This information is about liver tumours in children. It is helpful to read it alongside our general information on children's cancer, which contains more information about cancers in children, their diagnosis and treatment, and the support services available.

Approximately 10 children in the UK develop liver tumours each year. Boys are affected more commonly than girls. Liver tumours can be non-cancerous (benign) or cancerous (malignant). This section is about malignant liver tumours.

The liver is the largest organ in the body, and the main heat-producing organ. It is surrounded by a fibrous capsule and is divided into sections called lobes. It lies across the upper part of the abdomen and is larger on the right hand side of the body than on the left. It is surrounded, and protected from injury, by the lower ribs.

The liver is an extremely important organ that has many functions. One of these functions is to produce proteins that circulate in the blood. Some of the proteins help the blood to clot and prevent excessive bleeding, while others are essential to maintain the balance of fluid in the body. The liver also destroys harmful substances, and breaks down waste products not used by the body, so that they can be passed out in the urine or stools (bowel motions).

The liver is also responsible for breaking down food containing carbohydrates (sugars) and fats, so they can be used by the body for energy. It stores substances such as glucose and vitamins so that they can be used by the body when needed. The liver also produces bile, a substance that breaks down the fats in food so that they can be absorbed from the bowel (intestine).

The liver is connected to the small intestine (duodenum) by a tube called the bile duct. This duct takes the bile produced by the liver to the intestine.

The liver has an amazing ability to repair itself. It can function normally even if only a small part of it is in working order.

Liver cancer can be either primary or secondary. Primary liver cancers start in the liver and secondary liver cancers have spread into the liver from a different part of the body. This factsheet is about primary liver cancer in children.

There are two main types of primary liver cancer:

• Hepatoblastoma, which usually occurs in children under five years of age. About 8 children in the UK are diagnosed with hepatoblastoma each year
• Hepatocellular carcinoma (HCC), which is rarer and usually occurs in older children. About 2 children in the UK are diagnosed with hepatocellular carcinoma each year.

The cause of most primary liver tumours in the western world is unknown. However, in other parts of the world, hepatocellular cancer (HCC) is commonly related to the presence of liver infection. For example, in countries where mothers commonly have hepatitis B, and vaccination is not readily available at birth for their children. Children who are infected with hepatitis B have a higher risk of developing HCC in late childhood than uninfected children.

The most common symptom is a lump or swelling in the abdomen, which can be painful. Other possible symptoms include weight loss, a loss of appetite, feeling sick (nausea) and vomiting.

A variety of tests and investigations may be needed to diagnose a liver tumour. An ultrasound scan and x-rays will be taken, which can show if there is a tumour in the liver. Further tests including CT scans (computerised tomography)or MRI scans (magnetic resonance imaging) will be done to find the extent of the disease, both inside and outside the liver. Blood tests will also be taken.

Most hepatoblastomas and hepatocellular carcinomas produce a protein which is released into the bloodstream. This protein is known as alpha-fetoprotein (AFP). It is possible to measure levels of AFP in the blood, which can be a useful indicator of whether the liver tumour is responding to treatment or whether it may have come back after treatment. AFP is also known as a tumour marker.

Any tests and investigations that your child needs will be explained to you. Our general information on children’s cancers gives details of what the tests and scans involve.

To assess the extent of the cancer, a grouping system called PRETEXT (the 'pre-treatment extent of disease') is used. It uses an MRI scan to measure the amount of cancer in the liver at diagnosis (by an MRI scan), and is used to help establish the best treatment for each child.

This grouping system is unique to liver tumours and is used all over the world, except for in some parts of North America, Germany and Japan.

This grouping process is essential because liver tumours need to be removed surgically. Grouping divides the liver into 4 surgical areas (sectors) and gives an indication right from diagnosis of the kind of surgery needed to remove the tumour. Grouping also helps doctors to know whether a liver transplant surgeon needs to be involved from the start.

• PRETEXT 1 One liver sector is affected and the tumour can be removed by straightforward surgery
• PRETEXT 2 Two sectors are affected and the tumour can be removed with more extensive surgery
• PRETEXT 3 Three sectors are affected and the tumour can be removed with major surgery
• PRETEXT 4 All four sectors of the liver are affected and the tumour cannot be removed without replacing the liver with a donor liver transplant.

Doctors also look at the extent of the spread of cancer beyond the liver:

• In the blood vessels Sometimes the tumour gets into the blood vessels that are entering or leaving the liver. This may affect the type of surgery needed to remove the tumour
• In the abdomen Sometimes the tumour spreads outside the liver and into the abdomen (tummy). This makes it impossible to remove the tumour completely
• In the lungs or other organs If the tumour spreads outside the liver through the bloodstream (called metastatic disease), it usually goes to the lungs. In about 1 in 5 children, their lungs are found to be affected when they are diagnosed. Doctors use x-rays and CT or MRI scans to assess whether the lungs are affected.

