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Neuroblastoma Treatment (PDQ®)

Patient Version
Last Modified: 04/12/2012

General Information About Neuroblastoma

Key Points for This Section


Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of 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 the abdomen, in the chest, in nerve tissue near the spine in the neck, or in the spinal cord.

Enlarge 1
Anatomy of the female urinary system; drawing shows a front view of the right and left kidneys, the ureters,  urethra, and bladder filled with urine. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. The spine, adrenal glands, and uterus are also shown. 1
Anatomy of the female urinary system showing the kidneys, adrenal glands, ureters, bladder, and urethra. Urine is made in the renal tubules and collects in the renal pelvis of each kidney. The urine flows from the kidneys through the ureters to the bladder. The urine is stored in the bladder until it leaves the body through the urethra.


Neuroblastoma most often begins during early childhood, usually in children younger than 5 years. It sometimes forms before birth but is usually found later, when the tumor begins to grow and cause symptoms. In rare cases, neuroblastoma may be found before birth by fetal ultrasound.

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

(See the PDQ summary on Neuroblastoma Screening 2 for more information.)

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

Neuroblastoma is sometimes inherited (passed from the parent to the child). It usually occurs at a younger age than neuroblastoma that is not inherited. There also may be more than one tumor in the adrenal medulla in inherited neuroblastoma.

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

The most common 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 symptoms may be caused by neuroblastoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • 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 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.
  • Jerky muscle movements.
  • Uncontrolled eye movement.
  • Swelling of the legs, ankles, feet, or scrotum.

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.
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of the substances homovanillic acid (HMA) and vanillyl mandelic acid (VMA) may 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 in the organ or tissue that makes it. A higher than normal amount of the hormones dopamine and norepinephrine may be a sign of neuroblastoma.
  • 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.
  • 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.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
  • 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.
  • 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.
  • 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.
  • Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
  • MIBG (metaiodobenzylguanidine) scan: A procedure used to find neuroendocrine tumors, such as neuroblastomas and pheochromocytomas. A small amount of radioactive material called MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive material 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 prevent the thyroid gland from absorbing too much of the MIBG.

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 when diagnosed.
  • Stage of the cancer.
  • Where the tumor is in the body.
  • Tumor histology (the shape, function, and structure of the tumor cells).
  • Whether there is cancer in the lymph nodes.
  • Whether there are certain changes in the chromosomes.
  • How much time passed between diagnosis and when the cancer recurred.

Prognosis and treatment decisions 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.

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 infants, neuroblastoma may disappear without treatment. The infant is closely watched for symptoms of neuroblastoma. If symptoms occur, treatment may be needed.



