected?

Many different tests can be done to determine if your child has Neuroblastoma and how far the disease has progressed. Most of these tests are relatively painless and easy for your child.

• One of the best ways to test for Neuroblastoma is through a urine test. Neuroblastomas give off a substance called VMA (vanillyl mandellic acid). If VMA is found in your child’s urine, it is probable that there are Neuroblastomas present in the body. The urine test is almost 100% effective as a Neuroblastoma indicator.
• Another type of test, known as MIBG (meta-iodo-benzyl guanidine), can also detect Neuroblastoma in the body. In this test, a substance known as MIBG is injected into the body. Neuroblastoma cells take up MIBG. After the injection, the MIBG levels are measured. If a significant amount of the substance has been taken up and the patient’s MIBG level is low, it is likely that Neuroblastoma cells are present.
• CT (computed tomography) scans and MRI (magnetic resonance imaging) tests can be done to detect tumors resulting from Neuroblastoma. Both of these tests require that your child sit or lay still while pictures are taken by a machine. These machines allow doctors to see inside your child’s body on a very detailed level.

Is it curable?

The prognosis for Neuroblastoma depends greatly on the child’s age and the stage of the disease. The farther along the disease has progressed, the more difficult it can be to cure. Children under the age of 1 year tend to demonstrate a greater survival rate.

Unfortunately, diseases in very young children can be difficult to diagnose early on because the child cannot always verbalize a particular complaint.

Every child is different, and there is no universal generalization that can be made as to the future of a patient with Neuroblastoma. Your doctor will best be able to gauge the stage and prognosis of your child’s disease. The following is intended to provide an overview of some statistics from the National Cancer Institute regarding past cases of Neuroblastoma.

• Children of any age with Stage 1 Neuroblastoma have a cure rate greater than 90%.
• Long-term survival in Stage 2 patients over the age of 1 year can range from 75% to 90%, depending on the age of the child and the success of the treatments administered.
• Patients with Stage 2B or 3 Neuroblastoma over the age of 1 year have a survival rate of approximately 50% to 70%. Those younger than 1 year of age have a cure rate of around 80%.
• Patients with Stage 4 or 4S Neuroblastoma under the age of 1year tend to have a cure rate of 50% to 80%. Those patients over the age of 1 year have a cure rate of 10% to 40%.