About this Cancer

Neuroblastoma is a relatively rare childhood cancer that occurs in one out of every 100,000 children. It is most common in infants and children five-years-old and younger.  Neuroblastoma is a cancer of the nerve cells. These cells are involved in the development of the nervous system and other tissues, such as the adrenal gland.

Neuroblastoma most often occurs in the adrenal glands (specialized glands that release hormones to maintain blood pressure and enable us to respond to stress), located above the kidneys, though it can occur in other areas.  If left untreated, neuroblastoma can spread to the lymph nodes, liver, bones, and bone marrow.

Risk Factors

As with most cancers, the cause of neuroblastoma is unknown. In infants, neuroblastoma may be an abnormality of development that will disappear spontaneously. But, in some infants, as well as toddlers and most pre-school children, the disease will need aggressive treatment.  Neuroblastoma is most common in infants and children five-years-old and younger, and is more common in boys than girls.

Cancer Symptoms

The first symptoms of neuroblastoma are often vague, such as loss of appetite and tiredness. As a result, many neuroblastomas have already spread by the time they are diagnosed.

The symptoms of neuroblastoma vary depending on where the tumor is located. Often, the tumor is in the abdomen. The the child's abdomen may be swollen or distended and may complain of constipation or diarrhea. Though rare, sometime the child may have difficulty passing urine. If the tumor affects the chest area, the child may be breathless and have difficulty in swallowing. A tumor on the adrenal gland can cause high blood pressure and an increased heart rate. Though less common, some children may have weakness in the legs and walk unsteadily due to the tumor pressing on the spinal cord. 

 Diagnostic Tests

A biopsy, in which a sample of the tumor is surgically removed and examined by a pathologist under a microscope for cancer cells specific to neuroblastoma, will be performed. Tests such as X-rays, CT (computerized tomography) or MRI (magnetic resonance imaging) scans, and bone marrow and blood tests are carried out to see if the cancer has spread. A urine test will also be done, as urine will often contain a substance made by neuroblastomas, known as VMA (vanillyl mandellic acid).

An MIBG (meta-iodo-benzyl guanidine) scan may also be done. MIBG is a substance that is taken up by neuroblastoma cells. It is given by injection.  Attaching a small amount of radioactive iodine to the MIBG enables the tumors to be seen by a radiation scanner. MIBG may also be used as a treatment.

Treatment

The stage of neuroblastoma at diagnosis will determine the treatment. If the tumor is in only one place, surgery to remove the tumor may be all that is needed. Often, however, the tumor has already spread by the time the diagnosis is made, and intensive chemotherapy (oral or intravenous treatment with anticancer drugs) is needed. Radiation therapy (treatment with targeted X-rays) may be given externally, or, can sometimes be given internally using radioactive MIBG.

If the neuroblastoma is at an advanced stage, high-dose chemotherapy, toxic to the bone marrow, is given and followed by a stem cell or bone marrow transplant. In this treatment, bone marrow is taken from the patient after recovery from chemotherapy. This self-donated (autologous) collection of stem cells or bone marrow is purged for cancer cells using drugs or antibodies.  It is returned to the patient intravenously and travels to the bone marrow space to repopulate the bone marrow destroyed by chemotherapy.