Introduction

Lung cancer is the leading cause of cancer deaths in the United States, among both men and women. It claims more lives each year than colon, prostate, lymph and breast cancers combined.

Yet most lung cancer deaths could be prevented. That’s because smoking accounts for nearly 90 percent of lung cancer cases. Your risk of lung cancer increases with the length of time and number of cigarettes you smoke. If you quit smoking, even after smoking for many years, you can significantly reduce your chances of developing lung cancer. Protecting yourself from other risk factors for lung cancer, such as exposure to asbestos, radon and secondhand smoke, also decreases your risk.

Signs and symptoms

Lung cancer typically doesn’t cause signs and symptoms in its earliest stages. Signs and symptoms of lung cancer typically occur only when the disease is advanced. Signs and symptoms of lung cancer may include:

• A new cough that doesn’t go away
• Changes in a chronic cough or “smoker’s cough”
• Coughing up blood, even a small amount
• Shortness of breath
• Chest pain
• Wheezing
• Hoarseness

Causes

Lung cancer most commonly begins in the cells that line your lungs. Smoking causes the majority of lung cancers — both in smokers and in people exposed to secondhand smoke. But lung cancer also occurs in people who never smoked. In these cases, there may be no clear cause of lung cancer. Doctors have identified factors that may increase the risk.

How smoking causes lung cancer
Doctors believe smoking causes lung cancer by damaging the cells that line the lungs. When you inhale cigarette smoke, which is full of cancer-causing substances (carcinogens), changes in the lung tissue begin almost immediately. At first your body may be able to repair this damage. But with each repeated exposure, normal cells that line your lungs are increasingly damaged. Over time, the damage causes cells to act abnormally and eventually cancer may develop.

Your lungs are full of blood vessels and lymph vessels, giving lung cancer cells easy access to travel to other parts of your body. For this reason, lung cancer may spread to other parts of your body before you experience any signs or symptoms. In many cases, lung cancer may spread before it can even be detected in the lungs.

Types of lung cancer
Doctors divide lung cancer into two major types based on the appearance of lung cancer cells under the microscope. Your doctor makes treatment decisions based on which major type of lung cancer you have. The two general types of lung cancer include:

• Small cell lung cancer. Small cell lung cancer, sometimes called oat cell carcinoma, occurs almost exclusively in heavy smokers and is less common than non-small cell lung cancer.
• Non-small cell lung cancer. Non-small cell lung cancer is an umbrella term for several types of lung cancers that behave in a similar way. Non-small cell lung cancers include squamous cell carcinoma, adenocarcinoma and large cell carcinoma.

Risk factors

A number of factors may increase your risk of lung cancer. Some risk factors can be controlled, for instance, by quitting smoking. And other factors can’t be controlled, such as your sex. Risk factors for lung cancer include:

• Smoking. Smoking remains the greatest risk factor for lung cancer. Your risk of lung cancer increases with the number of cigarettes you smoke each day and the number of years you have smoked. Quitting at any age can significantly lower your risk of developing lung cancer.
• Your sex. Current or former women smokers have a greater risk of lung cancer than do men who have smoked an equal amount. Though it isn’t known why, some experts speculate that women have a greater susceptibility to the cancer-causing substances found in tobacco. Others believe that estrogen may play a role. Women also are known to inhale more than men do, and they are less likely to quit.
• Exposure to secondhand smoke. Even if you don’t smoke, your risk of lung cancer increases if you’re exposed to secondhand smoke.
• Exposure to radon gas. Radon is produced by the natural breakdown of uranium in soil, rock and water that eventually becomes part of the air you breathe. Although unsafe levels of radon can accumulate in any building, the greatest exposure risk most people face is at home. Radon testing can determine whether levels are safe.
• Exposure to asbestos and other chemicals. Workplace exposure to asbestos and other substances known to cause cancer — such as arsenic, chromium, nickel and tar soot — also can increase your risk of developing lung cancer, especially if you’re a smoker.
• Family history of lung cancer. People with a parent, sibling or other first-degree relative with lung cancer have an increased risk of the disease.
• Excessive alcohol use. Drinking more than a moderate amount of alcohol — no more than one drink a day for women or two drinks a day for men — may increase your risk of lung cancer.

Screening and diagnosis

Screening
Doctors aren’t sure whether you should undergo screening for lung cancer. Even if you have an increased risk of lung cancer — for instance, if you’re a smoker — it isn’t clear that a chest X-ray or computerized tomography (CT) scan can be beneficial. Some studies show that these tests can find cancer earlier, when it may be treated more successfully. But other studies find that these tests often reveal more benign conditions that require invasive testing and expose people to unnecessary risks.

Screening for lung cancer is controversial among doctors. Studies are ongoing to determine what types of tests may be helpful and who would benefit from lung cancer screening. In the mean time, talk with your doctor if you’re concerned about your risk of lung cancer. Together you can determine strategies to reduce your risk and decide whether screening tests are appropriate for you.

Diagnosis
If there’s reason to think that you may have lung cancer, your doctor can order a number of tests to look for cancerous cells and to rule out other conditions. In order to diagnose lung cancer, your doctor may recommend:

• Imaging tests. An X-ray image of your lungs may reveal an abnormal mass or nodule. A CT scan can reveal small lesions in your lungs.
• Sputum cytology. If you have a cough and are producing sputum, looking at the sputum under the microscope can sometimes reveal the presence of lung cancer cells. Before the test, you may be asked to breathe a mildly irritating mist to help you produce more sputum.
• Tissue samples (biopsy). A sample of abnormal cells may be removed in a procedure called a biopsy in order to diagnose lung cancer. Your doctor can perform a biopsy in a number of ways, including bronchoscopy, in which your doctor examines abnormal areas of your lungs using a lighted tube that is passed down your throat and into your lungs; mediastinoscopy, in which an incision is made at the base of your neck and surgical tools are inserted behind your breastbone to take tissue samples; and needle biopsy, in which your doctor uses X-ray or CT images to guide a needle through your chest and into a suspicious lump or nodule to collect cells. A biopsy sample may also be taken from lymph nodes or other areas where cancer has spread, such as your liver.

Staging
Once your lung cancer has been diagnosed, your doctor will work to determine the extent, or stage, of your cancer. Your cancer’s stage helps your doctor decide what treatment is most appropriate. Staging tests may include imaging procedures that allow your doctor to look for signs that cancer has spread beyond your lungs, such as magnetic resonance imaging (MRI), positron emission testing (PET) and bone scans. Not every test is appropriate for every person, so talk with your doctor about which procedures are appropriate for you.

Stages of non-small cell lung cancer

• Stage I. Cancer at this stage has invaded the underlying lung tissue but hasn’t spread to the lymph nodes.
• Stage II. This stage cancer has spread to neighboring lymph nodes or invaded the chest wall.
• Stage IIIA. At this stage, cancer has spread from the lung to lymph nodes in the center of the chest.
• Stage IIIB. The cancer has spread locally to areas such as the heart, blood vessels, trachea and esophagus — all within the chest — or to lymph nodes in the area of the collarbone or to the tissue that surrounds the lungs within the rib cage (pleura).
• Stage IV. The cancer has spread to other parts of the body, such as the liver, bones or brain.

Stages of small cell lung cancer

• Limited. Cancer is confined to one lung and to its neighboring lymph nodes.
• Extensive. Cancer has spread beyond one lung and nearby lymph nodes, and may have invaded both lungs, more remote lymph nodes or other organs.

Complications

Lung cancer can cause complications, such as:

• Fluid in the chest (pleural effusion). Lung cancer can cause fluid to accumulate in the space that surrounds the lungs in the chest cavity (pleural space). Pleural effusion can result from cancer spreading outside the lungs or in reaction to lung cancer inside the lungs. Fluid accumulating in the chest can cause shortness of breath. Treatments are available to drain the fluid from your chest and reduce the risk that pleural effusion will occur again. Cancer that spreads to the pleura is considered inoperable, so surgery isn’t an option for treatment.
• Cancer that spreads to other parts of the body (metastasis). Lung cancer often spreads (metastasizes) to other parts of the body — most commonly the opposite lung, brain, bones, liver and adrenal glands. Cancer that spreads can cause signs and symptoms, including pain, nausea, headaches or others based on what organ is affected. In some cases, treatments are available for isolated metastasis, but in most cases, the goal of treatment for metastasis is only to relieve signs and symptoms.
• Death. Unfortunately, survival rates haven’t improved for people diagnosed with lung cancer. In most cases, the disease is fatal. Almost 60 percent, or three out of every five people, diagnosed with lung cancer die within a year. Keep in mind, however, that this number includes people diagnosed with all types of lung cancer at all stages of the disease. People diagnosed at the earliest stages have the greatest chances for a cure. Your doctor can discuss more relevant statistics about your chances for survival with you.

Treatment

You and your doctor choose a cancer treatment regimen based on a number of factors, such as your overall health, the type and stage of your cancer, and your own preferences. Treatment options typically include one or more treatments, including surgery, chemotherapy, radiation therapy or targeted drug therapy.

In some cases you may choose not to undergo treatment. For instance, you may feel that the side effects of treatment will outweigh the potential benefits. When that’s the case, your doctor may suggest comfort (palliative) care to treat only the symptoms the cancer is causing, such as pain.

Surgery
During surgery your surgeon works to remove the lung cancer and a margin of healthy tissue. Procedures to remove lung cancer include:

• Wedge resection to remove a small section of lung that contains the tumor along with a margin of healthy tissue
• Lobectomy to remove the entire lobe of one lung
• Pneumonectomy to remove an entire lung

If you undergo surgery, your surgeon may also remove lymph nodes from your chest in order to check them for signs of cancer. If your lymph nodes contain cancer cells, this usually indicates that cancer has spread, even if cancer hasn’t been detected outside of your chest.

Lung cancer surgery carries risks, including bleeding and infection. Expect to feel short of breath after lung surgery. Your lung tissue will expand over time and make it easier to breathe. You may also feel pain in the muscles of your chest and in your arm on the side where you had the operation. Your doctor may recommend physical therapy or a rehabilitation program to help you restore your strength and range of motion.

Chemotherapy
Chemotherapy uses drugs to kill cancer cells. One or more chemotherapy drugs may be administered through a vein in your arm (intravenously) or taken orally. A combination of drugs usually is given in a series of treatments over a period of weeks or months, with breaks in between so that your body can recover.

Chemotherapy can be used as a first line treatment for lung cancer or as additional treatment after surgery. In some cases, chemotherapy can be used to lessen side effects of your cancer.

Radiation therapy
Radiation therapy uses high-powered energy beams, such as X-rays, to kill cancer cells. Radiation therapy can be directed at your lung cancer from outside your body (external beam radiation) or it can be put inside needles, seeds or catheters and placed inside your body near the cancer (brachytherapy).

Radiation therapy can be used alone or along with other lung cancer treatments. Sometimes it’s administered at the same time as chemotherapy. Radiation therapy can also be used to lessen side effects of lung cancer.

Targeted drug therapy
Targeted therapies are newer cancer treatments that work by targeting specific abnormalities in cancer cells. Targeted therapy options for treating lung cancer include:

• Bevacizumab (Avastin). Bevacizumab stops a tumor from creating a new blood supply. Blood vessels that connect to tumors can supply oxygen and nutrients to the tumor, allowing it to grow. Bevacizumab is usually used in combination with chemotherapy and is approved for advanced and recurrent non-small cell lung cancer. Bevacizumab carries a risk of severe bleeding.
• Erlotinib (Tarceva). Erlotinib blocks chemicals that signal the cancer cells to grow and divide. Erlotinib is approved for people with advanced and recurrent non-small cell lung cancer that haven’t been helped by chemotherapy. Erlotinib side effects include a skin rash and diarrhea.

Clinical trials
Clinical trials are studies of new lung cancer treatment methods. You may be interested in enrolling in a clinical trial if lung cancer treatments aren’t working or if your treatment options are limited. The treatments studied in a clinical trial may be the latest innovations, but they don’t guarantee a cure. Carefully weigh your treatment options with your doctor. Your participation in a clinical trial may help doctors better understand how to treat lung cancer in the future.

Supportive (palliative) care
When treatments offer little chance for a cure, your doctor may recommend you avoid harsh treatments and opt for supportive care instead. If you’re receiving supportive care, your doctor may treat any signs and symptoms you experience to make you feel more comfortable, but you won’t receive treatment aimed at stopping your cancer. Supportive care allows you to make the most of your final weeks or months without enduring treatment side effects that can negatively impact your quality of life.

Prevention

There’s no sure way to prevent lung cancer, but you can reduce your risk if you:

• Don’t smoke. If you’ve never smoked, don’t start. Talk to your children about not smoking, so they can understand how to avoid this major risk factor for lung cancer. Many current smokers began smoking in their teens. Begin conversations about the dangers of smoking with your children early, so they know how to react to peer pressure.
• Stop smoking. Stop smoking now. Quitting reduces your risk of lung cancer, even if you’ve smoked for years. Talk to your doctor about strategies and stop-smoking aids that can help you quit. Options include nicotine replacement products, medications and support groups.
• Avoid secondhand smoke. If you live or work with a smoker, urge him or her to quit. At the very least, ask him or her to smoke outside. Avoid areas where people smoke, such as bars and restaurants, and seek out smoke-free options.
• Test your home for radon. Have the radon levels in your home checked, especially if you live in an area where radon is known to be a problem. High radon levels can be remedied to make your home safer. For information on radon testing, contact your local department of public health or a local chapter of the American Lung Association.
• Avoid carcinogens at work. Take precautions to protect yourself from exposure to toxic chemicals at work. In the United States, your employer must tell you if you’re exposed to dangerous chemicals in your workplace. Follow your employer’s precautions. For instance, if you’re given a face mask for protection, always wear it. Ask your doctor what more you can do to protect yourself at work. Your risk of lung damage from these carcinogens increases if you smoke.
• Eat a diet full of fruits and vegetables. Choose a healthy diet with a variety of fruits and vegetables. Food sources of vitamins and nutrients are best. Avoid taking large doses of vitamins in pill form, as there may be unknown harms. For instance, researchers hoping to reduce the risk of lung cancer in heavy smokers gave them beta carotene supplements. Results showed the supplements actually increased the risk of cancer in smokers.
• Drink alcohol in moderation, if at all. Limit yourself to one drink a day if you’re a woman or two drinks a day if you’re a man. Anyone age 65 and older should drink no more than one drink a day.
• Exercise. Aim to achieve at least 30 minutes of exercise on most days of the week. Check with your doctor first if you aren’t already exercising regularly. Start out slowly and continue adding more activity. Biking, swimming and walking are good choices. Add exercise throughout your day — park farther away from work and walk the rest of the way or take the stairs rather than the elevator.

Coping skills

A diagnosis of lung cancer is devastating. It may take some time to come to terms with your feelings. When you’re ready, you can take steps to take control of your situation. Taking an active role in your health care may make you feel more empowered in coping with lung cancer. Try to:

• Learn all you can about lung cancer. Find out everything you can about your lung cancer — the type, the stage, your treatment options and their side effects. The more you know, the more active you can be in your own care. Write down your questions and ask them at your next appointment. Ask your health care team about additional sources of information. The National Cancer Institute will answer questions from the public. You can reach them at 800-4-CANCER, or 800-422-6237, or contact the American Cancer Society (ACS) at 800-227-2345.
• Take an active role in your treatment. Although you may feel tired and discouraged, don’t let others — including your family or your doctor — make important decisions for you. Take an active role in your treatment and work with your doctors to make your health care decisions.
• Build a strong support system. A strong support system helps you cope with everyday difficulties, such as fatigue and pain. Friends and family are worried about you and want to help, so learn to accept help when you need it. Staying connected with friends and family helps them cope with your illness and it gives you a chance to talk about your hopes and fears. Sometimes you’ll feel like your friends and family can’t understand your feelings if they’ve never had cancer. In these cases, support groups — both in your community and on the Internet — can be a good source for practical information and unique support. You may also find that you develop deep and lasting bonds with people who are going through the same things you are.
• Set reasonable goals. Having goals helps you feel in control and can give you a sense of purpose. But don’t choose goals you can’t possibly reach. You may not be able to work a 40-hour week, for example, but you may be able to work at least half the time. In fact, many people find that continuing to work can be helpful.
• Take time for yourself. Eating well, relaxing and getting enough rest can help combat the stress and fatigue of cancer. Also, plan ahead for the downtimes when you may need to rest more or limit what you do.
• Stay active. A diagnosis of cancer doesn’t mean you have to stop doing the things you enjoy. For the most part, if you feel well enough to do something, go ahead and do it. It’s important to stay involved with life.

Complementary and alternative medicine

If your doctor has told you that your lung cancer can’t be cured, you may be tempted to turn to complementary and alternative medicine for answers. Flashy Web sites claim cures are available at clinics in foreign countries, and secret herbal remedies seem to offer hope at a time when you feel you have few treatment options. But alternative lung cancer treatments aren’t backed by scientific research, so there’s no evidence that these therapies work. And, in many cases, the side effects of these treatments are unknown.

Alternative lung cancer treatments may be expensive and require travel to distant places. Consider how you want to spend the time you have remaining. You may prefer to spend your time at home with friends and family, rather than a faraway place where you have no support system.

Rather than forgoing mainstream cancer treatments, using complementary and alternative treatments along with care from your doctor may be a reasonable option. Work with your doctor to determine what complementary and alternative treatments may help relieve any signs and symptoms you experience. Your doctor can help you weigh the benefits and risks of complementary and alternative treatments.

The American College of Chest Physicians reviewed available complementary and alternative treatments and found some therapies may be helpful for people with lung cancer, including:

• Acupuncture. During an acupuncture session, a practitioner inserts small needles into precise points on your body. Acupuncture may relieve pain and ease cancer treatment side effects, such as nausea, vomiting and dry mouth, but there’s no evidence that acupuncture has any effect on your cancer. Acupuncture can be safe when done by a certified practitioner. Ask your doctor to recommend someone in your community. But acupuncture isn’t safe if you have low blood counts or take blood thinners.
• Hypnosis. Hypnosis is a type of therapy that puts you in a trance-like state that can be relaxing. Hypnosis is typically done by a therapist who leads you through relaxation exercises and asks you to think pleasing and positive thoughts. Hypnosis may reduce anxiety, nausea and pain in people with cancer, and it may improve appetite.
• Massage. During a massage, a massage therapist uses his or her hands to apply pressure to your skin and muscles. Massage can help relieve anxiety, distress, fatigue and pain in people with cancer. Some massage therapists are specially trained to work with people who have cancer. Ask your doctor for names of massage therapists in your community. Massage shouldn’t hurt. Your massage therapist shouldn’t put pressure anywhere near your tumor or any surgical wounds. Don’t have a massage if your blood counts are low or if you’re taking blood thinners.
• Meditation. Meditation is a time of quiet reflection in which you focus your mind on something, such as an idea, image or sound. Meditation may reduce stress and improve quality of life in people with cancer. Meditation can be done on your own, or there may be instructors in your community. Ask for recommendations from your health care team or friends and family.
• Yoga. Yoga combines gentle stretching movements with deep breathing and meditation. Yoga may help people with cancer sleep better. Yoga is generally safe when taught by a trained instructor, but don’t do any moves that hurt or don’t feel right. Many fitness centers offer yoga classes. Ask your friends and family for opinions on yoga classes they’ve taken.

Introduction

Colon cancer is cancer of the large intestine (colon), the lower part of your digestive system. Rectal cancer is cancer of the last 6 inches of the colon. Together, they’re often referred to as colorectal cancers. About 112,000 people are diagnosed with colon cancer annually, and about 41,000 new cases of rectal cancer are diagnosed each year, according to the American Cancer Society.

Most cases of colon cancer begin as small, noncancerous (benign) clumps of cells called adenomatous polyps. Over time some of these polyps become colon cancers.

Polyps may be small and produce few, if any, symptoms. Regular screening tests can help prevent colon cancer by identifying polyps before they become cancerous. If signs and symptoms of colon cancer do appear, they may include changes in bowel habits, blood in your stool, persistent cramping, gas or abdominal pain.

Signs and symptoms

Many people with colon cancer experience no symptoms in the early stages of the disease. When symptoms appear, they’ll likely vary, depending on the cancer’s size and location in your large intestine.

