What Is Hepatoblastoma?

Medically Reviewed by Dan Brennan, MD on July 23, 2022
5 min read

Hepatoblastoma is a type of liver cancer that affects children who are primarily under three years old. Although rare, it's the most common type of malignant form of liver tumor found in children. 

The tumor itself usually forms in the cells of the liver and often affects the right lobe. Unfortunately, hepatoblastoma can metastasize to other parts of the body and most often spreads to the lungs.  

While hepatoblastoma causes are primarily unknown, it's thought that some genetic conditions can increase a child’s risk of developing hepatoblastoma.

Hepatoblastoma occurs mostly during infancy, affecting children who are 18 months or younger. However, it can also appear in children anywhere from 3 to 5 years old. White children seem to be at an increased risk of developing hepatoblastoma than black children and it's more frequently diagnosed in boys than girls. 

Premature babies who were born with a lower-than-normal birth weight are also at an increased risk of developing hepatoblastoma as are children who have been exposed to hepatitis B and those who have  biliary atresia.

There are also genetic factors that are connected to hepatoblastoma. These conditions include Beckwith-Wiedemann syndrome, familial adenomatous polyposis, Aicardi syndrome, glycogen storage disease, and Simpson-Golabi-Behmel syndrome.

Hepatoblastomas may not present symptoms in the beginning, so you may not realize that your child’s body is experiencing any changes at first. Once the tumor begins to grow, it may start to affect your child’s health and behavior. 

When hepatoblastoma symptoms present themselves, you may notice a painful lump that has formed in your child’s abdomen, typically on the upper right side or in the middle. Your child may experience chronic nausea and diarrhea and they may have a poor appetite and undergo sudden weight loss. There may also be  jaundice, itchy skin, and anemia.

Like with all cancers, hepatoblastoma is divided into four stages of varying degrees. The stage of hepatoblastoma that a patient is diagnosed with will depend on whether the tumor has spread to other parts of the body and how it has affected the liver.

The stages are: 

  • Stage I: The tumor has not metastasized and has been confined to the liver. The tumor is removed during diagnostic surgery. Once removed, a pathologist will examine the tissue under a microscope to determine whether or not the margins are free from cancer cells. In Stage I, they usually are.
  • Stage II: Similar to Stage I, Stage II sees no metastasis of the tumor outside of the liver. However, upon removal of the tumor during the diagnostic surgery, cancerous cells are found within the margins.
  • Stage III: The tumor has grown too big to be removed during diagnostic surgery. It may have grown into, or be pressing against, the liver’s important tissues. It may also have spread to the lymph nodes that are close to the liver, but not elsewhere.
  • Stage IV: The tumor has spread beyond the liver and has reached other vital organs, such as the lungs.

If hepatoblastoma is suspected as a result of any of the associated symptoms, a doctor will do a thorough exam. During this exam, he or she will perform blood tests that test the functionality of the liver as well as a blood test to determine if there is liver damage. There may also be imaging tests including ultrasounds, X-rays, CAT scans, and MRIs, and  biopsies on any tumor tissue present to determine whether or not it's malignant.

Thankfully, with the advancement of treatment options and technology, the hepatoblastoma prognosis has improved over the last two decades with a survival rate of 80%–90%. Of course, the survival rate depends on how early hepatoblastoma is caught and what stage it's at when diagnosed.

Those who have caught hepatoblastoma early and have had it fully removed survive at a rate of more than 85%. The rate decreases to around 60% if the tumor has metastasized but is still responsive to chemotherapy treatment. 

Due to a more aggressive chemotherapy treatment plan and the option for a liver transplant, there is strong proof that children with Stage IV have a better chance of survival than they did in the past.

With the advancements in science and medicine, there are many treatment options available for hepatoblastoma today. 

The hepatoblastoma treatment options available to your child will depend upon the stage of the disease and other factors such as where it is located and how it has affected your child’s liver. 

After consulting with your child’s oncologist and determining the stage, location, and size, they may offer you and your child the following treatment options: 

  • Surgery. Surgery is performed in order to conduct a biopsy and determine the stage of hepatoblastoma. It's also an option to remove a large portion of the tumor, if possible, and to eliminate tumors that have metastasized to other areas such as the lungs.
  • Chemotherapy. Chemotherapy is the most common treatment method for most cancer types, and hepatoblastoma is no different. This treatment option allows the medicine to be administered through an IV, through an injection, or by mouth, and the medicine works to eliminate cancer cells present in the body. There is also a chemotherapy option that allows a catheter to deliver medicine straight to the liver in order to block blood flow to the tumor.
  • Radiation. Radiation therapy is another common treatment option for many cancer types, and is currently being studied for the effects it has on hepatoblastoma. Radiation therapy happens when high-powered radiation, often in the form of X-rays, is administered to the patient in order to kill cancer cells or to stop them from growing.
  • Transplant. If the liver has sustained significant damage, or if the tumor is too large to be surgically removed, the liver may need to be removed and replaced with a donor’s liver.

There may also be clinical trials available that your child can qualify for, so be sure to ask your oncologist about any current clinical trials.