Hepatoblastoma is a very rare liver cancer (malignant tumor) that affects babies and toddlers ages 1 to 3. It’s treated with surgery. Rarely, hepatoblastoma may spread outside children’s livers. Hepatoblastoma may be “cured” with chemotherapy to shrink cancerous tumors and surgery to remove sections of the liver affected by cancer.


What is hepatoblastoma?

Hepatoblastoma (pronounced “hep-ah-to-blas-to-mah”) is a very rare liver cancer that typically affects children ages 1 to 3. Surgeons may be able to cure hepatoblastoma if it’s diagnosed when liver cancer tumors are small enough to be completely removed with surgery. Medical researchers are evaluating ways to treat hepatoblastoma that surgery can’t remove or that’s spread (metastasized).

How common is this condition?

Hepatoblastoma is very rare. A global study estimates it affects 1 to 2 in 1 million children.


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Symptoms and Causes

What are hepatoblastoma symptoms?

Hepatoblastoma develops over time. If your child has hepatoblastoma, you may not notice any changes in your child’s body until the tumor in their liver has grown large enough to affect their overall health or behavior. Common symptoms include:

What causes hepatoblastoma?

Medical researchers haven’t identified the exact cause, but they have pinpointed some risk factors, including certain genetic (inherited) conditions. Risk factors include:

  • Premature birth: Most babies are born at 37 weeks or later. A baby born before 37 weeks is considered premature.
  • Low birth weight: Babies weighing less than 5.5 pounds at birth.
  • Early exposure to hepatitis B, which attacks your child’s liver.
  • Biliary atresia: This condition affects newborn babies, keeping bile from flowing to their small intestines. The backed-up bile damages their livers.
  • Hemihyperplasia: This condition causes one side of your child’s body to be larger than the other side.
  • Beckwith-Wiedemann syndrome: This inherited growth disorder increases the risk of developing childhood cancer, including hepatoblastoma.
  • Familial adenomatous polyposis (FAP): People with this condition are born with a mutated APC gene. FAP causes pre-cancerous polyps in your child’s large intestine.
  • Aicardi syndrome: This condition affects your child’s brain and eyes and causes them to have seizures.
  • Glycogen storage disease (GSD): This disease affects the way your child’s body processes glucose (sugar).
  • Simpson-Golabi-Behmel syndrome (SGB): Children born with SGB grow and gain weight at an unusually rapid rate. They may develop conditions in early childhood, including umbilical hernias and tumors.
  • Edwards syndrome (trisomy 18): Recent studies have some links between this inherited disorder and hepatoblastoma in the small number of children with Edwards syndrome who live for more than a year.


What are the complications of hepatoblastoma?

Hepatoblastoma may spread (metastasize) to other areas of your child’s body, particularly to their lungs.

Diagnosis and Tests

How is hepatoblastoma diagnosed?

Healthcare providers use several tests to diagnose hepatoblastoma. Those tests include:

My child may be upset and scared during these tests. How can I help them?

Tests are essential. But needles can sting and MRI machines make loud, scary noises, so it makes sense if your child becomes tearful or upset before or during tests. Talk to your child’s healthcare provider. They may recommend you work with a child life specialist to help you and your child. Here are some ways you can make essential tests easier:

  • Many times, children cope better with pain and tests if they know what to expect. Ask your child’s provider to walk you through the test so you can talk to your child about the process.
  • Play “let’s pretend” with your child, using the test as the game. For example, if your child will have an MRI, you and your child can practice holding still for the same amount of time the procedure will take.
  • Very young children want their parents nearby. Ask your provider about your child’s tests so you know when you can stay with your child and when you can’t.
  • When you can stay with your child, plan ways to distract them during the procedure like reading a favorite book, telling a favorite story or helping them imagine a favorite activity. Sometimes, simply being there to hold their hand is all they need.
  • When you can’t stay with your child, ask if they can have a favorite soft toy or blanket to hold during the test. It may help to tell your child that even if they can’t see you, you’ll be right there during the test.
  • Do your best to stay calm for your child’s sake.

How do healthcare providers stage hepatoblastoma?

Many healthcare providers use a process called pre-treatment extent of disease (PRETEXT) to stage or evaluate your child’s condition so they can create a surgical treatment plan.

Your liver has two lobes. Providers who use PRETEXT to stage hepatoblastoma divide the two lobes into four sections, or groups. Each group is based on the number of liver sections with tumors. The more sections with tumors, the higher the group number.

Under the PRETEXT system, providers establish a stage for the tumor in your child’s liver by treating them with chemotherapy before surgery. Chemotherapy may eliminate some tumors and shrink others, enabling providers to remove entire tumors, essentially eliminating the cancer.

For example, if your child has two tumors in two different sections of their liver, providers may say your child has a Group II cancer. But if pre-surgery chemotherapy eliminated one of the tumors so there’s a tumor in one section only, your child’s condition would be classified as a Group I cancer.


