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Primary Liver Cancers

by Steven K. Herrine, MD

Primary liver cancers are cancers that originate in the liver. The most common is hepatocellular carcinoma (hepatoma—see 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.

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 removed, people may live several years or longer.

Cholangiocarcinoma

This relatively slow-growing cancer 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 (see 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 part of the abdomen, jaundice (a yellowish discoloration of the skin and the whites of the eyes), weight loss, and abdominal discomfort.

Fibrolamellar carcinoma

This type of hepatocellular carcinoma is rare. It usually affects relatively young adults. It is not caused by preexisting cirrhosis or hepatitis B or C and has no other known risk factors.

People with fibrolamellar carcinoma usually fare better than those with other types of hepatocellular carcinoma. Many live several years after this cancer is removed.

Hepatoblastoma

This cancer is rare but is one of the most common primary liver cancers in infants. Occasionally, it occurs in older children and may produce hormones (called gonadotropins) that result in early (precocious) puberty (see Early Puberty). No cause has been identified.

Health may generally deteriorate, and a mass may be felt in the upper right part of the abdomen.

Angiosarcoma

This rare cancer originates 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 doctors feel a large mass in the upper right part of an infant's abdomen and the infant’s health is deteriorating. Results of blood tests to measure levels of alpha-fetoprotein levels and imaging tests may help doctors make the diagnosis. Levels of alpha-fetoprotein—a protein normally produced by immature liver cells in fetuses—usually increase when liver cancer is present.

Cholangiocarcinoma in the liver, fibrolamellar carcinoma, hepatoblastoma, and angiosarcoma are diagnosed by liver biopsy (removal of a sample of liver tissue with a needle for examination under a microscope—see Biopsy of the Liver).

Cholangiocarcinoma of the bile ducts outside the liver is usually diagnosed using special x-ray techniques (such as endoscopic retrograde cholangiopancreatography [ERCP] or percutaneous transhepatic cholangiography—see Imaging Tests of the Liver and Gallbladder) or 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.