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Liver Cancer in Children and Teens

What is liver cancer?

Liver cancer is rare in children and teens. It accounts for 1–2% of childhood cancers. Types of childhood liver cancers include:

What is liver cancer? Liver cancer is rare in children and teens. It accounts for 1-2% of pediatric cancers.

Liver cancer accounts for 1–2% of childhood cancers. Childhood liver cancers include hepatoblastoma, hepatocellular carcinoma, fibrolamellar hepatocellular carcinoma, and undifferentiated embryonal sarcoma of the liver.

The liver is a large organ on the top right side of the abdomen. The liver:

  • Helps clear waste from the blood
  • Makes bile to help digest food
  • Stores energy to fuel the body

Liver cancer occurs when cancer cells form tumors in the tissue of the liver.

Surgery is the main treatment option for liver cancers. The goal of surgery is to remove as much of the tumor as possible. Patients have a good chance for cure if the tumor can be completely removed.

Chemotherapy may be used to shrink the tumor before surgery. It can also help stop or slow the growth of cancer cells after surgery.

Survival depends on whether surgery can remove the tumor and whether the cancer has spread beyond the liver.

The liver is a large organ on the top right side of the abdomen. The liver helps clear waste from the blood, makes bile to help digest food, and stores energy to fuel the body.

The liver helps clear waste from the blood, makes bile to help digest food, and stores energy to fuel the body.

Symptoms of liver cancer

Symptoms of liver cancer may depend on the size of the tumor and if it has spread beyond the liver. Symptoms of liver cancer include:

  • Lump or swelling in the abdomen (belly), often without symptoms
  • Pain in the abdomen, shoulder, or back
  • Loss of appetite
  • Weight loss
  • Itchy skin
  • Yellow color to the eyes and/or skin (jaundice)
  • Pale skin and lips (may mean anemia)
  • Nausea and vomiting
  • Fever
  • Signs of early puberty due to hormones produced by the tumor

Diagnosis of liver cancer 

Doctors use several types of tests to evaluate liver tumors and make a diagnosis. These tests include:

  • A health history and physical exam to learn about symptoms, general health, past illnesses, and risk factors. Family history is important to find out if there might be an inherited risk.
  • Lab studies to look at blood and urine including:
    • Complete blood count to check the number and types of cells in the blood.
    • Other blood tests to look at liver function and check for inflammation or infection of the liver (hepatitis).
    • Alpha-fetoprotein (AFP) test to check for high levels. Increased AFP is usually seen in hepatoblastoma. High AFP may be seen in some patients with hepatocellular carcinoma, but this is less common. AFP levels are usually normal in children with UES and FLC, but may be high in FLC.
  • Imaging tests to learn about the size and location of the tumor and find out if it has spread to other places such as the lungs. These may include: 
  • Biopsy of the tumor to check for signs of cancer and learn more about the way cells look under a microscope (histology). This is important to make a diagnosis and plan treatment. In very young children with high AFP, a biopsy may not be needed.

Types of liver cancer

  1. Hepatoblastoma

    Hepatoblastoma is rare. But it is the most common type of liver cancer in children. It is usually found in infants and children under age 3. 

    Risk factors and causes of hepatoblastoma

    There is no known cause of liver cancer in most children. But some factors may increase risk. Risk factors for hepatoblastoma include:

    • Premature birth with very low birth weight
    • Hemi-hyperplasia (hemihypertrophy), a condition in which one side of the body grows faster than the other
    • Fetal alcohol syndrome
    • Familial adenomatous polyposis (FAP), an inherited condition where polyps develop in the colon
    • Beckwith-Wiedemann syndrome, a rare overgrowth disorder that includes large size at birth, low blood sugar, and other physical problems
    • Li-Fraumeni syndrome, an inherited condition linked to increased cancer risk
    • Trisomy 18, a genetic condition linked to multiple physical defects and health conditions

    Treatment of hepatoblastoma

    Surgery is the main treatment option for liver cancers. 

    Chemotherapy is typically used in hepatoblastoma to shrink the tumor before surgery and to keep it from coming back after. A small number of patients can be cured with surgery alone. Cisplatin is the main chemotherapy for hepatoblastoma. Combination therapy (treatment with more than one type of chemotherapy) is also used.

