Hepatoblastoma

Hepatoblastoma is a rare, malignant (cancerous) tumor of the liver. It is found 90 percent of the time before 3 years of age.

The cause of hepatoblastoma is usually unknown. Some genetic disorders put a child at greater risk.

An increased swelling of the belly is the most common symptom. The enlarged liver can sometimes be felt by your child's doctor. 

Most Common Symptoms

  • Swollen belly; a large mass in the belly
  • Pain in the belly
  • Pale skin and / or fatigue
  • Lower appetite and weight loss
  • Vomiting

Other Symptoms

  • Fever
  • Back pain
  • Decreased urination

Hepatoblastoma is usually first suspected based on the presence of a large mass in the belly. Once suspected, the initial diagnosis can be made using radiological imaging and blood tests.

Hepatoblastoma tumors usually secrete a protein called alpha-fetoprotein (AFP) into the blood. An increase of this protein level in the blood is helpful in making the diagnosis. Imaging tests such as ultrasoundCT scan and MRI of the abdomen may also be performed.

A definitive diagnosis is made by biopsy or removal of the tumor. A biopsy is a surgical procedure where a part of the suspicious mass is removed and looked at under a microscope. These procedures are done under general anesthesia so that the child is not conscious and will not feel any pain.

Staging depends on the extent of liver involvement and whether the tumor is in only one place or if it has spread. The most common sites for metastasis or spread of tumor are the lungs and abdominal lymph nodes.

There are two staging systems for hepatoblastoma.

  • The pre-treatment extent of disease (PRETEXT) staging system is based on imaging of the liver before surgery is attempted. There are four main lobes of the liver. The PRETEXT stage corresponds to the number of lobes that have tumor in them. For example, if one lobe is involved, the disease is considered stage I. If all four lobes are involved, the disease is considered stage IV.
  • The Children’s Oncology Group (COG) staging system is based on additional imaging of other parts of the body, as well as the results of surgery, if surgery was performed at diagnosis. COG staging reflects how much of the tumor was removed and how much the tumor has spread.

Stage I: There is no spread of tumor detectable outside of the liver. The tumor has been surgically removed at diagnosis and there are no cancer cells on the edges, or margins, of the removed tissue. A pathologist determines if the margins are clear of cancer cells by looking at the removed tissue under a microscope. 

Stage II: There is no spread of tumor detectable outside of the liver. The tumor has been surgically removed at diagnosis, but the margins of the removed tissue contain cancer cells.

Stage III: The tumor cannot be surgically removed at diagnosis because it is too big, it has grown into or presses on vital tissues in the liver, or it has spread to the lymph nodes that drain from the liver. In stage III disease, the tumor is not detectable in other parts of the body besides the liver and possibly the lymph nodes close to the liver.

Stage IV: The tumor has obvious spread through the bloodstream to other parts of the body, most commonly to the lungs.

There are several ways to treat hepatoblastoma. Treatment options might include chemotherapy, surgery to remove the tumor (also called tumor resection) and liver transplantation.

Chemotherapy is a key part of treatment for nearly all infants and children with hepatoblastoma. Chemotherapy is used to both shrink the primary liver tumor and to treat any hepatoblastoma that has escaped outside the liver. This is important for patients with all stages of the disease, because cancer cells might have traveled to other parts of the body, possibly in amounts too small to be detected by doctors. A rare exception to the need for chemotherapy is found in infants and children with COG stage I disease of a less aggressive type called pure fetal hepatoblastoma. For all other infants and children, chemotherapy is given before and / or after surgery. 

Tumor removal / resection: Many factors are considered when assessing whether a tumor can be safely removed from a child’s liver such that enough normal liver remains. One of the factors that doctors consider is the degree of liver involvement. For example, some children have a single tumor, while other children have multifocal disease (more than one tumor in the liver). Some tumors involve just one or two liver lobes, while others involve three or four liver lobes. Some tumors encroach upon vital structures within the liver, while others do not invade such structures. For patients who have limited disease when the tumor is discovered, tumor resection (removal) can be considered at diagnosis. This is true for approximately a third of children with hepatoblastoma. Of the children whose tumors are not resectable at diagnosis, their tumors can sometimes become resectable after a few cycles of chemotherapy.

Liver transplantation has also been used successfully in combination with chemotherapy to treat hepatoblastoma. Liver transplantation is usually used in cases where the tumor is located only in the liver and is unable to be removed by surgery. In these cases, the liver that contains the tumor is completely removed and replaced with a liver from a donor. Like with other surgeries for hepatoblastoma, chemotherapy is used before and often after liver transplantation to control the cancer. For infants and children who have unresectable hepatoblastoma (via conventional surgical approaches) and have cancer outside of the liver (aggressive COG stage IV disease), liver transplantation can still be used as part of the treatment plan successfully in certain circumstances, particularly when the disease outside the liver disappears with chemotherapy.

Many factors are considered when designing a treatment plan for an individual infant or child, and all treatment options should be discussed thoroughly between the care team and the family. Do not be afraid to ask questions and take an active role in the treatment plan for your child.

There has been dramatic improvement in outcomes over the last 20 to 25 years, with overall survival rates reaching 70 percent. Survival is dependent upon staging at diagnosis.  Patients with completely resected tumors have a greater than 85 percent chance of survival.  For those with unresectable disease that responds to chemotherapy, survival rates are about 60 percent.

The prognosis for an individual depends on the stage of the disease:

  • Stage I and II: When tumors are located only in the liver and can be surgically removed at diagnosis, the published cure rate is greater than 85 percent when surgery and chemotherapy are used together.
  • Stage III: When tumors are located only in the liver but cannot be surgically removed at diagnosis, the published cure rate is not as high, approximating 60 percent to 70 percent. The cure rate is higher if complete surgical removal of all tumor becomes possible. Anecdotally, the cure rate appears to be increasing for children with Stage III disease, at least in part due to increasing availability of liver transplantation as a surgical option.
  • Stage IV: When tumors are located in several places in the body, the published cure rate is 20 percent to 30 percent using surgery and chemotherapy. There is preliminary evidence to suggest that the cure rate may be increasing for children with Stage IV disease, possibly due to more aggressive chemotherapy options and the increasing availability of liver transplantation.

For additional information on this topic, contact us, cancer@cchmc.org.


Last Updated 10/2012