Hepatoblastoma

Hepatoblastoma is a malignant (cancerous) tumour of the liver which usually occurs in young children. This is different from hepatocellular carcinoma which usually occurs in adults and occasionally in older children.

Who gets hepatoblastoma?

About 10 to 15 children develop hepatoblastoma in the UK each year. The average age at diagnosis is one year and most cases occur before two years of age.

Hepatoblastoma is not associated with previous hepatitis, unlike hepatocellular carcinoma. Usually there are no associated genetic factors. There may be an increased risk of hepatoblastoma in children who have a family history of familial polyposis coli, or if the child has a condition called Beckwith-Wiedemann syndrome.

Signs and symptoms

Hepatoblastoma usually presents as a lump in the tummy. Other symptoms include:

poor appetite
weight loss
lethargy
fever
vomiting
jaundice.

Tests / investigations

We will need to carry out some tests to find out as much as possible about the type, position and size of the tumour. This will help us to decide on the best treatment for your child. The tests will include:

Blood samples – hepatoblastoma usually produces a protein called alpha-fetoprotein (AFP) which is released into the bloodstream. This is usually referred to as a tumour marker and is used to monitor response to treatment.
Chest X-ray – this will tell us if the tumour has spread to the lungs.
Ultrasound scan – this will tell us the position and size of the tumour. We can also use ultrasound scans to monitor the response to treatment.
CT scan – a scan of your child’s abdomen and lungs will be carried out. This will give us more detailed information about the tumour and may tell us if it has spread.
Biopsy – this is a small operation, usually carried out under general anaesthetic. The surgeon will remove a small piece of tissue from the lump, which will help us to make a diagnosis.

Staging

Staging is a measure of how far the tumour has spread beyond its original site. For treatment purposes, there are two groups. These are ‘standard risk’ and ‘high risk’ as follows:

standard risk is when the tumour is confined to the liver and involves at most three segments of the liver
high risk is when the tumour involves all four segments of the liver and/or the tumour has spread outside of the liver.