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What is neuroblastoma?

Neuroblastoma is a type of cancer that begins in the embryonic cells which normally develop into parts of our nervous system. These cells are called neuroblasts. A tumor arising from these cells is called a neuroblastoma.

The cause of neuroblastoma is not known. We do know it is not hereditary, does not result from injury and is not infectious.
Neuroblastoma is responsible for 8-10% of all childhood cancers. Although it is only the 5th most common cancer in childhood, it is responsible for a disproportionate number of deaths due to cancer in children. 

Neuroblastoma usually occurs in children under 5. It is the most common tumor in babies under 1 year of age. Although it is rare, neuroblastoma can occur in older children and adults.

Each year in Ontario about 25 children are diagnosed with neuroblastoma. Sixty percent of children diagnosed over the age of 1 have widespread (metastatic) disease at diagnosis.

Over the last 20 years there has been modest improvement, due to research, in the prognosis for children diagnosed at less than 1 year of age with neuroblastoma, however, despite research initiatives, there has been little improvement in the prognosis for older children with widespread disease at diagnosis. The vast majority of these children die.

Symptoms of the disease vary depending on where the tumor is located.

About 40% of neuroblastomas develop in the adrenal gland which is just above the kidney. The rest begin in a chain of nerve tissue that runs on each side of the spine. Tumors will then develop in the neck, chest, pelvis or abdomen.

Symptoms also result from the tumor spreading to different parts of the body. In 70% of patients the original tumor has already spread to other parts of the body before any symptoms are seen. Neuroblastoma may spread to bone, bone marrow, liver, skin and lymph nodes.

Ultrasound, CT Scans, liver scans, bone scans and MIBG scans are all used to diagnose neuroblastoma and to detect tumor locations throughout the body. Urine and blood tests are also done. A biopsy of the tumor is usually taken to confirm the diagnosis.

When neuroblastoma is diagnosed it is classified as being in one of 5 stages. The stage of disease tells us how far the disease has spread and determines what the best plan of treatment should be.

Sometimes surgery can be done to remove the tumor. If the tumor cannot be safely removed then chemotherapy may be used to help shrink the tumor so that it can possibly be removed.

Chemotherapy is used when the tumor cells have spread throughout the body.

Bone Marrow Transplant is often the treatment offered to patients with widespread disease and a poor prognosis once the neuroblastoma is under control.

The prognosis for neuroblastoma depends on the age of the child and the stage of the disease at diagnosis. The younger the child is at diagnosis the greater the chance for cure is.

If there is no sign of disease recurrence 5 years after diagnosis the chances are very good that the child has been cured.

The current 5 year survival rate after bone marrow transplant for children with neuroblastoma is 20-25%.

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