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Hepatoblastoma

What is hepatoblastoma?

Hepatoblastoma is a very rare cancerous tumor that originates 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. Most hepatoblastoma tumors originate in the right lobe.

This disease primarily affects children from infancy to about 5 years of age. Most cases appear during the first 18 months of life. Hepatoblastoma cancer cells can spread (metastasize) to other areas of the body. The most common sites of metastasis are the lungs, into the abdomen and abdominal structures, and rarely to bone, the central nervous system, and the bone marrow.

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 and excretes a product called "bile," which helps carry away waste products from the liver.

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, hemihypertrophy, and familial adenomatous polyposis. Other genetic conditions associated with liver cancer include several inborn errors of metabolism such as tyrosinemia, glycogen storage disease type I, galactosemia, and alpha1-antitrypsin deficiency.

Children who are exposed to hepatitis B 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 physician for a diagnosis.

How is hepatoblastoma diagnosed?

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

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 physician for information on staging. One method of staging hepatoblastoma is the following:

Treatment for hepatoblastoma:

Specific treatment for hepatoblastoma will be determined by your child's physician 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):

Long-term outlook for a child with hepatoblastoma:

Prognosis greatly depends on:

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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