Neuroblastoma

From the 'American Cancer Society'

Neuroblastoma

Neuroblastoma is a form of cancer that starts in early forms of nerve cells found in a developing embryo or fetus. It accounts for about 7% of childhood cancers. This type of cancer occurs in infants and young children. It is rarely found in children older than 10. This tumor can start anywhere but is usually in the belly (abdomen) and is noticed as swelling. It can also cause bone pain and fever.

What is neuroblastoma?

Neuroblastoma is a form of cancer that starts in certain types of very early forms of nerve cells found in an embryo or fetus. (The term neuro refers to nerves, while blastoma refers to a cancer that affects immature or developing cells). This type of cancer occurs in infants and young children. It is rarely found in children older than 10 years.

To understand neuroblastoma, it helps to know something about the sympathetic nervous system, which is where these tumors start.

About the sympathetic nervous system

The nervous system consists of the brain, spinal cord, and the nerves that reach out from them to all areas of the body. The nervous system is essential for thinking, sensation, and movement, among other things.

Part of the nervous system also controls body functions we are rarely aware of, such as heart rate, breathing, blood pressure, digestion, and other functions. This part of the nervous system is known as the autonomic nervous system.

The sympathetic nervous system is part of the autonomic nervous system. It includes:

  • Nerve fibers that run along either side the spinal cord.
  • Clusters of nerve cells called ganglia (plural of ganglion) at certain points along the path of the nerve fibers.
  • Nerve-like cells found in the medulla (center) of the adrenal glands. The adrenals are small glands that sit on top of each kidney. These glands make hormones such as adrenaline (epinephrine).

The main cells that make up the nervous system are called nerve cells or neurons. These cells interact with other types of cells in the body by releasing tiny amounts of chemicals (hormones). This is important, because neuroblastoma cells often release certain hormones that can cause symptoms (see the section, “How is neuroblastoma diagnosed?”).

Neuroblastomas

Neuroblastomas are cancers that start in early nerve cells of the sympathetic nervous system (sympathetic neuroblasts), so they can be found anywhere along this system.

A little more than 1 out of 3 neuroblastomas start in the adrenal glands. About 1 out of 4 begin in sympathetic nerve ganglia in the abdomen. Most of the rest start in sympathetic ganglia near the spine in the chest or neck or in the pelvis.

In rare cases, a neuroblastoma may have spread so widely by the time it is found that doctors can’t tell exactly where it started.

There is a wide spectrum of how neuroblastomas can behave. Some grow and spread quickly, while others grow slowly. Sometimes, in very young children, the cancer cells die without any cause and the tumor goes away on its own. In other cases, the cells sometimes mature on their own into normal ganglion cells and stop dividing. This causes the tumor to become a ganglioneuroma (see below).

Other autonomic nervous system tumors

Not all childhood autonomic nervous system tumors are malignant (cancerous).

Ganglioneuroma is a benign (non-cancerous) tumor made up of mature ganglion and nerve sheath cells.

Ganglioneuroblastoma is a tumor that has both malignant and benign parts. It contains neuroblasts (immature nerve cells) that can grow and spread abnormally, similar to neuroblastoma, as well as areas of more mature tissue that are similar to ganglioneuroma.

Ganglioneuromas are usually removed by surgery and looked at carefully under a microscope to be certain they do not have areas of malignant cells (which would make it a ganglioneuroblastoma). If the final diagnosis is ganglioneuroma, no other treatment is needed. If it is found to be a ganglioneuroblastoma, it is treated the same as a neuroblastoma.

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