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What are liver tumors?
Liver tumors are masses occurring in the liver that can be either benign or malignant (cancerous). Tumors that are found at birth or early after delivery are often benign in nature. While these require removal, no additional treatment with chemotherapy or radiotherapy is required. One type of benign tumor is called mesenchymal hamartoma. This tumor represents an abnormal collection of tissues seen in the developing fetus, but not normally occurring in the liver. Surgical removal of the tumor is the only treatment required
There are generally two types of rare malignant tumors and these require more extensive treatment. They are:
Hepatoblastoma is a cancerous liver tumor. The liver, the largest organ in the body, consists of right and left lobes. The disease occurs primarily in younger infants and children. With prompt treatment, hepatoblastoma is remarkably responsive to chemotherapy. Hepatoblastoma cancer cells can spread (metastasize) to other areas of the body. The most common sites of metastasis are the lungs, abdomen and abdominal structures, and rarely to bone, the central nervous system and bone marrow.
Hepatocellular (liver carcinoma):
Hepatocellular carcinoma is a rare disease in which cancerous cells are found in the tissues of the liver. This type of cancer is found in children from birth to 19 years of age, but usually does not occur before the age of 15. The median age is 12 years old.
Hepatocellular carcinoma may occur in multiple sites within the liver, and is much less responsive to chemotherapy than hepatoblastoma. Cancer cells can also 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. 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 liver tumors?
Although the exact cause of most liver tumors is unknown, there are a number of genetic conditions that are associated with an increased risk for developing childhood liver cancers. Hepatoblastoma is associated with Beckwith-Wiedemann syndrome, hemihypertrophy, and familial adenomatous polyposis.
Other genetic conditions associated with liver cancers include several inborn errors of metabolism such as tyrosinemia, glycogen storage disease type 1, galactosemia, and alpha-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 liver cancer.
Hepatocellular carcinoma may arise in livers with an underlying abnormality such as familial cholestatic cirrhosis, giant cell hepatitis of infancy, Fanconi's anemia, and glycogen storage disease. Children who are exposed to hepatitis B or C infections at an early age are at increased risk for developing hepatocellular carcinoma. Some hepatocellular carcinomas and hepatoblastomas have genetic alterations in tumor suppressor genes, which would explain the uncontrolled cell growth.
What are the symptoms of liver tumors?
The following are the most common symptoms of liver tumors. However, each child may experience symptoms differently. Symptoms may vary depending on the size of the tumor, whether it is benign or malignant and if malignant, the presence and location of metastases. Symptoms may include:
How are liver tumors diagnosed?
In addition to a complete medical history and physical examination, diagnostic procedures for hepatocellular carcinoma 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 hepatocellular carcinoma. Always consult your child's physician for information on staging. One method of staging is the following:
What are the treatments for liver tumors?
Specific treatment for hepatocellular carcinoma will be determined by your child's physician based on:
Treatment for benign tumors, such as mesenchymal hamartoma, requires surgical removal of the tumor, and usually, no further therapy.
Treatments for liver cancers require close coordination between the pediatric surgeons and pediatric oncologist who are managing your child's condition. Treatment for liver cancer usually includes a combination of the following:
What is the long-term outlook for patients with liver tumors?
Children who have had a benign tumor removed usually have no further problems. Children with hepatoblastoma can often be cured. Studies have shown that after the cancerous tumor is removed, along with the 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 continues to grow and often remains very close to what would be the predicted normal size of the liver despite removal of up to two-thirds of the liver during infancy. Long-term insufficiency of the liver is rarely seen except in those children who have the significant metabolic abnormalities associated with hepatocellular carcinoma. These children would require liver transplantation.
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 a liver cancer. New methods are continually being discovered to improve treatment and to decrease side effects.
What is the latest research on liver cancer?
Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat liver cancers.
Other types of treatment currently being studied include:
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