Definition of Childhood liver cancer

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Childhood liver cancer: Primary cancer of the liver (cancer that starts in the liver) in children, a relatively rare malignancy in children. There are 2 main types of primary liver cancer in children -- hepatoblastoma and hepatocellular carcinoma. Hepatoblastomas usually occur before 3 years of age, whereas the incidence of hepatocellular carcinoma varies little with age between 0 and 19 years. The overall survival rate for children with hepatoblastoma is 70% but is only 25% for hepatocellular carcinoma.

Most children with hepatoblastoma or hepatocellular carcinoma have a tumor marker in the blood called alpha-fetoprotein (AFP), that parallels the activity of their disease. Lack of a significant decrease of AFP levels with treatment may predict a poor response to therapy. If a hepatoblastoma can be completely removed, the majority of children survive. The inability to completely remove the primary liver tumor or the presence of metastatic disease is associated with a poor outcome. Resection (removal) is possible more often with hepatoblastoma than hepatocellular carcinoma, in which less than 30% are resectable. Chemotherapy prior to surgery for patients with hepatoblastoma may reduce the extent of the surgery needed. This approach is not usually done for hepatocellular carcinoma. Liver transplantation will be considered in selected cases and may be curative.

Hepatocellular carcinoma is associated with hepatitis B and hepatitis C infection, especially in children with virus they acquired in the perinatal period. Compared with adults, the incubation period from hepatitis virus infection to the start of hepatocellular carcinoma is extremely short in some children with perinatally-acquired virus.