Signs and symptoms of colon cancer include:

• A change in your bowel habits, including diarrhea or constipation or a change in the consistency of your stool for more than a couple of weeks
• Rectal bleeding or blood in your stool
• Persistent abdominal discomfort, such as cramps, gas or pain
• Abdominal pain with a bowel movement
• A feeling that your bowel doesn’t empty completely
• Weakness or fatigue
• Unexplained weight loss

Blood in your stool may be a sign of cancer, but it can also indicate other conditions. Bright red blood you notice on bathroom tissue more commonly comes from hemorrhoids or minor tears (fissures) in your anus, for example. In addition, certain foods, such as beets or red licorice, can turn your stools red. Iron supplements and some anti-diarrheal medications may make stools black. Still, it’s best to have any sign of blood or change in your stools checked promptly by your doctor because it can be a sign of something more serious.

Causes

In general, cancer occurs when healthy cells become altered. Healthy cells grow and divide in an orderly way to keep your body functioning normally. But sometimes this growth gets out of control — cells continue dividing even when new cells aren’t needed. In the colon and rectum, this exaggerated growth may cause precancerous polyps (adenomas, or adenomatous polyps) to form in the lining of your intestine. Over a long period of time — spanning up to several years — some of these polyps may become cancerous. In later stages of the disease, colon cancer may penetrate the colon walls and spread (metastasize) to nearby lymph nodes or other organs. As with most cancers, the exact cause for colon cancer is unknown.

Polyps can occur anywhere in your large intestine, the muscular tube that forms the last part of your gastrointestinal tract. The colon comprises the upper 4 to 5 feet of your large intestine, and the rectum makes up the lower 6 inches. Your colon absorbs water, salt and other minerals from food and stores waste until it’s eliminated from your body.

Polyps are either mushroom-shaped or flat and may be large or small. There are also several different types of colon polyps. Among the most common are:

• Adenomas. These polyps have the potential to become cancerous and are usually removed during screening tests such as flexible sigmoidoscopy or colonoscopy.
• Hyperplastic polyps. These polyps are rarely, if ever, a risk factor for colorectal cancer.
• Inflammatory polyps. These polyps may follow a bout of ulcerative colitis. Some inflammatory polyps may become cancerous, so having ulcerative colitis increases your overall risk of colon cancer.

Risk factors

Factors that may increase your risk of colon cancer include:

• Age. About 90 percent of people diagnosed with colon cancer are older than 50. Colon cancer can occur in younger people, but it occurs much less frequently.
• A personal history of colorectal cancer or polyps. If you’ve already had colon cancer or adenomatous polyps, you have a greater risk of colon cancer in the future.
• Inflammatory intestinal conditions. Long-standing inflammatory diseases of the colon, such as ulcerative colitis and Crohn’s disease, can increase your risk of colon cancer.
• Inherited disorders that affect the colon. Genetic syndromes passed through generations of your family can increase your risk of colon cancer. These syndromes cause only about 5 percent of all colon cancers. One genetic syndrome called familial adenomatous polyposis (FAP) is a rare disorder that causes you to develop thousands of polyps in the lining of your colon and rectum. People with untreated FAP have a greater than 90 percent chance of developing colon cancer by age 45. Another genetic syndrome, hereditary nonpolyposis colorectal cancer (HNPCC), also called Lynch syndrome, is more common than FAP. People with HNPCC have an increased risk of colon cancer and tend to develop colon cancer at an early age. Both FAP and HNPCC can be detected through genetic testing. Talk to your doctor about whether your family history suggests you have a risk of these conditions.
• Family history of colon cancer and colon polyps. You’re more likely to develop colon cancer if you have a parent, sibling or child with the disease. If more than one family member has colon cancer or rectal cancer, your risk is even greater. In some cases, this connection may not be hereditary or genetic. Instead, cancers within the same family may result from shared exposure to an environmental carcinogen or from diet or lifestyle factors.
• Diet. Colon cancer and rectal cancer may be associated with a diet low in fiber and high in fat and calories. Research in this area has had mixed results. Some studies have found an increased risk of colon cancer in people who eat diets high in red meat and processed meats.People who eat a diet similar to that of Western countries, such as the United States and Europe, have a higher risk of developing colon cancer than do people who eat diets typically seen in developing countries. When people move from a developing country to a Western country and adapt to the Western diet, their risk of colon cancer increases. Although many studies have tried to identify what part of the Western diet may cause colon cancer, the answer remains unknown.
• A sedentary lifestyle. If you’re inactive, you’re more likely to develop colon cancer. This may be because when you’re inactive, waste stays in your colon longer. Getting regular physical activity may reduce your risk.
• Diabetes. People with diabetes and insulin resistance may have an increased risk of colon cancer.
• Obesity. People who are obese have an increased risk of colon cancer and an increased risk of dying of colon cancer when compared with people considered normal weight.
• Smoking. People who smoke cigarettes may have an increased risk of colon cancer. They may also have an increased chance of dying of colon cancer.
• Alcohol. Heavy use of alcohol may increase your odds of colon cancer.
• Growth hormone disorder. Acromegaly, an uncommon disorder that causes an excess of growth hormone in your body, may increase your risk of colon polyps and colon cancer.
• Radiation therapy for cancer. Radiation therapy directed at the abdomen to treat previous cancers may increase the risk of colon cancer.

When to seek medical advice

If you notice any symptoms of colon cancer, such as blood in your stool or a persistent change in bowel habits, see your doctor as soon as possible. Keep in mind that colorectal cancer can occur in younger as well as older people. If you’re at high risk, don’t wait until symptoms appear. See your doctor for regular screenings.

Talk to your doctor about when you should begin screening for colon cancer. Guidelines generally recommend colon cancer screenings begin at age 50. Your doctor may recommend more frequent or earlier screening if you have other risk factors, such as a family history of the disease.

Screening and diagnosis

Screening
Most colon cancers develop from adenomatous polyps. Screening can detect polyps before they become cancerous. Screening may also detect colon cancer in its early stages when there is a good chance for cure.

You may be embarrassed by the screening procedures, worried about discomfort or afraid of the results. Discuss your screening options and your concerns with your doctor. Most procedures are only moderately uncomfortable, and working with a doctor you like and trust can help ease your embarrassment.

Common screening and diagnostic procedures include the following:

• Stool blood test. The fecal occult (hidden) blood test checks a sample of your stool for blood. It can be performed in your doctor’s office, but you’re usually given a kit that explains how to take the sample at home. You return the sample to a lab or your doctor’s office to be checked. Not all cancers can be detected with a stool blood test, since not all cancers bleed and those that do often bleed intermittently. This can result in a negative test result, even though you may have cancer. If blood is detected in your stool, your doctor may order other screening tests to determine the source of the blood. Blood in your stool can be the result of hemorrhoids or an intestinal condition other than cancer. Certain foods also may alter test results, suggesting blood in the colon when there is none.
• Flexible sigmoidoscopy. In this test, your doctor uses a flexible, slender and lighted tube to examine your rectum and sigmoid — approximately the last 2 feet of your colon. The test usually takes just a few minutes. It can sometimes be uncomfortable, and there’s a slight risk of perforating the colon wall. If a polyp or colon cancer is found during this exam, your doctor will recommend colonoscopy to look at the entire colon and remove any polyps that are present for examination under a microscope. Because sigmoidoscopy only examines the lower third of the colon, polyps or cancer in the rest of the colon won’t be detected.
• Barium enema. This diagnostic test allows your doctor to evaluate your entire large intestine with an X-ray. Barium, a contrast dye, is placed into your bowel in an enema form. During a double contrast barium enema, air is also added. The barium fills and coats the lining of the bowel, creating a clear silhouette of your rectum, colon and sometimes a small portion of your small intestine. There’s also a slight risk of perforating the colon wall, and the test has a significantly high rate of missing important lesions. A flexible sigmoidoscopy is often done in addition to the barium enema to aid in detecting small polyps that a barium enema X-ray may miss, especially in the lower bowel and rectum. Barium enema may miss small polyps or small cancers. If an abnormality is detected using barium enema, your doctor may recommend a colonoscopy.
• Colonoscopy. Colonoscopy is similar to flexible sigmoidoscopy, but the instrument used — a colonoscope, which is a long, flexible and slender tube attached to a video camera and monitor — allows your doctor to view your entire colon and rectum. If any polyps are found during the exam, your doctor may remove them immediately or take tissue samples (biopsies) for analysis. This is done through the colonoscope and is painless.You may receive a mild sedative to make you more comfortable. Preparation for the procedure involves drinking a large amount of fluid containing a laxative to clean out your colon — enemas are no longer necessary. Major risks of diagnostic colonoscopy include bleeding and perforation of the colon wall, but these are rare.
• Virtual colonoscopy (CT colonography). Though not available at all medical centers, virtual colonoscopy may be one screening option. Virtual colonoscopy uses a computerized tomography (CT) machine to take images of your colon, rather than using a scope inserted in your rectum to see inside your colon. Virtual colonoscopy still requires that you undergo a bowel preparation to remove any stool. If any abnormalities are detected on virtual colonoscopy, you’ll still need to undergo conventional colonoscopy. Research into virtual colonoscopy is still ongoing.

Diagnosis
If your doctor suspects you may have colon cancer based on your signs and symptoms, he or she may recommend colonoscopy to look for colon cancer. Colonoscopy allows your doctor to look for polyps or unusual areas in your colon. Your doctor can also remove a sample of tissue from your colon to look for cancer cells. In some cases, barium enema or flexible sigmoidoscopy may be used to diagnose colon cancer.

Staging your cancer
Once you’ve been diagnosed with colon cancer, your doctor will then order tests to determine the extent, or stage, of your cancer. Staging helps determine what treatments are most appropriate for you. Staging tests may include imaging procedures such as abdominal CT scan or chest X-ray. The stages of colon cancer are:

• Stage 0. Your cancer is in the earliest stage. It hasn’t grown beyond the inner layer (mucosa) of your colon or rectum. This stage of cancer may also be called carcinoma in situ.
• Stage I. Your cancer has grown through the mucosa but hasn’t spread beyond the colon wall or rectum.
• Stage II. Your cancer has grown into or through the wall of the colon or rectum but hasn’t spread to nearby lymph nodes.
• Stage III. Your cancer has invaded nearby lymph nodes but isn’t affecting other parts of your body yet.
• Stage IV. Your cancer has spread to distant sites, such as other organs — for instance to your liver or lung, to the membrane lining the abdominal cavity, or to an ovary.
• Recurrent. This means your cancer has come back after treatment. It may recur in your colon, rectum or other part of your body.

Treatment

The type of treatment your doctor recommends will depend largely on the stage of your cancer. The three primary treatment options are: surgery, chemotherapy and radiation.

Surgery (colectomy) is the main treatment for colorectal cancer. How much of your colon is removed and whether other therapies, such as radiation or chemotherapy, are an option for you depend on the location of your cancer, how far cancer has penetrated into the wall of your bowel, and whether it has spread to your lymph nodes or other parts of your body.

Surgical procedures

Your surgeon removes the part of your colon that contains the cancer, along with a margin of normal tissue on either side of the cancer to help ensure that no cancer is left behind. Nearby lymph nodes are usually also removed and tested for cancer. Your surgeon is often able to reconnect the healthy portions of your colon or rectum. But when that’s not possible, for instance if the cancer is at the outlet of your rectum, you may need to have a permanent or temporary colostomy. This involves creating an opening in the wall of your abdomen from a portion of the remaining bowel for the elimination of body waste into a special bag. Sometimes the colostomy is only temporary, allowing your colon or rectum time to heal after surgery. In some cases, however, the colostomy may be permanent.Side effects of colon cancer surgery may include short-term pain and tenderness, and temporary constipation or diarrhea. If you have a colostomy, you may develop an irritation on the skin around the opening (stoma).

Surgery to prevent cancer
In cases of rare, inherited syndromes such as familial adenomatous polyposis, or inflammatory bowel disease such as ulcerative colitis, your doctor may recommend removal your entire colon and rectum in order to prevent cancer from occurring in the future. Then, in a procedure known as ileal pouch-anal anastomosis, your surgeon will likely construct a pouch from the end of your small intestine that attaches directly to your anus. This allows you to expel waste normally, although you may have several watery bowel movements a day.

Surgery for early-stage cancer
If your cancer is small, localized in a polyp and in a very early stage, your surgeon may be able to remove it completely during a colonoscopy. If the pathologist determines that the cancer in the polyp doesn’t involve the base — where the polyp is attached to the bowel wall — then there’s a good chance that the cancer has been completely eliminated.

Some larger polyps may be removed using laparoscopic surgery. In this procedure, your surgeon performs the operation through several small incisions in your abdominal wall, inserting instruments with attached cameras that display your colon on a video monitor. He or she may also take samples from the lymph nodes that drain the area where the cancer is located. Studies have found that people undergoing this procedure need less pain medication and leave the hospital a day earlier on average. Also, people who have this procedure don’t have higher rates of recurrence than those who choose the open surgery.

Surgery for advanced cancer
If your cancer is very advanced or your overall health very poor, an operation to relieve a blockage of your colon or other conditions in order to improve your symptoms may be the best option. This type of surgery is referred to as palliative surgery. The goal of palliative surgery isn’t to cure your cancer, but to relieve signs and symptoms, such as bleeding and pain.

In specific cases where the cancer has spread only to the liver and if your overall health is otherwise good, your doctor may recommend surgery to remove the cancerous lesion from your liver. Chemotherapy may be used before or after this type of surgery. This treatment may improve your prognosis.

Chemotherapy

Chemotherapy uses drugs to destroy cancer cells. Chemotherapy can be used to destroy cancer cells after surgery, to control tumor growth or to relieve symptoms of colon cancer. Your doctor may recommend chemotherapy if your cancer has spread beyond the wall of the colon. In some cases, chemotherapy is used along with radiation therapy.

Possible side effects of chemotherapy include nausea and vomiting, mouth sores, fatigue, hair loss and diarrhea. Discuss the side effects and risks as well as the potential benefits with your doctor.

Radiation therapy

Radiation therapy uses powerful energy sources, such as X-rays, to kill any cancer cells that might remain after surgery, to shrink large tumors before an operation so that they can be removed more easily, or to relieve symptoms of colon cancer and rectal cancer.

Radiation therapy is rarely used in early stage colon cancer, but is a routine part of treating early stage rectal cancer, especially if the cancer has penetrated through the wall of the rectum or traveled to nearby lymph nodes. Radiation therapy, usually combined with chemotherapy, may be used after surgery to reduce the risk that the cancer may recur in the area of the rectum where it began.

Side effects of radiation therapy may include diarrhea, rectal bleeding, fatigue, loss of appetite and nausea.

Targeted drug therapy

Three drugs that target specific defects that allow cancer cells to proliferate are available to people with advanced colon cancer. The drugs bevacizumab (Avastin), cetuximab (Erbitux) and panitumumab (Vectibix) can be given along with chemotherapy drugs or alone. Bevacizumab works to prevent tumors from developing new blood vessels, which can deliver the oxygen and nutrients cancers need to survive. Cetuximab and panitumumab target a chemical signal that tells cells to divide and reproduce. Cetuximab was approved by the Food and Drug Administration in 2007 as a single-agent treatment for advanced colon cancer for which other treatments have failed. Panitumumab remains experimental.

Prevention

Get screened for colon cancer
Regular colon cancer screening should begin at age 50 for people thought to have an average risk of colon cancer. The U.S. Multisociety Task Force on Colorectal Cancer recommends the following screening options:

• Annual fecal occult blood testing
• Flexible sigmoidoscopy every five years
• Annual fecal occult blood testing plus flexible sigmoidoscopy every five years
• Double contrast barium enema every five years
• Colonoscopy every 10 years

More frequent or earlier screening may be recommended if you’re thought to have a high risk of colon cancer. Explore the benefits and risks of each screening option with your doctor. Together you can determine which colon cancer screening option is best for you. One factor to consider is whether your health insurance provider covers colon cancer screening.

Medicare covers colon cancer screening procedures. If you’re older than 50 and have Medicare benefits, Medicare will cover annual fecal occult blood tests and sigmoidoscopy every four years. If you’re at high risk of colorectal cancer, you’ll be covered for colonoscopy every two years, or every 10 years if you’re of average risk. Double contrast barium enema — which is sometimes supplemented with flexible sigmoidoscopy — can be used as an alternative, if your doctor thinks it’s a better choice for you.

Make lifestyle changes to reduce your risk
You can take steps to reduce your risk of colon cancer by making changes in your everyday life. Take steps to:

• Eat plenty of fruits, vegetables and whole grains. Fruits, vegetables and whole grains contain vitamins, minerals, fiber and antioxidants, which may play a role in cancer prevention. Try to eat five or more servings of fruits and vegetables every day, and to include a variety of produce in your diet.
• Limit fat, especially saturated fat. Eat a low-fat diet. Avoid saturated fats from animal sources such as red meat. Other foods that contain saturated fat include milk, cheese, ice cream, and coconut and palm oils.
• Eat a varied diet to increase the vitamins and minerals you consume. A number of vitamins and minerals have been linked to a lower risk of colon cancer, though results have been mixed. Studies haven’t proved certain vitamins and minerals will stop you from getting colon cancer, but it can’t hurt to vary the fruits and vegetables in your diet to ensure you get a wide selection of nutrients. Vitamins and minerals linked to a lower incidence of colon cancer include vitamin B-6 (pyridoxine), calcium, folic acid and magnesium.Food sources of calcium include skim or low-fat milk and other dairy products, shrimp, tofu and sardines with the bones. Magnesium is found in leafy greens, nuts, peas and beans. Food sources of vitamin B-6 include grains, legumes, peas, spinach, carrots, potatoes, dairy foods and meat. Folic acid is the synthetic form vitamin B-9, and it’s used in fortified breads, cereals and supplements. Vitamin B-9 occurs naturally in dark leafy greens such as spinach and lettuce, and in legumes, melons, bananas, broccoli and orange juice.
• Limit alcohol consumption. Limit the amount of alcohol you drink to no more than one drink a day for women and two for men. A drink is a 4- to 5-ounce glass of wine, a 12-ounce can of beer, or a 1.5-ounce shot of 80-proof liquor.
• Stop smoking. Talk to your doctor about ways to quit that may work for you.
• Stay physically active and maintain a healthy body weight. Try to get at least 30 minutes of exercise on most days. If you’ve been inactive, start slowly and build up gradually to 30 minutes. Also, talk to your doctor before starting any exercise program.

Talk to your doctor about drugs that may reduce your risk
Some medications have been found to reduce the risk of precancerous polyps or colon cancer. However, not enough evidence exists to recommend these medications to people who have an average risk of colon cancer. If you have an increased risk of colon cancer, you might discuss the benefits and risks of these medications with your doctor:

• Aspirin. Some evidence links a reduced risk of polyps and colon cancer to regular aspirin use. However, studies of low-dose aspirin or short-term use of aspirin haven’t found this to be true. It’s likely that you may be able to reduce your risk of colon cancer by taking large doses of aspirin over a long period of time. But using aspirin in this way is likely to cause side effects, such as gastrointestinal bleeding and ulcers.
• Nonsteroidal anti-inflammatory drugs (NSAIDs) other than aspirin. This class of pain-relief medications includes drugs such as ibuprofen (Advil, Motrin, others) and naproxen (Aleve, others). Some studies have found NSAIDs may reduce the risk of precancerous polyps and colon cancer. But side effects include ulcers and gastrointestinal bleeding. Some NSAIDs have been linked to an increased risk of heart problems.
• Celecoxib (Celebrex). Celecoxib and other drugs known as COX-2 inhibitors work similarly to NSAIDs to provide pain relief. Some evidence suggests COX-2 drugs can reduce the risk of precancerous polyps in people who’ve been diagnosed with these polyps in the past. But COX-2 drugs carry a risk of heart problems, including heart attack. Two COX-2 inhibitor drugs were removed from the market because of these risks.

Coping skills

A diagnosis of cancer can be extremely challenging. Even when a full recovery is likely, you may worry about a recurrence of the disease. But no matter what your concerns or prognosis, you’re not alone. Here are some strategies and resources that may make dealing with cancer easier:

• Know what to expect. Find out everything you can about your cancer — the type, stage, your treatment options and their side effects. The more you know, the more active you can be in your own care. In addition to talking with your doctor, look for information in your local library and on reliable Web sites on the Internet. The National Cancer Institute (NCI) answers questions from the public. You can reach the NCI at 800-4-CANCER, or 800-422-6237. Or contact the American Cancer Society (ACS) at 800-227-2345.
• Be proactive. Although you may feel tired and discouraged, don’t let others — including your family or your doctor — make important decisions for you. It’s vital that you take an active role in your treatment.
• Maintain a strong support system. Friends and family can be your best allies as you face your cancer diagnosis. They want to help you, so take them up on offers to make meals or provide transportation to and from appointments. Sometimes the concern and understanding of a formal support group or other cancer survivors can offer unique emotional support. Although support groups aren’t for everyone, they can be a good source for practical information. You may also find you develop deep and lasting bonds with people who are going through the same things you are. There are also support groups for the families of cancer survivors.
• Set reasonable goals. Having goals helps you feel in control and can give you a sense of purpose. But don’t choose goals you can’t possibly reach. You may not be able to work a 40-hour week, for example, but you may be able to work at least half time. In fact, many people find that continuing to work can be helpful.
• Take time for yourself. Eating well, relaxing and getting enough rest can help combat the stress and fatigue of cancer. Also, plan ahead for the downtimes when you may need to rest more or limit what you do.

