Robert Wood Johnson University Hospital


Pediatric Cancers - Hepatoblastoma

What is hepatoblastoma?

Hepatoblastoma is a very rare cancerous tumor that starts in the liver. The liver is one of the largest organs in the body. The primary functions of the liver include filtering and storing blood. The liver consists of right and left lobes.

This disease primarily affects children from infancy to about 3 years of age. Hepatoblastoma cancer cells can spread (metastasize) to other areas of the body, but this is rare.

Anatomy of the liver

The liver is located in the upper right-hand portion of the abdominal cavity, beneath the diaphragm and on top of the stomach, right kidney, and intestines. Shaped like a cone, the liver is a dark reddish-brown organ that weighs about three pounds.

Illustration of the anatomy of the biliary system
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The liver consists of two main lobes, both of which are made up of thousands of lobules. These lobules are connected to small ducts that connect with larger ducts to ultimately form the hepatic duct. The hepatic duct transports the bile produced by the liver cells to the gallbladder and duodenum (the first part of the small intestine). The liver regulates most chemical levels in the blood, excretes a product called bile, which makes clotting factors that keep the body from bleeding when injured, and helps remove waste products from the body.

What causes hepatoblastoma?

Although the exact cause of liver cancer is unknown, there are a number of genetic conditions that are associated with an increased risk for developing hepatoblastoma, including Beckwith-Wiedemann syndrome, Wilson disease, porphyria cutanea tarda, and familial adenomatous polyposis. Other genetic conditions associated with liver cancer include several inborn errors of metabolism such as tyrosinemia, glycogen storage diseases, and alpha1-antitrypsin deficiency.

Children who are exposed to hepatitis B or hepatitis C infection at an early age, or those who have biliary atresia, are also at increased risk for developing hepatocellular carcinoma. Some hepatoblastomas have genetic alterations in tumor suppressor genes, which would explain the uncontrolled cell growth.

What are the symptoms of hepatoblastoma?

The following are the most common symptoms of hepatoblastoma. However, each child may experience symptoms differently. Symptoms may vary depending on the size of the tumor and the presence and location of metastases. Symptoms may include:

The symptoms of hepatoblastoma may resemble other conditions or medical problems. Always consult your child's doctor for a diagnosis.

How is hepatoblastoma diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for hepatoblastoma may include:

If your child has been diagnosed with hepatoblastoma, you may want to consider getting a second opinion. In fact, some insurance companies require a second opinion for such diagnoses. According to the American Cancer Society, it is very rare that the time it will take to get a second opinion will have a negative impact on treatment. The peace of mind a second opinion provides may be well worth the effort.

What are the different stages of childhood liver cancer?

Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging symptoms that are used for hepatoblastoma. Always consult your child's doctor for information on staging. One method of staging hepatoblastoma is the following:

What is the treatment for hepatoblastoma?

Specific treatment for hepatoblastoma will be determined by your child's doctor based on:

Treatment for hepatoblastoma is generally aimed at resecting (removing) as much of the tumor as possible while maintaining adequate liver function. Liver tissue can regenerate when removed.

Other forms of treatment may include (alone or in combination):

What is the long-term outlook for a child with hepatoblastoma?

Prognosis greatly depends on:

Both the American Academy of Pediatrics and the National Cancer Institute recommend that children receive cancer diagnosis and treatment at one of the more than 200 medical centers in the U.S. that specialize in pediatric cancers. The organizations cite research studies that show higher survival rates when children receive care in such specialized centers. 

Finally, as with any cancer, prognosis and long-term survival can vary greatly from child to child. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with hepatoblastoma. New methods are continually being discovered to improve treatment and to decrease side effects.

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