Neuroblastoma Treatment (PDQ®)–Patient Version

NEUROBLASTOMA

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General Information About Neuroblastoma

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Neuroblastoma is a disease in which malignant (cancer) cells form in neuroblasts (immature nerve tissue) in the adrenal gland, neck, chest, or spinal cord.
Neuroblastoma is sometimes caused by a gene mutation (change) passed from the parent to the child.
Signs and symptoms of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.
Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma.
A biopsy is done to diagnose neuroblastoma.
Certain factors affect prognosis (chance of recovery) and treatment options.

Neuroblastoma is a disease in which malignant (cancer) cells form in neuroblasts (immature nerve tissue) in the adrenal gland, neck, chest, or spinal cord.

Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. The adrenal glands make important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in nerve tissue in the neck, chest, abdomen or pelvis.

Drawing shows parts of the body where neuroblastoma may be found, including the paraspinal nerve tissue and the adrenal glands. Also shown are the spine and right and left kidney. — Neuroblastoma may be found in the adrenal glands and paraspinal nerve tissue from the neck to the pelvis.

Neuroblastoma most often begins in infancy and may be diagnosed in the first month of life. It is found when the tumor begins to grow and cause signs or symptoms. Sometimes it forms before birth and is found during a fetal ultrasound.

By the time neuroblastoma is diagnosed, the cancer has usually metastasized (spread). Neuroblastoma spreads most often to the lymph nodes, bones, bone marrow, and liver. In infants, it also spreads to the skin.

Neuroblastoma is sometimes caused by a gene mutation (change) passed from the parent to the child.

Gene mutations that increase the risk of neuroblastoma are sometimes inherited (passed from the parent to the child). In children with a gene mutation, neuroblastoma usually occurs at a younger age and more than one tumor may form in the adrenal glands.

Signs and symptoms of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.

The most common signs and symptoms of neuroblastoma are caused by the tumor pressing on nearby tissues as it grows or by cancer spreading to the bone. These and other signs and symptoms may be caused by neuroblastoma or by other conditions.

Check with your child’s doctor if your child has any of the following:

Lump in the abdomen, neck, or chest.
Bulging eyes.
Dark circles around the eyes ("black eyes").
Bone pain.
Swollen stomach and trouble breathing (in infants).
Painless, bluish lumps under the skin (in infants).
Weakness or paralysis (loss of ability to move a body part).

Less common signs and symptoms of neuroblastoma include the following:

Fever.
Shortness of breath.
Feeling tired.
Easy bruising or bleeding.
Petechiae (flat, pinpoint spots under the skin caused by bleeding).
High blood pressure.
Severe watery diarrhea.
Horner syndrome (droopy eyelid, smaller pupil, and less sweating on one side of the face).
Jerky muscle movements.
Uncontrolled eye movements.

Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma.

The following tests and procedures may be used:

Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
Urine catecholamine studies: A procedure in which a urine sample is checked to measure the amount of certain substances, vanillylmandelic acid (VMA) and homovanillic acid (HVA), that are made when catecholamines break down and are released into the urine. A higher than normal amount of VMA or HVA can be a sign of neuroblastoma.
Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
Hormone test: A procedure in which a blood sample is checked to measure the amount of hormones released into the blood by the adrenal glands. A higher than normal amount of the hormones dopamine and norepinephrine may be a sign of neuroblastoma.
X-ray : An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
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Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
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Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.
mIBG (metaiodobenzylguanidine) scan : A procedure used to find neuroendocrine tumors, such as neuroblastoma. A very small amount of a substance called radioactivemIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive mIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the mIBG. This test is also used to find out how well the tumor is responding to treatment. mIBG is used in high doses to treat neuroblastoma.
Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
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Bone marrow aspiration and biopsy. After a small area of skin is numbed, a bone marrow needle is inserted into the child’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
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Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

A biopsy is done to diagnose neuroblastoma.

Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. The way the biopsy is done depends on where the tumor is in the body. Sometimes the whole tumor is removed at the same time the biopsy is done.

The following tests may be done on the tissue that is removed:

Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
Light and electron microscopy : A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.
MYCN amplification study : A laboratory study in which tumor or bone marrow cells are checked for the level of MYCN. MYCN is important for cell growth. A higher level of MYCN (more than 10 copies of the gene) is called MYCN amplification. Neuroblastoma with MYCN amplification is more likely to spread in the body and less likely to respond to treatment.

Children up to 6 months old may not need a biopsy or surgery to remove the tumor because the tumor may disappear without treatment.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

Age of the child at the time of diagnosis.
The child's risk group.
Whether there are certain changes in the genes.
Where in the body the tumor started.
Tumor histology (the shape, function, and structure of the tumor cells).
Whether there is cancer in the lymph nodes on the same side of the body as the primary cancer or whether there is cancer in the lymph nodes on the opposite side of the body.
How the tumor responds to treatment.
How much time passed between diagnosis and when the cancer recurred (for recurrent cancer).

Prognosis and treatment options for neuroblastoma are also affected by tumor biology, which includes:

The patterns of the tumor cells.
How different the tumor cells are from normal cells.
How fast the tumor cells are growing.
Whether the tumor shows MYCN amplification.
Whether the tumor has changes in the ALK gene.

The tumor biology is said to be favorable or unfavorable, depending on these factors. A favorable tumor biology means there is a better chance of recovery.

In some children up to 6 months old, neuroblastoma may disappear without treatment. This is called spontaneous regression. The child is closely watched for signs or symptoms of neuroblastoma. If signs or symptoms occur, treatment may be needed.

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Stages of Neuroblastoma