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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:
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 (teenagers). There are two main types of childhood liver cancer:
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 vessel that occurs in the liver and other organs. (See the Hemangioendothelioma section in the PDQ summary on Childhood Soft Tissue Sarcoma Treatment for more information.)
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. (See the PDQ summary on Adult Primary Liver Cancer Treatment 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:
Risk factors for hepatocellular carcinoma include the following:
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:
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:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
Prognosis may also 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 or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.
The following tests and procedures may be used in the staging process:
There are 2 staging systems for childhood liver cancer.
Two staging systems are used for childhood liver cancer:
The following stages are used before surgery:
The liver is divided into 4 vertical sections.
PRETEXT Stage 1
PRETEXT Stage 1. 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 stage 1, 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 Stage 2
PRETEXT Stage 2. 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 stage 2, 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 Stage 3
PRETEXT Stage 3. 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 stage 3, one of the following is true:
PRETEXT Stage 4
PRETEXT Stage 4. Cancer is found in all four sections of the liver.
In stage 4, cancer is found in all four sections of the liver.
The following stages are used after surgery:
In stage I, the tumor was in the liver only and all of the cancer was removed by surgery.
In stage II, the tumor was in the liver only and all of the cancer that can be seen without a microscope was removed by surgery. A small amount of cancer remains in the liver, but it can be seen only with a microscope, or the tumor cells may have spilled into the abdomen before surgery or during surgery.
In stage III:
In stage IV, the cancer has spread to other parts of the body.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone 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.
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.
Because cancer in children is rare, taking part in a clinical trial should be considered. 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. 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.
Some of the 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 symptoms appear or change.
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).
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.
Treatment using more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated.
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, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Percutaneous ethanol injection
Percutaneous ethanol injection is a cancer treatment in which a small needle is used to inject ethanol (alcohol) directly into a tumor to kill cancer cells.
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 Web site.
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. An angiogenesis inhibitor is a type of targeted therapy being studied in the treatment of children with liver cancer. Angiogenesis inhibitors are substances that block the growth of new blood vessels. In cancer treatment, angiogenesis inhibitors prevent the growth of new blood vessels needed for tumors to grow.
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 stage of the cancer 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. This is sometimes called re-staging.
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.
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Treatment of stages I and II hepatoblastoma may include the following:
Treatment of stage III hepatoblastoma may include the following:
Treatment of stage IV hepatoblastoma may include the following:
Treatment of stages I and II hepatocellular carcinoma may include the following:
Treatment of stage III hepatocellular carcinoma and PRETEXT stage 4 hepatocellular carcinoma may include the following:
Treatment of postsurgically-staged stage IV hepatocellular carcinoma may be combination chemotherapy to shrink the tumor, followed by surgery to remove as much of the tumor as possible.
Undifferentiated Embryonal Sarcoma of the Liver
Treatment of undifferentiated embryonal sarcoma of the liver may include the following:
Infantile Choriocarcinoma of the Liver
Treatment of infantile choriocarcinoma may be combination chemotherapy to shrink the tumor, followed by surgery to remove as much of the tumor as possible.
Recurrent Childhood Liver Cancer
Treatment of recurrent hepatoblastoma may include the following:
Treatment of recurrent hepatocellular carcinoma may include the following:
Treatment Options in Clinical Trials
Check for U.S. clinical trials from NCI's list of 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. General information about clinical trials is available from the NCI Web site.
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:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary
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PDQ also contains information on clinical trials.
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." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
Last Revised: 2013-03-15
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