Hepatic tumours

03 May 2011

Tumours of the liver are rare in childhood; around 18 children are diagnosed each year in Britain, about one per cent of all childhood cancers.

Eighty per cent of childhood hepatic tumours are hepatoblastomas, a type of embryonal tumour; the remainder are hepatic carcinomas.

Incidence

Hepatic tumours account for one per cent of childhood cancers.

Eighty per cent of childhood hepatic tumours are hepatoblastomas, a type of embryonal tumour; the remainder are hepatic carcinomas.

Incidence of hepatoblastoma is highest in the first two years of life, declining steeply thereafter. Hepatoblastomas are more common in boys.

Incidence of hepatic carcinomas increases with age, with the majority of cases diagnosed at age 10-14 years. Boys and girls are affected equally.

In addition to hepatoblastoma and hepatic carcinomas, other types of childhood cancer can occur with the liver as the primary site. These include s oft tissue sarcomas, lymphomas, neuroblastoma and germ-cell tumours. Inclusion of these tumours increases the total incidence of liver cancer in childhood by about a quarter.

Causes

Some reports suggest possible connections between environmental exposures and hepatoblastoma, including parental occupational exposure to metals, petroleum products and paints or pigments.

There also appears to be a significant association between hepatoblastoma and low birth weight. A very small number of cases of hepatoblastoma occur in association with congenital anomalies and familial conditions.

Hepatic carcinomas are more common in countries that have a high incidence of hepatitis B; children who are infected with hepatitis B have a higher risk of developing hepatic carcinoma than uninfected children.

Survival

Overall five-year survival from hepatic tumours is 66 per cent.

Within this, the rate for hepatoblastoma is better at around 75 per cent.

The outlook for children with hepatic carcinoma, however, is very poor – only 20 per cent survive.

Diagnosis

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

A variety of tests and investigations will be carried out to confirm the diagnosis if a hepatic tumour is suspected.

An ultrasound scan and/or x-rays should reveal the presence of a tumour in the liver.

A biopsy will be taken to confirm the diagnosis.

Blood tests will also be carried out. Most hepatic cancers produce a protein called alpha-fetoprotein (AFP). This is known as a tumour marker. It is possible to measure levels of AFP in the blood – this can be a useful indicator of whether the tumour is responding to treatment or whether it may have come back after treatment.

Treatment

Liver tumours need to be removed surgically. A system called PRETEXT (the ‘pre-treatment extent of disease’) uses MRI scanning to establish the extent of the tumour in the liver and to determine whether it has spread to other parts of the body.

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 a liver transplant is required

PRETEXT 1, 2 and 3 tumours are classed as ‘standard risk’. PRETEXT 4 is ‘high risk’ and includes tumours which have spread to other parts of the body (metastatic disease).

For hepatoblastoma, chemotherapy is given first – to shrink the tumour and get rid of the metastatic tumours.

Surgery to remove the remaining tumour will take place a few weeks after chemotherapy. Further chemotherapy is usually given after surgery to make sure all last traces of the disease are destroyed.

The role of chemotherapy in the treatment of hepatic carcinomas is less certain. If the tumour is not too large, surgery usually takes place straight away and chemotherapy may be given afterwards. If the tumour is too large to remove straight away it needs to be shrunk using chemotherapy.

If there is cancer in the lungs - and this is not completely destroyed by chemotherapy - the lungs will be operated on first. If the liver tumour can be surgically removed, that operation will usually follow a week or two later.

A liver transplant will be necessary if the tumour involves all four sectors of the liver (PRETEXT 4). This is only possible if all the cancer outside the liver has gone. A liver transplant involves removal of the whole liver and replacement with a liver from a donor. It may be possible for a parent to donate half of their liver.

Side effects of treatment

Treatment often causes side effects and these will be discussed with you before treatment starts.

The possible side effects depend on the treatment given and the part of the body being treated but may include nausea and vomiting, hair loss, reduced resistance to infection, bruising and bleeding, tiredness and diarrhoea.

These problems are all temporary and can be minimised with good supportive care.

Read more: Side effects of treatment

Long-term follow up

Children will have regular follow-ups to check for any recurrence of the cancer and for any problems which may arise as a result of the treatment they were given.

The cancer could recur 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 reach levels of over 100. Small rises in AFP can occur in the weeks after surgery, as the liver regenerates as much as it can.

The chemotherapy used to treat liver cancer can cause late side effects. These may include hearing problems, kidney problems and possibly heart problems. The child will have a slightly increased risk of developing another type of cancer later in life.

Read more: Long-term and late effects of treatment

References:
Cancer in Children: Clinical Management (5th edition). Eds: P A Voute, Ann Barrett, Michael C G Stevens and Hubert N Caron. Oxford University Press, 2005.
Childhood Cancer in Britain: incidence, survival, mortality. Ed by Charles Stiller. Oxford University Press, 2007.
Macmillan Cancer Support factsheet – Liver tumours in children (2010).

Related Topics Hepatic tumours, types of childhood cancer