    Prognosis for hepatoblastoma

    Recovery from hepatoblastoma depends on successful removal of all tumor cells. This is possible at diagnosis in about 1/3 of patients. Chemotherapy makes successful surgery possible in another 1/3 of patients. Sometimes a liver transplant is needed to fully remove the tumor.

    The five-year survival rate for hepatoblastoma is about 70% if the tumor can be successfully removed with surgery. If cancer is found in more of the liver or has spread outside the liver, then patients have a lower chance of cure.

    Factors linked to a worse outcome include:

    • Older than age 8 at diagnosis
    • Low alpha-fetoprotein (AFP) level (below 100 ng/mL)

  2. Hepatocellular carcinoma

    Childhood hepatocellular carcinoma (HCC) is rare. It is usually diagnosed in children between ages 12–14. It is not often diagnosed in children under age 5.

    Risk factors and causes of hepatocellular carcinoma

    HCC is usually found in adults who have liver disease or damage (cirrhosis) from infection, certain health conditions, or use of drugs and alcohol. Older children and teens may develop HCC. About 1 in 3 children with HCC have a risk factor that makes them more likely to get this cancer. These include:

    • Hepatitis B or C infection
    • Exposure to ethanol (alcohol)
    • Exposure to vinyl chloride
    • Exposure to Aflatoxin B1
    • Use of androgenic anabolic steroids
    • Diabetes
    • Certain inherited or congenital conditions:
      • Metabolic conditions including tyrosinemia and glycogen storage disease
      • Familial cholestatic syndromes including Alagille syndrome and biliary atresia
      • Neurofibromatosis
      • Ataxia-telangiectasia
      • Fanconi anemia

    Treatment of hepatocellular carcinoma

    Surgery is the only way to cure hepatocellular carcinoma. Chemotherapy is used to try to shrink the tumor enough to fully remove it. But this cancer is not very sensitive to chemotherapy. 

    If the tumor has spread beyond the liver at diagnosis, then doctors may use chemotherapy or therapies that target the affected organ(s).

    The drug sorafenib may help slow the cancer. Regorafenib has been approved for patients who were treated with sorafenib in the past. Some drugs such as cisplatin may work better in children than in adults.

    Local therapies may help patients who await a liver transplant. The main goal of these therapies is to slow cancer growth and extend life, not provide a cure.

    Prognosis for hepatocellular carcinoma

    Hepatocellular carcinoma is harder to treat because it rarely responds to chemotherapy. Complete removal of the tumor through surgery or a liver transplant offers the best chance for cure. The overall survival rate is less than 30%. Factors that influence chance of recovery include whether:

    • The cancer has spread to other parts of the body
    • Surgery can completely remove the tumor

  3. Fibrolamellar hepatocellular carcinoma

    Fibrolamellar hepatocellular carcinoma (FLC) is a rare type of liver cancer. Only 1–5% of all liver cancers are FLCs. This tumor is usually found in teens and adults under age 40. Patients in the early stages of FLC may have no symptoms. The cancer may have already spread before diagnosis.

    Risk factors for fibrolamellar hepatocellular carcinoma

    There are no known risk factors for FLC. Because this cancer is so rare, scientists do not know if it is passed through families.

    The cancer is caused by changes in the DNAJB1 and PRKACA genes. The doctor may perform a biopsy to test your child’s tumor. If the DNAJB1 and PRKACA genes are affected, then the disease is FLC.

    Treatment of fibrolamellar hepatocellular carcinoma

    Surgery is the main treatment option. Doctors will try to remove as much tumor as possible. They may remove part of the liver. Or they may perform a liver transplant.

    Chemotherapy can be used if surgery is not possible or if the cancer has spread to other parts of the body.

    Embolization therapy may block or reduce blood flow to the tumor, causing it to die. This is done by injecting a substance into an artery that supplies blood to the tumor.

    Prognosis for fibrolamellar hepatocellular carcinoma

    If FLC has not spread beyond the liver, surgery can offer a cure. The 5-year survival rate for children who have surgery ranges from 44–68%. Children who do not have surgery have a survival outlook of 2–17%.