Introduction

Inflammatory breast cancer is a rare but aggressive type of breast cancer that develops rapidly, making the affected breast red, swollen and tender. It’s a locally advanced cancer, meaning it has spread from its point of origin to nearby tissue and possibly to nearby lymph nodes.

The early signs of more-common forms of breast cancer — a breast lump or suspicious area on a routine, screening mammogram — are often absent in inflammatory breast cancer. Instead, the breast may appear normal until tumor cells invade and block lymphatic vessels in the overlying skin. Fluid backs up, and the breast swells and becomes discolored.

Inflammatory breast cancer can easily be confused with a breast infection, but if it’s cancer, symptoms won’t go away with antibiotics. Seek medical attention promptly if you notice skin changes on your breast, to help distinguish a breast infection from other breast disorders.

Inflammatory breast cancer accounts for between 1 percent and 6 percent of all breast cancer cases in the United States. Survival rates are lower than those observed in other locally advanced breast cancers. But new treatment approaches offer greater odds for survival than ever before.

Signs and symptoms

Despite its name, inflammatory breast cancer does not cause inflammation the way an infection does. Signs and symptoms include:

• Rapid change in the appearance of one breast, over the course of days or weeks
• Thickness, heaviness or visible enlargement of one breast
• Discoloration, giving the breast a red, purple, pink or bruised appearance
• Unusual warmth of the affected breast
• Dimpling or ridges on the skin of the affected breast, similar to an orange peel
• Itching
• Tenderness, pain or aching
• Enlarged lymph nodes under the arm, above the collarbone or below the collarbone
• Flattening or turning inward of the nipple
• Swollen or crusted skin on the nipple
• Change in color of the skin around the nipple (areola)

Other conditions have symptoms resembling those of inflammatory breast cancer. A breast infection (mastitis) also causes redness, swelling and pain, but breast infections usually develop during breast-feeding. With an infection, you’re likely to have a fever, which is unusual (but not unheard of) in inflammatory breast cancer.

Breast surgery or radiation therapy may block the lymphatic vessels in breast skin, temporarily making the breast swell and become discolored. When caused by surgery or radiation treatments, however, these changes gradually subside.

Causes

As with other types of breast cancer, the exact cause of inflammatory breast cancer is unknown. All cancers are characterized by unregulated cell division, starting with one abnormal cell, in this case usually in one of the breast’s ducts. In inflammatory breast cancer, the abnormal cells rapidly infiltrate and clog the lymphatic vessels in the skin over your breast. The blockage in the lymphatic vessels causes red, swollen and dimpled skin — a classic sign of inflammatory breast cancer.

Risk factors

Inflammatory breast cancer tends to affect women at an average age of 59 — about three to seven years younger than the average age at which other types of breast cancer are diagnosed. Men can develop the disease, but at an older age. Black women are slightly more likely than are white women to have inflammatory breast cancer.

When to seek medical advice

If you have an apparent breast infection that fails to improve despite a week of treatment with antibiotics, ask your doctor to do a further breast evaluation. This may include imaging studies such as ultrasound, mammogram or MRI scan and a biopsy. If test results show no signs of cancer, but your signs and symptoms appear to be getting worse, talk with your doctor about performing another biopsy — especially one that includes a skin sample — or ask for a referral to a breast specialist.

Screening and diagnosis

A diagnosis of inflammatory breast cancer is based on your medical history, your physical examination and an excisional biopsy — taking a small sample of skin and some of the underlying tissue to examine under a microscope. An excisional biopsy that includes the skin is helpful because a hallmark of inflammatory breast cancer is finding tightly packed clumps of cancer cells (tumor emboli) in the lymphatic vessels of the skin. Imaging tests — mammogram and breast ultrasound — also may be used to confirm the diagnosis by showing areas of skin thickening.

If the biopsy results confirm that you have inflammatory breast cancer, the next step is to determine how advanced your cancer is — its stage, or extent and severity. Your doctor may perform additional tests, such as a CT scan of your chest and abdomen, chest X-ray, and bone scan, to check for the presence of cancer cells in other parts of your body (metastases).

Your cancer will also be tested for the presence or absence of receptors for the hormones estrogen and progesterone, and to see if the cancer produces too much of a protein called HER2. Inflammatory breast cancers are often hormone receptor negative and HER2-positive.

Inflammatory breast cancer is classified as stage IIIB or stage IV breast cancer. Stage IIIB is locally advanced cancer — meaning it has spread to nearby lymph nodes and to the fibrous connective tissue inside the breast. Stage IV cancer has spread to other parts of your body, such as bones or liver. About one-third of newly diagnosed inflammatory breast cancers are stage IV.

Treatment

Treatment for inflammatory breast cancer starts with chemotherapy, followed by surgery and radiation therapy. This combined-treatment approach has improved the outlook for women with inflammatory breast cancer. About half the women diagnosed with the condition survive five or more years, and nearly one-third are alive 20 years after diagnosis.

Chemotherapy (anti-cancer drugs)
Treatment of inflammatory breast cancer usually begins with several rounds of chemotherapy to kill or control cancer cells. This pre-surgical treatment, referred to as neoadjuvant therapy, is needed to shrink the cancer and resolve skin problems before the operation, since swelling can prevent the surgical incision from healing properly. The exact number of chemotherapy treatments will depend on how well the cancer responds to the treatments.

Surgery
After chemotherapy, women with inflammatory breast cancer usually have an operation to remove the affected breast (mastectomy). Surgery alone — without chemotherapy — offers a much smaller chance of a cure. Breast-conserving surgery (lumpectomy) isn’t recommended for women with inflammatory breast cancer. Most women receive additional doses of chemotherapy after healing from the operation.

Radiation therapy
After surgery and any further chemotherapy, a course of radiation therapy is given to kill any remaining cancer cells in the breast and under your arm. This can help decrease the chance of cancer coming back in the area. Radiation typically involves about 30 treatments over six weeks.

Further treatments
Even after treatment with chemotherapy, surgery and radiation, recurrence rates remain high for inflammatory breast cancer. Because of this, your doctor may recommend further treatment (adjuvant therapy) to prevent the cancer from returning. This might include more chemotherapy or hormone therapy if your cancer tests positive for estrogen receptors. Hormone therapy, such as tamoxifen or anastrozole (Arimidex), interferes with the effects of the female hormone estrogen, which can promote cancer cell growth.

If your cancer is HER2-positive, your doctor will recommend a course of trastuzumab (Herceptin). You may also be offered the opportunity to participate in a clinical trial to test new treatments for inflammatory breast cancer.

Coping skills

Inflammatory breast cancer is aggressive and difficult to treat. Until recently, the prospects for long-term survival were poor. A further challenge is the relatively high risk that inflammatory breast cancer will return. Fortunately, the outlook has improved — but that doesn’t make coping any easier.

Because inflammatory breast cancer progresses rapidly, you may need to start treatment before you’ve had time to process everything. If you feel rushed, try thinking about your treatment as a series of short-term challenges rather than as one long process. At every stage of treatment, someone on your medical team should be available to explain what’s happening and answer your questions. Don’t be afraid to speak up about any issue that arises — anxiety, pain or ongoing trouble with treatment side effects.

Don’t try to go it alone. Seek support from your family and friends, and ask your doctor what community and hospital resources may be available.

There’s no “right” way to feel and act when you’re dealing with cancer. Despite often-repeated anecdotes about the power of a positive attitude, research shows that personality and coping style have no consistent effect on cancer outcomes. If you’re feeling sad, anxious or just plain mad, know that these feelings are normal, and try to let go of the added burden of blaming yourself.

Introduction

Breast cancer isn’t just a woman’s disease. Men also have breast tissue that can undergo cancerous changes. While women are about 100 times more likely to get breast cancer, any man can develop breast cancer. Male breast cancer is most common between the ages of 60 and 70.

The prognosis for male breast cancer is the same as for breast cancer in women. In the past, male breast cancer was often diagnosed at a more advanced stage, which may have led people to believe it had a worse prognosis. Although male breast cancer and breast cancer in women are similar, important distinctions such as breast size and awareness affect early diagnosis and survival in cases of male breast cancer.

Signs and symptoms

Knowing the signs and symptoms of breast cancer may help save your life. The earlier the disease is discovered, the more treatment options and the better chance of recovery you have.

The most common sign of breast cancer for both men and women is a lump or thickening in the breast. Often the lump is painless. Other male breast cancer symptoms include:

• Skin dimpling or puckering
• Development of a new retraction or indentation of the nipple
• Changes in the nipple or breast skin, such as scaling or redness
• Nipple discharge

Causes

Cancer is a group of abnormal cells that grow more rapidly than do normal cells. Cancer cells also have the ability to invade and destroy normal tissues, either by growing directly into surrounding structures or after traveling to another part of your body through your bloodstream or lymphatic system. Microscopic cancer cells form small clusters that continue to grow, becoming more densely packed and hard.

In most cases it isn’t clear what triggers abnormal cell growth in breast tissue in men. But doctors do know that about one in six cases of breast cancers in men are inherited, compared with about 5 percent to 10 percent of breast cancers in women. Defects in breast cancer gene 1 or 2 (BRCA 1 or BCRA 2) put you at greater risk of developing breast cancer. Other inherited genes also may increase your risk of developing breast cancer. Knowing your family history is important to determine your chance of inheriting an abnormal gene.

Most genetic mutations related to breast cancer aren’t inherited, but instead develop during your lifetime. These acquired mutations may result from radiation exposure, such as receiving chest radiation therapy in childhood, or from other, as yet unknown, factors.

Risk factors

A risk factor is anything that makes it more likely you’ll get a particular disease. But not all risk factors are created equal. Some, such as your age, sex and family history, can’t be changed. Others, including smoking and a poor diet, are personal choices over which you have some control.

Having one or even several risk factors doesn’t necessarily mean you’ll become sick — some men with more than one risk factor never get breast cancer, whereas others with no identifiable risk factors do.

Factors that may make you more susceptible to breast cancer include:

• Age. Breast cancer is most commonly diagnosed in men between the ages of 60 and 70, with an average age range of 65 to 67.
• Family history. If you have a close relative, such as a mother or sister, with breast cancer, you have a greater chance of also developing the disease. About one in five men with breast cancer have a relative who’s had it, too. Just because you have a family history of breast cancer doesn’t mean it’s hereditary, though.
• Genetic predisposition. In men, nearly 20 percent of breast cancers are inherited. Defects in one of several genes, especially BRCA1 or BRCA2 put you at greater risk of developing breast and prostate cancers. Usually these genes help prevent cancer by making proteins that keep cells from growing abnormally. But if they have a mutation, the genes aren’t as effective at protecting you from cancer.Men with a BRCA2 mutation have a 6 percent lifetime risk of breast cancer — about 100 times more than other men’s risk. Inherited mutations in the cell-cycle checkpoint kinase 2 (CHEK-2) gene and the p53 tumor suppressor gene also make it more likely that you’ll develop breast cancer.
• Radiation exposure. If you received radiation treatments to your chest as a child or young adult, you’re more likely to develop breast cancer later in life.
• Klinefelter’s syndrome. This condition results from an abnormality of the sex chromosomes, X and Y, present at birth (congenital). A male normally has only one X and one Y chromosome. In Klinefelter’s syndrome, two or more X chromosomes are present in addition to one Y chromosome. The Y chromosome contains the genetic material that determines the sex of a child and related development.The extra X chromosome that occurs in Klinefelter’s syndrome causes abnormal development of the testicles. As a result, men with this syndrome produce lower levels of certain male hormones — androgens — and more female hormones — estrogens, which can cause noncancerous breast growth (gynecomastia). Men with this condition may be at greater risk of breast cancer, though this connection is still unclear.
• Exposure to estrogen. If you take estrogen-related drugs, such as those used as part of a sex change procedure, you have a much higher risk of breast cancer. Estrogen drugs may also be used in hormone therapy for prostate cancer. Such drugs may slightly increase your risk of breast cancer, though not enough to outweigh the benefit of treating prostate cancer.
• Liver disease. If you have liver disease, such as cirrhosis of the liver, your body’s androgen activity may be reduced and its estrogen activity greater. This can increase your risk of gynecomastia and breast cancer.
• Excess weight. Obesity may be a risk factor for breast cancer in men, because it increases the number of fat cells in the body. Fat cells convert androgens into estrogen, increasing the amount of estrogen in your body and, therefore, your risk of breast cancer.
• Excessive use of alcohol. If you drink heavy amounts of alcohol, you have a greater risk of breast cancer.

When to seek medical advice

Most breast lumps in men are a result of enlarged breasts (gynecomastia), not breast cancer. However, it’s important to have lumps evaluated promptly. If a problem exists, you can have it identified and treated as soon as possible. See your doctor if you discover a lump or any of the other warning signs of breast cancer.

Screening and diagnosis

Because male breast cancer is rare, routine screening mammograms (mammography) generally aren’t recommended for men. If, however, you have a strong family history of breast cancer, consider talking to your doctor about developing a breast-screening program.

If your doctor suspects breast cancer, to diagnose your condition he or she may conduct a number of tests including breast examination (clinical breast exam), mammograms (mammography) or other tests:

• Clinical breast exam. During this exam, your doctor examines your breasts for lumps or other changes. He or she may be able to feel lumps you missed and will assess how large the lumps are, how they feel, and how close they are to your skin and muscles. Your doctor will also examine the rest of your body for signs that the cancer has spread, such as an enlarged liver or lymph nodes.
• Mammogram. A mammogram uses a series of X-rays to show images of your breast tissue. This test may be even more accurate in men than in women, because men don’t have dense breast tissue that can make it difficult to distinguish abnormal from normal tissue or breast cysts. During a mammogram, your breasts are compressed between plastic plates while a radiology technician takes the X-rays. If you find the compression too uncomfortable, tell the technician.
• Breast ultrasound (ultrasonography). Your doctor may use this technique to evaluate an abnormality seen on a mammogram or found during a clinical exam. Ultrasound uses sound waves to form images of structures within the body.
• Nipple discharge examination. Your doctor may collect nipple discharge if you’re experiencing it. The discharge is then examined for cancerous cells.
• Biopsy. A biopsy is the only way for your doctor to know whether a lump or abnormality is cancer. Biopsies can provide important information about an unusual breast change and help determine whether treatment is needed and, if so, the type of treatment required. To obtain a tissue sample, your doctor may use one of several procedures.Fine-needle aspiration biopsy is used for lumps you or your doctor can feel. During the procedure your doctor uses a thin, hollow needle to withdraw cells from the lump. He or she then sends the cells to a lab for analysis.

  In core needle biopsy, a radiologist or surgeon uses a hollow needle to remove tissue samples from a breast lump. A number of samples, each about the size of a grain of rice, may be taken, and a pathologist then analyzes them for malignant cells. The advantage of a core needle biopsy is that it removes tissue, rather than just cells, for analysis.

  In surgical biopsy, your surgeon removes all or part of a breast lump. In general, a small lump will be completely removed (excisional biopsy). If the lump is larger, only a sample will be taken (incisional biopsy). The biopsy is generally performed on an outpatient basis in a clinic or hospital.

• Estrogen and progesterone receptor tests. If a biopsy reveals malignant cells, your doctor will recommend additional tests — such as estrogen and progesterone receptor tests — on the malignant cells. These tests help determine whether female hormones affect the way the cancer grows. About 90 percent of male breast cancers have estrogen receptors, and more than 80 percent have progesterone receptors. If the cancer cells have receptors for estrogen or progesterone or both, your doctor may recommend treatment with a drug such as tamoxifen, which prevents estrogen from binding to these cells and stimulating growth.
• HER2 testing. If the biopsy shows malignant cells, your doctor may also test the sample for the presence of a protein called human epidermal growth factor receptor-2 (HER2), which promotes the growth of cancer cells. About 30 percent of male breast cancers have too much of this protein. Such cancers are usually more aggressive, growing and spreading more quickly than do other breast cancers. Once identified, this type of cancer is treated with a drug called trastuzumab (Herceptin). This medication keeps the protein from stimulating the growth of breast cancer cells.

Staging tests
If your doctor finds cancer, he or she will examine you further to determine if and how far the cancer has spread. Staging tests help determine the size and location of your cancer and whether it has spread. They also help your doctor determine the best treatment for you. Cancer is staged using the numbers 0 through IV:

• Stage 0 cancers are also called noninvasive or in situ (in one place) cancers. Although they haven’t spread to other parts of your body or invaded normal breast tissue, it’s important to have them removed, because they eventually can become invasive cancers. Finding and treating a cancerous lump at this stage offers the best chance for a full recovery.
• Stage I to IV cancers are invasive tumors that have the ability to spread to other areas. A stage I cancer is small and well localized and has a very successful treatment rate. But the higher the stage number, the lower the chances of cure. By stage IV, the cancer has spread beyond your breast to other organs, such as your bones, lungs or liver. Although it may not be possible to eliminate the cancer at this stage, its spread and some symptoms may be controlled with radiation, hormonal therapy, chemotherapy or all three.

Tests to determine a cancer’s spread may include:

• Chest X-ray. Your doctor may take a chest X-ray to see whether the cancer has spread to your lungs.
• Computerized tomography. A CT scan is an X-ray technique that produces more-detailed images of your internal organs than do conventional X-ray exams. Conventional X-ray exams produce 2-D images. But CT uses an X-ray-sensing unit that rotates around your body and a large computer to create cross-sectional images (like slices) of the inside of your body. A CT scan can help your doctor see if cancer has spread to your liver or other organs. Some CT scans require you to ingest a contrast medium before the scan. A contrast medium blocks X-rays and appears white on images, which can help emphasize some structures in your body.
• Magnetic resonance imaging (MRI). This test uses a magnetic field and radio waves to create cross-sectional images. Most MRI machines are large, cylindrical-shaped magnets. The strong magnetic field is produced by passing an electric current through wire loops or coils, which are located inside a protective housing. Other coils in the housing send and receive radio waves. When you’re in the machine, your body produces very faint signals in response to the radio waves. These signals are detected by coils within the machine, or by additional coils designed to surround a specific body part needing examination. A computer then processes the signals and generates an image. The collected signals create a composite, 3-D representation of your body.
• Positron emission tomography (PET) scan. Unlike other scanning techniques, a PET scan doesn’t produce clear structural images of organs. Instead, it shows images containing areas of more or less intense color to provide information about chemical activity within certain organs and tissues. Tumors often use more energy than healthy tissues do and may absorb more of a radioactive tracer, which allows the tumors to appear on the scan.

Genetic testing
The discovery of BRCA2 and other genes that may increase breast cancer risk has raised a number of emotional and legal questions about genetic testing. A simple blood test can help identify defective BRCA genes, but it’s not 100 percent accurate. And it’s important to know that having a defective BRCA gene doesn’t mean you’ll get breast cancer. In addition, test results can’t determine at what age you might develop cancer, how aggressively the cancer might progress or what your risk of death might be.

In general, testing is most beneficial if the results will help you make a decision about how you might best reduce your chance of developing breast cancer, such as modifying your lifestyle or closer screening. It may also help family members decide if they should be tested or evaluated for the presence of an abnormal gene. Be sure to thoroughly discuss all your options with a genetic counselor before any testing is done, so that you can understand the risks and the benefits of such testing.

Treatment

Breast cancer in men is generally treated the same as it is in women. In most cases no one right treatment exists. Instead, you’ll want to find the approach that’s best for you. To do that, you’ll need to consider many different factors, including the stage of your cancer and your age.

Before making any decisions, learn as much as you can about the many treatment options. Talk extensively with your health care team. Consider a second opinion. Don’t be afraid to ask questions. In addition, look for breast cancer books, Web sites, and information from organizations such as the American Cancer Society and Susan G. Komen for the Cure. Talking to others who have faced the same decision also may help. This may be the most important decision you ever make.

Treatments exist for every type and stage of breast cancer. Some men may need only surgery. Others will need surgery and an additional (adjuvant) therapy such as radiation, chemotherapy or hormone therapy.

