Hepatoblastoma (liver) in Children
The Pediatric Solid Tumor Program at the Cancer Center at The Children's Hospital of Philadelphia consists of a multidisciplinary team of experts working together to develop a care plan for your child's hepatoblastoma. Many of our oncologists are also on the forefront of researching new therapies for treatment of hepatoblastoma.
What is hepatoblastoma?
Hepatoblastoma is a rare tumor (an abnormal tissue growth) that originates in cells in the liver. It is the most common cancerous (malignant) liver tumor in early childhood. Most hepatoblastoma tumors begin in the right lobe of the liver. Hepatoblastoma cancer cells also can spread (metastasize) to other areas of the body. The most common site of metastasis is the lungs.
Who is diagnosed with hepatoblastoma?
Hepatoblastoma primarily affects children from infancy to about 5 years of age. Most cases appear during the first 18 months of life. Hepatoblastoma affects white children more frequently than black children, and is more common in boys than girls up to about age 5, when the gender difference disappears. It occurs more frequently in children who were born very prematurely (early) with very low birth weights.
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. They include:
- Beckwith-Wiedemann syndrome. This syndrome is characterized by a combination of Wilms' tumor, kidney failure, genitourinary malformations and gonad (ovaries or testes) abnormalities.
- Familial adenomatous polyposis. This is a group of rare inherited diseases of the gastrointestinal tract.
- Hemihypertrophy. This condition is the faster growth of one limb on one side of the body in comparison with the other side.
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 liver cancer.
What are the signs and symptoms of pediatric hepatoblastoma?
The signs and symptoms of pediatric hepatoblastoma often depend on the size of the tumor and whether it has spread to other parts of the body. Symptoms may include:
- A large mass in the abdomen
- Swollen abdomen
- Weight loss
- Decreased appetite
- Vomiting
- Jaundice (yellowing of eyes and skin)
- Itchy skin
- Anemia
- Back pain
How do we diagnose hepatoblastoma in your child?
After taking a complete medical history and doing a physical examination of your child, we may use the following tests:
- Alpha-fetoprotein (AFP) test. AFP levels in the blood can be used both to diagnose hepatoblastoma and to monitor its response to treatment.
- Computerized tomography scan (CT or CAT scan). This imaging test uses X-rays and computer technology to produce detailed cross-sectional images (slices) of the body, including the bones, muscles, fat and organs. It can show a mass in the liver and whether the cancer has spread to other organs such as the lungs.
- Magnetic resonance imaging (MRI). An MRI scan uses radio waves and strong magnets with computer technology. MRI shows more detailed images than CT and ultrasound and can help doctors see if the cancer has invaded one of the major blood vessels located near the liver.
- Blood tests. These laboratory tests will help evaluate liver function.
- Biopsy. A biopsy (a sample of tissue that is removed and examined under a microscope) will help confirm the diagnosis and help plan treatment.
- Bone scan. This imaging test uses a special radioactive material that is injected into a vein. The substance collects in areas of diseased bone and is sensed by special cameras that pick up radioactivity.
How is pediatric hepatoblastoma treated?
Treatment for hepatoblastoma is aimed at removing (resecting) as much of the tumor as possible. Liver tissue can regenerate after a portion is removed.
Most hepatoblastomas also require treatment with chemotherapy to shrink the tumor before surgery. This makes it easier and safer for the surgeon to remove the mass. Chemotherapy is also given after surgery to minimize the chance of recurrence (cancer regrowth).
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 because side effects of radiation and chemotherapy may occur, as well as second malignancies.
Late effects/cancer survivorship
Some children treated for cancer develop complications years later. Our Cancer Survivorship Program will provide you with information about the potential long-term effects of the specific treatment your child received, including ways of monitoring and treating these effects.
Reviewed by: Edward F. Attiyeh, MD, The Children's Hospital of Philadelphia
Date: November 2009
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