Skip to main content

Childhood Undifferentiated Embryonal Sarcoma of the Liver

Childhood undifferentiated embryonal sarcoma of the liver is a rare cancer type that forms in the tissues of the liver. This type of liver cancer usually occurs in children between 5 and 10 years. It often spreads all through 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 are 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
EnlargeAnatomy of the liver; drawing shows the right and left lobes of the liver. Also shown are the bile ducts, gallbladder, stomach, spleen, pancreas, small intestine, and colon.
Anatomy of the liver. The liver is in the upper abdomen near the stomach, intestines, gallbladder, and pancreas. The liver has a right lobe and a left lobe. Each lobe is divided into two sections (not shown).

Signs and symptoms of childhood undifferentiated embryonal sarcoma of the liver

Signs and symptoms are more common after the tumor gets big. These and other signs and symptoms may be caused by undifferentiated embryonal sarcoma of the liver or by other conditions. 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

Diagnosis of childhood undifferentiated embryonal sarcoma of the liver

Tests that examine the liver and the blood are used to diagnose childhood undifferentiated embryonal sarcoma of the liver and find out whether the cancer has spread.

The following tests and procedures may be used:

  • Physical exam and health history: A physical exam of the body will be done to check a person’s 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.
  • Complete blood count (CBC): 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: These blood 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: These blood tests 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: This procedure 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.
    EnlargeMagnetic resonance imaging (MRI) of the abdomen; drawing shows a child lying 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.
    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.
  • CT scan (CAT scan): This procedure 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.
    EnlargeComputed tomography (CT) scan of the abdomen; drawing shows a child lying on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
    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.
  • Ultrasound exam: This procedure uses high-energy sound waves (ultrasound) that 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.
    EnlargeAbdominal ultrasound; drawing shows a child lying on an exam table during an abdominal ultrasound procedure. A technician is shown pressing a transducer (a device that makes sound waves that bounce off tissues inside the body) against the skin of the abdomen. A computer screen shows a sonogram (picture).
    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).
  • Abdominal x-ray: An x-ray of the organs in the abdomen may be done. 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: During a biopsy, a doctor removes a sample of cells or tissue. A pathologist then views the cells or tissue under a microscope to look for cancer cells and find out the type of cancer. The doctor may remove as much tumor as safely possible during the same biopsy procedure.

    The following test may be done on the sample of tissue that is removed:

    • Immunohistochemistry: This laboratory test 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 check for a certain gene mutation, to help diagnose cancer, and to help tell one type of cancer from another type of cancer.

Prognostic factors for childhood undifferentiated embryonal sarcoma of the liver

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

  • the size of the tumor
  • the health of the patient
  • how the cancer responds to chemotherapy
  • whether the cancer can be removed completely by surgery
  • whether the patient can have a liver transplant
  • whether the cancer has just been diagnosed or has recurred

For childhood undifferentiated embryonal sarcoma of the liver that recurs (comes back) after initial treatment, the prognosis and treatment options depend on the following:

  • where in the body the tumor recurred
  • the type of treatment used to treat the initial cancer

Types of treatment for childhood undifferentiated embryonal sarcoma of the liver

Children with undifferentiated embryonal sarcoma of the liver 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 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. Other specialists may include the following:

  • pediatrician
  • radiation oncologist
  • pediatric nurse specialist
  • rehabilitation specialist
  • psychologist
  • social worker
  • nutritionist

For more information about having a child with cancer and ways to cope and find support, see Childhood Cancers.

Every child will not receive all the treatments listed below. Your child’s care team will help you make treatment decisions based on your child’s unique situation. To learn more about factors that help determine the treatment plan for this cancer, see Prognostic factors for childhood undifferentiated embryonal sarcoma of the liver.

Surgery

A partial hepatectomy (surgery to remove the part of the liver where cancer is found) may be done. A wedge of tissue, an entire lobe, or a larger part of the liver, along with some of the healthy tissue around it is removed. The remaining liver tissue takes over the functions of the liver and may regrow.

Liver transplant

In a liver transplant, the entire liver is removed by surgery and replaced with a healthy donated liver. 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

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). Treatment using more than one anticancer drug is called combination chemotherapy.

To learn more about chemotherapy and its side effects, see Chemotherapy and You: Support for People with Cancer.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. 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.

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. For some patients, taking part in a clinical trial may be the best treatment choice.

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. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

To learn more about clinical trials, see Clinical Trials Information for Patients and Caregivers.

Long-term side effects of treatment

Side effects from cancer treatment that begin after treatment and continue for months or years are called long-term or 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. For more information, see Late Effects of Treatment for Childhood Cancer.

Follow-up tests

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 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 of newly diagnosed childhood undifferentiated embryonal sarcoma of the liver

Treatment of newly diagnosed childhood undifferentiated embryonal sarcoma of the liver may include the following:

  • Combination chemotherapy may be used 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 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.

Treatment of progressive or recurrent childhood undifferentiated embryonal sarcoma of the liver

Sometimes childhood undifferentiated embryonal sarcoma of the liver continues to grow or comes back after treatment.

  • Progressive disease is cancer that continues to grow, spread, or worsen. Progressive disease may be a sign that the cancer has become refractory to treatment.
  • Recurrent childhood undifferentiated embryonal sarcoma of the liver 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.

To learn more about metastatic cancer (cancer that has spread from where it started to other parts of the body), see Metastatic Cancer: When Cancer Spreads.

Treatment of recurrent childhood undifferentiated embryonal sarcoma of the liver may include the following:

  • a clinical trial that checks a sample of the patient's tumor for certain gene changes to determine the type of targeted therapy that will be given
  • Updated:

If you would like to reproduce some or all of this content, see Reuse of NCI Information for guidance about copyright and permissions. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., “Childhood Undifferentiated Embryonal Sarcoma of the Liver was originally published by the National Cancer Institute.”