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.
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:
- A large abdominal mass, or swollen abdomen
- Weight loss, decreased appetite
- Early puberty in boys
- Abdominal pain
- Nausea and vomiting
- Jaundice (yellowing of the eyes and skin)
- Itching skin
- Enlarged veins on the belly that can be seen through the skin
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:
- Biopsy. A sample of tissue is removed from the tumor and examined under a microscope.
- Complete blood count (CBC). A measurement of size, number, and maturity of different blood cells in a specific volume of blood.
- Additional blood tests. These tests may include blood chemistries, evaluation of liver and kidney functions, and genetic studies.
- Multiple imaging studies, including:
- Computed tomography scan (also called a CT or CAT scan). A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general X-rays.
- Magnetic resonance imaging (MRI). A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
- X-ray. A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
- Ultrasound (also called sonography). A diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
- Alpha-fetoprotein (AFP) test. Alpha-fetoprotein (AFP) levels in the blood can be used to diagnose and follow response to treatment.
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:
- Stage I. This is usually a tumor that is only in the liver and that can be completely removed with surgery.
- Stage II. This is usually a tumor that can mostly be removed by surgery but very small amounts of the cancer are left in the liver.
- Stage III. This is usually a tumor that cannot be completely removed or cancer cells are found in nearby lymph nodes.
- Stage IV. Cancer that has spread (metastasized) to other parts of the body.
- Recurrent. The disease has returned after it has been treated. It may come back in the liver or in another part of the body.
What is the treatment for hepatoblastoma?
Specific treatment for hepatoblastoma will be determined by your child's doctor based on:
- Your child's age, overall health, and medical history
- Extent of the disease
- Your child's tolerance for specific medications, procedures, and therapies
- Expectations for the course of the disease
- Your opinion or preference
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):
- Surgery to remove tumor and part or all of the liver
- Liver transplant
- Radiation therapy
- Percutaneous ethanol injection. A small needle is used to inject an alcohol called ethanol right into the tumor to kill cancer cells.
- Watchful waiting. Not starting treatment until symptoms appear or change.
What is the long-term outlook for a child with hepatoblastoma?
Prognosis greatly depends on:
- The extent of the disease.
- The size and location of the tumor.
- Presence or absence of metastasis.
- The tumor's response to therapy.
- The age and overall health of your child.
- Your child's tolerance of specific medications, procedures, or therapies.
- New developments in treatment.
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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