Hepatoblastoma

Hepatoblastoma (pronounced “heh-puh-tow-blas-tow-mah”) is a very rare liver cancer. Only 1 to 2 children in 1 million children in the U.S. have it. This condition typically affects children ages 1 to 3. Early diagnosis and surgery to remove the cancerous tumor(s) may cure it. A liver transplant is another option.

Symptoms of hepatoblastoma

Common hepatoblastoma symptoms include:

• Abdominal (belly) pain
• Lump in the middle or upper right side of your child’s belly (abdomen)
• Loss of appetite and unexplained weight loss
• Persistent nausea and vomiting
• Yellow-colored skin or eyes from jaundice

Hepatoblastomas grow very slowly. Your child may not have symptoms until the tumor is large enough to affect their body. For example, your child may have belly pain that doesn’t go away or appear to be losing weight. Chances are, hepatoblastoma isn’t the reason why your child has a stomachache. Your intuition may be telling you that your child’s touchy tummy is a serious issue. In that case, don’t hesitate to call their pediatrician.

Hepatoblastoma causes

Experts don’t know exactly what causes healthy liver cells to become cancer cells. But there are conditions that increase hepatoblastoma risk:

• Very low birth weight (birth weight below 5 pounds, 5 ounces or 2,408 grams)
• Preterm birth (being born at 37 weeks gestation)
• Aicardi syndrome, which causes malformations in your child’s brain and other parts of their body
• Beckwith-Wiedemann syndrome that can affect your child’s growth and increase their risk of cancer
• Biliary atresia, which blocks babies’ bile ducts and damages their livers
• Edwards syndrome (trisomy 18) that affects how your child’s body develops and grows
• Familial adenomatous polyposis (FAP), which causes large numbers of precancerous polyps (growths) in your colon
• Hemihyperplasia that causes one side of your child’s body to grow faster than the other
• Simpson-Golabi-Behmel syndrome (SGB) that causes abnormalities in children’s liver and other organs

Complications of hepatoblastoma

Cancer treatments for hepatoblastoma may cause late effects. These are health issues that surface months and years after diagnosis or treatment. Your child may need long-term follow-up and medical care to manage them.

Second cancers are examples of a late effect. This is a new type of cancer. It appears months or years after your child completes hepatoblastoma treatment.

Late effects may make an impact on your child’s:

• Ability to think, learn and remember information
• Growth and development
• Moods, feelings and mental health
• Organs and tissues

How doctors diagnose hepatoblastoma

Your child’s pediatrician will do a physical examination. They’ll ask about your child’s symptoms and when you first noticed symptoms. They may do some of the following tests:

• Alpha-fetoprotein (AFP) tests. AFP is a substance produced by your child’s liver.
• Complete blood count (CBC). This test measures and counts the blood cells in your child’s blood.
• Comprehensive metabolic panel (CMP). A CMP tests your child’s blood for 14 different substances and liver function.
• Liver and Doppler ultrasounds. Healthcare providers use these tests to get images of the inside of your child’s liver.
• Magnetic resonance imaging (MRI). This test produces very clear images of the organs and blood vessels.
• Vascular ultrasound. Providers do this test to see the network of blood vessels that enter and leave your child’s liver.

Your child’s pediatrician may refer you to a pediatric oncologist. This is a doctor with experience treating cancer in children.

Stages of hepatoblastoma

Cancer staging is how oncologists set treatment plans. Pediatric oncologists use two grouping systems instead of cancer stages. Those groups are:

• PRETEXT stands for “pre-treatment extent of disease.” It refers to how cancer affects your child’s liver before pre-surgery chemotherapy. Your child’s pediatric oncologist looks for the number of tumors. They also check the tumor(s) size and appearance
• POSTTEXT refers to “post-treatment extent of disease” after pre-surgery treatment.

Your liver has two lobes. Oncologists divide the two lobes into four groups. They classify each group by the number of liver sections with tumors. The more sections with tumors, the higher the group number.

Here’s an example: Tests detect two tumors in different sections of your child’s liver. That puts the cancer in PRETEXT Group II. If pre-surgery chemotherapy removes one tumor, the cancer is POSTEXT Group I. Your child’s oncologist may say your child now has Group I hepatoblastoma. Other hepatoblastoma groups are:

• Group I. There’s cancer in one section of your child’s liver but none in the three other sections.
• Group II. This group has two scenarios. In one, there’s cancer in two sections of the liver that are next to each other, but not in the other two sections. In the other, there’s cancer in one section, but not in two sections that are next to each other.
• Group III. One or more sections of the liver contain cancer. But there isn’t cancer in the sections that are right next to those with cancer.
• Group IV. There’s cancer in all four sections of your child’s liver.

Specific treatments and procedures

The most common treatment is surgery to remove the part of your child’s liver that cancer affects. The liver is the only organ that can grow back after surgery. That means your child’s liver will eventually grow back to its original size.

Your child’s pediatric oncologist may do more chemotherapy before and after surgery. They do this treatment to shrink the tumor.

Your child may receive chemotherapy for four to six weeks after surgery. Chemotherapy after surgery reduces the chance that cancer will come back.

Other hepatoblastoma treatments include:

• Ablation therapy is a minimally invasive procedure for hepatoblastoma that comes back.
• Liver transplant is an option if cancer affects the central part of your child’s liver or many sections of their liver.
• Transarterial chemoembolization (TACE) cuts off the tumor’s blood supply and delivers chemotherapy directly to the tumor.

Can hepatoblastoma be cured?

Yes, but it depends on when your child is diagnosed and receives treatment. Your child’s surgeon may be able to cure hepatoblastoma if they can remove the entire tumor or all of the tumors.

Hepatoblastoma survival rates

Cancer survival rates are estimates based on people’s experiences with different kinds of cancer. Overall, 4 out of 5 children with hepatoblastoma will be alive five years after diagnosis.

When you think about survival rates, it’s important to keep in mind that your child’s experience with hepatoblastoma may be different from what other children experience.

Many things can make a difference in hepatoblastoma. If you have questions about survival rates, your child’s pediatric oncologist is your best resource for information. They’ll explain what survival rate data means in your child’s case.

When should my child see their healthcare provider?

Your child should have regular follow-up appointments with their cancer care team. The team will check your child’s health, including any late effect health issues. Team members will look for signs of returning hepatoblastoma or a new cancer.

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:

• Have a fever that’s higher than 100.4 degrees Fahrenheit (38.3 degrees Celsius)
• The surgery site turns dark red, or your child says it hurts when you touch it
• There are green or yellow substances oozing from the surgery site

What can I do to help my child manage hepatoblastoma tests and treatment?

Tests are essential to find out why your child is sick. But needles for blood tests can sting. MRI machines make loud scary noises. Your child may feel anxious about having chemotherapy or surgery. These are very normal reactions when a child has cancer. Working with a child life specialist may help your child (and you) get through tests and treatment.