Hepatoblastoma - Rare Childhood Liver Cancer

Hepatoblastoma is a rare cancer that develops in a normal liver, and primarily affects children from infancy to 5 years of age with most cases appearing in the first 18 months of a child's life. Our liver is one of the largest organs in our body and is responsible for storing nutrients from foods and filtering chemicals from our blood that can harm our bodies. It also has two lobes, a right lobe and a left lobe. Hepatoblastoma typically develops in the right lobe of the liver. Once these cancer cells develop, they do have the potential to spread to other areas of the body. Most common sites include the lungs and abdomen. However, it is very rare that it spreads to the bones and central nervous system. The information that follows is for informational purposes only. You should always consult your child's personal physician if you suspect something is medically wrong.

What are the causes?

The exact cause of hepatoblastoma is actually unknown, but there are a number of genetic conditions that may make a child more at risk for this form of liver cancer. Children of families that carry a gene related to a specific kind of colon cancer (familial adenomatus polyposis) and children with Beckwith-Wiedemann syndrome or hemihypertrophy (both overgrowth conditions) could be more at risk of developing hepatoblastoma. Children who have been exposed to hepatitis B at an early age are also at an increased risk for developing hepatoblastoma.

What are the symptoms?

Every child is different and may experience symptoms differently. Symptoms can vary depending on the size of the tumor and if the cancer has spread to other areas in the body. However, symptoms may include:

- Large abdominal mass, abdominal pain

- Weight loss, decreased appetite

- Vomiting

- Jaundice

These symptoms can also resemble other medical conditions and problems so it is always best to consult your physician.

What are the stages?

The stages of liver cancer determine where the cancer has spread to, if at all. Stages for hepatoblastoma include:

- Stage I: a tumor that can be removed with surgery.

- Stage II: the tumor can be partially removed through surgery, but small amounts of the cancer cells are left in the liver.

- Stage III: Cannot completely remove the tumor and cancer cells have reached the lymph nodes.

- Stage IV: Cancer has spread to other areas of the body.

- Recurrent: Cancer returns after treatment, either in the liver or somewhere else in the body.

How is it treated?

Treatment depends on a variety of factors, but is generally focused on the removal of the tumor while not compromising the overall liver function. The liver is a very resilient organ so it has great abilities to regenerate cells and tissues that are removed. Other forms of treatment can include chemotherapy and a liver transplant.

What is the outlook ?

Every child is different so the survival rate will vary from child to child, and the treatment of the condition. Cure is definitely possible when the tumor can be completely removed during surgery, but this is not a possible scenario in everyone. In fact, complete removal is usually only possible in about half of patients. Hepatoblastoma tends to respond very well to chemotherapy, but with children whose tumor has spread to other areas outside of the liver, they rarely survive long-term.

Again, prognosis depends on a variety of factors. Prompt medical attention and aggressive therapies are, of course, important for the best outcome, but it also depends on the extent of the disease, the age and overall health of the child, how well the tumor responds to treatment, etc.

Boston Children's Hospital

St. Jude Children's Research Hospital

Lucile Packard Children's Hospital at Stanford