Surgery
Breast-sparing procedures are often an option for women, but are not typically feasible for men. This is because a man’s breast doesn’t contain much tissue, so removing the cancer usually means removing all of the breast. Breast cancer operations include the following:

• Simple mastectomy. During a simple mastectomy, your surgeon removes all of your breast tissue — the lobules, ducts, fatty tissue, and a strip of skin with the nipple and areola. Depending on the results of the operation and follow-up tests, you may also need treatment with radiation to the chest wall, chemotherapy or hormone therapy.
• Modified radical mastectomy. Most men with breast cancer require a modified radical mastectomy. In this procedure, a surgeon removes your entire breast and some underarm (axillary) lymph nodes, but leaves your chest muscles intact. If the cancer has spread into the chest wall, your doctor may need to do a radical mastectomy that removes the chest wall muscles. Serious arm swelling (lymphedema) is more likely to occur in modified radical mastectomy than in simple mastectomy with sentinel node biopsy. Your lymph nodes will be tested to see if the cancer has spread. Depending on those results, you may need further treatment.
• Sentinel lymph node biopsy. Breast cancer first spreads to the lymph nodes under the arm. That’s why you need to have these nodes examined. If your surgeon doesn’t plan to do this, be sure you understand the reason. Until recently surgeons would remove as many lymph nodes as possible. But this greatly increased the risk of numbness, recurrent infections and serious swelling of the arm. That’s why a procedure has been developed that focuses on finding the sentinel nodes — the first nodes to receive the drainage from breast tumors and therefore the first to develop cancer. If a sentinel node is removed, examined and found to be healthy, the chance of finding cancer in any of the remaining nodes is very small, and no other nodes need to be removed. This spares the need for a more extensive operation and decreases the risk of complications. It’s important that the procedure be performed by an experienced team.

Radiation therapy
Radiation therapy uses high-energy X-rays to kill cancer cells and shrink tumors. It’s administered by a radiation oncologist at a radiation center. It may be used to shrink the tumor before surgery or to eliminate any remaining cancer cells in the breast, chest muscles or armpit after surgery.

Most men who undergo radiation therapy for breast cancer receive external beam radiation. In this procedure you receive radiation directed at the cancerous area from a machine outside your body. Radiation is usually started three to four weeks after surgery, to allow some time for your body to heal.

If your doctor recommends chemotherapy, the radiation will be delayed until all of the chemotherapy treatments are completed. You’ll typically receive treatment five days a week for about six consecutive weeks. The treatments are painless, and each treatment takes just a few minutes. The effects are cumulative, however, and you may become quite tired toward the end of the series. Your breast may be pink, puffy and somewhat tender, as if it had been sunburned.

Chemotherapy
Chemotherapy uses drugs to destroy cancer cells. Your doctor may recommend chemotherapy after surgery to kill any cancer cells that may have spread outside your breast. Treatment often involves receiving two or more drugs in different combinations. These may be administered intravenously, in pill form or both. You may have treatments every two or three weeks for three to six months.

For many people, chemotherapy can feel like another illness. The side effects may include hair loss, nausea, vomiting and fatigue. These effects occur because chemotherapy affects healthy cells — especially fast-growing cells in your digestive tract, hair and bone marrow — as well as cancerous ones. Not everyone has side effects, however, and there are now better ways to control some of them.

New drugs can help prevent or reduce nausea. Relaxation techniques, including guided imagery, meditation and deep breathing, also may help. In addition, exercise has been shown to be effective in reducing fatigue caused by chemotherapy.

One side effect of chemotherapy that has only recently been described is called “chemobrain.” This refers to the difficulties some patients have with thinking or concentrating while receiving chemotherapy or after. It’s uncertain how commonly this occurs, or if this is even due to the chemotherapy. Many people who receive chemotherapy don’t experience this side effect. Those who do may have difficulty with word finding, memory, multitasking and learning new things. Studies suggest it may effect between 20 percent and 30 percent of people undergoing chemotherapy. There’s no way to predict who will experience this mild cognitive impairment, and it’s not clear whether the treatment or the cancer is the actual cause of chemobrain.

In rare cases, certain chemotherapy medications may lead to cancer of the white blood cells (acute myeloid leukemia) — usually within one to two years after treatment ends. Some chemotherapy medications have the potential to damage the heart.

Hormone therapy
Estrogen receptor positive cancer means that estrogen might encourage the growth of breast cancer cells in your body. Estrogen is present in men, though in smaller amounts than in women. But 90 percent of breast cancers in men have estrogen receptors. Normally estrogen binds to certain cells in your breast and in other parts of your body. Hormone-blocking agents such as tamoxifen block this binding of estrogen to those receptors. This may help destroy cancer cells that have spread or reduce the chances that your cancer will recur.

The primary medication used to reduce the effect of estrogen in your body is tamoxifen (Nolvadex). This synthetic hormone belongs to a class of drugs known as selective estrogen receptor modulators (SERMs). It’s used as a treatment for men with hormone-sensitive metastatic breast cancer and as an adjuvant therapy for men with early-stage estrogen receptor positive breast cancer. You take tamoxifen daily, in pill form, for five years.

The male hormones — androgens — also play a role in the growth of breast cancer in men, although the role is unclear. Limiting androgens through the use of certain drugs does appear to effectively reduce the spread of the cancer. These drugs include:

• Luteinizing hormone-releasing hormone analogues. These drugs cause the testicles to reduce their androgen production.
• Anti-androgen drugs. Anti-androgens block the effect of male hormones on breast cancer cells.

Side effects of hormonal therapies may include hot flashes, decreased sexual desire, loss of erection, weight gain and mood swings.

A new group of hormonal therapy medications called aromatase inhibitors (anastrozole, exemestane and letrozole) are used to treat hormone-sensitive breast cancer in postmenopausal women. At this time, no data exist as to the usefulness of these medications in the treatment of male breast cancer.

Herceptin therapy
One new medication, trastuzumab (Herceptin), is a monoclonal antibody that attacks and blocks the activity of a certain protein made by some breast cancers. Only about one-third of breast cancers make too much of this protein called HER-2-neu. This protein stimulates the cancer cells to grow. Trastuzumab binds onto this protein and blocks its effect and kills the cancer cells. This treatment only works in those breast cancers that make too much HER-2-neu. Side effects of trastuzumab are uncommon, but may include heart problems, fever, chills, nausea and vomiting, weakness, diarrhea and headache.

Biological therapy
Sometimes called biological response modifier or immunotherapy, this treatment tries to stimulate your body’s immune system to fight cancer. Using substances produced by the body or similar substances made in a laboratory, biological therapy seeks to enhance your body’s natural defenses against specific diseases. Many of these therapies are experimental and available only in clinical trials.

Prevention

To help reduce your risk of breast cancer, maintain a healthy body weight and avoid heavy alcohol use. Early detection also increases your chances of surviving the disease. So if you develop a breast lump or other abnormality, seek prompt care.

Coping skills

After a diagnosis of breast cancer, it may take some time to sort through all your emotions. But you can still feel in charge of your life. One of the best ways to regain control is to educate yourself about breast cancer and its treatment. You’ll have many decisions to make in the weeks and months ahead. The more you know, the better prepared you’ll be to make the best choices.

In addition to talking to your medical team — your surgeon, a specialist in chemotherapy and hormone therapy (medical oncologist) and a specialist who administers radiation therapy (radiation oncologist) — you may also want to talk to a counselor or medical social worker. Or you may find it helpful and encouraging to talk to other men with cancer.

There are also excellent books about breast cancer and many reputable resources on the Internet. Be sure to look for the most current information, however. Breast cancer treatments are changing rapidly, and information quickly becomes dated. It’s important not to rely on just one source. There are many different approaches to breast cancer treatment.

Telling others
Unfortunately, treatment decisions aren’t the only decisions you’ll face. Every day may present new challenges. One of the first will likely be how and when to tell those closest to you. If you have children, telling them — no matter what their ages — can be difficult. Yet it’s best to be as honest as you can. You don’t have to give all the details. How much and what you say will depend on each child’s age and ability to understand. But trying to hide your illness isn’t a good idea. Instead, tell your children you’re doing everything possible to get well.

The decision to tell friends and co-workers isn’t easy either. Especially in the beginning, you may not want anyone outside your family to know. But over time you may find it helpful to confide in a few close friends or co-workers. Still, how much and whom to tell is up to you.

Keep in mind that people may not always react as you expect. Some may have many of the same feelings you do — anger, fear, grief. Others may be incredibly supportive. And some may not say much at all or may even avoid you. That’s not because they don’t care, but because they may not know what to say. Let them know that there are no right words and that their concern is enough.

Maintaining a strong support system
More and more studies show that strong relationships are crucial in dealing with life-threatening illnesses. In fact, friends and family are often an integral part of your treatment. Sometimes, though, you may want or need different kinds of support. If so, you may find the concern and understanding of other men with cancer especially helpful. Your doctor or a medical social worker may be able to put you in touch with a group near you. Or contact one of the many cancer organizations.

Taking care of yourself
During your treatment, you’ll need to plan your schedule carefully. Allow yourself time to rest. And don’t be afraid to ask for help. Your friends and family want to help, but they may not always know what to do. Be specific about your needs.

At the same time, you’ll likely want to stay as independent as possible. Sometimes in their desire to help, other people may try to take over your life. Or they may act as if you’re terribly fragile. Both can be detrimental to your recovery. Don’t hesitate to tell friends and loved ones how you want to be treated.

If you haven’t done so before, now is a good time to start eating a healthy diet, getting regular exercise and reducing stress. In fact, stress-reduction techniques and exercise may help relieve some of the side effects associated with radiation and chemotherapy.

Take time to examine what’s most important to you. Think about the things you want to accomplish and how you can achieve your goals. And make it a priority to live your life to the fullest.

Introduction

Gallbladder cancer and bile duct cancer are rare cancers of the biliary tract. Your gallbladder stores and your bile duct transports bile, a fluid produced by your liver that’s essential to the digestive process.

Gallbladder cancer seldom produces symptoms in the early stages. In fact, early gallbladder cancer is often only discovered when the gallbladder is removed as a treatment for gallstones. Otherwise, gallbladder cancer is often quite advanced by the time it’s diagnosed.

When gallbladder cancer is caught early, removing your gallbladder or part of the bile duct may eliminate all the cancerous cells. In advanced cases, treatment won’t cure gallbladder cancer but can help relieve symptoms and improve your quality of life.

Signs and symptoms

Gallbladder cancer rarely produces early signs and symptoms. When symptoms do appear, they often resemble those of other, more common, gallbladder problems such as gallstones or infection.  Gallbladder cancer symptoms include:

• Abdominal pain. Many people with gallbladder cancer have some abdominal pain — usually in the upper right part of the abdomen.
• Nausea and vomiting. These symptoms can occur when a tumor blocks the common bile duct.
• Yellowing of your skin and the whites of your eyes (jaundice). Jaundice results from high blood levels of bilirubin — the residue from the breakdown of red blood cells. Normally, bilirubin is metabolized in your liver and eliminated through the bile ducts. But a blocked bile duct can cause bilirubin to accumulate in your blood. The built-up pigments may turn your skin and the whites of your eyes yellow and your urine dark brown. Because bilirubin isn’t being eliminated through your bile, your stools also may turn pale yellow or white.
• Unintended weight loss or loss of appetite. Tumors can prevent the normal passage of food or interfere with its absorption by blocking the flow of pancreatic enzymes.

Signs and symptoms of bile duct cancer (cholangiocarcinoma)

• Jaundice. A yellowing of your skin and the whites of your eyes, along with tea- or coffee-colored urine and light-colored stools, are common initial signs of bile duct cancer.
• Enlarged gallbladder. A blocked bile duct can cause bile to accumulate in your gallbladder, making it larger than normal. Although your gallbladder is hidden behind other organs in your abdomen, your doctor can sometimes feel this enlargement during a physical exam or it may be detected on an ultrasound.
• Intense itching (pruritis). When the flow of bile is obstructed by a tumor, bile salts may be deposited in your skin, leading to intense itching.

Causes

Your gallbladder is a small, pear-shaped organ on the right side of your abdomen, just beneath your liver. Its main function is to store bile, a bitter, yellow-green fluid that’s produced in the liver cells. Bile is essential for the proper digestion of fats and is one of the main ways your body eliminates drugs, cholesterol and waste products of metabolism. It flows from your liver through a thin tube called the common hepatic duct and enters your gallbladder through another small tube (cystic duct).

When you eat, your gallbladder releases a highly concentrated form of bile into the common bile duct, a continuation of the hepatic and cystic ducts. The bile flows through this duct to the upper part of your small intestine (duodenum), where it begins to break down the fat in your food.

How gallbladder cancer begins
Healthy cells grow and divide in an orderly way — a process that’s controlled by DNA, the genetic material that contains the instructions for every chemical process in your body. When DNA is damaged, changes occur in these instructions. One result is that cells may begin to grow out of control and eventually form a malignant tumor — a mass of cancerous cells.

Although the exact cause of gallbladder and bile duct cancers isn’t clear, researchers believe that DNA in the cells of your biliary tract may be damaged by toxins that are routinely metabolized by your liver. These toxins are released into bile so that they can be eliminated from your body. But if bile empties more slowly than normal, it increases the amount of time your cells are exposed to cancer-causing substances (carcinogens).

Most gallbladder tumors develop in the cells that line the inner surface of the gallbladder. These tumors are most commonly adenocarcinomas — a term that describes the way the cancer cells look when viewed under a microscope.

Gallbladder adenocarcinoma is highly invasive and can quickly penetrate deep into the gallbladder wall, moving through layers of tissue from the inner surface to the outside of the gallbladder. Eventually the cancer may spread to nearby lymph nodes, obstruct the bile duct or invade other organs such as the liver. Cancer cells may also travel through the bloodstream to more remote parts of the body.

Bile duct cancer (cholangiocarcinoma)
Cancer can develop in any part of the bile duct that stretches from your liver to your small intestine. Many tumors occur in the hepatic duct just as it leaves the liver (perihilar tumors). Other tumors may develop in the bile duct near your small intestine (distal tumors) or inside the liver itself (intrahepatic tumors).

The majority of bile duct cancers are adenocarcinomas that originate in the mucous glands lining the inside of the ducts. By the time these cancers are diagnosed, they often have spread to other tissues and organs.

Risk factors

A number of factors may increase your risk of both gallbladder and bile duct tumors, including:

• Gallstones. These solid deposits of cholesterol or calcium salts form in your gallbladder, they are the most common risk factor for gallbladder cancer. The stones may cause your gallbladder to release bile more slowly, which increases the amount of time cells are exposed to toxins. Although most people with gallbladder cancer also have gallstones, the vast majority of people with gallstones never develop gallbladder cancer.
• Age. The chances of developing gallbladder cancer increase as you get older. Most people diagnosed with gallbladder cancer are in their 70s. Bile duct cancer is most common in those over 65.
• Your sex. Women are at least twice as likely as men are to develop gallbladder cancer. They’re also more likely to have gallstones because the female hormone estrogen causes more cholesterol, the main component of most gallstones, to be excreted in bile. But, bile duct cancers are more likely to occur in men than they are in women.
• Bile duct abnormalities. The pancreatobiliary duct junction is the point where the common bile duct — which carries bile from your liver and gallbladder to your small intestine — joins the pancreatic duct carrying digestive juices from your pancreas. In some people, these two ducts connect in a way that allows pancreatic juices to back up into the bile duct and prevents bile from being quickly emptied into the small intestine. This may irritate the cells lining the gallbladder and bile ducts, increasing the risk of cancer.
• Gallbladder polyps. These are growths on the inner surface of your gallbladder. Most aren’t cancerous, but polyps can contain malignant cells.
• Diet. Eating food high in carbohydrates and low in fiber may increase your risk of gallbladder cancer.
• Hazardous chemicals and toxic substances. People exposed to certain industrial chemicals, especially azotoluene, have an increased risk of developing gallbladder cancer.  Thorium dioxide (Thorotrast), a dye once used in X-rays, can cause both liver and bile duct cancers. Although Thorotrast hasn’t been used for decades, exposure to other toxic materials — including dioxin, nitrosamines and polychlorinated biphenyls (PCBs) — may increase your risk of bile duct cancer.
• Obesity. If you are very overweight (about 30 pounds more than your ideal weight), you’re at increased risk of gallbladder cancer. It’s estimated that as many as one in every four cases of gallbladder cancer is linked to excess weight.
• Race. American Indians, especially those living in the Southwest, are five times as likely to develop gallstones and gallbladder cancer as whites are. Hispanics also have higher rates of gallbladder cancer than whites do.
• Primary sclerosing cholangitis (PSC). This autoimmune disorder causes your immune system to attack your bile ducts. PSC creates scar tissue that narrows the bile ducts and prevents bile from reaching your intestines. Over time, repeated injury to bile duct tissue can increase the likelihood of developing cancer.
• Ulcerative colitis. Another autoimmune disease, ulcerative colitis is characterized by severe bouts of bloody diarrhea with abdominal pain and cramping.
• Congenital abnormalities of the bile ducts. These include choledochal cysts, which is a dilation or stretching of the common bile duct, and Caroli’s disease — a dilation of the bile ducts within your liver (intrahepatic ducts). Over time bile that collects in these dilated spaces may seriously damage the duct lining.
• Bile duct stones. Gallstones sometimes escape the gallbladder and enter the cystic duct, the common bile duct or, occasionally, the duct leading to your pancreas. These migrating stones can cause serious complications, including an increased chance of developing bile duct cancer.
• Parasitic infections. These are primarily a concern in Asian countries. Parasitic infections generally occur when humans eat fish containing the cysts of certain flatworms. The worms migrate to the bile ducts, where they damage the duct lining.

When to seek medical advice

See your doctor right away if you develop any of the signs and symptoms of biliary tract problems, such as:

• Jaundice
• Nausea and vomiting
• Abdominal pain
• Severe itching
• Dark urine
• Clay-colored stools

Although these symptoms often aren’t related to cancer, they may indicate other conditions that require medical care.

Screening and diagnosis

Many gallbladder cancers are discovered after a laboratory examination of a gallbladder that’s been removed for other reasons. Only about one-quarter of gallbladder cancers are diagnosed before the cancer is advanced.

Diagnosing gallbladder cancer earlier than this is difficult because the gallbladder is hidden behind the liver and is relatively inaccessible, because signs and symptoms don’t develop until late in the disease, and because when symptoms do appear, they can easily be mistaken for those of many other, more common conditions.

To help detect the existence and spread of gallbladder cancer, you’re likely to have one or more of the following:

• Blood tests. Your doctor may order tests to check for elevated levels of bilirubin or the enzyme alkaline phosphatase, which is released by damaged bile duct cells. You might also have tests to measure certain substances (markers) in your blood that sometimes indicate the presence of a tumor. People with bile duct cancer tend to have high levels of the marker CA 19-9. But CA 19-9 levels can be elevated in people with other types of cancer as well as in people who are cancer-free. For that reason, this isn’t considered a definitive test.
• Ultrasound. This test uses high-energy sound waves to produce images of your internal organs, including your gallbladder. It has no side effects, isn’t invasive and generally takes less than 30 minutes. During the procedure, you lie on a bed or table, and a wand-shaped device (transducer) that emits high-frequency sound waves is placed on your body. The sound waves are reflected from your gallbladder back to the transducer and then translated into a moving image.Ultrasound is usually one of the first tests done in cases of jaundice and is especially good at providing information about the shape and texture of tumors as well as diagnosing the presence of gallstones and obstructed bile ducts.

  Endoscopic ultrasound (EUS) is a technique that can sometimes provide even better images. In this test, an ultrasound transducer is attached to the end of a flexible, lighted viewing tube (endoscope). The endoscope is passed down your throat into your stomach and duodenum, and from there into the common bile duct.

