|Classification and external resources|
Hepatoblastoma is an uncommon malignant liver neoplasm occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells. They usually present with an abdominal mass. The disease is most commonly diagnosed during a child's first three years of life Alpha-fetoprotein (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.
Patients with familial adenomatous polyposis (FAP), a syndrome of early-onset colonic polyps and adenocarcinoma, frequently develop hepatoblastomas. Also beta-catenin mutations have been shown to be common in sporadic hepatoblastomas, occurring in as many as 67% of patients.
One study suggests that low birth weight might be a risk factor for hepatoblastoma.
A Children's Cancer Group study by Buckley et al. (1989) found significant linkage between hepatoblastoma and maternal and paternal exposure to metals.
The most common method of testing for hepatoblastoma is to a blood test checking the Alpha-fetoprotein. Alpha-fetoprotein (AFP) is used as a biomarker to help determine the presence of liver cancer in children. At birth, infants have relatively high levels of AFP, which fall to normal adult levels by the first year of life. The Normal levels for AFP in children has been reported as lower than 50 nanograms per milliliter (ng/ml) and 10 ng/ml. AFP Blood Test AFP greater than 500 (ng/ml) is a significant indicator of hepatoblastoma. AFP is also used as an indicator of the success of treatments. If treatments are successful in removing the cancer it can be expected that AFP levels will fall back to normal.
Surgical resection, adjuvant chemotherapy prior to resection, and liver transplantation have been used to treat these neoplasms, primary transplantation provides high, long term, disease-free survival rate in the range of 80%, in cases of complete resection and adjuvant chemotherapy survival rates approach 100%. The presence of metastases is the most potent predictor of poor prognosis.
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- Tim, P, et al.. Hepatoblastoma.
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- Otte JB, Pritchard J, Aronson DC, et al. (January 2004). "Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience". Pediatr Blood Cancer 42 (1): 74–83. doi:10.1002/pbc.10376. PMID 14752798.
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- Czauderna P, Mackinlay G, Perilongo G, et al. (June 2002). "Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group". J. Clin. Oncol. 20 (12): 2798–804. doi:10.1200/JCO.2002.06.102. PMID 12065556.
- humpath #2775 (Pathology images)
- University of Minnesota hepatoblastoma epidemiology study
- What is Hepatoblastoma?