Help for Patients and Caregivers : Liver Cancer

Primary liver cancer begins in the cells of the liver itself. Although many cancers are declining in the United States, new cases of primary liver cancer are increasing.

In the United States, cancer affecting the liver is more commonly metastatic cancer, which occurs when tumors from other parts of the body spread (metastasize) to the liver. Cancers that commonly spread to the liver include colon, lung and breast cancers. These cancers aren't called liver cancer. Instead, they are named after the organ in which the cancer began — such as metastatic colon cancer to describe cancer that begins in the colon and spreads to the liver. These metastatic cancers are treated based on where the cancer began, rather than being treated as primary liver cancers.

Primary liver cancer is rarely discovered early and often doesn't respond to current treatments — thus, the prognosis is often poor. Even when treatments fail to provide much improvement in the liver cancer itself, pain and other signs and symptoms caused by liver cancer can be aggressively treated to improve quality of life. But the most important news about primary liver cancer is that you can greatly reduce your risk by protecting yourself from hepatitis infection and cirrhosis, the leading causes of the disease.

Most people don't have signs and symptoms in the early stages of liver cancer, which means the disease may not be detected until it's quite advanced. When symptoms do appear, they may include some or all of the following:

• Loss of appetite and weight
• Abdominal pain, especially in the upper right part of your abdomen, that may extend into your back and shoulder
• Nausea and vomiting
• General weakness and fatigue
• An enlarged liver
• Abdominal swelling (ascites)
• A yellow discoloration of your skin and the whites of your eyes (jaundice)

Your liver is a football-sized organ that sits in the upper right portion of your abdomen, beneath your diaphragm and above your stomach. Your liver processes most of the nutrients absorbed from your small intestine and determines how much sugar (glucose), protein and fat enter your bloodstream. It also manufactures blood-clotting substances and certain proteins. Your liver performs a vital detoxifying function by removing drugs, alcohol and other harmful substances from your bloodstream.

Liver cancer occurs when liver cells begin to grow abnormally. It's not completely understood why this happens, but researchers believe that cancer starts with damage to DNA — the material that contains the instructions for every chemical process in your body, including the rate of cellular growth. DNA damage causes changes in these instructions. One result is that cells may begin to grow out of control and eventually form a tumor — a mass of malignant cells.

Primary liver cancer
Primary liver cancer is divided into several types based on the type of cells that become cancerous. Types include:

• Hepatocellular carcinoma (HCC). This is the most common form of primary liver cancer in both children and adults. It starts in the hepatocytes, the main type of liver cell.
• Cholangiocarcinoma. This type of cancer begins in the small tube-like bile ducts within the liver. This type of cancer is sometimes called bile duct cancer.
• Hepatoblastoma. This rare type of liver cancer affects children younger than 4 years of age. Most children with hepatoblastoma can be successfully treated.
• Angiosarcoma or hemangiosarcoma. These rare cancers begin in the blood vessels of the liver and grow very quickly.

Metastatic cancer
In the United States, most cancer found in the liver has spread there from another part of the body. Rather than being referred to as liver cancer, this type of cancer is usually named after the organ where it originated and is described as "metastatic." For instance, cancer that has spread to the liver from the colon is referred to as metastatic colon cancer.

Metastatic cancers form when malignant cells detach from the primary cancer and travel through the body in the circulatory or lymphatic system. Cancers that begin in certain organs near the liver, such as the pancreas, can spread directly to the liver. Most metastatic cancers reach the liver through the bloodstream. Why the liver is so commonly affected by metastatic cancer isn't clear. One reason may be the liver's rich blood supply.