Management and Treatment

How is hepatoblastoma treated?

Healthcare providers typically treat hepatoblastoma with partial hepatectomy, removing the parts of your child’s liver that have tumors. They may combine surgery with the following treatments:

  • Chemotherapy: Providers may use chemotherapy to shrink tumors before surgery. Chemotherapy may be systemic or local. Systemic chemotherapy travels throughout your child’s body. Local chemotherapy directly targets the tumor in your child’s liver.
  • Transarterial chemoembolization (TACE): This treatment works the same way you might build a dam in a stream to stop the stream from flowing. Providers inject anti-cancer drugs into one of the arteries that supplies blood to your child’s liver. Then, they inject a substance to block the artery. The substance acts like a dam, keeping the anti-cancer drugs swirling around the tumor instead of floating away.
  • Radiation therapy: Providers may use radiation therapy after surgery to remove any remaining cancer cells or as an alternative way to treat tumors that surgery can’t remove.
  • Ablation therapy: This treatment destroys or removes tissue. Providers use ablation therapy to treat recurring hepatoblastoma.
  • Liver transplantation: Your child’s surgeon may recommend a liver transplant if tumors in your child’s liver are too large to remove with surgery.

What are treatment side effects?

All treatments may have some immediate side effects that vary based on the kind of treatment, your child’s overall health and how they react to treatment. Some treatments may cause late effects. Late effects are health issues that appear months and years after your child completes treatment.

What happens if my child’s hepatoblastoma spreads outside their liver?

Healthcare providers may recommend additional surgery to remove tumors that spread from your child’s liver to other areas of their body.


Can hepatoblastoma be prevented?

Medical researchers don’t know exactly what causes hepatoblastoma, so they can’t say what prevents it. They do know there are some risk factors that may increase your child’s risk of developing the condition.

If you’re worried your child may be at risk, ask their healthcare provider for help. Depending on your child’s situation, their provider may recommend imaging tests and blood tests every three months until your child is 4 years old. (Children at risk of hepatoblastoma typically develop symptoms between ages 1 and 3.)

Outlook / Prognosis

Can hepatoblastoma be cured?

Yes, but it depends on when your child is diagnosed and receives treatment. Many times, healthcare providers can use surgery to remove smaller tumors that were diagnosed early on, before the tumors could grow larger, and spread to other parts of your child’s liver or other parts of their bodies.

  • About 90% of children treated early on were alive five years after diagnosis.
  • About 75% to 85% of children who had larger tumors were alive five years after diagnosis.

Sometimes, children treated with partial hepatectomy develop new tumors in the same area as their original tumor. This isn’t common. When hepatoblastoma comes back, providers may use the same sorts of treatment that eliminated the first tumor. They may also recommend your child participate in a clinical trial.

Unfortunately, many children develop additional cancers later in life. Talk to your child’s healthcare provider about any additional cancers your child may develop. They may recommend setting up regular screening tests to detect new cancers and treat those cancers before they take hold. Providers also screen for long-term side effects from chemotherapy or surgery.

Living With

What should I expect once my child has finished treatment?

As each child’s situation and treatments are different, ask your healthcare provider what you may expect, including specific late effects your child may have. They’ll have suggestions for monitoring your child’s health and well-being.

When should my child see their healthcare provider?

Your child should have regular follow-up appointments so their provider can monitor their overall health and look for signs of returning hepatoblastoma, a new cancer or late effect health issues.

When should I take my child to the emergency room?

Hepatoblastoma treatment typically involves surgery. Surgery can have complications, including infection. You should take your child to the emergency room if they:

  • Develop a fever that’s higher than 100.4 degrees Fahrenheit (38.3 degrees Celsius).
  • You notice their surgery site is turning dark red and/or is very tender to the touch.
  • You notice green or yellow substances oozing from your child’s surgery site.

What questions should I ask my child’s healthcare provider?

Hepatoblastoma is a very rare liver cancer. Here are some questions you may want to ask.

  • What is hepatoblastoma?
  • How does this disease affect my child?
  • What stage is my child’s hepatoblastoma?
  • What are treatment options?
  • If surgery is an option, what surgery do you recommend, and why?
  • What are surgery side effects?
  • Are there other treatments?
  • What are these treatments’ side effects?
  • Can these treatments cure my child?
  • What is my child’s prognosis?

A note from Cleveland Clinic

Hepatoblastoma is a very rare liver cancer that affects 1 in 1 million babies and toddlers. If your child is that one in a million, you’re probably dealing with a whirl of emotions. You may be worried and afraid your child. You may feel overwhelmed, trying to stay calm on the outside while you’re crumbling on the inside. It may help to know many children are “cured” after surgery to remove cancerous liver tumors. It may also help to know your child’s care team understands what you’re going through. They’ll always be willing to explain what you can expect. They’ll be there for you and for your child, from diagnosis to treatment and beyond.

Medically Reviewed

Last reviewed on 03/10/2023.

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