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. 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:

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  The number of red blood cells, white blood cells, and platelets.

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  The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

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  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:

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  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.

After childhood liver cancer has been diagnosed, tests are done to find out if cancer cells have spread within the liver or to other parts of the body.

The process used to find out if cancer has spread within the liver, to nearby tissues or organs, or to other parts of the body is called staging. In childhood liver cancer, the PRETEXT and POSTTEXT groups are used instead of stage to plan treatment. The results of the tests and procedures done to detect, diagnose, and find out whether the cancer has spread are used to determine the PRETEXT and POSTTEXT groups.

There are two grouping systems for childhood liver cancer.

Two grouping systems are used for childhood liver cancer:

• The PRETEXT group describes the tumor before the patient has treatment.
• The POSTTEXT group describes the tumor after the patient has treatment.

There are four PRETEXT and POSTTEXT groups:

The liver is divided into 4 sections. The PRETEXT and POSTTEXT groups depend on which sections of the liver have cancer.

PRETEXT and POSTTEXT Group I

Liver PRETEXT I. Cancer is found in one section of the liver. Three sections of the liver that are next to each other do not have cancer in them.

In group I, the cancer is found in one section of the liver. Three sections of the liver that are next to each other do not have cancer in them.

PRETEXT and POSTTEXT Group II

Liver PRETEXT II. Cancer is found in one or two sections of the liver. Two sections of the liver that are next to each other do not have cancer in them.

In group II, cancer is found in one or two sections of the liver. Two sections of the liver that are next to each other do not have cancer in them.

PRETEXT and POSTTEXT Group III

Liver PRETEXT III. Cancer is found in three sections of the liver and one section does not have cancer, or cancer is found in two sections of the liver and two sections that are not next to each other do not have cancer in them.

In group III, one of the following is true:

• Cancer is found in three sections of the liver and one section does not have cancer.
• Cancer is found in two sections of the liver and two sections that are not next to each other do not have cancer in them.

PRETEXT and POSTTEXT Group IV

Liver PRETEXT IV. Cancer is found in all four sections of the liver.

In group IV, cancer is found in all four sections of the liver.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if childhood liver cancer spreads to the lung, the cancer cells in the lung are actually liver cancer cells. The disease is metastatic liver cancer, not lung cancer.

There are different types of treatment for patients with childhood liver cancer.

Different types of treatments are available for children with liver cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Taking part in a clinical trial should be considered for all children with liver cancer. Some clinical trials are open only to patients who have not started treatment.

Children with liver cancer should have their treatment planned by a team of healthcare providers who are experts in treating this rare childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with liver cancer and who specialize in certain areas of medicine. It is especially important to have a pediatric surgeon with experience in liver surgery who can send patients to a liver transplant program if needed. Other specialists may include the following:

• Pediatrician.
• Radiation oncologist.
• Pediatric nurse specialist.
• Rehabilitation specialist.
• Psychologist.
• Social worker.

Some cancer treatments cause side effects months or years after treatment has ended.

Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

• Physical problems.
• Changes in mood, feelings, thinking, learning, or memory.
• Second cancers (new types of cancer).

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).

Five types of standard treatment are used:

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the treatment group may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Hepatoblastoma

Treatment options for hepatoblastoma that can be removed by surgery at the time of diagnosis may include the following:

• Surgery to remove the tumor, followed by watchful waiting or chemotherapy, for hepatoblastoma with pure fetal histology.
• Surgery to remove the tumor, with combination chemotherapy given either before surgery, after surgery, or both, for hepatoblastoma that is not pure fetal histology. For hepatoblastoma with small cell undifferentiated histology, aggressive chemotherapy is given.

Treatment options for hepatoblastoma that cannot be removed by surgery or is not removed at the time of diagnosis may include the following:

• Combination chemotherapy to shrink the tumor, followed by surgery to remove the tumor.
• Combination chemotherapy followed by a liver transplant.
• Chemoembolization of the hepatic artery to shrink the tumor, followed by surgery to remove the tumor.