Primary liver cancer can affect people of all ages and races, but certain factors may increase your risk, including:

• Sex. Men are more likely to develop liver cancer than are women, though it isn't clear why.
• Age. In the United States and Europe, liver cancer diagnosis occurs on average at about age 60. People in Asia and Africa tend to be diagnosed with liver cancer at younger ages — between 20 and 50.
• Chronic infection with HBV or HCV. Chronic infection with hepatitis B virus (HBV) or hepatitis C virus (HCV) is by far the most important risk factor for liver cancer.
• Cirrhosis. This progressive and irreversible condition causes scar tissue to form in your liver and increases your chances of developing liver cancer.
• Diabetes. People with this blood sugar disorder have a greater risk of liver cancer than do people who don't have diabetes. Having both diabetes and hepatitis C infection increases the risk even more.
• Exposure to aflatoxins. Consuming foods contaminated with fungi that produce aflatoxins greatly increases the risk of liver cancer. Crops such as corn, soybeans and peanuts can become contaminated with aflatoxins.
• Excessive alcohol consumption. Consuming more than a moderate amount of alcohol can lead to irreversible liver damage and increase your risk of liver cancer. Moderate consumption is defined as no more than two drinks a day for men and one drink for women. A drink is one 4- to 5-ounce glass of wine, 12 ounces of beer or a 1.5-ounce shot of 80-proof distilled spirits.
• Smoking. Smoking tobacco of any kind makes it more likely that you'll develop liver cancer.
• Bile duct disease. A disease called primary sclerosing cholangitis can cause inflammation and scarring of the liver's bile ducts. This increases your risk of bile duct cancer.

Screening
Screening for liver cancer hasn't been definitively proved to reduce the risk of dying of liver cancer. For this reason, many medical groups don't recommend liver cancer screening. However, the American Association for the Study of Liver Diseases recommends liver cancer screening for those thought to have a high risk, including people who have:

• Hepatitis B and one or more of the following: Are an Asian male older than 40, Asian female older than 50 or African and older than 20, have liver cirrhosis, or have a family history of liver cancer
• Liver cirrhosis from alcohol use
• Hepatitis C
• An inherited form of hemochromatosis
• Primary sclerosing cholangitis

Discuss the pros and cons of screening with your doctor. Together you can decide whether screening is right for you based on your risk. Screening typically involves blood tests and an ultrasound exam once or twice each year.

Diagnosis
If you experience any of the symptoms of liver cancer, your doctor will ask you about your medical history and perform a physical exam. Tests and procedures used to diagnose liver cancer include:

• Ultrasound (ultrasonography). This test uses sound waves to produce a picture of internal organs, including the liver. Ultrasound is painless and usually takes less than 30 minutes. While you lie on a bed or examining table, a wand-shaped device (transducer) is placed on your body. It emits sound waves that are reflected from your liver and transformed into a computer image. Ultrasound provides information about the shape, texture and makeup of tumors.
• Computerized tomography (CT) scan. This test uses X-rays to produce cross-sectional images of your body. You may also have a variation of the test — known as a CT angiogram — in which contrast dye is injected into an artery in your liver. X-rays then track the dye as it flows through the blood vessels in your liver. A CT angiogram, which may take up to an hour to perform, can provide detailed information on the number and location of liver tumors, but a CT scan exposes you to more radiation than conventional X-rays do, and some people may experience an allergic reaction to the contrast dye.
• Magnetic resonance imaging (MRI). MRI creates images using a magnetic field and radio waves. Sometimes a contrast dye also may be used. The test can take from 15 minutes to an hour. Newer MRIs can show images of the ducts that transport bile from the liver to the upper part of the small intestine (duodenum) as well as of the arteries and veins within the liver.
• Liver biopsy. In this procedure, a sample of tissue is removed from your liver and examined under a microscope. Liver biopsy is considered the only definitive way to diagnose liver cancer. Your doctor may use a thin needle or a lighted instrument (laparoscope) to obtain the sample. Biopsy carries a risk of bleeding, bruising and infection.
• Blood tests. Doctors sometimes use a blood test that checks for the presence of alpha-fetoprotein (AFP) — a type of protein found in small amounts in adults — to detect liver cancer. But the test isn't perfect. Not all malignant liver tumors produce AFP, and those that do may be advanced by the time protein levels become elevated. In addition, other types of cancer and even some noncancerous liver diseases can raise AFP levels.

