Solid Tumor: Hepatoblastoma
Alternate Names: None
Hepatoblastoma is a form of liver cancer that usually occurs in infants. In contrast to hepatocellular carcinoma, it arises in an otherwise normal liver.
- 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.
- 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.
- 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.
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.
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