Hepatoblastoma is a highly malignant solid tumor of the liver. It is the most common primary liver tumor in childhood — a tumor that starts in the liver itself, rather than spreading there from another organ. It develops from immature precursor cells of liver tissue, and the change in these cells begins before birth. It is rare, making up about 1% of cancers in children.
Hepatoblastoma mostly affects very young children. Most are between six months and three years old, and the average age at diagnosis is 1.5 years. Children over age four are rarely affected. Boys are affected more often than girls, at a ratio of about 1.4 to 1.
Most hepatoblastomas are a single, large tumor, often in the right lobe of the liver. About 15% of children have tumors in several areas of the liver, which signals more aggressive growth. The tumor rarely grows outside the liver. When it spreads, it most often goes to the lungs through the bloodstream, and about 10% to 20% of children already have lung metastases at diagnosis.
Doctors describe how far the tumor has grown using a system called PRETEXT, which stands for PRE-Treatment EXTent of disease. This stage guides whether surgery is possible and how intense chemotherapy should be.