What is Hepatocellular Carcinoma (HCC)?

Hepatocellular carcinoma (HCC), or childhood liver cancer, is a very rare disease in which cancer cells develop in the tissues of the liver and form a tumor.

The liver is one of the largest organs in the body. It is in the upper right-hand side of the abdomen under the ribs. The liver has many functions. For instance, it plays a vital role in turning food into energy. It also filters and stores blood.

HCC is one of two types of primary liver cancer. Hepatoblastoma is the other type of primary liver cancer. Metastatic liver tumors are those that start elsewhere and spread to the liver.

HCC is found in children from birth to 19 years of age. Children that are more likely than other children to get HCC include:

• Those with metabolic liver disease that causes liver scarring (such as tyrosinemia, alpha-1 antitrypsin deficiency, and others)
• Those who have hepatitis B or C (viral infections of the liver that cause swelling)
• Those with underlying conditions affecting the structure of their body (Abernathy-absence of the portal vein; Fontan procedure patients)

Cause of Hepatocellular Carcinoma

The cause of HCC is not known. It is not clear what causes the tumor to develop.

Signs and Symptoms of HCC

The early symptoms of HCC are often not unique to liver disorders. At the time of diagnosis, patients often:

• Are tired
• Have a fever
• Have pain in the belly
• Have a loss of appetite
• Appear thin and sick

As the cancer tumor grows, the patient may also have symptoms such as:

• Pain in the upper right part of the belly that stretches into the back and shoulder
• A buildup of fluid in the abdominal cavity (space inside the walls of the belly that contains most of the organs of digestion)
• Bleeding in the digestive tract (tube of the digestive system through which food passes and digestion takes place)
• Jaundice (a condition that causes the skin to turn yellow and the urine to become dark-colored)

Diagnosis of Hepatocellular Carcinoma

If your child's doctor suspects liver cancer, your child's history will be checked for risk factors. Your child's belly is also closely looked at during exams. Masses or lumps in the liver and belly often can be felt while your child is lying flat on the exam table. The liver most often is swollen and hard in patients with liver cancer.

The doctor will use a stethoscope to listen to the blood vessels that lie near the liver. They may be able to hear a sound that is not normal or a rubbing noise. The pressure of the tumor on the blood vessels causes the noises.

Your child's doctor may use other methods to diagnose HCC. They include:

Blood tests. Between 50 percent to 70 percent of liver tumor patients have high levels of serum alpha-fetoprotein. Blood tests can tell if high levels are present in your child's blood. This test cannot be used by itself to diagnose HCC, as other diseases can also cause high levels. More blood work (such as hepatitis B, iron, total iron binding capacity, ferritin and alpha-1 antitrypsin) may help find any risk factors.

Imaging studies. Imaging studies help find tissue in the liver that is not normal. Liver tissue as small as an inch across now can be found by ultrasound, computed tomography scan (CT scan), or magnetic resonance imaging (MRI). But these imaging studies cannot reliably tell the difference between a hepatocellular carcinoma and other abnormal masses or lumps of tissue.

Biopsy. A biopsy (when a sample of tissue is removed and checked) of the tumor may be done to provide a clear diagnosis of HCC. Either a sample of the liver or tissue fluid will be removed or the tumor will be fully resected (removed). A microscope will be used to see if there are cancer cells present.

Once HCC is found, more tests are done to find out if cancer cells have spread to other parts of the body. This is called staging. Your child's doctor needs to know the stage of the disease to provide the right treatment.

These stages are used to define HCC:

• Stage I: The cancer can be removed with surgery.
• Stage II: Most of the cancer may be removed in surgery, but microscopic (cannot be seen) amounts of cancer may remain in the liver after surgery.
• Stage III: Some of the cancer may be removed in surgery, but some of the tumor cannot be removed. Macroscopic (visible by the surgeon or by imaging) amounts remain either in the liver or in the lymph nodes (part of the body that filters out and gets rid of cancer cells and other waste products from the body).
• Stage IV: The cancer has spread to other parts of the body.

Treatment for Hepatocellular Carcinoma

There are two main kinds of treatment used for children with HCC. Treatment calls for a team of doctors to work together: a hepatologist (liver doctor), a surgeon (doctor who operates) and an oncologist (cancer doctor).

Surgery. Surgery may be used to take out the cancerous tumor and part of the liver where the cancer is found. If surgery to remove the tumor is not able to be done, a child may have a liver transplant. Liver transplantation is a treatment option in some patients with Stage II or III cancer. The transplant removes the diseased liver and replaces it with a healthy one from a donor. This is not an option if the cancer has spread beyond the liver.

Chemotherapy. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be given to your child before surgery to help shrink the size of the liver cancer. Your child may be given chemotherapy after surgery to kill any cancer cells that are left.

Chemotherapy for HCC is most often put into the body through a needle in a vein or artery or taken by mouth. The drug enters the bloodstream, travels through the body, and can kill cancer cells outside the liver. Your child's doctor also may give the chemotherapy directly into the blood vessels that go into the liver.

Your doctor may decide to put chemotherapy drugs into the liver's main artery 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. It also prevents the cancer cells from getting oxygen or other materials that they need to grow.

Your doctor may decide to put radiation beads into the artery supplying the tumor(s) with high dose radiation directly to the tumor. This is done in effort to shrink the cancer and make it more resectable.

The stage of your child's disease and your child's age and health will help the team decide which treatment to use. Most often, treatment for HCC is based on its stage:

• Stage I: Your child's treatment will likely be complete removal of the liver cancer by surgery. At times this is followed by chemotherapy
• Stage II: Your child's treatment will likely be removal of the liver cancer by surgery. At times this is followed by chemotherapy
• Stages III and IV: Your child's treatment will likely be chemotherapy and / or other treatment through the artery to the tumor (as above) to reduce the size of the tumor. Then there will be surgery to remove as much of the cancer as possible.

Long-Term Outlook for Children with HCC

A child's chances of recovery depend on the stage of the cancer (whether it has spread to other parts of the body), the treatment used, and the child's overall health.