Childhood Undifferentiated Embryonal Sarcoma of the Liver
Childhood undifferentiated embryonal sarcoma of the liver is a rare cancer type that usually forms in the tissues of the right lobe of the liver. This type of liver cancer usually occurs in children between 5 and 10 years but can also occur in adolescence. It often spreads throughout the liver and/or to the lungs.
The liver is one of the largest organs in the body. It has two lobes and fills the upper right side of the abdomen inside the rib cage. Three of the many important functions of the liver include the following:
- to make bile to help digest fats from food
- to store glycogen (sugar), which the body uses for energy
- to filter harmful substances from the blood so they can be passed from the body in stools and urine
Symptoms of childhood undifferentiated embryonal sarcoma of the liver
Children may not have symptoms of undifferentiated embryonal sarcoma of the liver until the tumor has grown bigger. It's important to check with your child’s doctor if your child has any symptoms below:
- a lump in the abdomen
- abdominal pain
- weight loss for no known reason
- loss of appetite
- fatigue or loss of energy
These symptoms may be caused by conditions other than undifferentiated embryonal sarcoma of the liver. The only way to know is to see your child’s doctor.
Tests to diagnose childhood undifferentiated embryonal sarcoma of the liver
If your child has symptoms that suggest undifferentiated embryonal sarcoma of the liver, their doctor will need to find out if these are due to cancer or to another condition. The doctor will ask you when the symptoms started and how often your child has been having them. They will also ask about your child's personal and family medical history and do a physical exam. Based on these results, the doctor may recommend diagnostic tests to find out if your child has undifferentiated embryonal sarcoma of the liver. If your child has liver cancer, the results of these tests will also help you and your child's doctor plan treatment.
The following tests and procedures may be used to diagnose undifferentiated embryonal sarcoma of the liver:
Complete blood count (CBC)
A sample of blood is drawn and checked for the following:
Liver function tests
Liver function tests 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
Blood chemistry studies 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.
Magnetic resonance imaging (MRI) with gadolinium
MRI uses a magnet, radio waves, and a computer to take 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. The procedure is also called nuclear MRI.
CT scan (CAT scan)
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, taken from different angles. 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.
To learn more, see Computed Tomography (CT) Scans and Cancer.
An ultrasound exam is 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. In childhood liver cancer, an ultrasound exam of the abdomen to check the large blood vessels is usually done.
A chest x-ray is an x-ray of the lungs. An x-ray is a type of high-energy radiation that can go through the body onto film, making a picture of areas inside the body.
Biopsy is a procedure in which a sample of tissue is removed from the tumor so that a pathologist can view it under a microscope to check for signs of cancer. The doctor may remove as much tumor as safely possible during the same biopsy procedure.
Immunohistochemistry uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
Getting a second opinion
You may want to get a second opinion to confirm your child's diagnosis and treatment plan. If you seek a second opinion, you will need to get important medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes or another approach, or provide more information about your child’s cancer.
To learn more about choosing a doctor and getting a second opinion, see Finding Health Care Services. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your appointments, see Questions to Ask Your Doctor.
Prognostic factors for childhood undifferentiated embryonal sarcoma of the liver
If your child has been diagnosed with undifferentiated embryonal sarcoma of the liver, you may have questions about how serious the cancer is and your child's chances of survival. The likely outcome or course of a disease is called prognosis.
The prognosis depends on the following:
- the size of the tumor
- how the cancer responds to chemotherapy
- whether the cancer can be removed completely by surgery
- whether your child can have a liver transplant
- whether the cancer has spread to other places in the body, such as the lungs
- whether the cancer has just been diagnosed or has come back after treatment
For childhood undifferentiated embryonal sarcoma of the liver that comes back after initial treatment, the prognosis depends on the following:
- where in the body the tumor came back
- the type of treatment used to treat the initial cancer
No two people are alike, and responses to treatment can vary greatly. Your child's cancer care team is in the best position to talk with you about your child's prognosis.
Stages of childhood undifferentiated embryonal sarcoma of the liver
Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. There is no staging system used for childhood undifferentiated embryonal sarcoma of the liver, but the tests and procedures done to diagnose the cancer are also used to help plan treatment.
Types of treatment for childhood undifferentiated embryonal sarcoma of the liver
There are different types of treatment for children and adolescents with undifferentiated embryonal sarcoma of the liver. You and your child’s care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the cancer is newly diagnosed or has come back.
A pediatric oncologist, a doctor who specializes in treating children with cancer, will oversee treatment. The pediatric oncologist works with other healthcare providers who are experts in treating children with undifferentiated embryonal sarcoma of the liver 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. This may include the following specialists and others:
Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s care team before treatment begins about what to expect. For help every step of the way, see our downloadable booklet, Children with Cancer: A Guide for Parents.
When possible, the cancer is removed by surgery. The following types of surgery may be done:
- Partial hepatectomy removes the part of the liver where cancer is found by surgery. The part removed may be a wedge of tissue, an entire lobe, or a larger part of the liver, along with some of the healthy tissue around it. The remaining liver tissue takes over the functions of the liver and may regrow.
- Liver transplant is the removal of the entire liver by surgery, followed by a transplant of a healthy liver from a donor. A liver transplant may be done when the cancer is in the liver only, and a donated liver can be found. If the patient has to wait for a donated liver, other treatment is given as needed.
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing.
For childhood undifferentiated embryonal sarcoma of the liver, the chemotherapy is injected into a vein. When given this way, the drugs enter the bloodstream and can reach cancer cells throughout the body.
Chemotherapy drugs used to treat undifferentiated embryonal sarcoma of the liver in children are:
Combinations of these drugs may be used. Other chemotherapy drugs not listed here may also be used.
To learn more about chemotherapy and its side effects, see Chemotherapy to Treat Cancer.
A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. Because cancer in children is rare, taking part in a clinical trial should be considered.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. Some clinical trials are open only to patients who have not started treatment. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
To learn more, see Clinical Trials Information for Patients and Caregivers.
Targeted therapy uses drugs or other substances to block the action of specific enzymes, proteins, or other molecules involved in the growth and spread of cancer cells. Targeted therapy is being studied for the treatment of undifferentiated embryonal sarcoma of the liver that has come back after treatment.
To learn more about how targeted therapy works against cancer, what to expect when having targeted therapy, and targeted therapy side effects, see Targeted Therapy to Treat Cancer.
Treatment of childhood undifferentiated embryonal sarcoma of the liver
Treatment of newly diagnosed childhood undifferentiated embryonal sarcoma of the liver may include the following:
- Chemotherapy may be given before surgery to help shrink the tumor so that it can be more easily removed. Chemotherapy may also be given after surgery.
- Surgery may be done to remove the tumor, followed by chemotherapy. A second surgery may be done to remove tumor that remains, followed by more chemotherapy.
- Liver transplant may be done if surgery to remove the tumor is not possible.
Sometimes childhood undifferentiated embryonal sarcoma of the liver continues to grow or recur (comes back) after treatment. The cancer may come back in the liver or in other parts of the body. Your doctor will work with you to plan treatment if your child is diagnosed with recurrent undifferentiated embryonal sarcoma of the liver.
Side effects and late effects of treatment
For information about side effects that begin during treatment for cancer, see our Side Effects page.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
- 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 long-term effects cancer treatment can have on your child. To learn more, see Late Effects of Treatment for Childhood Cancer.
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer or to find out the treatment group may be repeated 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 child's condition has changed, or if the cancer has come back.
Coping with your child's cancer
When a child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, see Support for Families When a Child Has Cancer and the booklet Children with Cancer: A Guide for Parents.