Hepatoblastoma in Children

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Hepatoblastoma is a very rare cancer that starts in the liver. It usually affects children younger than 3 years of age. About half of all children with it are diagnosed before age 1. It usually doesn't spread (metastasize) to other parts of the body.

Causes

The cancer is caused by gene changes in liver cells. It's not known why this happens.

Children Who Are at Risk for Hepatoblastoma

Some genetic health conditions increase a child's risk. These include:

• Beckwith-Wiedemann syndrome.
• Familial adenomatous polyposis.
• Glycogen storage disease.
• Aicardi syndrome.
• Simpson-Golabi-Behmel syndrome.

Babies born at a very low birth weight also seem to be at higher risk.

Children with these types of genetic health conditions may benefit from having tests done to check for cancer before they have any symptoms.

Symptoms

Symptoms depend on the size of the tumor. They tend to be different in each child. They can include:

• Lump (mass) in the belly (abdomen).
• A swollen belly.
• Pain in the belly.
• Loss of appetite.
• Weight loss.
• Feeling tired.
• Nausea and vomiting.
• Yellow color to the skin or whites of the eyes (jaundice).
• Fever.
• Itchy skin.

The symptoms of hepatoblastoma are a lot like those of many other health conditions. It's important to take your child to see a doctor if you notice these symptoms. Only a doctor can tell if your child has cancer.

Diagnosis

You may take your child to a doctor because of a lump, swelling, pain in the belly (abdomen), or other symptoms. The doctor will ask about your child's symptoms. A physical exam, focusing on the abdomen, will be done. Your child may need to see a pediatric oncologist. This is a doctor with special training in diagnosing and treating cancer in children. Your child may need tests, such as:

• Blood tests. These tests look for signs of illness. They check for blood clotting problems, liver and kidney function, tumor markers, gene problems, and more.
• Ultrasound exam. Sound waves are used to create images of the inside of your child's belly. This is often one of the first tests used to look at the liver.
• CT (computed tomography) scan. A series of X-rays taken from different angles and a computer are used to make images of the inside of the body. This may be done to look at blood vessels in the liver.
• MRI (magnetic resonance imaging). Large powerful magnets, radio waves, and a computer are used to make detailed images of the inside of the body. This may also be done to look at blood vessels in the liver.
• Tumor biopsy. A tiny piece (called a sample) of the tumor can be taken and checked for cancer cells. A biopsy is needed to diagnose hepatoblastoma. It may be done with a needle or during surgery. Tests on the sample may be done to check for gene mutations.

After a diagnosis of hepatoblastoma, your child will need more tests. These help your child's doctors learn more about the cancer. They'll show how big the tumor is and how far the cancer has spread inside your child's liver. Two groupings are then assigned:

• The PRETEXT group is assigned at the time of diagnosis. It describes the tumor before treatment starts.
• The POSTTEXT group describes the tumor after treatment.

Both groupings use Roman numerals and can have a value of I (1), II (2), III (3), or IV (4). The higher the number, the more parts (lobes) of the liver involved and the more advanced the cancer is.

These groupings are important to know when deciding how to treat the cancer. Be sure to ask your doctor to explain your child's PRETEXT and POSTTEXT groupings.

Treatment

Talk with your child's health care team about your child's treatment choices, the goals of treatment, and what the risks and side effects may be.

Treatment will depend on the PRETEXT group number (I to IV) and other factors. Hepatoblastoma can be treated with any of these:

• Surgery. This may be done to take a sample of the tumor for diagnosis. It's also done to remove as much of the tumor as possible . This can be a partial hepatectomy (part of the liver is removed) or a total hepatectomy (all of the liver is removed). Surgery can be used to remove tumors in other parts of the body, like the lungs.
• Chemotherapy. These are strong medicines that kill cancer cells. They may be given before or after surgery. They are given right into the blood through a vein (IV), as a shot (injection), or by mouth (oral). The medicines may also be put right into the liver. This is done with a tube (catheter) that goes into the liver's main artery. This is called chemoembolization of hepatic artery. The chemo is mixed with a substance that blocks the flow of blood to the tumor. This keeps the tumor from growing and spreading.
• Liver transplant. If a tumor can't be removed, the entire liver may be removed and replaced with a liver from a donor.
• Radiation therapy. These are high-energy X-rays or other types of radiation. Radiation is used to kill cancer cells or stop them from growing. It isn't often used to treat hepatoblastoma. But radioembolization is an internal radiation therapy and might be a choice in some cases. To do this, tiny radioactive beads are put into the main artery in the liver that goes to the tumor. The beads collect there and the radiation kills nearby cancer cells.
• Clinical trials. Most children with cancer are treated as part of a clinical trial. Taking part in a clinical trial means your child gets the best treatment available today, and might also get new treatments that are thought to be even better. Before starting treatment, ask your child's doctor if there are any treatments being tested that may work well for your child.
• Supportive care. Treatment can cause side effects. Medicines and other treatments can be used for pain, fever, infection, and nausea and vomiting.

Your child may have just one treatment or a combination of treatments. Tests will be done during treatment to see how well it's working.

With any cancer, how well a child is expected to recover (prognosis) varies. Keep in mind:

• Getting medical treatment right away is important for the best outcomes. Cancer that has spread is harder to treat.
• Ongoing follow-up care during and after treatment is needed.
• New treatments are being tested to improve outcomes and reduce side effects.

Possible Complications

Problems (complications) can be caused by the tumor or treatment. These include:

• Infections and bleeding from surgery.
• Increased infections, bruising and bleeding, vomiting, diarrhea, hair loss, and tiredness with some chemotherapy medicines.
• Problems with growth and development.
• Heart, kidney, or lung problems.
• Problems with liver function.
• Spreading cancer (metastasis).
• Cancer that grows back.
• Growth of new cancers later in life.
• Rejection of the new liver or problems with antirejection medicines, or both, if a liver transplant is needed.

Helping Your Child Live with Hepatoblastoma

A child with a hepatoblastoma needs ongoing care. Your child will be seen by oncologists and other health care providers to treat any late effects of treatment and to watch for signs or symptoms of the tumor returning. Your child will be checked with imaging tests and other tests. And your child may see other providers for problems from the tumor or from treatment.

You can help your child manage cancer treatment in many ways. For instance:

• Your child may have trouble eating. A dietitian may be able to help.
• Your child may be very tired. They will need to balance rest and activity. Encourage your child to get some exercise. This is good for overall health. And it may help to reduce tiredness. Ask your child's doctor what exercises are safe for your child.
• Get emotional support for your child. Find a counselor or a children's support group that can help.
• Make sure your child attends all follow-up appointments.

When to Contact Your Child’s Doctor

Your child's doctor will talk with you about when to call. You may be told to call if your child has:

• Signs of infection, such as fever of 100.4°F (38.0°C) or higher, or as directed by your child's doctor.
• Symptoms that get worse.
• New symptoms.
• Side effects from treatment that affect their daily function or don't get better with treatment.

Ask your child's doctor what signs to watch for and when to call. Know how to get help after office hours and on weekends and holidays.