Glossary Terms

abdomen (AB-doh-men)
The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
adrenal gland (uh-DREE-nul...)
A small gland that makes steroid hormones, adrenaline, and noradrenaline. These hormones help control heart rate, blood pressure, and other important body functions. There are two adrenal glands, one on top of each kidney. Also called suprarenal gland.
adrenal medulla (uh-DREE-nul meh-DOO-luh)
The inner part of the adrenal gland (a small organ on top of each kidney). The adrenal medulla makes chemicals such as epinephrine (adrenaline) and norepinephrine (noradrenaline) which are involved in sending nerve signals.
antibody (AN-tee-BAH-dee)
A protein made by plasma cells (a type of white blood cell) in response to an antigen (a substance that causes the body to make a specific immune response). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.
antigen (AN-tih-jen)
Any substance that causes the body to make a specific immune response.
biopsy (BY-op-see)
The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
blood (blud)
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
blood chemistry study (blud KEH-mih-stree STUH-dee)
A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.
blood pressure (blud PREH-sher)
The force of circulating blood on the walls of the arteries. Blood pressure is taken using two measurements: systolic (measured when the heart beats, when blood pressure is at its highest) and diastolic (measured between heart beats, when blood pressure is at its lowest). Blood pressure is written with the systolic blood pressure first, followed by the diastolic blood pressure (for example 120/80).
blood sugar (blud SHUH-ger)
Glucose (a type of sugar) found in the blood. Also called glycemia.
bone marrow (bone MAYR-oh)
The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.
bone marrow aspiration (bone MAYR-oh AS-pih-RAY-shun)
A procedure in which a small sample of bone marrow is removed, usually from the hip bone, breastbone, or thigh bone. A small area of skin and the surface of the bone underneath are numbed with an anesthetic. Then, a special wide needle is pushed into the bone. A sample of liquid bone marrow is removed with a syringe attached to the needle. The bone marrow is sent to a laboratory to be looked at under a microscope. This procedure may be done at the same time as a bone marrow biopsy.
bone marrow biopsy (bone MAYR-oh BY-op-see)
A procedure in which a small sample of bone with bone marrow inside it is removed, usually from the hip bone. A small area of skin and the surface of the bone underneath are numbed with an anesthetic. Then, a special, wide needle is pushed into the bone and rotated to remove a sample of bone with the bone marrow inside it. The sample is sent to a laboratory to be looked at under a microscope. This procedure may be done at the same time as a bone marrow aspiration.
breastbone (brest-bone)
The long flat bone that forms the center front of the chest wall. The breastbone is attached to the collarbone and the first seven ribs. Also called sternum.
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
chromosome (KROH-muh-some)
Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.
condition (kun-DIH-shun)
In medicine, a health problem with certain characteristics or symptoms.
contrast material (KON-trast muh-TEER-ee-ul)
A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.
CT scan (… skan)
A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
cytogenetics (SY-toh-jeh-NEH-tix)
The study of chromosomes and chromosomal abnormalities.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
diarrhea (dy-uh-REE-uh)
Frequent and watery bowel movements.
fetus (FEE-tus)
In humans, an unborn baby that develops and grows inside the uterus (womb). The fetal period begins 8 weeks after fertilization of an egg by a sperm and ends at the time of birth.
fever (FEE-ver)
An increase in body temperature above normal (98.6 degrees F), usually caused by disease.
high blood pressure (hy blud PREH-sher)
A blood pressure of 140/90 or higher. High blood pressure usually has no symptoms. It can harm the arteries and cause an increase in the risk of stroke, heart attack, kidney failure, and blindness. Also called hypertension.
histology (his-TAH-loh-jee)
The study of tissues and cells under a microscope.
hormone (HOR-mone)
One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.
immunohistochemistry (IH-myoo-noh-HIS-toh-KEH-mih-stree)
A technique used to identify specific molecules in different kinds of tissue. The tissue is treated with antibodies that bind the specific molecule. These are made visible under a microscope by using a color reaction, a radioisotope, colloidal gold, or a fluorescent dye. Immunohistochemistry is used to help diagnose diseases, such as cancer, and to detect the presence of microorganisms. It is also used in basic research to understand how cells grow and differentiate (become more specialized).
inherited (in-HAYR-ih-ted)
Transmitted through genes that have been passed from parents to their offspring (children).
injection (in-JEK-shun)
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
iodine (I-oh-dine)
An element that is necessary for the body to make thyroid hormone. It is found in shellfish and iodized salt.
kidney (KID-nee)
One of a pair of organs in the abdomen. Kidneys remove waste from the blood (as urine), produce erythropoietin (a substance that stimulates red blood cell production), and play a role in blood pressure regulation.
laboratory test (LA-bruh-tor-ee...)
A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.
liver (LIH-ver)
A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.
lymph node (limf node)
A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.
medical history (MEH-dih-kul HIH-stuh-ree)
