Disease Information

Solid Tumor: Hepatoblastoma

Alternate Names: None

Definition

Hepatoblastoma is a form of liver cancer that usually occurs in infants. In contrast to hepatocellular carcinoma, it arises in an otherwise normal liver.


Incidence

  • Liver tumors account for 0.5-2 percent of pediatric tumors and are the tenth most frequent tumors in children.
  • Hepatoblastoma has an incidence of 0.9 per 1 million children.
  • The median age of diagnosis is 1 year; most patients are diagnosed by 2 years of age.

Influencing Factors

  • In contrast to hepatocellular carcinoma, prior hepatitis infection is not associated with an increased risk of hepatoblastoma.
  • Children of families whose members carry a gene related to a certain kind of colon cancer (Familial colonic polyposis) and those with Beckwith-Wiedemann syndrome or hemi-hypertrophy may be more likely to develop hepatoblastoma.

Clinical Features and Symptoms

The cancer typically appears as an asymptomatic abdominal mass found on a routine physical examination or discovered incidentally by the parents. Other nonspecific features include weight loss, abdominal pain, loss of appetite, anemia, fever, vomiting and jaundice.


Survival Rates

  • Cure of hepatoblastoma is possible when the tumor can be completely removed by surgery. However, this is possible for only about half of the patients at the time of initial diagnosis.
  • Unlike hepatocellular carcinoma, hepatoblastoma is usually sensitive to chemotherapy and with treatment the tumor will shrink enough to allow total resection.
  • Children whose tumor has spread outside the liver rarely survive long-term.

Treatment Strategies

  • Until effective chemotherapy became available, complete surgical resection was the only successful treatment option. However, the procedure carried significant risks because these tumors typically form an excessive number of blood vessels – making them difficult to remove. Tumors that couldn’t be removed progressed. This situation changed in the early 1980s when several studies, including one at St. Jude, produced cure rates of 70 percent by adding chemotherapy for initially unresectable tumors, followed by total resection after the tumor shrank sufficiently. This combined approach is now the standard of practice in the United States.
  • In tumors that involve the whole liver, liver transplantation from a donor is sometimes an option.

Current Research

Studies at St. Jude have suggested that irinotecan may be an effective agent in some children with hepatoblastoma. This drug will be tested further in a new Children's Oncology Study that is being planned.

 

The St. Jude Web site is designed for educational purposes only and is not engaged in rendering medical advice or professional services. The information provided through this site should not be used for diagnosing or treating a health problem or a disease. It is not a substitute for professional care. If you have or suspect you may have a health problem, you should consult your health care provider.

 

Email This Article Email This Article   |   Print Print