Hepatoblastoma is a solid tumor cancer of the liver. This primary liver tumor which accounts for half of all liver tumors in children is rare with approximately 50 to 70 new cases diagnosed in the United States each year. It occurs slightly more often in boys than girls.

Like most childhood cancers, scientists do not know the cause of this disease. Some children however are at an increased risk of being diagnosed with hepatoblastoma due to other genetic conditions. These include: Beckwith-Wiedemann syndrome, familial adenomatous polyposis, and Aicardi syndrome.

Hepatoblastoma is predominantly found in preschool aged children. Half of all patients are diagnosed in their first year of life, with the majority of patients being diagnosed by their third birthday.

Diagnosis and treatment monitoring can be done using X-ray, CT scan, MRI, scan and/or ultrasound.

• More information on hepatoblastoma on the NCI web site – note that you can toggle back and forth between ‘Health Professional’ and ‘Patient’ versions.

Liver Cancer Treatment

Once hepatoblastoma has been diagnosed, the tumor will be staged based upon the extent of the tumor and whether it can be surgically removed. Approximately half of all hepatoblastoma tumors diagnosed are small and can be successfully treated with surgery alone (Stage I). The liver is one organ which is capable of regenerating itself, thus making surgery the best chance for a complete cure. In cases where the tumor is large and disease has spread to the lymph nodes (Stage III), or has spread to other parts of the body, primarily the lung (Stage IV), chemotherapy is used to shrink the tumor prior to surgical resection. Children whose tumors have relapsed and have not spread outside of the liver could be candidates for a liver transplant.

Serum Tumor Marker Tests: Hepatoblastoma often produces a protein which can be detected in the blood. Levels of alpha-fetoprotein (AFP) can be used as a way to determine whether the tumor is responding to treatment (AFP levels decrease), whether there is no evidence of disease, or if there is an indication of relapse (increased levels of AFP).

Hepatoblastoma responds well to chemotherapy agents when used to shrink tumors prior to surgery. Chemotherapy agents include cisplatin, fluorouracil, doxorubicin, and vincristine. Other possible agents used are ifosfamide, dactinomycin, and etoposide.

Clinical trials for hepatoblastoma can be found by searching the clinical trial database on the NCI web site. Follow these directions:

• Start at the NCI clinical trials search page.
• Choose type of cancer: “hepatoblastoma.”
• You will be given more choices (e.g., type of cancer and type of trial). Choose the appropriate responses and click “search.”

Symptoms of Hepatoblastoma

Symptoms at diagnosis can include:

• abdominal lump or mass often located in the upper right side or middle of the abdomen
• loss of appetite, nausea, abnormal weight loss, diarrhea
• changes in the skin color: jaundice evidenced by yellow tone to the skin and whites of the eyes
• general itching
• fever, irritability, listlessness
• abdominal pain

Statistics, Risk Factors, Incidence

• Approximately 50-70 new cases/year in the US
• 1% of all pediatric cancers.
• 95% diagnosed under 4 years of age.
• In 50% of the cases the disease can be cured by surgery alone.
• The 5 year survival rate for the combined stages of hepatoblastoma is 60%-70%. The prognosis for early stage disease is excellent with a 90% five year survival.

Resources

Websites

• NCI: Excellent resource and links
• Ped-onc resource center
• Hepatoblastoma Resource Network

E-mail Support Lists

ACOR: Ped-Onc