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This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.
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:
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:
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:
The treatment of two less common types of childhood liver cancer is also discussed in this summary:
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 Soft Tissue Sarcoma 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:
Risk factors for hepatocellular carcinoma include the following syndromes or conditions:
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:
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:
The following test may be done on the sample of tissue that is removed:
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 prognosis (chance of recovery) and treatment options for hepatocellular carcinoma depend on the following:
For childhood liver cancer that recurs (comes back) after initial treatment, the prognosis and treatment options depend on:
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:
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:
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:
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.
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.
Recurrent childhood liver cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the liver or in other parts of the body. Cancer that is growing or worsening during treatment is progressive disease.
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:
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:
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:
When possible, the cancer is removed by surgery.
Factors that affect the type of surgery used include the following:
Chemotherapy is sometimes given before surgery, to shrink the tumor and make it easier to remove. This is called neoadjuvant therapy.
Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
Watchful waiting is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. In hepatoblastoma, this treatment is only used for small tumors that have been completely removed by surgery.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Treatment using more than one anticancer drug is called combination chemotherapy.
Chemoembolization of the hepatic artery (the main artery that supplies blood to the liver) is a type of regional chemotherapy used to treat childhood liver cancer. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with a substance that blocks the artery, cutting off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. This procedure is also called transarterial chemoembolization or TACE.
The way the chemotherapy is given depends on the type of the cancer being treated and the PRETEXT or POSTTEXT group.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, beads, seeds, wires, or catheters that are placed directly into or near the cancer.
Radioembolization of the hepatic artery (the main artery that supplies blood to the liver) is a type of internal radiation therapy used to treat hepatocellular carcinoma. A very small amount of a radioactive substance is attached to tiny beads that are injected into the hepatic artery through a catheter (thin tube). The beads are mixed with a substance that blocks the artery, cutting off blood flow to the tumor. Most of the radiation is trapped near the tumor to kill the cancer cells. This is done to relieve symptoms and improve quality of life for children with hepatocellular carcinoma.
The way the radiation therapy is given depends on the type of the cancer being treated and the PRETEXT or POSTTEXT group.
Hepatocellular carcinoma that is linked to the hepatitis B virus may be treated with antiviral drugs.
New types of treatment are being tested in clinical trials.
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.
Treatment options for hepatoblastoma that can be removed by surgery at the time of diagnosis may include the following:
Treatment options for hepatoblastoma that cannot be removed by surgery or is not removed at the time of diagnosis may include the following:
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:
Treatment options in clinical trials for newly diagnosed hepatoblastoma include:
Treatment options for hepatocellular carcinoma that can be removed by surgery at the time of diagnosis may include the following:
Treatment options for hepatocellular carcinoma that cannot be removed by surgery at the time of diagnosis may include the following:
Treatment for hepatocellular carcinoma that has spread to other parts of the body at the time of diagnosis may include:
Treatment options for hepatocellular carcinoma related to hepatitis B virus (HBV) infection include:
Undifferentiated Embryonal Sarcoma of the Liver
Treatment options for undifferentiated embryonal sarcoma of the liver (UESL) may include the following:
Infantile Choriocarcinoma of the Liver
Treatment options for choriocarcinoma of the liver in infants may include the following:
Recurrent Childhood Liver Cancer
Treatment of recurrent hepatoblastoma may include the following:
Treatment of progressive or recurrent hepatocellular carcinoma may include the following:
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.
For more information from the National Cancer Institute about childhood liver cancer, see the following:
For more childhood cancer information and other general cancer resources, see the following:
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government's center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
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.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials are listed in PDQ and can be found online at NCI's website. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).
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The best way to cite this PDQ summary is:
National Cancer Institute: PDQ® Childhood Liver Cancer Treatment. Bethesda, MD: National Cancer Institute. Date last modified <MM/DD/YYYY>. Available at: http://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq. Accessed <MM/DD/YYYY>.
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Last Revised: 2015-12-02
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