Staging
Staging tests help determine the size and location of cancer and whether it has spread. Liver cancer may be staged in different ways. One method uses the Roman numerals I through IV, with higher numbers indicating cancers that are more advanced. A stage I tumor is small and confined to one lobe of the liver. By stage IV, several tumors may exist in different lobes, or malignant cells may have spread to other parts of the body.

Doctors may also use the following stages to describe primary liver cancer in adults:

• Localized resectable. At this stage, the tumor is confined to one lobe of your liver and can be completely removed in an operation. The term "resectable" refers to a tumor that can be surgically removed.
• Localized unresectable. The cancer is found in only one part of your liver, but can't be completely removed, either because the noncancerous portion of your liver isn't healthy or because the cancer is located near your liver's main arteries, veins and bile ducts.
• Advanced. This stage of cancer has spread throughout the liver or to other parts of your body, particularly the bones or lungs. You're more likely to have advanced cancer if you also have cirrhosis or chronic hepatitis.
• Recurrent. This means the cancer has returned to your liver or to another part of your body after being treated.

Stages of primary cancer in children
Doctors use the following stages to describe childhood liver cancer:

• Stage I. At this stage, the cancer can be removed with surgery.
• Stage II. Most stage II liver cancers can be removed with an operation, but microscopic amounts of cancer remain in the liver after surgery.
• Stage III. At this stage, some of the cancer may be surgically removed, but some will remain in the lymph nodes or abdomen.
• Stage IV. This stage of cancer has spread to other parts of the body.
• Recurrent. This means the cancer has returned after it has been treated. It may recur in the liver or in another part of the body.

People with liver cancer may sometimes experience the following complications:

• Liver failure. This occurs when the liver is no longer able to function adequately. It usually develops when there is extensive damage to liver cells.
• Kidney failure. The kidneys also may fail, losing their ability to filter fluids and waste and causing dangerous levels of these substances to accumulate in the body.
• Spread of the cancer cells (metastasis). Cancer that spreads to areas outside the liver becomes more difficult to treat. Liver cancer most commonly spreads to the lungs and bones.

Treatments for primary liver cancer depend on the extent (stage) of the disease as well as your age, overall health, feelings and personal preferences. Discuss all of your options carefully with your treatment team.

The goal of any treatment is to eliminate the cancer completely. When that isn't possible, the focus may be on preventing the tumor from growing or spreading. In some cases palliative care only is appropriate. Palliative care refers to treatment aimed not at removing or slowing the disease but at helping relieve symptoms and making you as comfortable as possible.

Treatments for primary liver cancer in adults
Treatments for adults with primary liver cancer include:

