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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 pediatric cancers. Types of pediatric liver cancer include hepatoblastoma, hepatocellular carcinoma, and undifferentiated embryonal sarcoma of the liver.  

  • Hepatoblastoma (HB) is a type of liver cancer usually seen in infants and young children under age 3. It is the most common pediatric liver cancer.
  • Hepatocellular carcinoma (HCC) occurs in older children and teens. It is very rare in young children. It is usually diagnosed in children ages 12-14 years.
  • Undifferentiated embryonal sarcoma of the liver (UES) is a rare liver cancer that usually develops in children between the ages of 6-10 years. It is the 3rd most common type of pediatric liver cancer after hepatoblastoma and hepatocellular carcinoma. It makes up about 15% of pediatric liver cancers.
  • In UES, serum AFP is usually normal. This tumor often responds to chemotherapy. However, surgery is still essential for cure.
What is liver cancer? Liver cancer is rare in children and teens. It accounts for 1-2% of pediatric cancers.

Liver cancer is rare in children and teens. It accounts for 1-2% of pediatric cancers. Types of pediatric liver cancer include hepatoblastoma, hepatocellular carcinoma, and undifferentiated embryonal sarcoma of the liver.

The liver is a large organ on the top right side of the abdomen. It has several important functions. The liver helps clear waste from the blood, makes bile to help digest food, and 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 who have complete removal of tumors have a good chance for cure.

Chemotherapy may be used to shrink the tumor before surgery or stop or slow the growth of cancer cells after surgery. Survival of liver cancer depends on whether surgery can remove the tumor and whether the cancer has spread outside 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 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.

Liver Cancer Symptoms

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

  • Lump or swelling in the abdomen (belly), often without symptoms
  • Pain in the abdomen 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

Hepatoblastoma can be associated with signs of early puberty (precocious puberty) due to hormones secreted by the tumor.

Liver Cancer Diagnosis

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 determine whether there might be an inherited risk.
  • Lab studies to look at blood and urine including:
    • Complete blood count to check the number of red blood cells, white blood cells, and platelets; the amount of hemoglobin in the red blood cells; and the portion of blood made up of red blood cells.
    • 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 is usually normal in children with UES.
  • Imaging tests to look for cancer, see how big the tumor is, and find out if it has spread to other places such as the lungs. 
  • Biopsy of the some of the tissue from the tumor to check for signs of cancer in the cells and learn more about the histology. The way cells look under a microscope is important for making a diagnosis and planning treatment. In very young children with high AFP, a biopsy may not be needed.

Liver Cancer Stages

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. The higher the group, the more sections of the liver have tumor. 

PRETEXT Group Amount of the liver involved
PRETEXT Group I
  One section of the liver is involved.                                                                                 
PRETEXT Group II  One or two sections of the liver are involved.
PRETEXT Group III  Two or three sections of the liver are involved.
PRETEXT Group IV
  All four sections of the liver are involved.

Surgery is the main treatment option for liver cancers. Patients who have complete removal of tumors have 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.

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.

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 3 years of age. 

    Risk Factors and Causes of Hepatoblastoma

    For most children with liver cancer, there is no known cause. However, certain factors may increase risk in some patients. 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 associated with increased cancer risk
    • Trisomy 18, a genetic condition associated with multiple physical defects and health conditions

    Prognosis for Hepatoblastoma

    Recovery from hepatoblastoma depends on successful removal of all tumor. 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 remove all tumor.

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

    Factors that are associated with a worse outcome include:

    • Older age at diagnosis (>8 years old)
    • Low alpha-fetoprotein (AFP) level below 100 ng/mL
    • Small cell histology.

    Patients who are younger and have fetal cell histology are more likely to be cured.

    Treatment of Hepatoblastoma

    Surgery is the main treatment option for liver cancers. 

    Chemotherapy can be used before surgery in hepatoblastoma to shrink the tumor and make it easier to remove. Cisplatin is the main chemotherapy for hepatoblastoma. Combination therapy (treatment with more than one type of chemotherapy) is also used.

  2. Hepatocellular carcinoma

    Pediatric hepatocellular carcinoma is rare. It is usually diagnosed in children between the ages of 12 and 14, and is not often diagnosed in children under 5 years of age.

    Risk Factors and Causes of Hepatocellular Carcinoma

    Hepatocellular carcinoma is usually found in adults who have liver disease or damage (cirrhosis) due to infection, certain health conditions, or damage due to use of drugs and alcohol. Older children and teens may develop the cancer. However, most do not have any known risk factors. About 1/3 of children with hepatocellular carcinoma do 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 mellitus
    • 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

    Prognosis for Hepatocellular Carcinoma

    Hepatocellular carcinoma is more difficult 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.
    • Whether surgery can completely remove the cancer.

    Treatment of Hepatocellular Carcinoma

    Surgery is the only way to cure hepatocellular carcinoma. Chemotherapy is used to try to shrink the tumor enough so that it can be completely removed. However, this cancer is not very sensitive to chemotherapy. 
    If the tumor has already spread outside the liver at diagnosis, then chemotherapy or local therapies can be tried.

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

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

Life after Liver Cancer

Liver transplant

Patients who have had a liver transplant need lifelong immunosuppression medication. These drugs keep the body from attacking or rejecting the new liver. Because these medicines act to lower the body’s natural defenses, patients may have an increased risk for infection. However, 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

For general health and disease prevention, all cancer survivors should adopt healthy lifestyle and eating habits, as well as continue to have regular physical checkups and screenings by a primary physician at least annually. Childhood cancer survivors treated with systemic chemotherapy should be monitored for acute and late effects of therapy. Possible health concerns include hearing loss and kidney problems (cisplatin) and heart problems (anthracylcines).

—
Reviewed: June 2018

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Long-Term and Late Effects

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