A record of information about a person’s health. A personal medical history may include information about allergies, illnesses, surgeries, immunizations, and results of physical exams and tests. It may also include information about medicines taken and health habits, such as diet and exercise. A family medical history includes health information about a person’s close family members (parents, grandparents, children, brothers, and sisters). This includes their current and past illnesses. A family medical history may show a pattern of certain diseases in a family.
metastasize (meh-TAS-tuh-size)
To spread from one part of the body to another. When cancer cells metastasize and form secondary tumors, the cells in the metastatic tumor are like those in the original (primary) tumor.
microscope (MY-kroh-SKOPE)
An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.
nerve (nerv)
A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.
neuroblastoma (NOOR-oh-blas-TOH-muh)
Cancer that arises in immature nerve cells and affects mostly infants and children.
neuroendocrine tumor (NOOR-oh-EN-doh-krin TOO-mer)
A tumor that forms from cells that release hormones in response to a signal from the nervous system. Some examples of neuroendocrine tumors are carcinoid tumors, islet cell tumors, medullary thyroid carcinomas, pheochromocytomas, and neuroendocrine carcinomas of the skin (Merkel cell cancer). These tumors may secrete higher-than-normal amounts of hormones, which can cause many different symptoms.
neurological exam (NOOR-oh-LAH-jih-kul eg-ZAM)
A series of questions and tests to check brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk, and how well the muscles, sensory systems, and deep tendon reflexes work.
norepinephrine (NOR-eh-pih-NEH-frin)
A chemical made by some nerve cells and in the adrenal gland. It can act as both a neurotransmitter (a chemical messenger used by nerve cells) and a hormone (a chemical that travels in the blood and controls the actions of other cells or organs). Norepinephrine is released from the adrenal gland in response to stress and low blood pressure. Also called noradrenaline.
organ (OR-gun)
A part of the body that performs a specific function. For example, the heart is an organ.
paralysis (puh-RA-lih-sis)
Loss of ability to move all or part of the body.
pathologist (puh-THAH-loh-jist)
A doctor who identifies diseases by studying cells and tissues under a microscope.
PDQ
PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.
petechiae (peh-TEE-kee-ee)
Pinpoint, unraised, round red spots under the skin caused by bleeding.
pheochromocytoma (FEE-oh-KROH-moh-sy-TOH-muh)
Tumor that forms in the center of the adrenal gland (gland located above the kidney) that causes it to make too much adrenaline. Pheochromocytomas are usually benign (not cancer) but can cause high blood pressure, pounding headaches, heart palpitations, flushing of the face, nausea, and vomiting.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
radioactive (RAY-dee-oh-AK-tiv)
Giving off radiation.
radioisotope (RAY-dee-oh-I-suh-tope)
An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.
recover (ree-KUH-ver)
To become well and healthy again.
recur (ree-KER)
To come back or to return.
scan (skan)
A picture of structures inside the body. Scans often used in diagnosing, staging, and monitoring disease include liver scans, bone scans, and computed tomography (CT) or computerized axial tomography (CAT) scans and magnetic resonance imaging (MRI) scans. In liver scanning and bone scanning, radioactive substances that are injected into the bloodstream collect in these organs. A scanner that detects the radiation is used to create pictures. In CT scanning, an x-ray machine linked to a computer is used to produce detailed pictures of organs inside the body. MRI scans use a large magnet connected to a computer to create pictures of areas inside the body.
scanner (SKA-ner)
In medicine, an instrument that takes pictures of the inside of the body.
scrotum (SKROH-tum)
In males, the external sac that contains the testicles.
sonogram (SAH-noh-gram)
A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called ultrasonogram.
spinal cord (SPY-nul kord)
A column of nerve tissue that runs from the base of the skull down the back. It is surrounded by three protective membranes, and is enclosed within the vertebrae (back bones). The spinal cord and the brain make up the central nervous system, and spinal cord nerves carry most messages between the brain and the rest of the body.
spine (spine)
The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spine encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spinal column, and vertebral column.
stage (stayj)
The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.
stomach (STUH-muk)
An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.
stress (stres)
The response of the body to physical, mental, or emotional pressure. This may make a person feel frustrated, angry, or anxious, and may cause unhealthy chemical changes in the body. Untreated, long-term stress may lead to many types of mental and physical health problems.
symptom (SIMP-tum)
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
thyroid gland (THY-royd...)
A gland located beneath the larynx (voice box) that makes thyroid hormone and calcitonin. The thyroid gland helps regulate growth and metabolism. Also called thyroid.
tissue (TIH-shoo)
A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer)
An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.
ultrasound (UL-truh-SOWND)
A procedure in which high-energy sound waves are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.
urine (YOOR-in)
Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
x-ray (EX-ray)
A type of radiation used in the diagnosis and treatment of cancer and other diseases. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.

Table of Links

1http://www.cancer.gov/PublishedContent/MediaLinks/291550.html
2http://www.cancer.gov/cancertopics/pdq/screening/neuroblastoma/Patient