LIVER CANCER (HEPATOBLASTOMA) IN CHILDREN

Patient Education Material

The Liver

The liver is the largest solid organ in the human body. It lies within the tummy on the right side of the ribs and just below the lungs. The liver has two lobes and is further divided into 8 segments.

The Liver and Bile Duct System

Unlike other organs in the human body and the liver’s blood supply is double by two vessels that supply blood, oxygen, and nutrients. The portal vein brings blood from the digestive system and accounts for 70-80% of the liver’s blood supply. The hepatic artery carries 20-30% of blood to the liver. Liver tumors get to take blood and, supply from branches of the hepatic artery.  The liver is your body’s metabolic factory and helps to control more than 500 chemical reactions, including:

  • Storing carbohydrates
  • Breaking down proteins
  • Converting ammonia to urea a substance found in urine.
  • Using fats to provide energy
  • Producing cholesterol which is needed for the production of bile (which in turn helps to digest fats in the food) and hormones.
  • Storing minerals and vitamins.
  • Processing blood (breaking down old red blood cells, and producing clotting factors).

Hepatoblastoma:

It is a malignant (cancerous) tumor of the liver which occurs in young children. This is a different type of cancer than the adult type of liver cancer (hepatocellular cancer or HCC). The true incidence of this tumor in Pakistan is unknown. The average age at diagnosis one a year and most cases occur before two years of age. Hepatoblastoma not associated with previous hepatitis because unlike hepatocellular carcinoma. Usually, there are no associated genetic factors. There may an increased risk of hepatoblastoma in children who have a family history of familial polyposis cold, 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 tumor. This will help us to decide and 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 tumor marker and is used to monitor response to treatment.
  • Chest X-ray – this will tell us if the tumor has spread to the lungs.
  • Ultrasound scan – this will tell us the position and size of the tumor. 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 and information about the tumor and may tell us if it has spread.
  • Biopsy – This is usually carried out under general anesthetic under ultrasound guidance. The radiologist will take a small piece of tissue from the lump with a small needle. The biopsy will help us to make a diagnosis.

Staging

Staging a measure of how far the tumor has spread beyond its original site. For treatment purposes so there are two groups. These are ‘standard risk’ and ‘high risk’ as follows:

  • Standard risk is when the tumor is confined to the liver and involves at most three segments of the liver.
  • High risk when the tumor involves all four segments of the liver and/or the tumor spread outside of the liver.
Pre-text staging system is used for staging of hepatoblastomaPre-text staging system of hepatoblastoma

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 tumor. The aim to remove the tumor completely and this is undertaken by a specialist liver surgeon. If it not possible to remove the tumor completely after further chemotherapy, then liver transplantation considered.

Chemotherapy

Treatment with anti-cancer drugs used to destroy cancer cells. It 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 tumor.

Effects of treatment

  • The prognosis depends on the risk group of the tumor, 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 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 common.

  • A small number of children develop side effects many years later because of the treatment they have received. These may include hearing problems or impaired heart and kidney function, and a small risk of developing second cancer and 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 and indefinitely. so that we can help with any long-term effects of the treatment.