Primary liver cancers are cancers that originate in the liver. The most common is a hepatoma (hepatocellular carcinoma). At first, liver cancer usually causes only vague symptoms (such as weight loss, loss of appetite, and fatigue). As a result, the diagnosis is often made late, and the prognosis is usually poor.

Hepatoma

A hepatoma (hepatocellular carcinoma) is a cancer that begins in the liver cells.

• Having hepatitis B or hepatitis C or drinking excess alcohol increases the risk of developing a hepatoma.
• People may have abdominal pain, lose weight, and feel a large mass in the upper right abdomen.
• Doctors base the diagnosis on results of a blood test and imaging tests.
• Hepatomas are usually fatal unless they are diagnosed early.

Hepatomas are the most common type of cancer originating in the liver. They usually occur in people who have severe scarring of the liver (cirrhosis—see Fatty Liver, Cirrhosis, and Related Disorders: Cirrhosis).

In certain areas of Africa and East Asia, hepatomas are more common than metastatic liver cancer and are a common cause of death. In these areas, many people have chronic infection with the hepatitis B virus. The presence of this virus in the body increases the risk of hepatomas more than 100-fold. Hepatitis B can cause cirrhosis but can lead to hepatomas whether cirrhosis develops or not and whether the infection is chronic or not. Cirrhosis due to chronic hepatitis C also increases the risk of hepatomas.

Hepatomas sometimes result from exposure to certain cancer-causing substances (carcinogens). In subtropical regions where hepatomas are common, food is often contaminated by carcinogens called aflatoxins, substances that are produced by certain types of fungi.

In North America, Europe, and other geographic areas where hepatomas are less common, most people with hepatomas are alcoholics with long-standing cirrhosis. Other types of cirrhosis may also lead to hepatomas, but the risk is lower with primary biliary cirrhosis than with other types.

Symptoms

Usually, the first symptoms are abdominal pain, weight loss, and a large mass that can be felt in the upper right abdomen. Or people who have had cirrhosis for a long time may unexpectedly become much more ill. A fever may occur. Occasionally, the first symptoms are sudden abdominal pain and shock (dangerously low blood pressure) caused by rupture or bleeding of the hepatoma.

Occasionally, hepatomas interfere with how the body processes certain substances (metabolism). For example, hepatomas can lead to low sugar levels (hypoglycemia), high calcium levels (hypercalcemia), and high fat levels (hyperlipoproteinemia) in the blood.

Diagnosis

Detecting a hepatoma early is difficult because, at first, the symptoms do not provide many clues. If a doctor feels an enlarged liver or an imaging test detects a mass in the upper right abdomen during an examination for other purposes, the doctor may suspect a hepatoma, especially in people with long-standing cirrhosis.

If a hepatoma is suspected, the following are done:

• Blood tests to measure levels of alpha-fetoprotein: This protein is normally produced by the fetus, and levels decrease markedly by age 1 year. Levels are high in about half the people with a hepatoma.
• Physical examination: Doctors feel the upper right abdomen to check for an enlarged liver or mass. They may place a stethoscope over the liver to check for sounds caused by hepatomas. For example, they occasionally hear rushing sounds (hepatic bruits, which are due to blood rushing through blood vessels inside the cancer) or scratchy sounds (friction rubs, which are due to the cancer rubbing against the liver surface and surrounding structures).
• Imaging tests: Ultrasonography, computed tomography (CT), or magnetic resonance imaging (MRI) of the abdomen can sometimes detect hepatomas that have not yet caused symptoms. If the diagnosis is unclear, x-rays may be taken after a radiopaque dye (a dye visible on x-rays) is injected into the main artery in the liver (hepatic artery) to check for a hepatoma. This procedure (called hepatic arteriography) is particularly useful before surgical removal of the hepatoma because the x-rays show the precise location of the liver's blood vessels.

If the diagnosis is still unclear, a liver biopsy (removal of a small sample of liver tissue with a needle for examination under a microscope) can confirm the diagnosis (see Diagnostic Tests for Liver, Gallbladder, and Biliary Disorders: Biopsy of the Liver). The risk of bleeding or other injury during a liver biopsy is usually low.

Staging: If cancer is diagnosed, doctors determine how large the hepatoma is and whether it has spread to nearby structures or other parts of the body. The imaging tests used for diagnosis can provide some of this information. Doctors may also use a small viewing tube (laparoscope), inserted through a small incision in the abdomen, to look directly at the liver and nearby organs.

The cancer is classified ranging from stage I (a single tumor that has not spread) to stage IV (spread to distant parts of the body). Staging helps doctors decide on treatment and estimate survival.