For hepatoblastoma that has spread to other parts of the body at the time of diagnosis, combination chemotherapy is given to shrink the cancer in the liver and cancer that has spread to other parts of the body. After chemotherapy, imaging tests are done to check whether the cancer can be removed by surgery.

Treatment options may include the following:

• If the cancer in the liver and other parts of the body can be removed, surgery will be done to remove the tumors followed by chemotherapy to kill any cancer cells that may remain.
• If the cancer in the liver cannot be removed by surgery but there are no signs of cancer in other parts of the body, the treatment may be a liver transplant.
• If the cancer in other parts of the body cannot be removed or a liver transplant is not possible, chemotherapy, chemoembolization of the hepatic artery, or radiation therapy may be given.

Treatment options in clinical trials for newly diagnosed hepatoblastoma include:

• A clinical trial of new treatment regimens based on how likely it is the cancer will recur after initial treatment.

Hepatocellular Carcinoma

Treatment options for hepatocellular carcinoma that can be removed by surgery at the time of diagnosis may include the following:

• Surgery alone to remove the tumor.
• Surgery to remove the tumor, followed by chemotherapy.
• Combination chemotherapy followed by surgery to remove the tumor.

Treatment options for hepatocellular carcinoma that cannot be removed by surgery at the time of diagnosis may include the following:

• Chemotherapy to shrink the tumor, followed by surgery to completely remove the tumor.
• Chemotherapy to shrink the tumor. If surgery to completely remove the tumor is not possible, further treatment may include the following:

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  Liver transplant.

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  Chemoembolization of the hepatic artery to shrink the tumor, followed by surgery to remove as much of the tumor as possible or liver transplant.

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  Chemoembolization of the hepatic artery alone.

• Radioembolization of the hepatic artery as palliative therapy to relieve symptoms and improve the quality of life.

Treatment for hepatocellular carcinoma that has spread to other parts of the body at the time of diagnosis may include:

• Combination chemotherapy to shrink the tumor, followed by surgery to remove as much of the tumor as possible from the liver and other places where cancer has spread. Studies have not shown that this treatment works well but some patients may have some benefit.

Treatment options for hepatocellular carcinoma related to hepatitis B virus (HBV) infection include:

• Surgery to remove the tumor.
• Antiviral drugs that treat infection caused by the hepatitis B virus.

Undifferentiated Embryonal Sarcoma of the Liver

Treatment options for undifferentiated embryonal sarcoma of the liver (UESL) may include the following:

• Combination chemotherapy to shrink the tumor, followed by surgery to remove as much of the tumor as possible. Chemotherapy may also be given after surgery to remove the tumor.
• Surgery to remove the tumor followed by chemotherapy. A second surgery may be done to remove tumor that remains, followed by more chemotherapy.
• Liver transplant if surgery to remove the tumor is not possible.
• A clinical trial of a combination of targeted therapy, chemotherapy and/or radiation therapy before surgery.

Infantile Choriocarcinoma of the Liver

Treatment options for choriocarcinoma of the liver in infants may include the following:

• Combination chemotherapy to shrink the tumor, followed by surgery to remove the tumor.
• Surgery to remove the tumor.

Recurrent Childhood Liver Cancer

Treatment of recurrent hepatoblastoma may include the following:

• Surgery to remove isolated (single and separate) metastatic tumors with or without chemotherapy.
• Combination chemotherapy.
• Liver transplant.
• A clinical trial of a new treatment.

Treatment of progressive or recurrent hepatocellular carcinoma may include the following:

• Chemoembolization of the hepatic artery to shrink the tumor before liver transplant.
• Liver transplant.
• A clinical trial of a new treatment.

Treatment Options in Clinical Trials

Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood liver cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

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This PDQ cancer information summary has current information about the treatment of childhood liver cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Liver Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389318]

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