Key Points for This Section

Childhood liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. Certain diseases and disorders can increase the risk of developing childhood liver cancer. Possible signs of childhood liver cancer include a lump or pain in the abdomen. Tests that examine the liver and the blood are used to detect (find) and diagnose childhood liver cancer. Certain factors affect prognosis (chance of recovery) and treatment options.

Childhood liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver.

The liver is one of the largest organs in the body. It has four lobes and fills the upper right side of the abdomen inside the rib cage. The liver has many important functions, including:

• Filtering harmful substances from the blood so they can be passed from the body in stools and urine.
• Making bile to help digest fats from food.
• Storing glycogen (sugar), which the body uses for energy.

There are two main types of childhood liver cancer:

• Hepatoblastoma: A type of liver cancer that usually does not spread outside the liver. This type usually affects children younger than 3 years old.
• Hepatocellular carcinoma: A type of liver cancer that often spreads to other places in the body. This type can affect children of any age.

This summary refers to the treatment of primary liver cancer (cancer that begins in the liver). Treatment of metastatic liver cancer, which is cancer that begins in other parts of the body and spreads to the liver, is not discussed in this summary. Primary liver cancer can occur in both adults and children. Treatment for children, however, is different than treatment for adults. (Refer to the PDQ summary on Adult Primary Liver Cancer Treatment ¹ for more information.)

Certain diseases and disorders can increase the risk of developing childhood liver cancer.

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for hepatoblastoma include the following:

• Being male.
• Having familial adenomatous polyposis (FAP).
• Having Beckwith-Wiedemann syndrome.
• Having had a very low weight at birth.

Risk factors for hepatocellular carcinoma include the following:

• Being male.
• Having hepatitis B or hepatitis C. The risk is greatest when the virus is passed from mother to child at birth.
• Having liver damage caused by certain diseases, such as biliary cirrhosis or tyrosinemia.

Possible signs of childhood liver cancer include a lump or pain in the abdomen.

Symptoms are more common after the tumor becomes large. Other conditions can cause the same symptoms. A doctor should be consulted if any of the following problems occur:

• A painless lump in the abdomen.
• Swelling or pain in the abdomen.
• Weight loss for no known reason.
• Loss of appetite.
• Early puberty in boys.
• Nausea and vomiting.

Tests that examine the liver and the blood are used to detect (find) and diagnose childhood liver cancer.

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.
• Serum tumor marker test: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The blood of children who have liver cancer may have increased amounts of a protein called alpha-fetoprotein (AFP) or a hormone called beta-human chorionic gonadotropin (β-hCG). Other cancers and certain noncancerous conditions, including cirrhosis and hepatitis, may also increase AFP levels.
• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • The number of red blood cells, white blood cells, and platelets.
  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  • The portion of the blood sample made up of red blood cells.
• Liver function tests: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver cancer.
• Abdominal x-ray: An x-ray of the organs in the abdomen. An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body.
• 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.
• 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. In childhood liver cancer, a CT scan of the chest and abdomen is usually done.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. The sample may be taken during surgery to remove or view the tumor. A pathologist views the sample under a microscope to determine the type of liver cancer.

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

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

• The stage of the cancer (the size of the tumor, whether it affects part or all of the liver, and whether it has spread to other places in the body, such as the lungs).
• Whether the cancer can be removed completely by surgery.
• The type of liver cancer (hepatoblastoma or hepatocellular).
• Whether the cancer has just been diagnosed or has recurred.

Prognosis may also depend on:

• Certain features of the cancer cell (how it looks under a microscope).
• Whether the AFP blood levels go down after chemotherapy begins.

Childhood liver cancer may be cured if the tumor is small and can be completely removed by surgery. Complete removal is possible more often for hepatoblastoma than for hepatocellular carcinoma.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Having to do with the abdomen, which is the part of the body between the chest and the hips that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

AFP. A protein normally produced by a fetus. AFP levels are usually undetectable in the blood of healthy adult men or women (who are not pregnant). An elevated level of AFP suggests the presence of either a primary liver cancer or germ cell tumor. Also called AFP.

A rare, overgrowth disorder in which babies are large at birth and may develop low blood sugar. Other common symptoms are a large tongue, large internal organs, and defects of the abdominal wall near the navel. Beckwith-Wiedemann syndrome increases the risk of developing certain cancers, especially Wilms tumor.