• Computerized tomography (CT) scan. This is essentially a highly detailed X-ray that allows your doctor to see your gallbladder in two-dimensional slices. Split-second computer processing creates these images while a series of thin X-ray beams passes through your body. In most cases, you’ll have a dye (contrast medium) injected into a vein before the test. By producing clearer images, the dye makes it easier to distinguish a tumor from normal tissue. A CT scan can also help determine if cancerous cells have spread to the common bile duct, lymph nodes or liver.Your greatest risk is a possible allergic reaction to the dye. CT scans also expose you to more radiation than do ordinary X-rays.
• Magnetic resonance imaging (MRI). Instead of X-rays, this test uses a powerful magnetic field and radio waves to create images. Used in combination with cholangiography — a test in which a small amount of dye is used to highlight the biliary tract — it can help determine whether the flow of bile is blocked or a tumor has invaded your liver.During the test, you’re encased in a cylindrical tube that can seem confining. The machine also makes a loud thumping noise. In most cases you’ll be given headphones for the noise. If you’re claustrophobic, mild sedation may be an option for you.
• Endoscopic retrograde cholangiopancreatography (ERCP). In this procedure, an endoscope is passed down your throat, through your stomach and into the upper part of your small intestine. Your intestinal tract is inflated with air so that your doctor can more easily see the openings of the bile and pancreatic ducts, and a dye is injected into the ducts through a catheter that’s passed through the endoscope. Finally, X-rays are taken of the ducts.In spite of pain medication, the procedure can be uncomfortable. Your throat also may be sore for a time after the procedure, and you may feel bloated from the air introduced into your intestine. Major complications are rare and include infection and bleeding. This test is most sensitive for detecting an obstruction of the bile ducts and its cause.
• Laparoscopy. A more invasive procedure than ERCP, laparoscopy also uses a small, lighted instrument (laparoscope) to view your gallbladder, liver and surrounding tissue. But in this case, the instrument is attached to a television camera and inserted through a small incision in your abdomen. During the procedure, your surgeon may take tissue samples to help confirm the diagnosis of cancer. Laparoscopy is often used to confirm how far cancer has spread. Risks include bleeding and infection and injury to your abdominal organs or blood vessels.
• Biopsy. In this procedure, a small sample of tissue is removed and examined for malignant cells under a microscope. It’s the only way to make a definitive diagnosis of cancer.Biopsies of the gallbladder and bile ducts can be obtained in several ways. Your doctor may take tissue samples during laparoscopy. Or you may have fine-needle aspiration (FNA), a procedure in which a very thin needle is inserted through your skin and into your gallbladder. An ultrasound or CT scan is often used to guide the needle’s placement. When the needle has reached the tumor, cells are withdrawn and sent to a lab for further study. Tissue samples can also be removed during or after gallbladder surgery.

  Bile duct cells and tiny fragments of duct tissue can be obtained through a procedure known as biliary brushing. As in ERCP, an endoscope is inserted into the bile duct where it empties into your small intestine. But instead of injecting dye and taking X-rays, your surgeon uses a small brush placed in the endoscope to scrape cells and bits of tissue from the lining of your bile duct.

  Staging biliary tract cancers
  Staging tests help determine the size and location of cancer and whether it has spread. This information helps determine the best treatment options.

  Doctors stage biliary tract cancers in several ways. One method is as follows:

  • Resectable. These cancers have not spread beyond the walls of the gallbladder or bile ducts and can be entirely removed in an operation. The term “resectable” refers to a cancer that can be removed.
  • Unresectable. At this stage, the cancer has spread to nearby lymph nodes or other organs such as the liver, pancreas, stomach or intestines and can’t be completely removed.
  • Recurrent. This refers to cancer that returns after it has been treated. It may recur in the gallbladder or bile duct or in some other part of the body.

Complications

Gallbladder and bile duct tumors can cause a number of complications including:

• Nausea and vomiting. These signs and symptoms, along with jaundice, can result when a tumor blocks the common bile duct.
• Malabsorption. A growing tumor in the common bile duct can press against the upper part of the small intestine, preventing food and digestive enzymes from passing normally into the duodenum. This can lead to malabsorption and malnutrition.
• Metastasis. This is the most serious complication of biliary tract cancer. Your gallbladder and bile ducts are surrounded by a number of vital organs, including your liver, stomach, pancreas and intestines. Because biliary tract cancers are rarely discovered in the early stages, they often have time to spread to these organs or to nearby lymph nodes.

Treatment

Gallbladder cancer treatment depends on the type and stage of cancer, as well as on your age, overall health, feelings and personal preferences. Especially when cancer is advanced, choosing a treatment plan is a major decision, and it’s important to take enough time to consider your choices.

You may also want to consider seeking a second opinion. This can provide additional information to help you feel more certain about the option you’re considering.

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 causing more harm. In some cases, an approach called palliative care may be best. 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.

Gallbladder cancer
Surgical removal (resection) of the gallbladder usually offers the best hope for people with gallbladder cancer. If the tumor is very small and hasn’t spread to the deeper layers of gallbladder tissue, your surgeon may perform a simple cholecystectomy, which removes only the gallbladder. Sometimes this may be done laparoscopically, using a camera and miniature instruments inserted through small incisions in your abdomen.

If the cancer is more advanced, your surgeon will likely perform what’s known as an extended cholecystectomy — an operation in which some liver tissue and nearby lymph nodes are removed along with your gallbladder.

Once the cancer has spread beyond the walls of your gallbladder, it can no longer be completely removed with an operation. In that case, your treatment team will discuss other options with you. These may include radiation — either external beam radiation (high-energy X-rays) or implanted radiation “seeds” — or chemotherapy, which is anti-cancer medication.

These additional treatments may be used alone or in combination. Some doctors believe that radiation together with chemotherapy after surgery is more effective than either alone.

Radiation and chemotherapy may also be used for palliative care to help make you more comfortable if your cancer is so advanced that treating the cancer is no longer an option. For example, either treatment may be used to help shrink a tumor that’s blocking a bile duct.

Bile duct cancer
Surgery usually offers the best chance for people with bile duct cancer. But the type of operation you may have will vary, depending on the location of the cancer and how extensive it is:

• Perihilar tumors. Tumors that develop where the hepatic duct leaves your liver (perihilar tumors) are usually treated by surgically removing a portion of the bile duct, the gallbladder and surrounding lymph nodes.
• Distal tumors. Treatment for these tumors, located in the bile duct near your small intestine, is often by what’s known as a Whipple resection. In this operation, your surgeon removes part of your pancreas, the common bile duct and your gallbladder, as well as a small portion of your intestine where the common bile duct and pancreatic duct enter the intestine together.
• Tumors that can’t be removed. When a tumor is too close to major blood vessels, your surgeon may suggest an operation to bypass some of your small intestine. This may help prevent further blockage of your bile duct and digestive tract and may also help relieve symptoms, but won’t cure the cancer. If a bypass isn’t an option, your surgeon may place metal or plastic tubes known as stents into the bile duct to keep it open. After surgery, your doctor may also recommend radiation or chemotherapy.

Clinical trials
Because standard treatments are rarely effective for advanced biliary tract cancers, you may want to consider participating in a clinical trial. This is a study that tests new therapies — typically new drugs, different approaches to surgery or radiation treatments, and novel methods such as gene therapy. If the therapy being tested proves to be safer or more effective than current treatments, it will become the new standard of care.

Treatments used in clinical trials haven’t yet been shown to be effective. They may have serious or unexpected side effects, and there’s no guarantee you’ll benefit from them. On the other hand, cancer clinical trials are closely monitored by the federal government to ensure they’re conducted as safely as possible. And they offer access to treatments that wouldn’t otherwise be available to you. New methods of chemotherapy and radiation are currently in clinical trials.

If you’re interested in finding out more about clinical trials, talk to your treatment team. You can also call the National Cancer Institute’s Cancer Information Service at 800-4-CANCER, or 800-422-6237. The call is free, and trained specialists are available to answer your questions. Or visit the National Cancer Institute’s Web site.

Prevention

Although it’s usually not possible to prevent gallbladder and bile duct cancers, you can take steps to reduce your risk. In general, eating a healthy diet and exercising regularly can lower your risk of many types of cancer.

Aim for at least five servings of fruits and vegetables every day, along with foods from other plant sources such as whole-grain breads, cereals, rice and beans. In addition, try to limit your intake of high-fat foods.

Other steps you can take to reduce your risk of many types of cancer include the following:

• Maintain a healthy weight. This is one of the best ways to reduce your chance of developing gallbladder cancer. If you need to lose weight, remember that a slow, steady loss is the healthiest way to reach your goals. Regular exercise can help you maintain a healthy weight.
• Stop smoking. You can also greatly reduce your risk of many types of cancer by stopping smoking. Cigarette smoke contains carcinogens that can damage the DNA that regulates cell growth. Tobacco use is associated with 180,000 cancer deaths annually. Talk to your doctor about the best ways to quit, or contact the American Cancer Society at 800-ACS-2345, or 800-227-2345, or the National Cancer Institute’s quit line at 877-44U-QUIT, or 877-448-7848, for more information.

Protecting yourself against bile duct cancer
Avoid toxic chemicals. Among these are highly hazardous substances such as dioxin — a byproduct of plastics and chlorinated pesticide manufacturing — and PCBs, which were used in a number of industrial materials in the past. Because PCBs don’t break down easily, they’re still found worldwide in the air, water, soil and especially in fish. Also implicated in bile duct cancers are nitrosamines, a group of carcinogenic chemicals found in products ranging from tobacco leaves to cured meats.

Coping skills

Learning you have any life-threatening illness can be devastating. But coping with a diagnosis of biliary tract cancer can be especially difficult. The more advanced the disease when it’s discovered, the less likely the chance of real recovery. As a result, you may feel especially overwhelmed just when you need to make crucial decisions. Although there are no easy answers for people dealing with biliary tract cancer, some of the following suggestions may help:

• Learn all you can about your illness. Learn everything you can about gallbladder and bile duct cancers — 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 a 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 can be helpful 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, a counselor, medical social worker, religious counselor or even a formal support group can be helpful.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.

Making your wishes known
Many people also take steps to ensure that their end-of-life wishes are known and respected. In fact, the greatest fear of many people with a life-threatening illness is being subjected to treatments they don’t want or spending their last weeks or months in a hospital away from loved ones and familiar surroundings. But many more choices now exist for people with a terminal illness.

• Hospice care. This approach provides a special course of treatment to terminally ill people. It allows family and friends — with the aid of nurses, social workers and trained volunteers — to care for a loved one themselves. It also provides emotional, social and spiritual support for people who are ill and those closest to them.Although most people under hospice care remain in their own homes, the program is available anywhere — including nursing homes and assisted living centers. For those who stay in a hospital, palliative care specialists can provide comfort, compassionate care and dignity.
• Advanced directives. It’s also important to discuss end-of-life issues with your family and medical team. Part of this discussion will likely involve advance directives — a general term for oral and written instructions you give concerning your medical care should you become unable to speak for yourself.One type of advance directive is known as a durable power of attorney (POA) for health care. In this case, you sign a legal document authorizing a person you respect and trust to make legally binding medical decisions for you if you’re unable to do so.

  A POA is often recommended because the appointed person can make decisions in situations not covered in a regular advance directive. Whatever you decide, it’s important to put your wishes in writing. Laws regarding advance directives and POAs vary from state to state, but a written document is more likely to be respected.

Complementary and alternative medicine

More and more people are interested in nontraditional approaches to healing, especially when standard treatments produce intolerable side effects or aren’t able to provide a cure. To address this growing interest, the National Institutes of Health established the National Center for Complementary and Alternative Medicine (NCCAM) in 1992. The center’s mission is to explore nontraditional therapies in a scientifically rigorous way.

Rather than simply addressing a problem with the body, complementary and alternative treatments often focus on the entire person — body, mind and spirit. As a result, they can be effective at reducing stress, alleviating the side effects of conventional treatments such as chemotherapy and improving quality of life.

However, be sure to check with your doctor before trying any complementary or alternative therapy. Some treatments may have potentially serious interactions with medications you’re already taking, or they may decrease the efficacy of your treatment.

Introduction

Cancer that originates in the bone — primary bone cancer — is rare. Fewer than 2,500 Americans are diagnosed with this type of cancer each year. The condition affects more children than adults.

The most common forms of primary bone cancer are:

• Osteosarcoma, which occurs primarily in growing bone tissue
• Chondrosarcoma, which occurs in cartilage
• Ewing’s sarcoma, which arises primarily in immature tissue in bone marrow

Osteosarcoma and Ewing’s sarcoma occur most often in children, adolescents and young adults, ages 10 to 20. Chondrosarcoma is more common in adults over 50.

Treatment of bone cancer depends on the type of bone cancer, as well as its location, size and stage.

Signs and symptoms

Pain is the most common bone cancer symptom. Although bone cancer can arise in any of your bones, it most frequently occurs in the long bones of your arms and legs.

Other possible symptoms and signs of bone cancer include:

• Weakened bones, sometimes leading to fractures
• Joint swelling and tenderness (for tumors in or near joints)
• Fatigue
• Fever
• Unintended weight loss
• Anemia

Causes

Primary bone cancer is different from secondary or metastatic bone cancer. In secondary or metastatic bone cancer, the cancer originates in a different place but spreads (metastasizes) to the bones. For example, someone with prostate cancer may develop bone lesions from the prostate cancer. But, even though the cancer has spread to the bone, it’s still prostate cancer.

Metastatic bone cancer is much more common than is primary bone cancer. Additionally, primary bone cancer doesn’t refer to cancer that begins in the bone marrow — the soft inner core of your bones that makes your blood cells. Bone marrow cancers include diseases such as multiple myeloma and acute and chronic leukemias.

In general, no one knows for certain what causes most primary bone cancers. Adults with Paget’s disease of bone, which involves abnormal development of new bone cells, may be at increased risk of osteosarcoma.

In a few cases, bone cancers may have a hereditary component, such as in:

• Li-Fraumeni syndrome. This condition is characterized by an increased risk of many different cancers, including osteosarcoma, breast cancer, brain cancer and others.
• Rothmund-Thomson syndrome. This condition causes short stature, skeletal problems and rashes, and increases risk of bone cancer.
• Hereditary retinoblastoma. Children with this rare cancer of the eye have an increased risk of osteosarcoma.
• Multiple exostoses. Children with this inherited condition that causes cartilage bumps to form on your bones have an increased risk of chondrosarcoma.

The association with radiation
Radiation is occasionally associated with bone cancer. Exposure to radiation from a diagnostic X-ray won’t harm you. But heavy doses of radiation, such as radiation therapy given for other cancers, can increase your risk of developing bone cancer, especially if you receive the therapy at a young age.

Still, radiation therapy is becoming more and more sophisticated, which may lead to fewer of such side effects. For example, doctors today are better able to regulate doses of radiation and more precisely target the tumor being treated.

When to seek medical advice

Talk to your doctor if you develop signs or symptoms that may indicate bone cancer, such as unexplained bone pain. Having bone pain doesn’t necessarily mean you have bone cancer. Bone pain is more likely due to injuries or arthritis. And bone tumors are often noncancerous (benign). However, the only way to determine whether a tumor is cancerous is for a doctor to examine a sample of the tissue.

Screening and diagnosis

If your doctor suspects you have bone cancer, he or she will take a medical history and perform a physical exam. In addition, the following tests help in diagnosing bone cancer:

• Imaging tests. Tests such as X-rays, computerized tomography (CT) scans, ultrasound and magnetic resonance imaging (MRI) enable your doctor to evaluate the area of concern.
• Bone scan. Your doctor may also request a bone scan, a procedure in which you’re injected with a tiny amount of radioactive material, called a tracer, which can be detected by a special camera used to create images of your bones.

Examining a tissue sample
Determining whether a tumor is malignant requires removal of a sample of tissue (biopsy) from the tumor for examination. If your doctor suspects you have bone cancer, ask for a referral to a cancer center that specializes in bone cancer before the biopsy is performed. Treatment of bone cancer requires a specialized team who work together to treat bone cancer. This team includes a medical oncologist, orthopedic surgeon, radiation oncologist and physical therapist.

Techniques for removing a sample of a suspected bone cancer include:

• Needle biopsy. Your doctor uses a thin needle to remove small pieces of tissue from the tumor. There are two types of needle biopsy — fine-needle aspiration and core biopsy. A core biopsy involves using a slightly larger needle to remove a small, solid core of tissue.
• Surgical biopsy. Your doctor makes an incision through your skin and removes either the entire tumor (excisional biopsy) or a portion of the tumor (incisional biopsy). In some cases you may need only local anesthesia. For a larger or deep-seated tumor, you’ll likely require general anesthesia for the procedure. It’s important that a doctor experienced in the treatment of sarcomas perform the excisional biopsy.

Grading and staging
Besides determining whether the tissue is cancerous, examining tissue can determine how aggressive the cancer is (its grade). Further testing, such as scans and blood tests, will determine if the cancer has spread and how far (its stage).

Childhood bone cancers, such as osteosarcoma or Ewing’s sarcoma, are generally grouped into two stages, depending on whether the cancer is contained in one part of the body (localized) or has spread to other parts (metastasized).

Survival is based on a number of factors, including the type of cancer, at what stage the cancer was discovered and where the tumor is located. If the tumor is very small and localized, the prognosis is generally more promising than if the cancer has begun to spread.

Complications

The complications of bone cancer may include weakened bones and bone fractures. If the cancer spreads to other organs, complications include dysfunction of the affected organ, such as shortness of breath if it spreads to your lungs.

Treatment

As with other cancers, bone cancer treatment depends on the size, type, location and stage of the cancer, including whether it has spread to the lungs or other parts of your body, and your overall health.

Surgery
Surgery is the most common treatment for bone cancer. Surgery for cancer that hasn’t spread involves removing the cancer and a rim of healthy bone surrounding it.

In the past, amputation was common for bone cancer in an arm or leg. Today, advances in surgical techniques and chemotherapy before surgery (neoadjuvant chemotherapy) or after surgery (adjuvant chemotherapy) and radiation therapy make limb-sparing surgery possible in many cases. With osteosarcoma, limb-sparing surgery involves replacing the cancerous bone with an artificial device (prosthesis) or bone from another part of your body or from another person (transplant).

A well-coordinated team of doctors — including surgeons, medical oncologists, radiation oncologists, and physical medicine and rehabilitation specialists who are familiar with treatment of sarcomas — is important for increasing the chance you’ll be able to have limb-sparing treatment. Specialized centers for treatment of sarcoma exist in many areas.

If osteosarcoma spreads, treatment may involve surgical removal of both the bone tumor and the metastasized cancer.

Ewing’s sarcoma has a tendency to metastasize rapidly. Treatment may involve chemotherapy with multiple drugs as well as radiation therapy and surgery to remove the primary tumor.

Radiation therapy
Radiation therapy — also called radiotherapy or X-ray therapy — involves treating cancer with beams of high-energy particles, or waves (radiation), such as gamma rays or X-rays. Although radiation can affect healthy cells as well as cancer cells, it’s much more harmful to cancer cells. In addition, normal cells can recover from the effects of radiation more easily than cancer cells can.

Many people with cancer undergo some type of radiation therapy. Your doctor may suggest using radiation therapy at different times during your cancer treatment and for different reasons, such as before surgery to shrink a cancerous tumor or after surgery to stop the growth of any remaining cancer cells. Radiation may also be used at the same time as chemotherapy. In addition, doctors sometimes use radiation therapy to shrink tumors to decrease the pressure, pain or other symptoms they may cause.

Chemotherapy
Chemotherapy uses medications to kill rapidly dividing cells. These cells include cancer cells, which continuously divide to form more cells, and healthy cells that divide quickly, such as those in your bone marrow, gastrointestinal tract, reproductive system and hair follicles. Healthy cells usually recover shortly after chemotherapy is complete — so, for example, your hair soon starts growing again.

Unlike radiation therapy, which treats only the part of your body exposed to the radiation, chemotherapy treats the body as a whole (systemically). Its purpose is to treat cells that may have escaped from where the cancer originated.

Depending on the type of cancer you have and whether it has spread, your doctor may use chemotherapy to:

• Shrink the cancer prior to an operation, making the operation easier
• Eliminate all cancer cells in your body, even when cancer is widespread
• Prolong your life by controlling cancer growth and spread
• Relieve symptoms and enhance your quality of life

In some cases, chemotherapy may be the only treatment you need. More often, doctors use it in conjunction with other treatments, such as surgery or radiation, to improve results.

Coping skills

A diagnosis of cancer, whether yours or your child’s, presents big challenges. Remember that no matter what your concerns or the prognosis, there are resources and strategies that may make dealing with cancer easier. Here are some suggestions for coping:

• Learn all you can. Find out everything you can about your or your child’s cancer — the type, stage and risks involved and your treatment options and their side effects. The more you know, the more you can participate in treatment decisions. In addition to talking with your doctor, look for information in your local library and from reliable Internet sources.The National Cancer Institute answers questions from the public over the phone at 800-4-CANCER, or 800-422-6237. Or contact the American Cancer Society at 800-ACS-2345, or 800-227-2345.
• Be proactive. Although you may feel tired and discouraged, don’t let others — including your family and your doctor — make important decisions for you. It’s vital that you take an active role in your own or your child’s treatment.
• Maintain a strong support system. Having a support system and a positive attitude can help you cope with the challenges cancer brings. Although friends and family can be your best allies, they sometimes may have trouble dealing with the illness of a loved one. If so, the concern and understanding of a formal support group or of others coping with their own cancer or that of their child can be especially helpful.Although support groups aren’t for everyone, they can be a good source for practical information for you and your family, too. You may also develop deep and lasting bonds with people who are going through the same things you are.
• Maintain hope. Although your hopes may change as you go through a cancer diagnosis and treatment, it’s important to have hope. For example, when you first hear the diagnosis, you may hope that there’s been a mistake. Once you accept the diagnosis, you’ll hope for a good result from treatment.One way to maintain hope is to set goals. Having goals helps you feel in control and can give you a sense of purpose. But don’t choose goals you can’t possibly reach. You may not be able work a 40-hour week, for example, but you may be able to work part time. In fact, many people find that continuing to work and engage in everyday activities can be helpful.
• Take time for yourself. Eating well, relaxing and getting enough rest can help combat the stress and fatigue of cancer. Also, plan ahead for the times when you may need to rest more or limit what you do. If your child has cancer, one of the most important things you can do is take care of yourself. As a caregiver, you need to have the strength and emotional reserves to meet your child’s needs.
• Stay active. Having cancer doesn’t mean you have to stop doing the things you enjoy or normally do. For the most part, if you feel well enough to do something, go ahead and do it. It’s important to stay involved as much as you can. If your child has cancer, try to keep his or her life as normal as possible.
• Look for a connection to something beyond yourself. Having a strong faith or a sense of something greater than yourself may help you successfully cope with cancer.

