• Hepatoblastoma Overview

    Hepatoblastoma is a rare type of childhood cancer that occurs in the liver.  Children who have had hepatitis B infection at an early age, those born prematurely and of low birth weight, or those who have had biliary atresia (blockage of the tubes that carry bile) are at increased risk of developing hepatoblastoma.   A number of genetic conditions also increase the risk for this disease.

    Hepatoblastoma Treatment at Dana-Farber/Boston Children's

    Patients with hepatoblastoma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Liver Tumor Program. Continue reading to learn more about hepatoblastoma or visit the Liver Tumor Program homepage to learn about our expertise.

    Symptoms & Diagnosis

    Depending on the size and location of the tumor, the symptoms of hepatoblastoma may vary. Common symptoms include:

    • A large abdominal mass or a swollen abdomen
    • Pain on the right side that may extend to the back and shoulder
    • Back pain from compression of the tumor
    • Decreased appetite and weight loss
    • Vomiting
    • Jaundice
    • Fever
    • Anemia 

    A physician may order a number of different tests to diagnose hepatoblastoma. In addition to a medical history and physical exam, these may include:

    • Blood tests, including a complete blood count and blood chemistries
    • Liver and kidney function tests
    • Alpha-fetoprotein, a protein often secreted by this type of tumor
    • Liver ultrasound
    • Liver and chest computerized tomography (CT or CAT) scan
    • Liver magnetic resonance imaging

    Ultimately, the diagnosis is confirmed by a biopsy of the tumor. Hepatoblastoma can spread to the lungs, and rarely to the bone.  Therefore, a full work-up at diagnosis is necessary to define disease extent. After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Complete surgical removal of the tumor is important to achieving a cure for hepatoblastoma. If removal is risky or there is a chance some of the tumor may be left behind, a child may be treated with chemotherapy prior to surgery. We participate in consortium clinical trials for children with hepatoblastoma. After three or four courses of chemotherapy, surgical removal is more readily accomplished. In very rare cases, when tumors are still inoperable after receipt of chemotherapy, doctors may consider a liver transplant.

    There can be side effects related to the tumor itself or its treatment. Knowing what these side effects are can help physicians prepare for and, in some cases, prevent these symptoms from occurring.

    Progressive or Recurrent Disease

    The prognosis for children with recurrent or progressive hepatoblastoma depends on several factors, including the site of recurrence and prior treatment of the disease. Aggressive surgery for isolated spread (metastases) may extend disease-free survival. Clinical trials may also be appropriate for children with recurrent or progressive disease.

    Long-term Outlook

    There has been a dramatic improvement in outcomes for hepatoblastoma over the past 20 years. Complete surgical removal at diagnosis, with or without chemotherapy, can allow for a survival rate of almost 100%.  The survival rate drops to approximately 70% for patients requiring chemotherapy upfront to assist with surgical excision. Patients with metastatic disease have an approximately 20 or 30% survival rate.

    There is preliminary evidence that the cure rate for children with advanced stages of the disease may be improving due to aggressive chemotherapy options and the possibility of a liver transplant.

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  • Watch: Chemotherapy for Children


    Lisa R. Diller, MD, Chief Medical Officer at Dana-Farber/Boston Chilren's explains how chemotherapy affects children.