Screening: In some areas where the hepatitis B virus is common, ultrasonography is used to screen people with hepatitis B for liver cancer. Screening usually involves measurement of the alpha-fetoprotein level and ultrasonography every 6 or 12 months.

Prognosis

In the United States, most people with a hepatoma do not live for more than a few years because the hepatoma is detected at a late stage. Screening and early detection result in a better prognosis. In some areas of East Asia, routine screening is more common. If the hepatoma is small and has not spread and liver transplantation can be done, the person can often live a number of years.

Prevention

Use of the vaccine against hepatitis B virus eventually reduces the incidence of hepatomas, especially in areas where they are common. Preventing the development of cirrhosis regardless of cause can also help. For example, treating chronic hepatitis C and alcoholism can help prevent hepatomas from developing.

Treatment

Only liver transplantation or surgical removal of the hepatoma offers any hope of cure. However, when the hepatoma is surgically removed, it often recurs. Also, removing the hepatoma in people who have cirrhosis may not be possible because too much of their liver is damaged.

If transplantation or surgery is not possible or if people are waiting for liver transplantation, treatments may be used to slow the hepatoma's growth and relieve symptoms. For example, the hepatoma's blood supply may be blocked by inserting particles into blood vessels to the hepatoma (a procedure called selective hepatic artery embolization). As a result, the hepatoma shrinks. Cold (cryoablation) or electrical energy (radiofrequency ablation) may be directly applied to the hepatoma to destroy cancer cells. These methods do not destroy all the cancer cells.

Chemotherapy drugs can be injected into a vein or into the hepatic artery, providing a high concentration of the drugs directly to cancer cells in the liver. Chemotherapy drugs can temporarily slow the growth of the hepatoma.

Radiation therapy is usually ineffective.

Other Primary Liver Cancers

Other primary liver cancers are uncommon or rare. For diagnosis, a biopsy is usually needed. Most people with these cancers have a poor prognosis. If the cancer has not spread, it can sometimes be removed. When it can be, people may live several years or longer.

A cholangiocarcinoma is a relatively slow-growing cancer that originates in the lining of the bile ducts in or outside the liver. In China, infestation with liver flukes (a parasite) contributes to the development of this cancer. People with primary sclerosing cholangitis are at risk of developing cholangiocarcinoma. Symptoms of the cancer are often vague but may include sudden deterioration of the person's general health, a mass in the upper right abdomen, jaundice (a yellowish discoloration of the skin and the whites of the eyes), weight loss, and abdominal discomfort.

A fibrolamellar carcinoma is a rare type of hepatoma that usually affects relatively young adults. It is not caused by preexisting cirrhosis, hepatitis B or C, or other known risk factors. People with fibrolamellar carcinoma usually fare better than those with other types of hepatoma. Many live several years after this hepatoma is removed.

A hepatoblastoma is a relatively common cancer in infants. Boys are affected twice as often as girls. Occasionally, it occurs in older children and may produce hormones (called gonadotropins) that result in early (precocious) puberty (see When Puberty Starts Too Early). No cause has been identified. Doctors may be able to diagnosis this cancer based on elevated alpha-fetoprotein levels and results of imaging tests.

An angiosarcoma is a rare cancer originating in the blood vessels of the liver. An angiosarcoma may be caused by exposure to vinyl chloride in the workplace, as occurs in the manufacture of polyvinyl chloride (PVC), or by exposure to arsenic. However, in most people, no cause is identified.

Diagnosis and Treatment

A hepatoblastoma is usually suspected when a doctor feels a large mass in an infant's upper right abdomen and the infant's general health is deteriorating.

Cholangiocarcinoma in the liver, hepatoblastoma, and angiosarcoma are diagnosed by liver biopsy (removal of a sample of liver tissue with a needle for examination under a microscope—see Diagnostic Tests for Liver, Gallbladder, and Biliary Disorders: Biopsy of the Liver).

Cholangiocarcinoma of the bile ducts outside the liver is usually diagnosed with special x-ray techniques (such as endoscopic retrograde cholangiopancreatography [ERCP] or percutaneous transhepatic cholangiography) or during surgery. In two thirds of people with this type of cancer, the cancer has already spread to nearby lymph nodes by the time it is detected.

Usually, treatment of these cancers has little effect, and most people die within a few months of when the cancer was detected. However, if the cancer is detected relatively early, it may be surgically removed, offering the hope of long-term survival.

Last full review/revision September 2007 by Steven K. Herrine, MD