There are two main types of primary liver cancer:
- Hepatoblastoma - this usually occurs in children under five years of age. About 17 children in the UK are diagnosed with hepatoblastoma each year.
- Hepatocellular carcinoma (HCC) - this is rarer and usually occurs in older children. About three children in the UK are diagnosed with hepatocellular carcinoma each year.
The liver
The liver is the largest organ in the body and is divided into sections called lobes.
It is in in the upper part of the tummy and is larger on the right-hand side of the body than on the left. It is surrounded, and protected, by the lower ribs.
The liver is good at repairing itself. It can still do its job even if only a small part of it is working. The liver is a vital organ that has lots of important jobs:
- makes proteins to help prevent too much bleeding
- helps maintain the balance of fluid in the body
- breaks down harmful substances so that they can be passed out in wee (urine) or poo (stools)
- breaks down food and converts it to energy
- stores sugars and fats for when they are needed
- produces bile which breaks down the fats in food so that they can be absorbed by the body
Causes
We don’t know what causes most liver tumours. In some parts of the world, hepatocellular carcinoma (HCC) is related to liver infection. This happens in countries where hepatitis B is common in pregnant mothers and vaccination is not readily available for children at birth. Children who are infected with hepatitis B have a higher risk of developing HCC than uninfected children. There is a link to increased rates of hepatoblastoma in premature and low birth weight babies. It is important to remember that nothing you have done has caused your child’s cancer.
Signs and symptoms
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 being sick (vomiting).
How liver tumours are diagnosed
A variety of tests and investigations may be needed to diagnose a liver tumour.
An ultrasound scan and x-rays will be taken to show if there is a tumour in the liver. Further tests, including CT or MRI scans, of the tummy and chest will be done to find out the extent of the disease. Blood tests will also be carried out.
Most liver tumours produce a protein called alpha-fetoprotein (AFP). The levels of AFP in the blood can be measured and this 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 by your child’s medical team.
Grouping
All liver tumours are assessed by using a grouping system called PRETEXT (pre-treatment extent of disease). It helps your child’s doctors to decide which treatment is best for your child.
The grouping system is essential because liver tumours need to be removed surgically. The grouping divides the liver into four areas and helps decide the kind of surgery that is needed. Grouping also helps doctors decide whether a liver transplant surgeon needs to be involved from the start or not.
PRETEXT 1 - One liver sector is affected and the tumour can be removed by straightforward surgery.
PRETEXT 2 - Two adjoining sectors are affected and the tumour can be removed with more extensive surgery.
PRETEXT 3 - Two or three sectors are affected with no two adjoining sectors free of disease, and the tumour can be removed with major surgery but sometimes may require a liver transplant.
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 whether the cancer has spread beyond the liver:
In the blood vessels - sometimes the tumour gets into the blood vessels which enter or leave the liver. This may affect the type of surgery needed.
In the tummy (abdomen) - sometimes the tumour spreads outside the liver and into the tummy.
In the lungs or other organs - if the tumour spreads outside the liver (metastatic disease), it usually goes to the lungs. About 1 in 5 children are found to have affected lungs when they are diagnosed. X-rays and CT scans are used to see whether the lungs are affected.
Treatment
Treatment depends on the type of liver cancer and the grouping of the tumour. Your child’s doctor will discuss the treatment options with you.
Hepatoblastoma
Treatment will usually be a combination of chemotherapy and surgery. The exact timing of surgery and the intensity of chemotherapy is decided using a combination of ‘risk’ factors which includes grouping, whether the tumour has spread, AFP levels and the age of the child.
- Very low risk tumours are usually small and only in the liver. It may be safe to remove the tumour at diagnosis. Most children will need some chemotherapy after surgery.
- Low risk tumours are only in the liver but cannot be removed at diagnosis. Chemotherapy will be given to shrink the tumour before it is removed. More chemotherapy is usually given after surgery.
- Intermediate risk tumours have other risk factors but are usually only in the liver. Intense chemotherapy is given at the start. Many children will be able to have their tumour removed with standard surgery, some children will need a liver transplant to safely remove all of the tumour. More chemotherapy is usually given after surgery.
- High risk tumours have usually spread outside of the liver (metastases). Intense chemotherapy is given at the start to try and clear the disease that has spread outside the liver before having surgery or a transplant. If the cancer outside of the liver is not cleared after initial chemotherapy, treatment may be more challenging and more courses of chemotherapy may be needed.
Hepatocellular carcinoma
For hepatocellular carcinoma, surgery is the main treatment. If the tumour is small enough, it will usually be removed at diagnosis. Chemotherapy may be given afterwards. If the tumour is too large to remove at first, or has spread, chemotherapy is given to shrink it so that surgery can be done later.
Hepatocellular tumours don’t always respond well to chemotherapy, so other treatments, such as chemoembolisation and targeted drugs, may be used.
Chemotherapy
Chemotherapy is the use of anti-cancer drugs to destroy cancer cells. It is usually given directly into a vein (intravenously).
Surgery
All children, if they are able to, will have surgery, either upfront (very low risk group), or after chemotherapy. If there’s still cancer in the lungs, this will usually be operated on first. If the liver tumour can be surgically removed, this operation usually follows shortly after.
If the tumour involves all four sectors of the liver (pretext 4), a liver transplant will probably be needed. This is recommended for hepatoblastoma but only in some 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 to your child, or for your child to have a liver from a donor. A liver transplant is only possible if all the cancer outside the liver has gone. The transplant team will be able to answer all of your questions.
Chemoembolisation
Chemoembolisation is giving drugs directly into the artery going into the liver. This may be used for hepatocellular cancer.
Targeted drugs
Targeted drugs work differently to conventional chemotherapy. Some drugs may cause cancer cells to die directly. Others cut off the blood supply to the tumour cells (antiangiogenic drugs). Some drugs, called multi-targeted agents, work in both ways.
Clinical trials
Many children have their treatment as part of a clinical research trial. Clinical trials are carried out to try to improve our understanding of the best way to treat an illness, usually by comparing the standard treatment with a new or modified version. Clinical trials mean there are now better results for curing children’s cancers compared with just a few years ago.
Your child’s medical team will talk to you about taking part in a clinical trial and will answer any questions you have. Taking part in a research trial is completely voluntary, and you’ll be given plenty of time to decide if it’s right for your child. You may decide not to take part, or you can withdraw from a trial at any stage. You will then receive the best standard treatment available.
National treatment guidelines
Sometimes, clinical trials are not available for your child’s tumour. In these cases, your doctors will offer the most appropriate treatment, using guidelines which have been agreed by experts across the UK. Children’s Cancer and Leukaemia Group (CCLG) is an important organisation which helps to produce these guidelines.
Recurrence
If the cancer comes back after initial treatment, often (before anything is seen on scans) the levels of alpha-fetoprotein (AFP) in the child’s blood will start to rise again. Small rises in AFP can happen in the weeks after surgery, as the liver repairs itself.
Your doctor will discuss all the treatment options with you.
Follow-up care
More than three quarters of 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.
Once treatment has finished, the doctors will monitor your child closely with regular appointments to be sure that the cancer has not come back and there are no complications. 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. After a while you will not need to visit the clinic so often.
If you have specific concerns about your child’s condition and treatment, it’s best to discuss them with your child’s doctor, who knows their situation in detail.