Hepatoblastoma

This 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.

In this section

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 or MRI scan – an MRI or CT  scan of your child’s abdomen and CT scan of the  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.

Treatment of hepatoblastoma

Treatment for hepatoblastoma depends on which risk group your child falls into. The treatment usually includes chemotherapy and surgery, and occasionally liver transplantation.

Surgery

Surgery is carried out following chemotherapy to shrink the tumour. The aim is to remove the tumour completely and this is undertaken by a specialist liver surgeon. If it is not possible to remove the tumour completely after further chemotherapy, then liver transplantation maybe considered.

Chemotherapy

Treatment with anti-cancer drugs is used to destroy the cancer cells. It is usually given by injections and drips into a vein (intravenous infusions). The type of chemotherapy will vary in intensity and duration according to the risk group of the tumour.

If your child takes part in a clinical trial, the treatment is explained in more detail in the specific trial information sheet. You will be given an outline of the treatment in the form of a ‘road map’.

Clinical trials

Clinical trials are medical research trials involving patients that are carried out to try and find new and better treatments. In cancer, clinical trials are most commonly used to try and improve different forms of treatment such as surgery, radiotherapy or chemotherapy.

The treatment being tested may be aimed at:

  • improving the number of people cured (for example, trying new types of surgery or chemotherapy)
  • improving survival
  • relieving the symptoms of the cancer
  • relieving the side effects of treatment
  • improving the quality of life or sense of wellbeing for people with cancer.

Clinical trials may also involve research aimed at understanding more about the tumour’s biology. You may be asked to allow us to do research on the tumour sample removed at surgery, or on blood samples.

Well-run clinical trials have led to a significant improvement in the treatment of children with cancer. You can find more general information on clinical trials in the separate information sheet. If you are asked to consider entering your child into a clinical trial you will be given specific information about the trial before deciding whether to take part.

Effects of treatment

The prognosis depends on the risk group of the tumour, but many children with hepatoblastoma are cured.

General side effects of chemotherapy

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low red blood cells (anaemia), low white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. We will give anti-sickness drugs at the same time to stop nausea and vomiting. These are usually very effective.

Sore mouth (mucositis)

Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. We will give your child painkillers for this, and explain how to care for your child’s mouth during treatment.

Hair loss

Temporary hair loss is common.

Specific drugs

For details of the side effects of individual drugs please see Macmillan specific drug information leaflets.

What are the possible long-term effects of treatment?

A small number of children develop side effects many years later because of the treatment they have received. These may include hearing problems, impaired heart and kidney function and a small risk of developing a second cancer later in life.

About five years after treatment finishes we will transfer your child’s care to our long term follow-up clinic. Your child will be seen at regular intervals in this clinic, indefinitely, so that we can help with any long-term effects of the treatment.