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Study Led by Dr. Rakesh Sindhi Shows Increase in Liver Transplantation for Hepatoblastoma
Hepatoblastoma is the most common liver cancer of childhood, although it affects only 2 to 3 people in a million. It affects children during the first three years of life and usually presents as an abdominal mass which causes pain and discomfort. A large percentage of these tumors occur in children who are born premature. The tumor can be cured with surgical removal. If surgical removal is hazardous because of size and location of this tumor inside the liver, cure is still possible with liver transplantation.
It is estimated that a fifth of hepatoblastoma tumors may require liver transplantation. Also, hepatoblastoma now accounts for 7.5% of all liver transplants in children compared with <3% for other pediatric liver cancers. Chemotherapy is started immediately upon diagnosis and is also given after liver transplantation. Up to 80% of children treated with liver transplantation survive longer than 20 years without experiencing recurrences, if the tumor is confined to the liver.
Recurrences can occur in 16% of children after transplantation. Recurrences are most common during the first two years, and rarely occur thereafter. Hepatoblastoma most commonly spreads to the lungs. Interestingly, even if the tumor is present outside the liver, eliminating it with surgery or chemotherapy before transplantation can still bring about cure in up to half of such children.
This potential for cure, even if the tumor has spread outside the liver, or if the tumor is extremely large makes it imperative that children with hepatoblastoma be referred for surgery and/or transplantation as soon as possible.
Hepatoblastoma must be categorically distinguished from the more common hepatocellular cancer. Hepatocellular cancer affects adults, usually occurs in individuals with preexisting infectious hepatitis or cirrhotic liver diseases and responds poorly to chemotherapy. Also, survival is poor if tumors are large, invade blood vessels, or have spread outside the liver. Because so much more is known about hepatocellular carcinoma, one can easily assume that a large hepatoblastoma which invades blood vessels or has spread to a single site outside the liver is inoperable because it likely behaves like the "usual" liver cancer. Multiple studies from several centers have shown that this is not the case. Another unique aspect of treating hepatoblastoma with liver transplantation concerns a more primitive type of hepatoblastoma referred to as "anaplastic". Surgical removal of this type of hepatobalstoma is followed by recurrence inside the liver in the majority. Among 35 children with hepatoblastoma treated at the Hillman Center for Pediatric Transplantation at Children's Hospital of Pittsburgh of UPMC, recurrences were not any more or less common after liver transplantation for this type of hepatoblastoma.
Therefore, the best option once hepatoblastoma has been diagnosed is to work with an expert hematologist-oncologist who cares for pediatric liver cancer. Over the last decade, excellent chemotherapy protocols have been tested by the Pediatric Cooperative Oncology Group. These protocols can be implemented by pediatric oncologists while referrals are being made for removal of the tumor by surgery or transplantation.
Symptoms of liver cancer in the early stages are vague and often go unnoticed. Liver cancer can cause the following symptoms:
These symptoms may also be caused by other, less serious health conditions. A person experiencing these symptoms should see a doctor.
Your child's doctor will ask about your child's symptoms and review his or her medical history, as well as performing a physical examination. If the doctor suspects liver cancer, he or she will recommend tests to confirm or rule out the diagnosis. Tests may include:
Once liver cancer is found, more tests will be done to find out if cancer cells have spread to other parts of the body. Doctors call this process "staging" because they need to find out what stage the cancer is in. Once they have that information, your child's doctors can plan the best treatment for your child. The following stages are used for childhood liver cancer:
Treatments for childhood liver cancer depend on the type (hepatoblastoma or hepatocellular carcinoma) and stage of your child's disease, as well as your child's age and general health.
Surgery may be used to take out the cancer and part of the liver where the cancer is found. Surgery may also be used to remove any cancer that has spread to other parts of the body.
For some patients with earlier stages of liver cancer (particularly hepatocellular cancer), liver transplantation may be an option. Surgeons can replace the liver with a donor's healthy liver. Your child's doctor will discuss this option with you if it is a feasible treatment.
Chemotherapy uses drugs to kill cancer cells. Your child may receive chemotherapy before surgery to help reduce the size of the liver cancer, or after surgery to kill any remaining cells. Chemotherapy given after surgery when the doctor has removed the cancer is called adjuvant chemotherapy. Chemotherapy for childhood liver cancer is usually put into the body through a needle in a vein or artery. This type of chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells outside the liver. In another type of chemotherapy, called direct infusion chemotherapy, drugs are injected directly into the blood vessels that go into the liver.
A special treatment called chemo-embolization is sometimes used to treat childhood liver cancer. Chemotherapy drugs are injected into the main artery of the liver with substances that block or slow the flow of blood into the cancer. This lengthens the time the drugs have to kill the cancer cells and it also prevents cancer cells from getting the oxygen and nutrients they need to grow.
Radiation therapy uses X-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external radiation therapy) or from putting materials that produce radiation through thin plastic tubes in the area where the cancer cells are found (internal radiation therapy).
Treatment that uses medications or substances made by the body to increase or restore the body's natural defenses against cancer is called biological therapy, or biological response modifier (BRM) therapy.
Once treatment is completed, your child will have regular blood tests to measure the level of alpha-fetoprotein (if appropriate), as well as scans and chest X-rays. This allows your child's doctors to monitor the effectiveness of the treatment and catch any recurrence of cancer early.
Like his or her treatment plan, your child's chance of recovery (prognosis) depends on the stage of liver cancer (particularly whether or not it has spread), how the cancer cells look under a microscope (the histology), and your child's general state of health. As with most cancers, cure rates for children are much higher than for adult cancers. More than half of the children with hepatoblastoma are cured, and for children with small tumors only in the liver the prospects are even better.
Learn about other Liver Disease States.
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One Children’s Hospital Way
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Pittsburgh, PA 15224
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