Introduction

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.

Signs and symptoms

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)

Causes

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.

Risk factors

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 and diagnosis

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.

Complications

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.

Treatment

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.

Prevention

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.

Coping skills

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.

Introduction

Hurthle cell cancer (HCC) is a rare form of cancer affecting the butterfly-shaped thyroid gland in the front part of the lower neck. The thyroid secretes hormones that are essential for regulating your body’s metabolism.

Among new cases of thyroid cancer, less than 5 percent are Hurthle cell cancers. The condition is also called Hurthle cell carcinoma and sometimes oxyphil cell carcinoma.

Hurthle cell cancer can be aggressive, and surgery to remove the thyroid gland is the most common treatment. Most people with Hurthle cell cancer have a good prognosis, but the outlook for Hurthle cell cancer is better the earlier that a diagnosis occurs.

Signs and symptoms

A number of signs and symptoms are associated with thyroid cancer — and specifically Hurthle cell cancer — although these characteristics may not be present in the earliest phases of the disease. These same signs and symptoms may also be indications of other medical conditions — such as an infection of the thyroid gland or a noncancerous enlargement of the thyroid (goiter).

Signs and symptoms of Hurthle cell cancer may include:

• A fast-growing lump (nodule) in your neck, just below your Adam’s apple (the most common clinical sign)
• Enlarged and swollen lymph nodes in your neck
• Pain in your neck or throat, with pain sometimes extending to your ears
• Hoarseness or other changes in your voice
• Shortness of breath
• Trouble swallowing
• A continuous cough not associated with an upper respiratory infection

Causes

The precise cause of Hurthle cell cancer is not known, although researchers believe the disease is associated with alterations in DNA, including some related to the aging process.

DNA is the genetic material that contains the instructions for every chemical process in your body. Some of the genes in your DNA promote cell division, and some slow cell division or program cells to die at the right time. Still other genes control processes that help repair DNA. When DNA is altered or damaged, these genes may not function properly, causing cells to grow out of control and eventually form a mass (tumor) of cancerous (malignant) cells.

Like other thyroid cancers, Hurthle cell cancer has been linked to radiation exposure.

Doctors still have unanswered questions about Hurthle cell cancers, including how to classify them. Hurthle cell cancer is often considered a subgroup of follicular tumors of the thyroid, which develop in the thyroid’s follicle cells. These are cells in which thyroid hormones — thyroxine and triiodothyronine — are manufactured. Some researchers, however, believe that Hurthle cell cancer should be considered a distinct category of thyroid malignancies.

Risk factors

Some studies have suggested the following factors can raise your likelihood of developing Hurthle cell cancer:

• Being female
• Older age
• Radiation treatments to the head and neck

When to seek medical advice

If you have any signs or symptoms associated with Hurthle cell cancer, contact your doctor. An evaluation by your doctor and appropriate diagnostic tests can determine whether these signs or symptoms are caused by Hurthle cell cancer or another medical condition.

Most people with Hurthle cell cancer have a good prognosis and excellent long-term survival. However, if Hurthle cell cancer is diagnosed in its later stages, the outlook may be poorer than for other forms of thyroid cancer.

Screening and diagnosis

If your doctor suspects Hurthle cell cancer, the first steps will be to obtain your medical history and conduct a physical exam. Your doctor will examine your neck, checking the size of your thyroid and seeing whether lymph nodes in the neck are swollen. He or she will also likely order blood tests to check your thyroid function.

To help make a definite diagnosis, your doctor may recommend one or more of the following tests:

• Ultrasound scan. This painless imaging technique, also called a sonogram, uses high-frequency sound waves to outline the neck anatomy and detect a thyroid tumor. A doctor or person who is trained to perform ultrasound exams (sonographer) will place gel on your neck and then place a hand-held transducer on the area. The transducer transmits sound waves into the neck region and sends information to a computer to create a series of pictures of the thyroid — including images of its shape and size.While very good at identifying whether a tumor is present, ultrasound scans can’t tell for sure whether a growth or nodule is malignant or benign. Ultrasound is safe, with virtually no complications associated with its use.
• Fine-needle aspiration (FNA) biopsy. This test may determine whether a tumor or nodule is cancerous or noncancerous. In this procedure, you lie down and the doctor passes a fine needle through the skin of your neck. Your doctor may use ultrasound imaging to help direct the needle into the tumor. As the needle is withdrawn, cellular material is taken out (aspirated). Then the process is repeated to obtain cells from several regions of the growth.These cell samples are placed on glass slides and sent to a laboratory for microscopic analysis to determine whether the nodule is cancerous. In many instances, the results of the biopsy are inconclusive, and the test may need to be repeated or surgical removal of the nodule may be necessary to confirm whether cancer is present. Most tumors turn out to be benign.

  Fine-needle aspiration can be performed in your doctor’s office or at an ambulatory surgical center. Because the needle is very thin (much thinner than a needle used to withdraw blood from your arm), it causes only slight discomfort. Your doctor may apply a local anesthetic to the area to eliminate any chance of pain. Some people experience a small amount of temporary bleeding, bruising or swelling at the site where the needle was inserted.

You won’t need to do much preparation for either of these tests. No fasting is required. In most cases, you won’t need to stop taking any medications.

Other diagnostic tests also may be used, including:

• Magnetic resonance imaging (MRI)
• Computerized tomography (CT) scan
• Positron emission tomography (PET) scan

Complications

Possible complications of Hurthle cell cancer and other thyroid tumors include:

• Difficulty swallowing and breathing. This can occur if the tumor grows and presses on the esophagus.
• Spread of the cancer. If Hurthle cell cancer isn’t treated appropriately, or if treatment begins late, the cancer can spread (metastasize) to other tissues and organs, making therapy more difficult and diminishing the chances of recovery.

Treatment

Several approaches are available to treat Hurthle cell cancer:

• Surgery. Surgery is the best treatment for Hurthle cell cancer. During this operation all or nearly all of the thyroid gland is removed, in a procedure is called a near total thyroidectomy. The surgeon leaves tiny edges of thyroid tissue near the adjacent parathyroid glands to lower the likelihood of parathyroid injury. These small parathyroid glands regulate your body’s calcium level. Surrounding lymph nodes are removed to determine if the cancer has spread to them.Before the surgery, your doctor will conduct a complete physical exam, including a heart evaluation to determine the risk of cardiac complications during the operation. Blood tests can reveal if you have a bleeding disorder that could become problematic. You’ll be asked to avoid eating and drinking, beginning the night before the operation.

  The greatest risk associated with the operation is unintended injury to the recurrent laryngeal nerve, which could cause temporary or permanent hoarseness or a loss of your voice. Other complications include damage to the parathyroid, excessive bleeding and infection. You’ll probably need to stay in the hospital for a day or two after the operation to ensure that post-surgical bleeding hasn’t occurred and that you’re able to breathe normally.

• Thyroid hormone medication. After surgery for Hurthle cell cancer, your doctor will prescribe the synthetic drug levothyroxine (Levoxyl, Synthroid, Levothroid), which replaces the hormones no longer being produced because of the absence of the thyroid. You’ll need to take these synthetic hormones for the rest of your life. These medications are safe, and blood tests help determine the correct dose, which minimizes the risk of side effects.
• Radioactive iodine therapy. Radiation therapy using a capsule containing radioactive iodine 131 is often prescribed as an adjunct to surgery for Hurthle cell cancer. It can be used to destroy any microscopic thyroid tissue remaining after the operation, using a modest dose of radiation in a procedure called “remnant ablation.” At times, a higher dose of radioiodine can be used to eliminate any malignant cells that have spread to other parts of the body, such as the lungs and bones.Radioactive iodine therapy is typically administered about six weeks after surgery, generally as an outpatient procedure.

  In rare cases, radioiodine therapy can cause temporary side effects including sore throat, dry mouth, decrease in taste sensations, neck tenderness, nausea and vomiting. Nearly all traces of the radioactive iodine are excreted from the body within about two days after the start of treatment. Doctors avoid this form of treatment in pregnant and breast-feeding women because of risks to the fetus and newborn. Women are advised to wait at least six months before attempting to conceive, and men are advised to wait several months before attempting conception.

• External beam radiation. This approach delivers radiation treatment to attack malignant cells. It’s sometimes an option for people in whom radioiodine therapy hasn’t been successful. It uses a special X-ray machine called a linear accelerator to deliver radiation to cancer cells. This treatment can target specific areas where cancer cells are present, minimizing damage to nearby healthy tissue. Side effects may include fatigue and a temporary reddening and sensitivity of the skin.

Prevention

There are few specific measures you can take to prevent Hurthle cell cancer. However, you may be able to lower your risk by reducing your exposure to radiation from X-rays and other sources whenever possible.

Complementary and alternative medicine

Although many alternative or nontraditional treatments — from vitamins to herbs — are sometimes promoted for use in patients with cancer, none has been proved helpful in the management of Hurthle cell cancer or other types of thyroid cancer. If you have questions about a specific alternative treatment, talk with your doctor.

Introduction

Pancreatic cancer is one of the most serious of cancers. It develops when cancerous cells form in the tissues of your pancreas — a large organ that lies horizontally behind the lower part of your stomach. Your pancreas secretes enzymes that aid digestion and hormones that help regulate the metabolism of carbohydrates.

Pancreatic cancer spreads rapidly and is seldom detected in its early stages, which is a major reason why it’s a leading cause of cancer death. Signs and symptoms may not appear until the disease is quite advanced. By that time, the cancer is likely to have spread to other parts of the body and surgical removal is no longer possible.

For years, little was known about pancreatic cancer. But researchers are beginning to understand the genetic basis of the disease — knowledge that may eventually lead to new and better treatments. Just as important, you may be able to reduce your risk of pancreatic cancer with some lifestyle changes.

Signs and symptoms

Signs and symptoms of pancreatic cancer often don’t occur until the disease is advanced. When symptoms do appear, they may include:

• Upper abdominal pain that may radiate to your middle or upper back. Pain is a common symptom of advanced pancreatic cancer. Abdominal pain occurs when a tumor presses on surrounding organs and nerves. Pain may be constant or intermittent and is often worse after you eat or when you lie down. Because many conditions other than cancer can cause abdominal pain, be sure to discuss your symptoms carefully with your doctor.
• Loss of appetite and unintentional weight loss. Unintended weight loss is a common sign of pancreatic cancer. Weight loss occurs in most types of cancer because cancerous (malignant) cells deprive healthy cells of nutrients, and this is especially true in pancreatic cancer.
• Yellowing of your skin and the whites of your eyes (jaundice). About half of people with pancreatic cancer develop jaundice, which occurs when bilirubin, a breakdown product of worn-out blood cells, accumulates in your blood. Normally, bilirubin is eliminated in bile, a fluid produced in your liver. But if a pancreatic tumor blocks the flow of bile, excess pigment from bilirubin may turn your skin and the whites of your eyes yellow. In addition, your urine may be dark brown and your stools white or clay-colored. Although jaundice is a common sign of pancreatic cancer, it’s more likely to result from other conditions, such as gallstones or hepatitis.
• Itching. In the later stages of pancreatic cancer, you may develop severe itching when high levels of bile acids, another component of bile, accumulate in your skin.
• Nausea and vomiting. In advanced cases of pancreatic cancer, the tumor may block a portion of your digestive tract, usually the upper portion of your small intestine (duodenum), causing nausea and vomiting.
• Digestive problems. When cancer prevents pancreatic enzymes from being released into your intestine, you’re likely to have a hard time digesting foods — especially those high in fat. Eventually, this may lead to significant weight loss — as much as 25 pounds or more — and malnutrition.

Causes

Your pancreas is about 6 inches long and looks something like a pear lying on its side. The wider end (head) is located near the center of your abdomen next to the upper part of your small intestine (duodenum). The main part (body) of the pancreas stretches behind your stomach, and the narrow end (tail) is on your left side, next to your spleen.

A part of your digestive system, your pancreas performs two essential functions:

• It produces digestive juices and enzymes that help break down proteins, carbohydrates and fats so the food you eat can be digested in your small intestine.
• It secretes the hormones insulin and glucagon that regulate the way your body metabolizes sugar (glucose).

Most of your pancreas is composed of cells that produce digestive enzymes and juices. Pancreatic juices flow into the main pancreatic duct, which leads to your small intestine (duodenum). The pancreatic duct joins up with the tube leading from your gallbladder to form the common bile duct, which then empties into the small intestine. Your pancreas also contains small “islands” of cells that secrete the hormones insulin and glucagon, along with somatostatin.

Types of pancreatic cancer
Most pancreatic tumors originate in the duct cells or in the cells that produce digestive enzymes (acinar cells). Called adenocarcinomas, these tumors account for nearly 95 percent of pancreatic cancers.

Tumors that begin in the islet cells (endocrine tumors) are much less common. When they do occur, they may cause the affected cells to produce too much hormone. For example, tumors in glucagon cells (glucagonomas) might cause excess amounts of glucagon to be secreted, while tumors in insulin cells (insulinomas) may lead to an overproduction of insulin.

Tumors can also develop in the ampulla of Vater — the place where your bile and pancreatic ducts empty into your small intestine. Called ampullary cancers, these tumors often block the bile duct, leading to jaundice. Because even a small tumor can obstruct the bile duct, signs and symptoms of ampullary cancer usually appear earlier than do symptoms of other pancreatic cancers.

Why pancreatic cancer occurs
Healthy cells grow and divide in an orderly way. This process is controlled by DNA — the genetic material that contains the instructions for every chemical process in your body. When DNA is damaged, changes occur 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.

Researchers don’t know exactly what damages DNA in the vast majority of cases of pancreatic cancer. But it is known that a small percentage of people develop the disease as a result of a genetic predisposition. These people who have a close relative, such as a parent or sibling, with pancreatic cancer have a higher risk of developing pancreatic cancer themselves.

In addition, a number of genetic diseases have been associated with an increased risk of pancreatic cancer, including familial adenomatous polyposis, nonpolyposis colon cancer, familial breast cancer associated with the BRCA2 gene, hereditary pancreatitis, and familial atypical multiple mole-melanoma syndrome — a serious type of skin cancer. This means that people who have a hereditary predisposition to develop these cancers are also more likely to develop pancreatic cancer.

Yet only about 10 percent of pancreatic cancers result from an inherited tendency. A greater number are caused by environmental or lifestyle factors, such as smoking, diet and chemical exposure.

Risk factors

The vast majority of pancreatic cancers occur in people older than 65. Other important risk factors include:

• Race. Black men and women have a higher risk of pancreatic cancer.
• Sex. More men than women develop pancreatic cancer.
• Cigarette smoking. If you smoke, you’re two to three times more likely to develop pancreatic cancer than nonsmokers are. This is probably the greatest known risk factor for pancreatic cancer, with smoking associated with almost one in three cases of pancreatic cancer.
• Abnormal glucose metabolism. Having diabetes may increase your risk of pancreatic cancer. Insulin resistance or high insulin levels may also be risk factors for pancreatic cancer.
• Hereditary pancreatitis. Your chances of developing pancreatic cancer increase if you have hereditary chronic pancreatitis. Hereditary pancreatitis (HP) is a rare genetic condition marked by recurrent attacks of pancreatitis — a painful inflammation of your pancreas.
• Excess weight. People who are very overweight or obese may have a greater risk of developing pancreatic cancer than do people of normal weight.
• Diet. A diet high in animal fat and low in fruits and vegetables may increase your risk of pancreatic cancer.
• Chemical exposure. People who work with petroleum compounds, including gasoline and other chemicals, have a higher incidence of pancreatic cancer than people not exposed to these chemicals.

When to seek medical advice

See your doctor if you experience an unexplained weight loss, abdominal pain or jaundice. Many problems other than cancer may cause similar signs and symptoms, so your doctor will check for these conditions as well as for pancreatic cancer. If cancer is present, early diagnosis and treatment offer the best chance of recovery.

Screening and diagnosis

Detecting pancreatic cancer in its early stages is difficult. Signs and symptoms usually don’t appear until the cancer is large or has spread (metastasized) to other tissues. And because your pancreas is relatively hidden — tucked behind your stomach and inside a loop of your small intestine — small tumors can’t be seen or felt during routine exams.

For this reason, and because pancreatic cancer spreads so quickly, researchers have focused on finding a reliable screening test. At one time, scientists thought a substance called CA 19-9 was the answer. CA 19-9 is produced by pancreatic cancer cells and can be detected by a blood test. But by the time blood levels are high enough to be measured, the cancer is no longer in its early stages. Currently there is no effective screening test for pancreatic cancer.

If your doctor suspects pancreatic cancer, you may have one or more of the following tests to diagnose the cancer:

• Ultrasound imaging. In this test, a device called a transducer is placed on your upper abdomen. High-frequency sound waves from the transducer reflect off your abdominal tissues and are translated by a computer into moving images of your internal organs, including your pancreas. Ultrasound tests are safe, noninvasive and relatively brief — a typical test takes less than an hour.
• Computerized tomography (CT) scan. This imaging test allows your doctor to visualize your organs, including your pancreas, in two-dimensional slices. Split-second computer processing creates these images as a series of very thin X-ray beams pass through your body. Sometimes you may have a dye (contrast medium) injected into a vein before the test. The clearer images produced with the dye make it easier to distinguish a tumor from normal tissue. A CT scan exposes you to more radiation than do conventional X-rays, but in most cases, the benefits of the test outweigh the risks.
• Magnetic resonance imaging (MRI). Instead of X-rays, this test uses a powerful magnetic field and radio waves to create images of your pancreas. During the test, you’re placed in a cylindrical tube that can seem confining to some people. The machine also makes a loud thumping noise you might find disturbing. In most cases you’ll be given headphones for the noise.
• Endoscopic retrograde cholangiopancreatiography (ERCP). In this procedure, a thin, flexible tube (endoscope) is gently passed down your throat, through your stomach and into the upper part of your small intestine. Air is used to inflate your intestinal tract so your doctor can more easily see the openings of your pancreatic and bile ducts. The bile ducts are thin tubes that carry bile, a fluid produced in your liver that helps digest fats. These ducts are often the site of pancreatic tumors. A dye is then injected into the ducts through a small hollow tube (catheter) that’s passed through the endoscope. Finally, X-rays are taken of the ducts. Your throat may be sore for a time after the procedure, and you may feel bloated from the air introduced into your intestine.
• Endoscopic ultrasound (EUS). In this test, an ultrasound device is passed through an endoscope into your stomach. The device directs sound waves to your pancreas. A computer then translates the sound waves into close-up images of your pancreas and your bile and pancreatic ducts. The images are superior to those produced by standard ultrasound and are particularly useful for detecting small pancreatic tumors.
• Percutaneous transhepatic cholangiography (PTC). In this test, your doctor carefully inserts a thin needle into your liver while you lie on a movable X-ray table. A dye is then injected into the bile ducts in your liver, and a special X-ray machine (fluoroscope) tracks the dye as it moves through the ducts. Any obstructions should show up on the X-ray. The table is rotated several times during the procedure so you can assume a variety of positions. During the test, you may have a feeling of pressure or fullness, or have slight discomfort in the right side of your back.
• Biopsy. In this procedure, a small sample of tissue is removed and examined for malignant cells under a microscope. It’s the only way to make a definitive diagnosis of cancer. Biopsies of the pancreas and bile ducts can be performed in several ways. If you have a mass that can be reached with a needle, your doctor may choose to perform a fine-needle aspiration (FNA) — a procedure in which a very thin needle is inserted through your skin and into your pancreas. An ultrasound or CT scan is often used to guide the needle’s placement. When the needle has reached the tumor, cells are withdrawn and sent to a lab for further study. Tissue samples can also be removed during ERCP or EUS. Sometimes, in a procedure similar to ERCP, your surgeon uses an endoscope to pass a catheter into your bile duct where it empties into your small intestine. But instead of injecting dye, your surgeon uses a small brush introduced through the catheter to scrape cells and bits of tissue from the lining of the duct.
• Laparoscopy. This procedure uses a small, lighted instrument (laparoscope) to view your pancreas and surrounding tissue. The instrument is attached to a television camera and inserted through a small incision in your abdomen. The camera allows your surgeon to clearly see what’s happening inside you. During laparoscopy, your surgeon can take tissue samples to help confirm a diagnosis of cancer. Laparoscopy may also be used to determine how far cancer has spread. Risks include bleeding and infection and a slight chance of injury to your abdominal organs or blood vessels.