Treatment will depend on a number of factors, including the type of liver cancer (hepatoblastoma or hepatocellular carcinoma), the PRETEXT of the tumour, and whether it has spread. Treatment always includes chemotherapy and surgery. For hepatocellular carcinoma, it might also include antiangiogenic treatment or chemoembolisation (these are explained below).

Initially, the diagnosis will be made by taking of a small sample of cells from the tumour (biopsy). This will be done under general anaesthetic, either through the skin or through a cut made in the tummy (abdomen). Once the diagnosis, grouping and extent of spread (if it has spread) have been confirmed, treatment will begin with chemotherapy. Before chemotherapy is given, a risk assessment is made. This helps doctors to determine the type and amount of chemotherapy necessary:

• Standard risk is PRETEXT 1, 2 and 3
• High risk is PRETEXT 4 and disease that has spread beyond the liver to other parts of the body (metastatic disease).

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is usually given as injections and drips (infusions) into a vein. Your child's doctor will discuss with you the type and amount of chemotherapy needed, based on the result of their risk assessment. Sometimes, further chemotherapy is given after surgery.

Chemoembolisation refers to the giving of drugs directly into the vein going into the liver. Very occasionally, this may be used for hepatocellular cancer.

Antiangiogenic treatment is medication that prevents the tumour from developing a blood supply. This is sometimes used in the treatment of hepatocellular carcinoma. The best known antiangiogenic medication is thalidomide. Although this drug is dangerous for pregnant women, it can help to control the growth of cancer cells.

Surgery All children who are able to, will have surgery following chemotherapy. If there was cancer in the lungs, and this has not completely disappeared with the chemotherapy, the lungs will be operated on first. If the liver tumour can be surgically removed, that operation will follow, usually a week or two later.

If the tumour involves all four sectors of the liver (PRETEXT 4), a liver transplant will be necessary. This is recommended for hepatoblastoma, but only in very particular circumstances for hepatocellular carcinoma. In a transplant, the whole liver is removed and replaced with a liver from another person. This will be discussed with you from the beginning, and you will be given the opportunity to think about donating half of your liver, or for your child to have a liver from a donor. The transplant team will be there to answer all of your questions. Liver transplant is only possible if all the cancer outside the liver has gone.

Treatment often causes side effects, and your child's doctor will discuss these with you before treatment starts. Side effects can include: feeling sick (nausea) and vomiting, hair loss, an increased risk of infection, bruising and bleeding, tiredness and diarrhoea.

Late side effects

The chemotherapy used in liver cancer can cause late side effects. These may include hearing problems, kidney problems and possibly heart problems. There will be a slightly increased risk of your child developing another type of cancer later in life. Most children will develop some late effects and so will need to have follow-up. Your child's doctor or nurse will explain more about any possible late side effects, and there is more detail in our general information on children's cancer.

If the cancer comes back after initial treatment, this is known as a recurrence. It can come back in the liver or in other parts of the body. If the cancer comes back, often (before anything is seen on scans) the levels of alpha-fetoprotein (AFP) in the child's blood will start to rise again, and reaches levels of over 100. Small rises in AFP can occur in the weeks after surgery, as the liver regenerates as much as it can.

Many children have their treatment as part of a clinical research trial. Trials aim to improve our understanding of the best way to treat an illness (usually by comparing the standard treatment with a new or modified version of the standard treatment). Specialist doctors carry out trials for children's cancer. Your child's medical team will talk to you about taking part in a clinical trial (if appropriate) and will answer any questions you may have. Written information is often provided to help explain things. Taking part in a research trial is completely voluntary, and you'll be given plenty of time to decide if it is right for your child.

When the treatment ends, your child will have regular blood tests to measure the level of AFP in the blood (if appropriate), as well as scans and chest x-rays. More than three quarters of the children with hepatoblastoma are cured, and for children with small tumours that are confined to the liver, the outlook is even better. The outcome for hepatocellular carcinoma is not quite as good. The staff at the hospital can give you information about the likely outcome for your child. Our nurses can also offer support.

If you have specific concerns about your child’s condition and treatment, it is best to discuss them with your child’s doctor, who knows the situation in detail.

As a parent, the fact that your child has cancer is one of the worst situations you can be faced with. You may have many different emotions, such as fear, guilt, sadness, anger and uncertainty. These are all normal reactions, and are part of the process that many parents go through at such a difficult time.

Your child may have a range of powerful emotions throughout their experience of cancer. Our booklet, Peppermint Ward, is a storybook for younger children with cancer. It looks at the issues that they and their family may face, and helps them to explore their feelings.

This section has been compiled using information from a number of reliable sources, including:

• Cancer in Children: Clinical Management (5th ed). Eds PA Voute et al. Oxford University Press, 2005.
• Oxford Textbook of Oncology (2nd edition). Eds. Souhami et al, Oxford University Press, 2002.

For further references, please see the general bibliography.