Hepatoblastoma: childhood liver cancer, symptoms, diagnosis and treatment

The Royal Marsden describes hepatoblastoma as a malignant (cancerous) tumour of the liver that usually occurs in young children. It is different from hepatocellular carcinoma, which usually occurs in adults and occasionally in older children. The Children's Cancer and Leukaemia Group (CCLG) says there are two main types of primary liver cancer in children, hepatoblastoma, which usually occurs in children under five years of age, and hepatocellular carcinoma, which is rarer in children and usually occurs in older children.

The liver is described by the CCLG as the largest organ in the body, sitting in the upper part of the tummy, larger on the right-hand side, and protected by the lower ribs. It makes proteins that help prevent too much bleeding, helps maintain fluid balance, breaks down harmful substances, converts food to energy, stores sugars and fats and produces bile. The CCLG notes that the liver is good at repairing itself and can still do its job even if only a small part of it is working, which is one reason surgery to remove part of the liver is possible.

Children with Cancer UK describes liver cancer as accounting for 1% of childhood cancers, with hepatoblastoma the most common type and more common in boys.

The CCLG says we do not know what causes most liver tumours. The Royal Marsden says hepatoblastoma is not associated with previous hepatitis, unlike hepatocellular carcinoma, and that usually there are no associated genetic factors. It adds that there may be an increased risk in children who have a family history of familial polyposis coli, or who have a condition called Beckwith-Wiedemann syndrome.

The CCLG notes a link to increased rates of hepatoblastoma in premature and low birth weight babies. Children with Cancer UK says that a very small number of cases occur in association with congenital anomalies and familial conditions. These are associations that help researchers understand risk; they do not mean a parent could have prevented the cancer.

The CCLG says the most common symptom is a lump or swelling in the abdomen, which can be painful. Other possible symptoms are weight loss, loss of appetite, feeling sick and being sick. The Royal Marsden lists a lump in the tummy together with poor appetite, weight loss, lethargy, fever, vomiting and jaundice.

Many of these signs are non-specific and have common explanations in children. A persistent swelling or lump in a child's abdomen is worth showing to a GP, who can arrange an ultrasound scan and refer on if needed. If a child seems seriously unwell, use NHS 111 or emergency services.

The CCLG says an ultrasound scan and X-rays will show if there is a tumour in the liver, and that further tests, including CT or MRI scans of the tummy and chest, are done to find out the extent of the disease, along with blood tests. The Royal Marsden lists blood samples, a chest X-ray to see if the tumour has spread to the lungs, an ultrasound to show position and size, CT or MRI of the abdomen and a CT scan of the lungs, and a biopsy, which is usually a small operation under general anaesthetic, to make the diagnosis.

Most liver tumours produce a protein called alpha-fetoprotein, or AFP. The CCLG says levels in the blood can be measured and are a useful indicator of whether the tumour is responding to treatment or may have come back, which is why AFP is called a tumour marker.

The CCLG says all liver tumours are assessed using a system called PRETEXT (pre-treatment extent of disease). It divides the liver into four areas and helps decide what kind of surgery is needed, and whether a liver transplant surgeon needs to be involved from the start. PRETEXT 1 means one liver sector is affected and the tumour can be removed with straightforward surgery, PRETEXT 2 two adjoining sectors, PRETEXT 3 two or three sectors with no two adjoining sectors free of disease, and PRETEXT 4 all four sectors, where the tumour cannot be removed without replacing the liver.

Doctors also look at whether the cancer has spread beyond the liver, into blood vessels, into the tummy or into the lungs or other organs. The CCLG says that if it spreads outside the liver it usually goes to the lungs, and about 1 in 5 children are found to have affected lungs at diagnosis. The Royal Marsden divides treatment into standard risk, where the tumour is confined to the liver and involves at most three segments, and high risk, where it involves all four segments and/or has spread outside the liver.

The CCLG says treatment for hepatoblastoma is usually a combination of chemotherapy and surgery, and the timing of surgery and intensity of chemotherapy are decided using risk factors including grouping, whether the tumour has spread, AFP levels and the age of the child. The Royal Marsden similarly says that treatment usually includes chemotherapy and surgery, and occasionally liver transplantation.

The CCLG says all children who are able will have surgery, either upfront or after chemotherapy. If the tumour involves all four sectors of the liver, a liver transplant will probably be needed, which it says is recommended for hepatoblastoma. In a transplant the whole liver is removed and replaced with a liver from another person. The family is told about this from the beginning and may be given the opportunity to think about donating half of their liver to their child, or the child may have a liver from a donor. A transplant is only possible if all the cancer outside the liver has gone.

The CCLG notes that many children have treatment as part of a clinical research trial, that taking part is voluntary and that you will be given time to decide. When no trial is available, doctors use guidelines agreed by experts across the UK.

Outcomes depend on risk group and whether the tumour can be completely removed. Children with Cancer UK gives a five-year survival rate of 73% for childhood liver cancer overall, a figure for liver cancer in children as a whole rather than for hepatoblastoma alone. Your child's own team is the right source for what the outlook means for them.

A clinical nurse specialist and the wider team at a specialist children's cancer centre support families through treatment. Charities such as the CCLG and Children with Cancer UK provide information for parents, and it is worth asking your team about psychological support for the whole family.

Because hepatoblastoma is so rare, it is managed by specialist children's cancer teams, with surgery carried out by a specialist liver surgeon and a transplant team involved where needed. Families sometimes seek a second opinion privately, which can sit alongside NHS care. Whichever route you use, the child's pathology, PRETEXT group and AFP results should travel with them.