Neuroblastoma

Neuroblastoma is a rare and potentially deadly cancer that affects children. Striking various systems of the body, it can arise from the adrenal gland. But what are its symptoms? How should it be treated? The following information should help you talk to a urologist about this condition.

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What is neuroblastoma?

Neuroblastoma is a solid, cancerous tumor that usually originates in portions of the adrenal gland, located in the abdomen near the kidney. Tumors may develop in nerve tissues in the neck, chest, abdomen and pelvis.

Neuroblastoma is a very common tumor of childhood, accounting for 7 to 8 percent of all malignancies. About one in 6,000 children will be diagnosed with neuroblastoma by the age of five. There are no gender-related differences in incidence and it is the most common malignant tumor of infancy. Fifty percent of the cases occur in children less than two years of age with 75 percent occurring in children less than four years of age. Neuroblastoma has a wide range of severity. Children with localized neuroblastoma can be cured by surgery alone but those who have disease that has spread to the bone (metastasized) usually have a fatal outcome.

Who is at risk for neuroblastoma?

It is suggested that 20 percent of neuroblastoma cases are inherited. However, the risk of neuroblastoma developing in a sibling or offspring of a patient with neuroblastoma is less than six percent.

What are the symptoms of neuroblastoma?

The symptoms of neuroblastoma vary because the location of the tumor determines the symptoms that are noticed by the parents. Most neuroblastomas are found in the abdomen. Parents may feel a lump or mass while dressing or bathing their child. A tumor in the abdomen may cause the child to feel "full", experience stomach pain, loss of appetite, constipation and difficulty urinating. Other tumors can be found in the head, neck or chest. Tumors located in the head or neck may appear as lumps. The child may have "black eyes", much like bruises, an eyelid that droops or pupil that does not constrict properly. Chest tumors may cause pain, difficulty in breathing or a persistent cough. Tumors that grow in spinal areas may cause the child to have pain, numbness of the lower legs or feet, constipation and difficulty urinating. Spread of the tumor is present in 70 percent of patients with neuroblastoma when they are first diagnosed. It is this spread that accounts for the majority of the symptoms.

How is neuroblastoma diagnosed?

The diagnosis of neuroblastoma is made by a variety of techniques. After the physician performs a physical examination, he/she may use screening tests to confirm the diagnosis. The most common ones used are CT scan and MRI. Laboratory evaluation will include a complete blood count (CBC) to test for anemia. Studies will be performed as this tumor may affect blood clotting. All children will have tissue taken from the bone marrow to examine under the microscope. Once a tumor is found, it may be necessary either to remove the tumor or to remove some tissue from the tumor and examine it under the microscope.

A staging system has been developed for neuroblastoma to help the physician determine the best treatment for the disease. The stage is based on how far the disease has spread from its original site. In stage 1, the cancer is found only in the place where it started. In stage 2a, the cancer is on one side and can only be partially removed. It has not spread to the lymph nodes. In stage 2b, there is cancer on one side with complete or incomplete removal but with lymph nodes that are positive for spread from the same side. It has not spread to lymph nodes on the other side of the body. In stage 3, the cancer has spread to tissues on the other side of the body and/or it has spread to lymph nodes relatively near the original cancer but on the other side of the body.  Stage 4 indicates that the cancer has spread to distant lymph nodes, bone, liver, skin, bone marrow or other organs. Finally, stage 4 indicates that the primary tumor has not directly spread to the opposite side of the body. Lymph nodes on the same side of the body may be involved, but nodes on the other side are not involved. Neuroblastoma may have spread to the liver or skin and may involve the bone marrow.

How is neuroblastoma treated?

More than one of these treatments may be used, depending on the stage of the cancer. Very often, more than one treatment base may be utilized in the same child. The four types of treatment used are:

• surgical removal of the tumor
• radiation therapy
• chemotherapy
• salvage chemotherapy with bone marrow transplantation (using very toxic chemotherapy that wipes out all the bone marrow and then replacing it in the child with new marrow)

Surgery is used whenever possible to remove the tumor. It also may be used to reduce the size of mass of the tumor in order to make treatment of some other form more effective. Radiation is very often used after surgery to sterilize the area where the tumor used to be. Chemotherapy may also be used to kill tumor cells after surgery or radiation. In some situations, the tumor is so invasive and large that chemotherapy may be used prior to surgery in order to shrink the mass.

What can be expected after treatment for neuroblastoma?

The chances of recovery from neuroblastoma depend on the stage of the cancer, the age of the child at diagnosis, the location of the tumor and the state and nature of the tumor cells evaluated under the microscope. Those tumors that originate in non-adrenal sites have a better prognosis that those from the adrenal gland. Children less than one year of age have far better survival rates than those diagnosed at older ages. Children under six months of age have a higher chance that their tumors will disappear with little or no treatment. Almost all patients with stage 1 disease who have undergone surgical removal of the tumors will survive. Children with stage 3 or 4 disease require much more aggressive treatment and do not do well. It is also known that the prognosis is poor if there is an increase in N-mye within the child's DNA. 

