The Esophagus

The esophagus is a muscular tube that connects the mouth to the stomach. It carries food and liquids to the stomach. It is about 10-13 inches long. At its smallest point, it is a little less than one inch wide. The wall of the esophagus has several layers. Cancer of the esophagus starts from the inner layer and grows outward. At the top of the esophagus is a special muscle that relaxes to let food or liquid enter. This muscle is called a sphincter . The lower part of the esophagus connects to the stomach. Another muscle here opens to allow food to enter the stomach. This muscle also keeps stomach acid and juices out of the esophagus. When stomach juices escape into the esophagus, we feel heartburn. The medical term for heartburn is reflux. Long-term reflux of stomach acid into the esophagus sets up a special problem. It can change the cells in the lower end of the esophagus. They become more like the cells that line the stomach. These changed cells produce a condition called Barrett's esophagus . The altered cells can change into cancer, so they should be closely watched by a doctor.

The stomach and esophagus are part of the upper digestive system.

Esophageal Cancer

There are two main types of cancer of the esophagus. One type grows in the cells that form the top layer of the lining of the esophagus. These are called squamous cells, and that cancer is known as squamous cell carcinoma. Squamous cell cancer can grow anywhere along the length of the esophagus. It accounts for about half of all cancers of the esophagus. The other type usually starts near the opening to the stomach. It is called adenocarcinoma. This type of cancer cannot start unless squamous cells have been changed by acid reflux.

In the United States, the American Cancer Society estimates that there will be about 16,470 new cases of this cancer in 2008. About 14,280 people will die of the disease. This cancer is 3 to 4 times more common among men than among women and 50% more common among African Americans than among whites. Some countries such as Iran, northern China, India, and southern Africa have rates that are 10-100 times higher than that of the United States.

Risk Factors

While we don't know the exact cause of esophageal cancer, we do know some of the risk factors that make this cancer more likely. A risk factor is something that increases a person's chance of getting cancer. Some risk factors, such as smoking, can be controlled. Others, like a person's age or race, can't be changed. The risk of this cancer goes up with age. It is rarely found in people under 40. Men are three times more likely to get this cancer than women. African Americans are 50% more likely to develop cancer of the esophagus than whites. Barrett's esophagus occurs because of long-term reflux of fluid from the stomach into the lower esophagus. Some people feel heartburn from this reflux and others do not. Over time this reflux can change the cells at the end of the esophagus. This raises the risk of adenocarcinoma of the esophagus. Not everyone with Barrett's esophagus will get cancer of the esophagus. Long-standing heartburn, also called reflux (or GERD: gastroesophageal reflux disease), increases the risk of this cancer even if it has not progressed to Barrett's esophagus. Using any form of tobacco raises the risk of this cancer. The longer a person uses tobacco, the greater the risk. More than half of all squamous cell esophageal cancer is linked to smoking. Long-term heavy drinking is a major risk factor for cancer of the esophagus. For people who both smoke and drink, the risk is further increased. The risk of this cancer is higher for people who are overweight or obese. Diets that do not include enough fruits or vegetables and certain minerals and vitamins can raise the risk of this cancer. A diet with plenty of green and yellow fruits and vegetables and cruciferous vegetables (such as cabbage, broccoli, and cauliflower) may lower the risk of developing squamous cell cancer of the esophagus. Overeating, which leads to being overweight, also raises the risk of this cancer. Drinking lots of very hot liquids might increase the risk of this cancer. Working with a chemical used in dry cleaning may lead to a greater risk of this cancer. Breathing in other chemical fumes may also increase the risk. Lye is found in strong cleaners like drain cleaners. It can burn and destroy cells. If someone accidentally drinks one of these cleaning liquids, the lining of the esophagus will scar. A person who has swallowed lye has a higher risk of squamous cell esophageal cancer because the muscle at the bottom of the esophagus does not open to release food into the stomach. In that case the lower end of the esophagus expands, and food collects there instead of moving into the stomach. This raises the risk for squamous cell cancer. Tylosis is a rare, inherited disease that causes extra skin to grow on the palms of the hands and soles of the feet. People with tylosis are at a very high risk for esophageal cancer. Infection with the Helicobacter pylori bacteria causes inflammation and ulcers in the stomach lining, which may lead to a condition called gastric atrophy ( cells that line the stomach are destroyed). This condition may increase the risk of developing squamous cell cancer of the esophagus. Other risk factors for developing esophageal cancer include: being malnourished (lacking nutrients and/or calories ), or being infected with human papillomavirus , or having achalasia. Achalasia is a disease that involves abnormal esophageal peristalsis (the muscular milking action of the esophagus that moves fluids and food downward into the stomach).