A hormone normally found in the blood and urine during pregnancy. It may also be produced by some tumor cells. An increased level of beta-human chorionic gonadotropin may be a sign of cancer of the testis, uterus, ovary, liver, stomach, pancreas, or lung. Beta-human chorionic gonadotropin may also be produced in response to certain conditions that are not cancer. Beta-human chorionic gonadotropin is being studied in the treatment of Kaposi sarcoma. Also called ß-hCG.

A fluid made by the liver and stored in the gallbladder. Bile is excreted into the small intestine, where it helps digest fat.

Having to do with the liver, bile ducts, and/or gallbladder.

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.

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.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is 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.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

A type of chronic, progressive liver disease in which liver cells are replaced by scar tissue.

CBC. A test to check the number of red blood cells, white blood cells, and platelets in a sample of blood. Also called blood cell count and CBC.

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.

Computed tomography scan. 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 computed tomography scan, computerized tomography, computerized axial tomography scan, and CAT scan.

The process of identifying a disease, such as cancer, from its signs and symptoms.

An inherited condition in which numerous polyps (growths that protrude from mucous membranes) form on the inside walls of the colon and rectum. It increases the risk of colorectal cancer. Also called FAP and familial polyposis.

The substance inside red blood cells that binds to oxygen in the lungs and carries it to the tissues.

Disease of the liver causing inflammation. Symptoms include an enlarged liver, fever, nausea, vomiting, abdominal pain, and dark urine.

A virus that causes hepatitis (inflammation of the liver). It is carried and passed to others through blood or sexual contact. Also, infants born to infected mothers may become infected with the virus.

A virus that causes hepatitis (inflammation of the liver). It is carried and passed to others through blood or sexual contact. Also, infants born to infected mothers may become infected with the virus.

A type of liver tumor that occurs in infants and children.

A type of adenocarcinoma, the most common type of liver tumor.

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.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

Primary liver cancer is cancer that forms in the tissues of the liver. Secondary liver cancer is cancer that spreads to the liver from another part of the body.

A blood test to measure the blood levels of certain substances released by the liver. A high or low level of certain substances can be a sign of liver disease.

A portion of an organ, such as the liver, lung, breast, thyroid, or brain.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

Cancer that has spread from the place in which it started to other parts of the body.

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, nuclear magnetic resonance imaging, and NMRI.

A feeling of sickness or discomfort in the stomach that may come with an urge to vomit. Nausea is a side effect of some types of cancer therapy.

A part of the body that performs a specific function. For example, the heart is an organ.

A colorless, odorless gas. It is needed for animal and plant life. Oxygen that is breathed in enters the blood from the lungs and travels to the tissues.

A doctor who identifies diseases by studying cells and tissues under a microscope.

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.

An exam of the body to check for general signs of disease.

A type of blood cell that helps prevent bleeding by causing blood clots to form. Also called a thrombocyte.

The original tumor.

The likely outcome or course of a disease; the chance of recovery or recurrence.

A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.

To come back or to return.

RBC. A cell that carries oxygen to all parts of the body. Also called RBC and erythrocyte.

Something that may increase the chance of developing a disease. Some examples of risk factors for cancer include age, a family history of certain cancers, use of tobacco products, certain eating habits, obesity, lack of exercise, exposure to radiation or other cancer-causing agents, and certain genetic changes.

A blood test that measures the amount of substances called tumor markers (or biomarkers). Tumor markers are released into the blood by tumor cells or by other cells in response to tumor cells. A high level of a tumor marker may be a sign of cancer.

A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called an ultrasonogram.

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.

The material in a bowel movement. Stool is made up of food that was not digested, bacteria, mucus, and cells from the intestines. Also called feces.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A group or layer of cells that work together to perform a specific function.

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 cancerous), or malignant (cancerous). Also called neoplasm.

A substance that may be found in tumor tissue or released from a tumor into the blood or other body fluids. A high level of a tumor marker may mean that a certain type of cancer is in the body. Examples of tumor markers include CA 125 (in ovarian cancer), CA 15-3 (in breast cancer), CEA (in ovarian, lung, breast, pancreas, and gastrointestinal tract cancers), and PSA (in prostate cancer).

A procedure in which high-energy sound waves (ultrasound) 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.

Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.

A blood vessel that carries blood to the heart from tissues and organs in the body.

In medicine, a very simple microorganism that infects cells and may cause disease. Because viruses can multiply only inside infected cells, they are not considered to be alive.

To eject some or all of the contents of the stomach through the mouth.

Refers to a blood cell that does not contain hemoglobin. White blood cells include lymphocytes, neutrophils, eosinophils, macrophages, and mast cells. These cells are made by bone marrow and help the body fight infections and other diseases. Also called WBC.

A type of high-energy radiation. 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.