Staging pancreatic cancer
Staging tests help determine the size and location of cancer and whether it has spread. They’re crucial in helping your doctor determine the best treatment for you. Pancreatic cancer may be staged in several ways. One method is to use these terms:

• Resectable. All the tumor nodules can be removed.
• Locally advanced. Because the cancer has spread to tissues around the pancreas or into the blood vessels, it can no longer be completely removed.
• Metastatic. At this stage, the cancer has spread to distant organs, such as the lungs and liver.

Your doctor may also refer to your cancer as stage 1, 2, 3, or 4:

• Stage 1 pancreatic cancer is confined to the pancreas.
• Stage 2 pancreatic cancer has spread somewhat, possibly to the lymph nodes, but not into large blood vessels nearby.
• Stage 3 pancreatic cancer has invaded large blood vessels, may be in the lymph nodes, but hasn’t spread to distant sites.
• Stage 4 means the cancer has spread to a distant site or sites in your body.

Complications

Your pancreas produces a number of enzymes that break down food so your body can absorb the nutrients it contains. But pancreatic tumors often interfere with the production or flow of these enzymes. As a result, your body can’t easily absorb nutrients, which can lead to diarrhea and severe weight loss.

Other complications of pancreatic cancer include:

• Problems with glucose metabolism. Tumors that affect the ability of your pancreas to produce insulin can lead to problems with glucose metabolism, including diabetes.
• Jaundice, sometimes with severe itching. Yellowing of your skin and the whites of your eyes can develop when a pancreatic tumor blocks your bile duct, the thin tube that carries bile from your liver to your duodenum. The yellow color comes from excess bilirubin. Bile acids may cause intense itching when they build up in your skin.
• Pain. Large pancreatic tumors may press on surrounding nerves, leading to back or abdominal pain that may sometimes be severe. Often, your doctor can prescribe medications that help relieve pain. When medications aren’t enough, cutting or injecting alcohol into some of the affected nerves may be an option.
• Metastasis. This is the most serious complication of pancreatic cancer. Your pancreas is surrounded by a number of vital organs, including your stomach, spleen, liver, lungs and intestine. Because pancreatic tumors are rarely discovered in the early stages, they often have time to spread to these organs or to nearby lymph nodes.

Treatment

Treatment for pancreatic cancer depends on the stage and location of the cancer as well as on your age, overall health and personal preferences. Especially when cancer is advanced, choosing a treatment plan is a major decision, and it’s important to carefully evaluate your choices.

You may also want to consider seeking a second opinion. This can provide additional information to help you feel more certain about the option you’re considering.

The first goal of treatment is always to eliminate the cancer completely. When that isn’t possible, the focus may be on preventing the tumor from growing or causing more harm. In some cases, an approach called palliative care may be best. 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.

Surgical options
The only way to eliminate pancreatic cancer is an operation to remove the tumor. Unfortunately, this is possible only in a small percent of people. Once the cancer has spread beyond the pancreas to other organs, lymph nodes or blood vessels, surgery is usually no longer an option. When surgery is possible, your surgeon may use one of the following procedures, depending on the extent and location of the tumor:

• Whipple procedure (pancreatoduodenectomy). This is the most common procedure for treating pancreatic cancer, including resectable cancers of the ampulla of Vater. In general, the Whipple procedure involves removing the wide end (head) of your pancreas. To do that, your surgeon must also remove your duodenum, gallbladder and the end of the common bile duct. Sometimes part of your stomach is removed as well. The end of your bile duct and remaining part of your pancreas are then connected to your small intestine so that bile and pancreatic enzymes continue to reach the small intestine. The procedure has risks, including infection and bleeding.
• Total pancreatectomy. In this procedure, your surgeon removes your entire pancreas as well as your bile duct, gallbladder and spleen; part of your small intestine and stomach; and most of the lymph nodes in the area. After a total pancreatectomy, you’ll need insulin injections and pancreatic enzymes, and the operation presents serious risks. Total pancreatectomy isn’t often used for people with pancreatic cancer because there doesn’t appear to be enough benefit from the procedure to justify the risks.
• Distal pancreatectomy. In this procedure, which is primarily used to treat islet cell cancers, only the tail — or the tail and a small portion of the body of your pancreas — is removed. Sometimes your spleen may also be removed.

Operations for pancreatic cancer are complex. The most successful outcomes generally occur when these procedures are performed in cancer centers by highly experienced surgeons.

Radiation therapy
Radiation therapy uses high-energy X-rays to destroy cancer cells. You may receive radiation treatments before or after cancer surgery, often in combination with chemotherapy. Or, your doctor may recommend a combination of radiation and chemotherapy treatments when your cancer can’t be treated surgically.

Radiation that comes from a machine outside your body (external beam radiation) is generally used to treat pancreatic cancer. Side effects of radiation therapy may include a burn on your skin similar to sunburn where the radiation enters your body, nausea, vomiting and fatigue.

Doctors at some cancer centers are studying a new approach to radiation therapy, called intraoperative electron beam radiation. In this procedure, a type of external beam radiation that uses high-energy particles (electrons) is directed at your pancreas during surgery. This allows doctors to treat a pancreatic tumor with a high dose of radiation while sparing nearby organs.

Chemotherapy
Chemotherapy uses drugs to help kill cancer cells. Injected into a vein or taken orally, these drugs travel through your bloodstream. For that reason, they’re often used to treat cancers that have spread. Chemotherapy, or chemotherapy in combination with radiation, is the usual treatment for pancreatic cancers that have spread to nearby tissues or distant organs. Although chemotherapy won’t eliminate the cancer, it may help relieve symptoms. It may also help improve survival when used as an adjuvant therapy after an operation to remove a tumor in the pancreas.

For years, the drug fluorouracil (5-FU) was the only chemotherapy option for people with pancreatic cancer. But fluorouracil wasn’t always effective. Now doctors are having more success with a newer drug, gemcitabine. The drug is normally used alone but may be used in combination with other drugs as part of a clinical trial. In November 2005, the U.S Food and Drug Administration (FDA) approved the combination of gemcitabine and erlotinib to treat advanced pancreatic cancer that hasn’t responded to other treatments. While this combination isn’t a cure, it does increase the life expectancy for some people. Doctors are also testing a number of other new medications and new combinations of older medicines.

Chemotherapy drugs affect normal cells as well as malignant ones, especially fast-growing cells in your digestive tract and bone marrow. For that reason, side effects — including nausea and vomiting, mouth sores, an increased chance of infection due to a shortage of white blood cells, and fatigue — are common. Not everyone experiences side effects, however, and there are new and better ways to control them if you do. Be sure to discuss any questions you may have about side effects with your treatment team.

Clinical trials
If you have advanced pancreatic cancer, you may want to consider participating in a clinical trial. This is a study that is used to test new forms of therapy — typically new drugs, different approaches to surgery or radiation treatments, and novel methods such as gene therapy. If the therapy being tested proves to be safer or more effective than current treatments, it will become the new standard of care.

Remember that the treatments used in clinical trials haven’t yet been shown to be effective. They may have serious or unexpected side effects, and there’s no guarantee you’ll benefit from them.

On the other hand, cancer clinical trials are closely monitored by the federal government to ensure they’re conducted as safely as possible. And they offer access to treatments that wouldn’t otherwise be available to you.

If you’re interested in finding out more about clinical trials, talk to your treatment team. You can also call the National Cancer Institute’s Cancer Information Service at (800) 4-CANCER, or (800) 422-6237.

Palliative procedures
If your cancer has spread too far to be completely removed by an operation, the primary goal will be to relieve your signs and symptoms. Treatments that focus on making you more comfortable include:

• Surgical bypass. Tumors that block your bile duct, pancreatic duct or duodenum can cause pain, digestive difficulties, nausea, vomiting, jaundice and severe itching. To help ease some of these symptoms, you may have an operation to reroute the flow of bile by going around (bypassing) the tumor.
• Stent insertion. When a bypass operation isn’t an option, your surgeon may place a stainless steel or plastic tube (stent) in the bile duct to keep it open. A stent is usually the best choice for people who have metastatic cancer or who are very weak.
• Pain management. Tumors pressing on surrounding nerves can cause severe pain, especially in the later stages of the disease. Although pain is a real concern for people with pancreatic cancer, treatment with morphine or similar medications can provide relief in many cases. Long-lasting forms of morphine that need to be taken only once or twice a day may be especially helpful. When medication isn’t enough, your doctor may discuss other options with you, such as cutting some of the nerves that transmit pain signals or injecting alcohol into these nerves to block the sensation of pain.
• Pancreatic enzyme tablets. By replacing the digestive enzymes your pancreas no longer produces, these tablets can improve your body’s ability to absorb nutrients and may help reduce diarrhea and weight loss.
• Insulin therapy. When pancreatic cancer affects insulin production, you may need insulin injections to help control your blood sugar levels.

New treatments
Researchers are studying a number of other approaches to treating pancreatic cancer, including:

• Anti-angiogenesis factors. Cancer cells need angiogenesis factors to produce new blood vessels so they can grow and spread. Scientists have developed drugs that stop this process.
• Farnesyl transferase inhibitors. Most people with pancreatic cancer have cells that contain a specific genetic mutation. In order to function, the mutated cells need an enzyme called farnesyl transferase. Now researchers have developed drugs that block the action of farnesyl transferase, causing the mutated cells to die. Studies of these drugs are under way.
• Growth factor inhibitors. Growth factor receptors help some malignant cells grow. Drugs that block these receptors, such as cetuximab, may help fight pancreatic cancer.

Prevention

Although it’s not always possible to prevent pancreatic cancer, these lifestyle changes may help reduce your risk:

• Quit smoking. Cigarette smoke contains carcinogens that can damage the DNA that regulates cell growth. Talk to your doctor about the best ways to quit, or contact the American Cancer Society or American Lung Association for more information.
• Maintain a healthy weight. Being overweight increases your risk of pancreatic cancer. If you need to lose weight, keep in mind that a slow, steady loss is the healthiest way to reach your goals. Aim for no more than 1 to 2 pounds a week. Add 30 minutes or more of aerobic exercise — such as walking, jogging or biking — on most days, and you can increase the amount of weight you lose.
• Exercise regularly. Experts believe that getting even a moderate amount of exercise every week can cut your risk of pancreatic cancer. For overall health, aim for 30 minutes of exercise on most days. If you’re not used to exercising, start out slowly and work up to your goal.
• Eat a healthy diet. A diet high in fruits and vegetables and low in animal fat can reduce your risk of pancreatic cancer.

Self-care

Poor appetite, weight loss and muscle wasting are often problems for people with pancreatic cancer. These symptoms may be compounded by cancer treatments as well as by the emotional toll of living with the disease. For that reason, your doctor may recommend talking to a registered dietitian. He or she can help you find ways to get the nourishment you need.

These suggestions also may help:

• Reduce the size of your meals. Try eating several small meals throughout the day instead of two or three larger ones. If you’re nauseous, choose foods that are soothing and easy-to-digest, such as soups, rice or a plain baked potato.
• Restrict dietary fat. Follow your doctor’s recommendations for reducing fat in your diet. Pancreatic cancer affects your body’s ability to digest fats.
• Consider dietary supplements. With pancreatic cancer, your body can’t easily absorb nutrients. Talk to your doctor about vitamin and mineral supplements.
• Have nourishing snacks within easy reach. That way, you’re more likely to eat. Fresh fruit, nonfat yogurt or carrot sticks are all good choices.
• Don’t worry if you have days when you can’t eat at all. In the meantime, do whatever you can to make yourself feel better. Let your doctor know if you don’t feel better in a couple of days.
• Try to drink plenty of fluids. Water is essential for your body’s proper functioning.

Coping skills

Learning you have any life-threatening illness can be devastating. But coping with a diagnosis of pancreatic cancer can be especially difficult. The more advanced the disease when it’s discovered, the less likely the chance of real recovery. As a result, you may feel especially overwhelmed just when you need to make crucial decisions. Although there are no easy answers for people dealing with pancreatic cancer, some of the following suggestions may help:

• Learn all you can about your illness. Learn everything you can about pancreatic 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 a 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. Some reliable sites are listed at the end of this article. In addition, the National Cancer Institute offers a toll-free information line called the Cancer Information Service that you can reach at (800) 4-CANCER, or (800) 422-6237.
• Maintain a strong support system. Strong relationships are crucial to 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, pastoral or religious counselor, 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 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.A great fear of many people with a life-threatening illness is being subjected to treatments they don’t want or spending their last weeks or months in a hospital away from loved ones and familiar surroundings. The welcome news is that many choices exist for people with a terminal illness.

  Hospice care, for example, provides a special course of treatment to terminally ill people. This allows family and friends — with the aid of nurses, social workers and trained volunteers — to care for and comfort a loved one at home or in hospice residences. It also provides emotional, social and spiritual support for people who are ill and those closest to them. Although most people under hospice care remain in their own homes, the program is available anywhere — including nursing homes and assisted-living centers. For people who stay in a hospital, palliative care specialists can provide comfort, compassionate care and dignity.

  Although it can be extremely difficult, discuss end-of-life issues with your family and medical team. Part of this discussion will likely involve advance directives — a general term for oral and written instructions you give concerning your medical care should you become unable to speak for yourself.

  One type of advance directive is known as a durable power of attorney (POA) for health care. In this case, you sign a legal document authorizing a person you respect and trust to make legally binding medical decisions for you if you’re unable to do so. A POA is often recommended because the appointed person can make decisions in situations not covered in a regular advance directive. Whatever you decide, it’s important to put your wishes in writing. Laws regarding advance directives and POAs vary from state to state, but a written document is more likely to be respected.

  To learn more about advance directives, you can contact the National Hospice and Palliative Care Organization at (800) 658-8898.

Complementary and alternative medicine

More and more people are interested in nontraditional approaches to healing, especially when standard treatments produce intolerable side effects or aren’t able to provide a cure. To address this growing interest, the National Institutes of Health established the National Center for Complementary and Alternative Medicine (NCCAM).

The center’s mission is to explore nontraditional therapies in a scientifically rigorous way. NCCAM and the National Cancer Institute have teamed up specifically to look at the role complementary and alternative medicine may play in the treatment of cancer.

In general, alternative medicine refers to therapies, such as mistletoe or coenzyme Q-10, that may be used instead of conventional treatments. Complementary or integrative medicine, on the other hand, usually means therapies used in conjunction with traditional treatments.

Rather than simply addressing a problem with the body, complementary and alternative treatments often focus on the entire person — body, mind and spirit. As a result, they can be especially effective at reducing stress, alleviating the side effects of conventional treatments such as chemotherapy, and improving quality of life. However, these products aren’t regulated by the FDA, so you can’t be sure of a product’s quality. Additionally, some of these substances can interfere with your treatment. Check with your doctor before taking alternative medications.

NCCAM’s findings are available on its Web site. You can also talk to information specialists at NCCAM’s clearinghouse by calling (888) 644-6226.

Introduction

Although the incidence of stomach cancer has declined dramatically in the United States and Western Europe in the last 60 years, the disease remains a serious problem in much of the rest of the world, where it’s a leading cause of cancer death.

This global variation is almost certainly linked to two factors that play a major role in the development of stomach cancer — infection with Helicobacter pylori (H. pylori) bacteria and diet, especially the type of diet that’s high in salted, smoked and pickled foods common in areas that lack refrigeration as a means of preserving food.

Stomach cancer is more readily treated when caught early. Unfortunately, by the time stomach cancer causes symptoms, it’s often at an advanced stage and may have spread beyond the stomach. Yet there is encouraging news. You can reduce your risk of stomach cancer by making a few changes in your lifestyle.

Signs and symptoms

One early sign of stomach cancer is microscopic internal bleeding, which is usually only detected by tests that check your stool for blood. You may also feel tired if this bleeding causes the loss of too many healthy red blood cells (anemia). Early stomach cancer may also cause symptoms such as heartburn and abdominal pain, which can be mistaken for other, more common problems.

When the cancer is more advanced, you may experience signs and symptoms such as:

• Discomfort in the upper or middle region of your abdomen that may not be relieved by food or antacids (In the early stages of stomach cancer, pain is often relieved by food or acid-buffering medications.)
• Abdominal discomfort aggravated by eating
• Black, tarry stools
• Vomiting blood
• Vomiting after meals
• Weakness and fatigue
• Unintended weight loss
• Full feeling after meals, even when eating less than normal

Having one or more of these signs and symptoms doesn’t necessarily mean you have stomach cancer. Other more common conditions, especially peptic ulcers, can cause similar problems.

Causes

Your stomach is a muscular sac located in the upper middle of your abdomen, just below your ribs. The stomach walls are lined with three layers of powerful muscles that mix food with enzymes and acids produced by glands in the stomach’s inner lining. Your stomach’s delicate tissues are protected from this acidic mix by a thick, jelly-like mucus that coats the stomach lining.

Types of stomach cancer

• Adenocarcinomas. The great majority of stomach cancers are adenocarcinomas, which start in the glandular cells in the stomach’s innermost lining. Adenocarcinomas account for about 95 percent of all stomach cancers.
• Lymphomas. These are cancers of immune system tissue in the stomach wall. Some lymphomas are aggressive, whereas others grow much more slowly. The latter, known medically as mucosa-associated lymphoid tissue (MALT) lymphomas, usually stem from H. pylori infection and are often curable when found in the early stages.
• Carcinoid tumors. A small percentage of stomach cancers are carcinoid tumors that originate in the stomach’s hormone-producing cells. Carcinoid tumors tend to grow less quickly and spread (metastasize) less frequently than do the more common stomach cancers.
• Gastrointestinal stromal tumors (GISTs). Doctors believe that these rare tumors develop from cells called interstitial cells of Cajal, which are part of your autonomic nervous system. Your autonomic nervous system consists of the nerves that regulate the part of your nervous system that you can’t control, such as your heart rate, blood pressure and intestinal function.Although GISTs can occur anywhere from the esophagus to the rectum, most are found in the stomach. Yet GISTs are not the same as other gastric cancers, differing not only in the cells in which they originate but also in their prognosis and treatment. A majority of GISTs have a specific genetic mutation that allows for treatment with a new form of cancer-specific therapy.

Why stomach cancer develops
Healthy cells grow and divide in an orderly way. This process is controlled by DNA — the genetic material that contains the instructions for every chemical process in your body. Some of the genes in your DNA promote cell division and some slow cell division or program cells to die at the right time. Still other genes control processes that help repair DNA. When DNA is damaged, these genes may not function properly, causing cells to grow out of control and eventually form a tumor — a mass of malignant cells.

Although the causes of many types of cancer aren’t clear, researchers have made progress in pinpointing factors that damage DNA in stomach cells and in understanding how that damage leads to cancer. These factors include:

• H. pylori infection. A majority of the world’s population is infected with corkscrew-shaped bacteria called Helicobacter pylori (H. pylori) that live deep in the mucous layer that coats the lining of the stomach. Although it’s not entirely clear how the bacteria are transmitted, it’s likely they spread from person to person through the oral-fecal route or are ingested in contaminated drinking water. H. pylori infection frequently occurs in childhood and can last throughout life if not treated. It’s the primary cause of stomach ulcers.Having ulcers doesn’t necessarily put you at higher risk of stomach cancer, but having H. pylori infection does. That’s because long-term infection causes inflammation that can lead to precancerous changes in the stomach lining. One of these changes is atrophic gastritis, a condition in which the acid-producing glands are slowly destroyed. It’s likely that low acid levels prevent cancer-causing toxins from being properly broken down or flushed out of your stomach.
• Nitrates and nitrites. These are nitrogen-based chemicals that are added to certain foods, especially cured meats such as ham and bacon, hot dogs and deli meats. Both nitrates and nitrites combine with other nitrogen-containing substances in your stomach to form N-nitroso compounds — carcinogens that are known to cause stomach cancer.
• Salted, smoked or pickled foods and red meat. Before the advent of refrigeration, people commonly preserved food by salting, smoking or pickling. But these foods often contain large amounts of nitrites and nitrates, which can be converted in your stomach into cancer-causing compounds. Countries where consumption of salted meat and fish and pickled vegetables is high — Japan and Korea are notable examples — tend to have correspondingly high rates of stomach cancer. Eating a diet high in red meat, especially when the meat is barbecued or well-done, also has been linked to stomach cancer.
• Tobacco and alcohol use. Tobacco use can irritate the stomach lining, which may help explain why smokers have twice the rate of stomach cancer that nonsmokers do. Alcohol has been associated with an increased risk of stomach cancer, but the link between the two isn’t clear.

