Hepatoblastoma (Liver Cancer)

Overview

What is hepatoblastoma?

Hepatoblastoma (hep″ah-to-blas-to´mah) is a very rare liver cancer that typically affects children ages 1 to 3. Healthcare providers treat hepatoblastoma with chemotherapy and surgery, including liver transplantation. Approximately 75% to 80% of children with hepatoblastoma are alive five years after diagnosis. That survival rate increases to 90% for children whose tumors were diagnosed and treated early on, usually with a combination of surgery and other therapies. Healthcare providers are developing new surgical techniques to help children who have more advanced forms of hepatoblastoma.

Is hepatoblastoma common?

Hepatoblastoma affects about 1 in 1 million young children living in developed countries. The number of cases has increased over the past few years, as more children who are born prematurely or with very low birth weights survive their challenging start in life.

How does hepatoblastoma affect my child’s body?

Hepatoblastoma damages your child’s liver, one of the more important organs in our bodies. We rely on our livers to break down, store and absorb nutrients. Our livers also eliminate toxins and keep us from bleeding too much when we’re cut or injured.

Symptoms and Causes

What are hepatoblastoma symptoms?

Hepatoblastomas develop over time. 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. Here are some signs your child may have hepatoblastoma:

  • They may have a painful lump in the upper right side or the middle of their abdomen.
  • They may have persistent nausea and diarrhea.
  • They may not be interested in eating and might be losing weight.

What causes hepatoblastoma?

Researchers haven’t identified hepatoblastoma’s exact cause, but they have identified a few risk factors, including hereditary conditions. Here are some conditions that might increase your child’s risk of developing hepatoblastoma:

  • Premature birth. Most babies are born 37 weeks after their mothers become pregnant. A baby born before 37 weeks is considered premature.
  • Low birth weight.
  • Early exposure to Hepatitis B. This is a virus that 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 babies’ livers.
  • Hemihyperplasia. This condition affects your child’s body, causing one side of your child’s body to be larger than the other side.

What genetic conditions are linked to hepatoblastoma?

Genetic conditions that may be linked to hepatoblastoma include:

  • Beckwith-Wiedemann syndrome. This is a growth disorder that increases your child’s risk of developing certain childhood cancers, including hepatoblastoma.
  • Familial adenomatous polyposis (FAP). This condition causes pre-cancerous polyps in your child’s large intestine.
  • Aicardi syndrome. This is a rare condition that 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.
  • 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.

Diagnosis and Tests

How do healthcare providers diagnose hepatoblastoma?

Provider use several tests to diagnose hepatoblastoma, including:

  • Complete blood count. Providers check your child’s alpha-fetoprotein (AFP) levels as part of this test. AFP is a substance produced by your child’s liver.
  • Comprehensive metabolic panel (CMP). A CMP tests your child’s blood for 14 different substances and liver function.
  • Vascular ultrasound. This test gives healthcare providers a view of the network of blood vessels entering and leaving your child’s liver.
  • Magnetic resonance imaging (MRI). This is a painless test that uses a large magnet, radio waves and a computer to produce very clear images of organs and structures within your child’s body.

My child might 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. Ask your healthcare provider about child life support services. The team members will help you guide your child through their care. Here are some ways you can make essential tests easier:

  • Often, children cope better with pain and tests if they know what to expect. Ask your child’s healthcare 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 is going to have an MRI, you and your child can practice holding still for the amount of time the procedure will take.
  • Very young children want their parents nearby. Ask your healthcare 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 during a procedure, plan ways to distract them during it, 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 your child may 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’ll be right there during the test.
  • Do your best to stay calm for your child’s sake.

How do 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.

Our livers have two lobes. Healthcare 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, healthcare 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 healthcare providers to remove entire tumors, essentially eliminating the cancer.

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

Management and Treatment

Can hepatoblastoma be cured?

Often, healthcare providers can use surgery to remove smaller tumors, eliminating the tumors in your child’s liver and essentially curing the disease. Types of surgery your healthcare provider may recommend are:

  • Partial hepatectomy. This surgery removes the tumor from the section of the liver where it was found.
  • Total hepatectomy and liver transplantation. Your healthcare provider may remove your child’s entire liver, replacing it with a healthy liver.

Are there other treatments for hepatoblastoma?

