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General Information About Childhood Liver Cancer

Key Points

Childhood liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver.
There are different types of childhood liver cancer.
Certain diseases and disorders can increase the risk of childhood liver cancer.
Signs and symptoms 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 and find out whether the cancer has spread.
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. Three of the many important functions of the liver are:

To filter harmful substances from the blood so they can be passed from the body in stools and urine.
To make bile to help digest fats from food.
To store glycogen (sugar), which the body uses for energy.

Anatomy of the liver; drawing shows the right and left front lobes of the liver, bile ducts, gallbladder, stomach, spleen, pancreas, colon, and small intestine. The two back lobes of the liver are not shown. — Anatomy of the liver. The liver is in the upper abdomen near the stomach, intestines, gallbladder, and pancreas. The liver has four lobes. Two lobes are on the front and two small lobes (not shown) are on the back of the liver.

Liver cancer is rare in children and adolescents.

There are different types of childhood liver cancer.

There are two main types of childhood liver cancer :

Hepatoblastoma : Hepatoblastoma is the most common type of childhood liver cancer. It usually affects children younger than 3 years of age.
In hepatoblastoma, the histology (how the cancer cells look under a microscope) affects the way the cancer is treated. The histology for hepatoblastoma may be one of the following:
- Pure fetal histology.
- Small cell undifferentiated histology.
- Non-pure fetal histology, non-small cell undifferentiated histology.
Hepatocellular carcinoma : Hepatocellular carcinoma usually affects older children and adolescents. It is more common in areas of Asia that have high rates of hepatitis infection than in the U.S.

The treatment of two less common types of childhood liver cancer is also discussed in this summary:

Undifferentiated embryonal sarcoma of the liver: This type of liver cancer usually occurs in children between 5 and 10 years of age. It often spreads all through the liver and/or to the lungs.
Infantile choriocarcinoma of the liver is a very rare tumor that starts in the placenta and spreads to the fetus. The tumor is usually found during the first few months of life. Also, the mother of the child may be diagnosed with choriocarcinoma. Choriocarcinoma is a type of gestational trophoblastic disease and needs treatment. See the Gestational Trophoblastic Disease Treatment summary for information on the treatment of choriocarcinoma.

Epithelioid hemangioendothelioma is a rare cancer of the blood vessels that occurs in the liver and other organs. See the Epithelioid hemangioendothelioma section in the PDQ summary on Childhood Vascular Tumors Treatment for more information.

This summary is about 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. However, treatment for children is different than treatment for adults. See the PDQ summary on Adult Primary Liver Cancer Treatment for more information on the treatment of adults.

Certain diseases and disorders can increase the risk of 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. Talk with your child’s doctor if you think your child may be at risk.

Risk factors for hepatoblastoma include the following syndromes or conditions :

Aicardi syndrome.
Beckwith-Wiedemann syndrome.
Familial adenomatous polyposis (FAP).
Glycogen storage disease.
A very low weight at birth.
Simpson-Golabi-Behmel syndrome.
Certain genetic changes, such as Trisomy 18.

Risk factors for hepatocellular carcinoma include the following syndromes or conditions:

Alagille syndrome.
Glycogen storage disease.
Hepatitis B virus infection that was passed from mother to child at birth.
Progressive familial intrahepatic disease.
Tyrosinemia.

Some patients with tyrosinemia or progressive familial intrahepatic disease will have a liver transplant before there are signs or symptoms of cancer.

Signs and symptoms of childhood liver cancer include a lump or pain in the abdomen.

Signs and symptoms are more common after the tumor gets big. Other conditions can cause the same signs and symptoms. Check with your child’s doctor if your child has any of the following:

A lump in the abdomen that may be painful.
Swelling in the abdomen.
Weight loss for no known reason.
Loss of appetite.
Nausea and vomiting.

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

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 hormone called beta-human chorionic gonadotropin (β-hCG) or a protein called alpha-fetoprotein (AFP). Other cancers and certain noncancer conditions, including cirrhosis and hepatitis, can 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 damage or cancer.
Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as bilirubin or lactate dehydrogenase (LDH), 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.
Epstein-Barr virus (EBV) test: A blood test to check for antibodies to the EBV and DNA markers of the EBV. These are found in the blood of patients who have been infected with EBV.
Hepatitis assay: A procedure in which a blood sample is checked for pieces of the hepatitis virus.
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 liver. 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).
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.
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. In childhood liver cancer, an ultrasound exam of the abdomen to check the large blood vessels is usually done.
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).
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.
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.
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.
Biopsy: The removal of a sample of cells or tissues so it 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 looks at the sample under a microscope to find out the type of liver cancer.
The following test may be done on the sample of tissue that is removed:
- 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 is used to check for a certain gene mutation and to tell the difference between different types of cancer.

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

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

The PRETEXT or POSTTEXT group.
Whether the cancer has spread to other places in the body, such as the lungs or certain large blood vessels.
Whether the cancer can be removed completely by surgery.
How the cancer responds to chemotherapy.
How the cancer cells look under a microscope.
Whether the AFP blood levels go down after treatment.
Whether the cancer has just been diagnosed or has recurred.
Age of the child.

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

The PRETEXT or POSTTEXT group.
Whether the cancer has spread to other places in the body, such as the lungs.
Whether the cancer can be removed completely by surgery.
How the cancer responds to chemotherapy.
How the cancer cells look under a microscope.
Whether the child has hepatitis B infection.
Whether the cancer has just been diagnosed or has recurred.

For childhood liver cancer that recurs (comes back) after initial treatment, the prognosis and treatment options depend on:

Where in the body the tumor recurred.
The type of treatment used to treat the initial cancer.

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.

CancerHelp Online® is a patient education program of The CancerHelp Institute, official Content Distribution Partner of the National Cancer Institute.

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