Risk factors

Having H. pylori infection makes you more likely to develop stomach cancer than someone who doesn’t have the infection. Even so, most people with H. pylori don’t get stomach cancer, and researchers believe that genetic factors make some people more susceptible to the disease.

Other risk factors for stomach cancer include:

• Your sex. Men have double the rate of stomach cancer that women do.
• Age. Most people who develop stomach cancer are older than 50 years.
• Diet. A diet high in foods preserved by smoking, salting or pickling increases your risk of stomach cancer. So do foods that contain nitrites and nitrates, such as bacon, ham and processed meats. Eating large amounts of red meat — particularly if it’s barbecued or well-done — also increases your risk. On the other hand, consuming plenty of fruits and vegetables, especially those that are red or deep yellow, such as tomatoes, carrots and sweet potatoes, helps protect against stomach cancer.
• Tobacco use. Smokers have twice the incidence of stomach cancers that nonsmokers do.
• Previous stomach surgery. The risk of stomach cancer may increase in people who have had part of their stomach and the opening to the small intestine (pyloric valve) removed — usually as a treatment for peptic ulcers. After stomach surgery, bile and sometimes pancreatic juices can back up, causing irritation and inflammation of the stomach lining (gastritis). In addition, the amount of protective stomach acid decreases while nitrite-producing bacteria may increase. These factors can lead to stomach cancer in some people. In general, the risk is greatest for the first 20 years after the initial surgery.
• Stomach polyps. These are small growths in the lining of your stomach. Most are noncancerous (benign), but adenomatous polyps may be precancerous.
• Familial cancer syndromes. These include hereditary nonpolyposis colon cancer and familial adenomatous polyposis, inherited disorders that slightly increase your risk of stomach cancer. People who carry mutations in the BRCA1 and BRCA2 genes also have an increased stomach cancer risk. These mutations were previously thought to be associated only with breast and ovarian cancers.
• Family history. You’re more likely to develop stomach cancer if you have a parent or sibling with the disease.
• Pernicious anemia. This condition, which is often associated with atrophic gastritis, develops when your stomach is no longer able to make a protein called intrinsic factor that helps your body absorb vitamin B-12. Although pernicious anemia is easily treated with B-12 injections, having the disease can slightly increase your risk of stomach cancer.
• Type A blood. Your blood type is determined by the presence or absence of two proteins — A and B — that occur on red blood cells. For reasons that aren’t clear, people with type A blood have a somewhat higher risk of stomach cancer than do people with other blood types.
• Country of origin. Stomach cancer is more common in some parts of the world — especially Japan, Korea, parts of Eastern Europe, and Latin America — than it is in the United States. These differences are likely related to diet and H. pylori infection. Stomach cancer occurs most often in countries where large amounts of meat or smoked, heavily salted or pickled foods are consumed, or where there is a lack of refrigeration as a means to preserve food.
• Obesity. Extra weight has been associated with an increased risk of some cancers, including stomach cancer.

When to seek medical advice

Stomach cancer is treatable if caught early. Unfortunately, it rarely causes symptoms in the beginning stages. When symptoms do occur, they’re often vague and can easily be mistaken for other, more common but less serious problems such as a stomach virus or heartburn. Less than one in five stomach cancers are diagnosed before they have spread outside the stomach.

See your doctor if you have a persistent feeling of discomfort in the upper or middle region of your abdomen, especially if it occurs in conjunction with fatigue and weight loss.

And see your doctor right away if you develop black, tarry stools or if you vomit after meals. Although not always indicators of stomach cancer, these signs may result from other conditions that require medical care.

Screening and diagnosis

To help diagnose stomach cancer and rule out other possibilities, your doctor may recommend one or more of the following diagnostic tests:

• Upper endoscopy. This procedure allows your doctor to see abnormalities in your upper gastrointestinal (GI) tract that may not be visible on X-rays. For the test, your doctor inserts a thin, flexible, lighted tube (endoscope) through your mouth and into your esophagus, stomach and the first part of your small intestine. Your throat is usually numbed before you’re asked to swallow the endoscope, and you’ll usually receive intravenous medication to ensure that you’re comfortable during the procedure.If any tissue in your upper intestinal tract looks suspicious, your doctor can remove a small sample (biopsy) using instruments inserted through the endoscope. The sample is then sent to a lab for examination by a pathologist.

  Upper endoscopy takes about 20 to 30 minutes, although you won’t be sent home until the medication wears off — usually one to two hours later. Risks of the procedure are rare and include bleeding and perforation of the stomach lining. The most common complication is a slight sore throat from swallowing the endoscope.

• Stomach X-ray (barium upper GI series). This test uses a series of X-rays to examine your esophagus, your stomach and the first part of your small intestine.Before the test, you’ll drink a thick liquid (barium) that temporarily coats the lining of your stomach so that it shows up clearly on the X-rays. You may also be asked to swallow a gas-producing liquid or pill, such as sodium bicarbonate, which stretches the stomach and separates its folds, thereby providing a better view of the inner lining.

  After the test you can eat normally and resume your usual activities, although you’ll need to drink extra water to help flush the barium from your system. The most common complication of the procedure is temporary constipation.

If you receive a diagnosis of stomach cancer, your doctor is likely to recommend additional tests to help determine the extent of the disease (staging tests) and the best course of treatment. These may include:

• Endoscopic ultrasound. This test helps determine whether cancer has spread into the walls of your stomach or to nearby tissues and lymph nodes. Endoscopic ultrasound is similar to upper endoscopy, but in this case, the endoscope carries a small ultrasound probe that uses high-frequency sound waves to create images of your stomach and surrounding tissues, including lymph nodes.
• Computerized tomography (CT) scan. Used to help check for the spread of cancer outside your stomach — especially to organs such as your liver and lungs — this test uses split-second computer processing and X-ray beams to produce detailed cross-sectional images of your internal organs. A CT scan exposes you to more radiation than conventional X-rays do, but in most cases, the benefits outweigh the risks.
• Magnetic resonance imaging (MRI). This test also looks for the spread of cancer beyond your stomach. But unlike a CT scan, MRI uses a powerful magnetic field and radio waves — not X-rays — to produce cross-sectional images of your body.
• Chest X-ray. This test checks whether cancer has spread to your lungs but isn’t as sensitive as a CT scan.

Treatment

The kind of treatment you receive for stomach cancer depends on a number of factors, including the location of the cancer, how advanced it is, your overall health and your own preferences. Especially when cancer is advanced, choosing a treatment plan is a major decision, and it’s important to take time to evaluate your choices.

You may also want to consider seeking a second opinion. This can provide additional information to help you feel more certain about the option you’re considering.

The goal of any treatment is always to eliminate the cancer completely. When that isn’t possible, the focus may be on preventing the tumor from growing or causing more harm. In some cases, an approach called palliative care may be best. 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.

Stomach cancer treatment options include the following:

• Surgery. This is the most common treatment for stomach cancer. Depending on the extent of the cancer, your doctor may remove part (subtotal, or partial, gastrectomy) or all (total gastrectomy) of your stomach as well as some of the surrounding tissue. Lymph nodes near the tumor also are often removed during surgery. After a subtotal gastrectomy, the remaining part of your stomach is connected to your esophagus and your small intestine. If your entire stomach is removed, your surgeon attaches your esophagus directly to your small intestine. A 2006 study suggested that chemotherapy before and after surgery may improve outcomes for certain people, so discuss this with your doctor.When stomach cancer is caught at an early stage and your surgeon is able to remove the entire tumor, a complete recovery is possible. Unfortunately, diagnosis usually doesn’t occur until stomach cancer has spread through the stomach wall to nearby lymph nodes or other organs. At this point, it’s not possible to remove all the cancer surgically, but your doctor may still recommend an operation to alleviate pain, bleeding or obstruction. In some cases of advanced stomach cancer, a laser beam directed through an endoscope can vaporize most of the tumor and relieve obstruction without an operation.

  After gastrectomy, some people experience leakage or obstruction where the intestinal tract has been reconstructed. More common problems associated with partial or total gastrectomy include diarrhea, vomiting and dumping syndrome, which occurs when the small intestine fills too quickly with undigested food. Signs and symptoms of dumping may occur immediately after eating (early dumping) or several hours after a meal (late dumping) and include nausea, vomiting, diarrhea, cramping and dizziness.

• Chemotherapy. This treatment uses drugs to help kill cancer cells. Injected into a vein or taken orally, chemotherapy medications travel through your bloodstream and are often used to eliminate cancer cells that may remain after surgery or to treat cancers that have spread to other parts of the body. Chemotherapy may also be used to control cancer growth, prolong life or relieve symptoms of advanced disease. Although it sometimes may be the only treatment needed, doctors most often use chemotherapy in conjunction with other therapies. For example, in locally advanced stomach cancer, which occurs when the tumor affects only the stomach and nearby tissues, chemotherapy and radiation (radiotherapy) may be offered after surgery to help increase survival and improve quality of life.A 2006 study found that people whose stomach cancer hadn’t spread had better outcomes when chemotherapy was used both before and after surgery to remove the cancer. In the study, people with stomach cancer were randomly assigned to receive surgery alone or to receive three cycles of chemotherapy before surgery and three cycles of chemotherapy after recovering from surgery. People in this study who received surgery combined with chemotherapy lived somewhat longer and had a lower risk of the cancer returning than did people who received surgery only.

  Because anti-cancer drugs affect healthy cells as well as cancerous ones — especially fast-growing cells in your digestive tract and bone marrow — side effects such as nausea and vomiting, fatigue, and an increased risk of infection due to a shortage of white blood cells are common. Although not everyone experiences these side effects, chemotherapy can sometimes feel like another illness and is often the part of treatment about which people are most apprehensive. But newer anti-nausea medications can prevent or reduce most nausea. Sometimes acupuncture or relaxation techniques, such as guided imagery, meditation and deep breathing, also may help control nausea and vomiting. Chemotherapy is normally administered in cycles, with periods of treatment alternating with periods of recovery during which your body can recover. Ask your treatment team about the side effects of any treatment you’re considering and the best ways to minimize those effects.

• Radiation therapy (radiotherapy). This therapy uses high-energy X-rays to kill cancer cells. Unlike chemotherapy, which affects your entire body, radiation affects only those parts of your body through which the radiation beam passes. Because any tissue touched by radiation can be damaged, doctors are careful to aim the beam in a way that’s least likely to harm healthy tissue. Radiation that comes from a machine outside your body (external beam radiation) is generally used to treat stomach cancer, especially in conjunction with chemotherapy. It may also help relieve pain and blockages. Side effects may include a burn similar to sunburn on your skin where the radiation enters your body, nausea, vomiting, and fatigue that may increase over the course of treatment.
• Antibiotics. Carefully selected individuals who have H. pylori-associated gastric lymphomas may be cured by antibiotic therapy that eliminates the bacteria causing this cancer. If you have this type of cancer, your doctor will need to carefully monitor your condition for recurrence or for the presence of more advanced disease that requires more aggressive therapy.

Targeted drug therapy
The Food and Drug Administration has approved the anti-leukemia drug imatinib mesylate (Gleevec) to treat gastrointestinal stromal tumors (GISTs) that contain a specific genetic mutation. Imatinib, which is taken in capsule form once a day, belongs to a class of medications that target a specific genetic mutation within cancer cells but leave healthy tissue relatively untouched. Side effects tend to be milder than with other types of cancer therapy and include fluid retention (edema), nausea, muscle cramps and rash.

Imatinib is effective only for GISTs that have a specific genetic mutation and even then, it isn’t for everyone — surgical removal remains the primary therapy for this type of cancer. Despite promising results in some people, imatinib isn’t effective in all cases nor are the long-term effects known.

Clinical trials
If you have advanced stomach cancer, you may want to consider participating in a clinical trial. This is a study that’s used to test new forms of therapy — typically new drugs, different approaches to surgery or radiation treatments, and novel methods such as gene therapy. If the therapy being tested proves to be safer or more effective than current treatments, it will become the new standard of care.

The treatments used in clinical trials haven’t yet proved effective. They may have serious or unexpected side effects, and there’s no guarantee you’ll benefit from them.

On the other hand, cancer clinical trials are closely monitored by the federal government to ensure they’re conducted as safely as possible. And they offer access to treatments that wouldn’t otherwise be available to you.

If you’re interested in finding out more about clinical trials, talk to your treatment team. You can also call the National Cancer Institute’s Cancer Information Service at 800-4-CANCER, or 800-422-6237. The call is free and trained specialists are available to answer your questions.

Prevention

Although it may not be possible to prevent stomach cancer, the following steps can help reduce your risk:

• Emphasize fruits and vegetables. A diet rich in fresh fruits and vegetables, especially those high in vitamin C and beta carotene, has been shown to help protect against stomach cancer. Look for deep green and dark yellow or orange fruits and vegetables, such as Swiss chard, bok choy, spinach, cantaloupe, mango, acorn or butternut squash, and sweet potatoes. Also try to eat vegetables from the cabbage family, including broccoli, brussels sprouts and cauliflower. Lycopene, a nutrient found in tomatoes and other red fruits and vegetables such as strawberries and red bell peppers, may be a particularly powerful anti-cancer chemical.
• Avoid nitrites and nitrates. These nitrogen compounds are known to contribute to stomach cancer. They’re found primarily in processed meats — bologna, salami and corned beef, for instance — and in cured meats such as ham and bacon.
• Limit smoked, pickled and heavily salted foods. These have been linked to an increased risk of stomach cancer. Countries where the consumption of smoked, pickled and salted food is high have correspondingly high stomach cancer rates.
• Don’t smoke. Tobacco use greatly increases your risk of stomach cancer, especially cancer that occurs at the junction of the esophagus and stomach.
• Limit alcohol consumption. Alcohol may cause changes in cells that can lead to cancer.
• Limit red meat. Eating large amounts of red meat — particularly when it’s barbecued or well-done — increases your risk of stomach cancer. Instead, choose fish or poultry.
• See your doctor if you have symptoms of an ulcer. Infection with H. pylori, the bacterium that causes most cases of gastric ulcers, is one of the leading causes of stomach cancer. Don’t ignore symptoms of ulcers, such as a gnawing pain in your abdomen or chest that’s worse when your stomach is empty or at night. Other, more severe signs and symptoms of ulcers include nausea, vomiting, bleeding and unintended weight loss.

Self-care

After gastrectomy, it’s not uncommon to experience nausea, vomiting, diarrhea, weight loss, nutritional deficiencies and dumping syndrome, which occurs when food enters your small intestine too quickly. These side effects usually result from eating more at one time than your digestive system can tolerate and can often be controlled with changes in the amount, frequency and kinds of food you eat.

You may also develop nutritional deficiencies because you’re no longer able to absorb certain vitamins. Vitamin B-12, for example, can only be absorbed when it’s attached to a protein produced in your stomach (intrinsic factor). For that reason, you’ll need to receive vitamin B-12 injections for life. Your doctor may also recommend supplementing your diet with other nutrients, especially folic acid, iron and calcium.

Although coping with the effects of gastrectomy can be challenging, the following measures may help improve or relieve your symptoms:

• Eat small, frequent meals. Normally, your stomach can expand to accommodate what you eat and drink, which it then releases slowly into your small intestine. After gastrectomy, you won’t be able to eat as much at one sitting as you once did, although you likely can consume the same amount overall. To get the calories you need while minimizing intestinal symptoms, try eating six small meals a day, rather than two or three large ones.
• Avoid drinking with meals. Although it’s important to drink plenty of fluids, especially water, drinking with meals hastens the movement of food through the upper part of your digestive tract and may reduce the absorption of nutrients. Instead, try to drink 30 minutes before or 60 minutes after you eat.
• Eat slowly and chew thoroughly. This can help reduce nausea and vomiting and increase your ability to absorb nutrients.
• Avoid extremely hot or cold foods or liquids. These may aggravate your symptoms.
• Rest after meals. It’s best to relax after you eat because activity increases the likelihood of nausea and vomiting. Don’t lie flat, however, for at least two to three hours after a meal.
• Avoid sugar. All forms of sugars and sweets aggravate dumping syndrome.
• Use dairy products cautiously. Some people find that dairy foods such as milk, cheese and even yogurt cause gas, bloating and abdominal pain. In that case, an enzyme product such as Lactaid or Dairy Ease may help break down lactose. Consuming small amounts of milk products or combining them with other foods to slow digestion also may help. In some cases, though, you may need to eliminate dairy foods completely. If so, be sure to get enough protein, calcium and B vitamins from other sources.
• Avoid troublesome foods. If certain foods make your symptoms worse, don’t eat them. Instead, try eating softly cooked or pureed fruits and vegetables, rice, plain baked potatoes, soups, broth, and chicken or fish cooked without fat.
• Talk to a dietitian. It’s difficult to get the calories and nutrition you need on a restricted diet. A dietitian can help you plan healthy meals that don’t aggravate your symptoms.

Coping skills

A diagnosis of cancer can be extremely challenging. Remember that no matter what your concerns or prognosis, you’re not alone. These strategies and resources may make dealing with cancer easier:

• Know what to expect. Find out everything you can about your cancer — the type, stage, your treatment options and their side effects. The more you know, the more active you can be in your own care. In addition to talking with your doctor, look for information in your local library and on the Internet. Representatives from the National Cancer Institute will answer questions from the public. You can reach them at 800-4-CANCER, or 800-422-6237. Or contact the American Cancer Society at 800-ACS-2345, or 800-227-2345.
• Be proactive. Although you may feel tired and discouraged, don’t let others — including your family or your doctor — make important decisions for you. Take an active role in your treatment.
• Maintain a strong support system. Having a support system can help you cope with any issues, pain and anxieties that might occur. Although friends and family can be your best allies, they sometimes may have trouble dealing with your illness. If so, the concern and understanding of a formal support group or others coping with cancer can be especially helpful. Although support groups aren’t for everyone, they can be a good source for practical information. You may also find you develop deep and lasting bonds with people who are going through the same things you are.
• Set reasonable goals. Having goals helps you feel in control and can give you a sense of purpose. But don’t choose goals you can’t possibly reach. You may not be able to work a 40-hour week, for example, but you may be able to work part time. In fact, many people find that continuing to work can be helpful.
• Take time for yourself. Eating well, relaxing and getting enough rest can help combat the stress and fatigue of cancer. Also, plan ahead for the downtimes when you may need to rest more or limit what you do.
• Stay active. Being diagnosed with cancer doesn’t mean you have to stop doing the things you enjoy or normally do. For the most part, if you feel well enough to do something, go ahead and do it. Stay involved as much as you can.

Talking about end-of-life issues
Although it can be extremely difficult, discuss end-of-life issues with your family and medical team. Part of this discussion will likely involve advance directives — a general term for verbal and written instructions you give concerning your medical care should you become unable to speak for yourself.

One type of advance directive is known as a durable power of attorney (POA) for health care. In this case, you sign a legal document authorizing a person you respect and trust to make legally binding medical decisions for you if you’re unable to do so. A POA is often recommended because the appointed person can make decisions in situations not covered in a regular advance directive. Whatever you decide, put your wishes in writing. Laws regarding advance directives and POAs vary from state to state, but a written document is more likely to be respected.

Coming to terms with your illness
Having a serious illness may be the hardest thing you’ve ever dealt with. 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.

In fact, the greatest fears of many people with a life-threatening illness include being subjected to treatments they don’t want, becoming a burden to their loved ones, and spending their last weeks or months in a hospital away from familiar surroundings. The welcome news is that many more choices now exist for people with a terminal illness.

Hospice care, for example, provides a special course of treatment to terminally ill people. This allows family and friends — with the aid of nurses, social workers and trained volunteers — to care for and comfort a loved one at home or in a hospice residence. It also provides emotional, social and spiritual support for people who are ill and those closest to them. Although most people under hospice care remain in their own homes, the program is available anywhere — including nursing homes and assisted living centers. For those who stay in a hospital, palliative care specialists can provide comfort, compassionate care and dignity.