 Hepatoblastoma
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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.
As you read further below, you will find general information about hepatoblastoma. If you would like to view summary information about cancer first, see the cancer overview.
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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 and excretes a product called "bile," which helps carry away waste products from the liver.
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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 hepatoblastoma. Some hepatoblastomas have genetic alterations in tumor suppressor genes, which would explain the uncontrolled cell growth.
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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:
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- a large abdominal mass, or swollen abdomen
- weight loss, decreased appetite
- abdominal pain
- vomiting
- jaundice (yellowing of the eyes and skin)
- fever
- itching skin
- anemia (pale skin and lips from decreased number of red blood cells)
- back pain from compression of the tumor
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The symptoms of hepatoblastoma may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
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In addition to a complete medical history and physical examination, diagnostic procedures for hepatoblastoma may include:
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- biopsy - a sample of tissue removed from the tumor and examined under a microscope; the surgeon may also look at the liver using an instrument called a laparascope, a small tube with a light on the end
- complete blood count (CBC) - a measurement of size, number, and maturity of different blood cells in a specific volume of blood
- additional blood tests - may include blood chemistries, evaluation of liver and kidney functions, and genetic studies
- multiple imaging studies, including:
- computerized 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 cross-sectional images (often called slices), both horizontally and vertically, 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 which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film
- ultrasound (also called sonography) - a diagnostic imaging technique which 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.
- liver scans - pictures or x-rays taken of the bone after a dye has been injected that is absorbed by liver tissue. These are used to detect tumors and liver abnormalities.
- alpha-fetoprotein (AFP) test - alpha-fetoprotein (AFP) levels in the blood can be used to diagnose and follow response to treatment
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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:
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- stage I - usually a tumor that can be completely removed with surgery
- stage II - usually a tumor that can mostly be removed by surgery but very small amounts of the cancer are left in the liver
- stage III - usually a tumor that cannot be completely removed and the cancer cells are found in the 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.
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Specific treatment for hepatoblastoma will be determined by your child's physician based on:
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- your child's age, overall health, and medical history
- extent of the disease
- your child's tolerance for specific medications, procedures, and therapies
- how your child's physician expects the disease to progress
- your opinion or preference
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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):
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- surgery - current treatments require close coordination between pediatric surgeons and the pediatric oncologists. Whether the tumor can be removed initially is a decision that must be made by the pediatric surgeon. If it is deemed that removal would be extremely risky or have a significant chance of leaving tumor behind, then the child may be treated initially with chemotherapy. After 3 or 4 courses of treatment, resection is often much more readily accomplished. Complete removal of the tumor remains very important to achieving a cure in children with hepatoblastoma.
It is important to note that studies have shown that after removal of the tumor, including removal of a major segment of the liver, the liver has a remarkable capacity to regenerate or grow towards the normal volume that it had prior to removal. Long-term follow-up studies of children have shown that the liver can continue to grow and often remains very close to what would be the predicted normal size of the liver despite removal of up to 2/3 of the liver in infancy.
- chemotherapy - as mentioned above, a treatment used in conjunction with surgery and other treatments. Chemotherapy is a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of drugs work in different ways to fight cancer cells and shrink tumors. Chemotherapy may be used alone for some types of cancer or in conjunction with other therapy such as radiation or surgery. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat. While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.
Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:
- as an injection into the muscle or fat tissue
- intravenously (directly to the bloodstream; also called IV)
- intrathecally - chemotherapy given directly into the spinal column with a needle
- liver transplant - the liver of the child affected with hepatoblastoma may be replaced with a liver from a donor. Your child's physician will provide more information on whether this is an option for your child and the process involved.
- supportive care - any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment
- continuous follow-up care - a schedule of follow-up care determined by your child's physician and other members of your care team to monitor ongoing response to treatment and possible late effects of treatment
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Prognosis greatly depends on:
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- 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
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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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Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat hepatoblastoma. Children's Hospital participates in the current treatment protocols of the national Pediatric Oncology Group/Children's Oncology Group.
Other types of treatment currently being studied include:
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- angiogenesis inhibitors - substances that may be able to prevent the growth of tumors by blocking the formation of new blood vessels that feed the tumors
- biological therapies - a wide range of substances that may be able to involve the body's own immune system to fight cancer or lessen harmful side effects of some treatments
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