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Neuroblastoma

Neuroblastoma is a cancer which develops from branches of nerves from the spinal cord. These branches may reach many areas of the body and tumors can be found in many different places. In half of the cases of children with neuroblastoma, the tumors are found in the abdomen and usually involve the adrenal gland which sits on top of the kidney. Neuroblastoma is sometimes (but less frequently) found in the chest, neck, pelvis or head.

What are some of the signs and symptoms of neuroblastoma?

The symptoms of neuroblastoma depend upon where the cancer is. For example, if the tumor is in the abdomen, the child's belly may look enlarged or bloated. If there is bone involvement with the tumor, there may be bone pain resulting in a limp or the child's refusal to walk or to use a certain arm or leg. Other general signs may be loss of appetite, failure to gain weight, unexplained fevers, a tired feeling, pale skin and minor aches and pains.

What causes neuroblastoma?

The cause of neuroblastoma is not known at this time. We do know that it cannot be spread to others. We also know that some infants are born with it, suggesting that it can occur while the unborn child's nervous system cells are growing and maturing.

Who gets neuroblastoma?

Although neuroblastoma is the third most common type of cancer in children, it is still rare. There are about 500 newly diagnosed cases of neuroblastoma each year in the United States. In most cases, it is a disease of early childhood. Fifty percent of these children are under 2 years old when diagnosed. Overall, 75 percent of all children with neuroblastoma are under 5 years old.

Is neuroblastoma inherited?

There is no information to support this. It does not tend to run in families or to occur in other family members.

What are metastases?

Metastases refers to the spread of cancer from its original location to other parts of the body. Neuroblastoma can spread, usually to the bones or the bone marrow. Neuroblastoma has often been called a “silent tumor” because 60 percent of children with this tumor already have metastases before any signs of the disease are noticed.

What is staging?

Staging is the process of finding out the extent of disease present at the time of initial diagnosis. Neuroblastoma has been divided into four stages for purposes of better matching the therapy to the disease:

Stage 1 — The tumor is confined to the organ and can be completely removed by surgery.

Stage 2 — The tumor has not been completely removed or has spread to lymph nodes located on the same side of the body as the tumor.

Stage 3 — The tumor has spread from its original site to lymph nodes in the opposite side of the body.

Stage 4 — The tumor has spread to distant locations in the body such as the bone marrow, bones, liver or lymph nodes.

Stage 4S — Same as Stage 4 but reserved for infants under 1 year of age, who have small tumors that have spread to the liver, bone marrow or skin, but not the bones.

How can neuroblastoma be treated?

The method of treatment proposed for your child will depend upon the stage of disease at the time of diagnosis and on certain features of your child's tumor.

There are three types of therapy commonly used to treat neuroblastoma. They are chemotherapy, surgery and bone marrow transplantation. The type of therapy chosen will depend upon the age of the child and the extent of the disease. The decision of how to treat the disease is made by both you and your child's doctor.

In a number of newborns with neuroblastoma the tumors disappear without therapy. In general, this is not true for older children with neuroblastoma. Over 60 percent of all children with neuroblastoma are older than 1 year and have metastases. Most children receive a combination of surgery and chemotherapy to treat their disease; a few children may also need radiation therapy.

Chemotherapy is the use of medications to kill cancer cells. Chemotherapy not only destroys cancer cells, but also affects the growth of normal cells and therefore has side effects which will be explained to you.

Surgery to remove the bulk of the tumor may be done early, during the diagnostic period, or later after the tumor has been shrunk with radiation or chemotherapy drugs. If there is wide spread of the disease, surgery is usually done after chemotherapy to minimize the risk of injury to vital organs that the neuroblastoma may be effecting.

Stem cell transplantation is the use of higher doses of chemotherapy and sometimes radiation therapy, followed by the infusion of stem cells (young cells from the bone marrow) that have been previously collected from the patient. If your child has Stage 3 or 4 neuroblastoma, more information will be given to you about this treatment option.

How long will my child's therapy last?

The usual length of treatment is about nine months. It involves one scheduled hospitalization lasting about a week, per month, and follow-up blood tests as an outpatient.

The above information is a brief overview of what you need to know initially about neuroblastoma in children. The nurses and doctors can answer your questions and help you find more information.

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