Frequently asked questions:

Can neuroblastoma be prevented?

Neuroblastoma may be genetic and passed down through generations. There are no known preventive measures.

There have been screening studies in Japan and Canada showing conflicting results. At present, it is generally not felt that screening will increase survival.

It depends on many factors such as age, stage, location and finding of an increase of N-mye within the child's DNA.

Many institutions are a part of larger national groups and gather the information on each child with neuroblastoma. These institutions include doctors of all specialties treating children with neuroblastoma.

This will be a very difficult time. Unless the child has a stage 1 tumor and can have it completely removed, there will be a combination of many different treatments with significant toxicity. This will produce a strain on the entire family unit.

Absolutely! One of the most magnificent aspects of the institutions participating in large children's cancer treatments is the commitment to family-centered care. Hospital personnel who are trained to deal with those affected by cancer will provide support.  Furthermore, there will be multiple other families who are going through the same thing you are so each of you will be able to give the other significant support.

Reviewed December, 2002

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Glossary Terms

abdomen:
	Also referred to as the belly. It is the part of the body that contains all of the internal structures between the chest and the pelvis.
adrenal:
	Glands that reside above the kidneys composed of an inner medulla and an outer cortex.
adrenal gland:
	One of a pair of small glands, each of which sits on top of one of the kidneys. These glands produce hormones that help control heart rate, blood pressure, the way the body uses food and other vital functions.
anemia:
	The condition of having too few red blood cells to carry oxygen throughout the body. People with anemia may be tired and pale, experience shortness of breath and/or may feel their heartbeat change. Anemia is common in people with chronic renal failure or those on dialysis.
bone marrow:
	A soft, reddish substance inside some bones that is involved in the production of blood cells.
cancer:
	An abnormal growth that can invade nearby structures and spread to other parts of the body and may be a threat to life.
CBC:
	Also known as a complete blood count. Standard set of measurement of the white blood cells and red blood cells.
chemotherapy:
	Treatment with medications that kill cancer cells or stop them from spreading.
complete blood count:
	Also known as a CBC. Standard set of measurement of the white blood cells and red blood cells.
constipation:
	A condition in which a person has difficulty eliminating solid waste from the body and the feces are hard and dry.
CT scan:
	Also known as computerized tomography, computerized axial tomography or CT scan. A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images of the body. Shows detailed images of any part of the body, including bones, muscles, fat and organs. CT scans are more detailed than general X-rays.
DNA:
	Also known as deoxyribonucleic acid. A molecule in the form of a twisted double strand that is the major component of chromosomes and carries genetic information.
gene:
	The basic unit capable of transmitting characteristics from one generation to the next.
genetic:
	Relating to the origin of something.
gland:
	A mass of cells or an organ that removes substances from the bloodstream and excretes them or secretes them back into the blood with a specific physiological purpose.
invasive:
	Having or showing a tendency to spread from the point of origin to adjacent tissue, as some cancers do. Involving cutting or puncturing the skin or inserting instruments into the body.
ions:
	Electrically charged atoms.
kidney:
	One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.
lymph:
	Fluid containing white cells. It can transport bacteria, viruses and cancer cells.
lymph nodes:
	Small rounded masses of tissue distributed along the lymphatic system most prominently in the armpit, neck and groin areas. Lymph nodes produce special cells that help fight off foreign agents invading the body. Lymph nodes also act as traps for infectious agents.
malignancies:
	Cancerous growths.
malignant:
	A cancerous growth that is likely to grow and spread which can cause serious disablement or death.
metastasized:
	Cancerous tumor that has spread to another part of the body.
MRI:
	Also referred to a magnetic resonance imaging. A diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.
N-mye:
	A gene.
pelvis:
	The bowl-shaped bone that supports the spine and holds up the digestive, urinary, and reproductive organs. The legs connect to the body at the pelvis.
radiation:
	Also referred to as radiotherapy. X-rays or radioactive substances used in treatment of cancer.
radiation therapy:
	Also referred to as radiotherapy or radiation. X-rays or radioactive substances used in treatment of cancer.
renal:
	Pertaining to the kidneys.
stage:
	Classification of the progress of a disease.
stent:
	With regard to treating ureteral stones, a tube inserted through the urethra and bladder and into the ureter. Stents are used to aid treatment in various ways, such as preventing stone fragments from blocking the flow of urine.
stoma:
	An opening.
tissue:
	Group of cells in an organism that are similar in form and function.
toxicity:
	Degree to which something is poisonous.
transplant:
	Replacement of a diseased organ with a healthy one. A kidney transplant may come from a living donor or from someone who has just died.
tumor:
	An abnormal mass of tissue or growth of cells.
urologist:
	A doctor who specializes in diseases of the male and female urinary systems and the male reproductive system. Click here to learn more about urologists. (Download the free Acrobat reader.)

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