    Factors that affect chance of cure include:

    • How much tumor is removed
    • Location of the tumor
    • Whether the cancer has spread to other parts of the body

    Balancing quality of life with cancer-directed therapy is important. Talk to your care team about what problems to expect and ways to help manage them. Palliative care can help you manage symptoms, promote quality of life, and make informed decisions that align your family’s wishes with goals of care.

  4. Undifferentiated embryonal sarcoma of the liver

    Undifferentiated embryonal sarcoma of the liver (UES) is a rare liver cancer that usually develops in children ages 5–10. It is the 3rd most common type of childhood liver cancer after hepatoblastoma and hepatocellular carcinoma. UES makes up between 9–15% of childhood liver cancers.

    Risk factors for undifferentiated embryonal sarcoma of the liver

    The risk factors for UES are unknown. In many cases, there are no signs or symptoms of UES until the tumor is large.

    Treatment of undifferentiated embryonal sarcoma of the liver

    Chemotherapy is used to shrink the tumor before surgery. Chemotherapy after surgery may be given to kill remaining cancer cells.

    If possible, your child will have surgery to remove the tumor. Doctors will try to remove as much tumor as possible. They may remove part of the liver. Or they may perform a liver transplant.

    Prognosis for undifferentiated embryonal sarcoma of the liver

    The outlook for children with UES depends on:

    • The tumor size at diagnosis
    • How much tumor can be removed with surgery
    • How well the UES responds to chemotherapy
    • Whether the cancer has spread to other parts of the body

    The 5-year survival rate for children with UES is 84%. If UES comes back after treatment, it will likely spread through the liver or lungs, with a lower survival rate. The long-term outlook depends on the location of the new tumor and the treatment that was used to treat the previous tumor.

Stages of liver cancer 

Doctors often classify liver tumors based on where the cancer is before the patient has treatment. This system is called pretreatment extent of disease (PRETEXT). PRETEXT groups (1, 2, 3, 4) depend on which parts of the liver have cancer. 

PRETEXT Group Amount of the liver involved
PRETEXT Group 1
 One section of the liver is involved.                                                                                 
PRETEXT Group 2 One or 2 sections of the liver are involved. Two side-by-side sections are free of tumor.
PRETEXT Group 3 Two or 3 sections of the liver are involved. One or 2 sections are free of tumor, but these sections do not touch.
PRETEXT Group 4
 All 4 sections of the liver are involved. 

If the tumor can be completely removed with surgery, the patient has a good chance for cure.

Doctors often classify liver tumors based on where the cancer is before the patient has treatment. This system is called pretreatment extent of disease (PRETEXT). PRETEXT groups (I, II, III, IV) depend on which parts of the liver have cancer.

Staging of liver tumors depends on where cancer is found in the liver.

Support for patients with liver cancer

Liver transplant

Patients who have a liver transplant need lifelong immunosuppression medication. These drugs keep the body from attacking or rejecting the new liver. Because the medicines lower the body’s natural defenses, patients may have an increased risk for infection. But many people live normal, healthy lives after an organ transplant. It is important to have regular medical care and take medicines as prescribed.

Late effects of treatment

Survivors of childhood liver cancer should have long-term follow-up care. Some treatments can cause late effects. These are health problems that happen months or years after treatment has ended.

For general health and disease prevention, all cancer survivors should adopt healthy lifestyle and eating habits. They should have regular checkups and screenings by primary health care providers. Childhood cancer survivors treated with chemotherapy should be monitored for late effects of therapy. Possible health concerns include hearing loss and kidney problems (cisplatin) and heart problems (anthracylcines).

Your child should receive a survivorship care plan after they complete treatment. It includes guidance on health screenings, disease risk factors, and how to improve health. Survivors should share this plan with their health care providers.

Questions to ask your care team

  • What are our treatment options?
  • What are the possible side effects of each treatment?
  • What can be done to manage side effects?
  • Will my child need to be in the hospital for treatment?
  • Where is the treatment available? Is it close to home or will we have to travel?

Key points about liver cancer

  • Liver cancer is rare. It occurs in only 1–2% of childhood cancers.
  • Most treatment for liver cancer involves surgery. Chemotherapy may also be used.
  • Survival depends on whether doctors can remove the tumor and whether the disease has spread beyond the liver.
  • Survivors of childhood liver cancer should follow a healthful lifestyle and get long-term follow-up health care.

Find more information about liver cancer

—
Reviewed: December 2022

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