• Surgery. The best treatment for localized resectable cancer is usually an operation known as surgical resection. In some cases, the area of the liver where the cancer is found can be completely removed. You aren't a candidate for surgical removal of liver tumors if you have cirrhosis or only a small amount of healthy liver tissue. Even when resections are successful, there is a chance the cancer can recur elsewhere in the liver or in other areas within a few years.
• Alcohol injection. In this procedure, pure alcohol is injected directly into tumors, either through the skin or during an operation. Alcohol dries out the cells of the tumor and eventually the cells die. Each treatment consists of one injection, although you may need a series of injections for the best results. Alcohol injection has been shown to improve survival in people with small hepatocellular tumors. It may also be used to help reduce symptoms in cases of metastatic liver cancer. The most common side effect is leaking of alcohol onto the liver or into the abdominal cavity.
• Radiofrequency ablation. In this procedure, electric current in the radiofrequency range is used to destroy malignant cells. Using an ultrasound or CT scan as a guide, your surgeon inserts several thin needles into small incisions in your abdomen. When the needles reach the tumor, they're heated with an electric current, destroying the malignant cells. Radiofrequency ablation is an option for people with small, unresectable hepatocellular tumors and for some types of metastatic liver cancers. Although the procedure has a somewhat higher risk of serious complications than alcohol injection does, it appears to provide better outcomes.
• Chemoembolization. Chemoembolization is a type of chemotherapy treatment that supplies strong anti-cancer drugs directly to the liver. Chemoembolization isn't curative, but it can shrink tumors in a certain percentage of people, which may provide symptom relief and improve survival. During the procedure, the hepatic artery — the artery from which liver cancers derive their blood supply — is blocked, and chemotherapy drugs are injected between the blockage and the liver. The idea is that by targeting the tumor directly, doctors can use potent doses of drugs without creating as many side effects as occur with systemic chemotherapy. But the fact is that chemoembolization causes many of the same side effects as other forms of chemotherapy, including abdominal pain, nausea and vomiting. Chemoembolization is less likely to cause some side effects such as lowered blood cell counts or hair loss.
• Cryoablation (cryosurgery or cryotherapy). This treatment uses extreme cold to destroy cancer cells. Cryoablation may be an option for people with inoperable primary and metastatic liver cancers. It may also be used in addition to surgery, chemotherapy or other standard treatments. During the procedure, your doctor places an instrument (cryoprobe) containing liquid nitrogen directly onto liver tumors. Ultrasound images are used to guide the cryoprobe and monitor the freezing of the cells. Side effects include damage to the bile ducts and major blood vessels, leading to bleeding or infection.
• Radiation therapy. This treatment uses high-powered energy beams to destroy cancer cells and shrink tumors. Radiation may come from a machine outside your body or from radiation-containing materials inserted into your liver. Radiation may be used on its own to treat localized unresectable cancer. Or you may have radiation therapy following surgical removal of a tumor to help destroy any remaining malignant cells. Radiation side effects may include fatigue, nausea and vomiting.
• Chemotherapy. This treatment uses powerful drugs to kill cancer cells. Chemotherapy may be systemic — meaning it travels throughout your body in your bloodstream — or regional. Systemic chemotherapy is generally not effective in treating liver cancer, but may be a treatment option in certain cases.
• Liver transplantation. In this surgical procedure, a diseased liver is removed and replaced with a healthy, donated organ. Liver transplantation may be an option for some people with small, early-stage liver tumors and for certain people with bile duct tumors. In other cases, especially when tumors are larger or blood vessels are involved, a transplant may not improve long-term outlook because the cancer may recur outside the new liver.
• Sorafenib (Nexavar). Sorafenib was approved by the Food and Drug Administration in 2007 for use in advanced inoperable liver cancer. Sorafenib is a targeted therapy designed to interfere with a tumor's ability to generate new blood vessels. Sorafenib has been shown to slow or stop advanced liver cancer from progressing for a few months longer than with no treatment. More studies are needed to understand how targeted therapies may be used to control advanced liver cancer.

Treatments for primary liver cancer in children
Liver cancer in young people is rare. As a result, most children with the disease are treated at centers that specialize in childhood cancers. In general, the treatments available for children are the same as for adults, and the best approach depends on the stage and type of cancer as well as the child's age and overall health.

Clinical trials
Because standard treatments often aren't effective in treating liver cancer, you may want to consider participating in a clinical trial — a research study that tries to improve current treatments or find new treatments. This can give you access to experimental therapies that might not otherwise be available. There are no guarantees with clinical trials, however, and you should fully understand the potential risks as well as possible benefits before taking this step.

In many cases it's not possible to prevent the spread of cancer from another site to the liver. And it may not always be possible to prevent primary liver cancer. But you can greatly reduce your risk by taking steps to protect yourself from hepatitis B and C, cirrhosis and other liver diseases.

Get vaccinated
The single most effective way to prevent hepatitis B is to receive the hepatitis B vaccine, which provides more than 90 percent protection for both adults and children. Protection lasts years and may even be lifelong. The vaccine can be given to almost anyone, including infants, older adults and those with compromised immune systems. Infants often receive the vaccine in the first year of life — typically at 2, 4 and 9 months of age.