Healthcare providers target your child’s disease in several ways. Here are some other treatments they may use:

  • Systemic chemotherapy. This is chemotherapy that travels throughout your child’s body.
  • Local chemotherapy. This chemotherapy directly targets the tumor in your child’s liver.
  • Transarterial chemoembolization or TACE. This treatment works the same way you might build a dam in a stream to stop the stream from flowing. Healthcare providers inject anti-cancer drugs into one of your child's arteries that supplies blood to their liver. Then they inject a substance to block the artery. The substances act like a dam, keeping the anti-cancer drug swirling around the tumor instead of floating away.
  • Radiation therapy. Healthcare providers may use radiation therapy after surgery to remove any remaining cancer cells or as an alternative way to treat tumors that can’t be removed using surgery.
  • Ablation therapy. This treatment destroys or removes tissue. Healthcare providers use ablation therapy to treat recurring hepatoblastoma.

What happens if my child’s hepatoblastoma has spread 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.

Prevention

Are there ways to prevent hepatoblastoma?

Researchers don’t know all the reasons why certain children develop hepatoblastoma, but they have discovered potential connections between a few hereditary conditions. They also believe babies who are born prematurely or born with a very low birth weight have an increased risk of developing hepatoblastoma.

Ask your child’s healthcare provider about the risk factors for hepatoblastoma. They’ll help you decide if your child is at risk. They’ll also develop a plan for monitoring your child’s health for signs of hepatoblastoma. Diagnosing and treating hepatoblastoma early on increases the chance your child’s treatment will be successful.

Outlook / Prognosis

More children are being successfully treated for hepatoblastoma. In many cases, early treatment means children are cured after surgery and chemotherapy. Unfortunately, many children who develop additional cancers later in life. Talk to your healthcare provider about any additional cancer your child may develop. They may recommend setting up regular screening tests to detect new cancers and treat those cancers before they take hold.

Living With

What can I expect if my child has hepatoblastoma?

Healthcare providers typically treat hepatoblastoma with surgery and chemotherapy. While each child’s situation is different, nearly all hepatoblastoma surgery is major surgery. Your child will need time to recover from their surgery

Will my child have late effects from hepatoblastoma treatment?

Late effects are health problems that appear after your child finishes cancer treatment. Treatments such as surgery, chemotherapy or liver transplant can cause health problems that appear months and years after treatment is through.

Since 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 wellbeing.

Here are some things you can do to help support your child after cancer treatment:

  • Make sure your child has regular wellness checks, including visits to their dentist and pediatric healthcare provider.
  • Encourage self-care. Even very young children can benefit from understanding how a healthy diet and fun exercise can keep them strong.

When should my child see their healthcare provider?

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

When should I take my child to the emergency room?

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

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

What questions should I ask my doctor?

Hepatoblastoma is a very rare liver cancer. If your child is diagnosed with hepatoblastoma, you may want to know why this happened and what you can do to help your child. Here are some suggestions to help you learn more:

  • 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

Healthcare providers can successfully treat hepatoblastoma, a very rare liver cancer that affects very young children. But hepatoblastoma casts a long shadow. Even after successful treatment, your child’s cancer may come back. They may develop a second cancer. Some children develop medical conditions years after their hepatoblastoma treatment, too. If you’re worried about what’s next, ask your child’s healthcare provider to explain the specific challenges your child may face. They’ll share more than insight — they’ll also share understanding and support.

Last reviewed by a Cleveland Clinic medical professional on 11/30/2021.

References

  • American Childhood Cancer Organization. Hepatoblastoma. (https://www.acco.org/hepatoblastoma/) Accessed 11/30/2021.
  • Childhood Liver Cancer Treatment (https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq) (https://www.cancer.gov/types/childhood-cancers/pediatric-care-pdq) (PDQ ®) (https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq) (https://www.cancer.gov/types/childhood-cancers/pediatric-care-pdq) Patient Version. (https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq) Accessed 11/30/2021.
  • Hiyama, Eiso. Pediatric Hepatoblastoma: Diagnosis and Treatment. Transl Pediatr. Accessed 11/30/2021.
  • Musick, Sierra R, Smith Melinda, Rouster Audra S. Hepatoblastoma. (https://www.ncbi.nlm.nih.gov/books/NBK534795/?ff=20211011030139&v=2.15.0%23_NBK534795_pubdet_) StatPearls. Accessed 11/30/2021.
  • National Cancer Institute. Pediatric Supportive Care (PDQ ®) – Patient Versio (https://www.cancer.gov/types/childhood-cancers/pediatric-care-pdq) n. Accessed 11/30/2021.
  • National Organization for Rare Diseases. Hepatoblastoma. (https://rarediseases.org/gard-rare-disease/hepatoblastoma/) Accessed 11/30/2021.
  • Yang, Tianyuo, Whitlock, Richard S, Vasudevan, Sanjeev A. Surgical Management of Hepatoblastoma and Recent Advances. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6966548/) Cancers. Accessed 11/30/2021.

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