Take measures to prevent hepatitis C
Because no vaccine for hepatitis C exists, the following measures can play a key role in protecting your health:

• Educate yourself and others. Make sure you understand what viral hepatitis is and how it's transmitted.
• Know the health status of any sexual partner. Don't engage in unprotected sex unless you're absolutely certain your partner isn't infected with HBV, HCV or any other sexually transmitted disease. If you don't know the health status of your partner, use a new latex condom every time you have vaginal or anal sex. If you don't have a male condom, use a female condom.
• Don't use IV drugs, but if you do, use a clean needle. The best way to protect yourself from HCV is not to inject drugs. But if that isn't an option for you, make sure any needle you use is sterile, and don't share it. Contaminated drug paraphernalia is responsible for about half of all new hepatitis C cases. Take advantage of needle exchange programs in your community and consider seeking help for your drug use.
• Avoid body piercing and tattooing. Needles that may not be properly sterilized can spread the virus.
• Be cautious about blood products in certain countries. Most Americans with HCV became infected through blood transfusions received before 1992 — the year improved blood-screening tests became available. Although the blood supply is now well screened in the United States, this isn't always the case in other countries. If an emergency requires that you receive blood or blood products in another country, get tested for HCV and HBV as soon as you return home.
• Avoid or limit alcohol. Alcohol speeds the progression of any liver disease you may have and is the leading cause of cirrhosis — a key factor in primary liver cancer.
• Avoid medications that may cause liver damage. Your doctor can advise you about these medications, which may include over-the-counter medications as well as prescription drugs. Avoid mixing alcohol and acetaminophen (Tylenol, others) — a combination known to cause liver damage.
• Avoid exposure to environmental toxins. Your liver filters every substance you ingest, inhale or apply to your skin. For that reason, avoid unnecessary chemical exposure.

Learning you have any life-threatening illness can be devastating. But coping with a diagnosis of liver cancer can be especially difficult. The more advanced the disease when it's discovered, the less likely the chance of cure. As a result, you may feel overwhelmed just when you need to make crucial decisions. You're also likely to be even more concerned about others than yourself. How will you tell your children? Will your partner be able to cope? Who will take care of all of the things you normally do if you can't?

Although there are no easy answers for people dealing with liver cancer, some of the following suggestions may be of help:

• Learn all you can about your illness. Learn everything you can about liver cancer — how the disease progresses, your prognosis and your treatment options, including both experimental and standard treatments and their side effects. Be sure you understand whether a particular approach is used to treat cancer or provide palliative care. Don't be afraid to seek a second opinion and to explore treatments available through clinical trials. You will have many decisions to make in the weeks and months ahead. The more you know, the more active role you can take in the decision-making process.

  In addition to talking to your medical team, look for information in books and reputable sources on the Internet. The National Cancer Institute offers a toll-free information line called the Cancer Information Service. It provides access to trained counselors and accurate, up-to-date information on all aspects of living with cancer. You can reach the Cancer Information Service 24 hours a day at 800-4-CANCER, or 800-422-6237.

• Maintain a strong support system. Strong relationships are crucial in dealing with life-threatening illnesses. Although friends and family can be your best allies, in some cases they may have trouble dealing with your illness. Or you may not have a large social network. If so, the concern and understanding of a counselor, medical social worker or even a formal support group can be helpful. Although support groups aren't for everyone, they can sometimes be a good resource for practical information about your disease. You may also find strength and encouragement in being with people who are facing the same challenges you are.

  If you're interested in learning more about support groups, talk to a doctor, nurse, social worker or psychologist. They may be able to put you in touch with a group in your area. Or check your local phone book, library or a cancer organization. The National Cancer Institute also can provide a list of support groups. After deciding to participate in a group, try it out a few times. If it doesn't seem useful or comfortable, you don't have to continue.

• Come to terms with your illness. Coming to terms with your illness may be the hardest thing you've ever done. For some people, having a strong faith or a sense of something greater than themselves makes this process easier. Others seek counseling from someone who understands life-threatening illnesses, such as a medical social worker, psychologist or chaplain. Many people also take steps to ensure that their end-of-life wishes are known and respected.