Prevention

At this time there is no sure way to prevent cancer of the esophagus. The risk of getting the disease can be lowered by avoiding tobacco and too much alcohol. Eating fruits and vegetables, especially raw, may offer some protection. Staying active and keeping a healthy weight can also help. Some studies have found that the risk can be lowered in people who take aspirin or other drugs such as ibuprofen that reduce inflammation. You should talk to your doctor about the pros and cons of taking these drugs. If you have a high risk of cancer of the esophagus, talk to your doctor about how often you should be seen and what tests you should have. People with Barrett's esophagus and others at high risk should have exams to look for cancer of the esophagus. The doctor might recommend surgery (or other methods) if certain abnormal cells (dysplasia) are found.

Symptoms

Trouble with swallowing (dysphagia). This is the most common symptom of cancer of the esophagus. It means that you feel as if food is lodged in the chest. By this time, if cancer is present it has grown to fill about half the opening of the esophagus. Solid foods like bread and meat often get stuck. People with dysphagia often switch to softer foods or even liquids to help with swallowing. To help the food go down, the body makes more saliva. This causes some people to bring up lots of thick mucus or saliva. Pain when swallowing, or pain in the mid-chest, or a feeling of pressure or burning can be a sign of cancer. But these symptoms can also be caused by something else, such as heartburn. About half of people with esophageal cancer lose weight without trying. This is because they are not getting enough food since they have trouble swallowing. Also, they may find they have less appetite. Of course these symptoms can be caused by other diseases as well.

Screening

Screening is looking for cancer before a person has any symptoms . This can help find cancer at an early stage . When abnormal tissue or cancer is found early, it may be easier to treat. Screening for esophageal cancer is under study. Screening may include endoscopy, biopsies, brush cytology (a procedure in which cells are brushed from the lining of the esophagus and viewed under a microscope to see if they are abnormal), balloon cytology (a procedure in which cells are collected from the lining of the esophagus using a deflated balloon that is swallowed by the patient), chromoendoscopy (a procedure in which a dye is sprayed onto the lining of the esophagus during esophagoscopy to detect early Barrett's esophagus by staining the lining of the esophagus), f luorescence spectroscopy (a procedure that uses a special light to view tissue in the lining of the esophagus),

Risks of Esophageal Cancer Screening

Screening test results may appear to be normal even though esophageal cancer is present (a false negative test result). A person who receives a false-negative test result may delay seeking subsequent medical care even if symptoms are worsening. Screening test results may appear to be abnormal even though no cancer is present (a false positive test result). A false-positive test result can cause anxiety and is usually followed by more tests (such as biopsy ), which also have risks.

Esophagoscopy. A thin, lighted tube is inserted through the mouth and into the esophagus to look for abnormal areas.

Tests

Barium swallow or upper gastrointestinal x-rays are taken after the patient swallows barium, a dense liquid. Barium coats the surface of the esophagus and helps create a good picture. Any lumps on the lining of the esophagus show up on the x-ray. A barium swallow is often the first test to be done in people who have trouble swallowing. Upper gastrointestinal endoscopy uses a flexible tube with a light and video camera on the end (endoscope). The doctor uses this to look at the esophagus and the stomach. The patient is given a sedative before the tube is passed down through the mouth and esophagus into the stomach. This test is useful because: 1) the doctor can see the esophagus clearly, and 2) a tissue sample can be taken to find out if there is cancer and if so, what type it is, and 3) if the cancer is blocking the opening of the esophagus, the opening can be made bigger, and 4) the doctor can learn more about whether the cancer can be removed with surgery. A CT (computed tomography) scan is a type of x-ray that takes many pictures. These pictures are combined by a computer to give a detailed view of organs inside the body. CT scans take longer than regular x-rays and the patient has to lie still on a table while the pictures are being done. A CT scan can be helpful in finding out the extent of the cancer, which in turn can help guide choices about surgery. CT scans can also be used to guide a biopsy needle into an area that might be cancer. The needle is used to remove a sample of tissue for study in the lab. Endoscopic ultrasonography uses an endoscope with a small ultrasound (sound wave) probe attached. It is like the test that doctors use to take pictures of the fetus during pregnancy. The ultrasound can show how far the cancer has grown into the esophagus to help in making choices about surgery. Recent studies suggest that it might be more accurate than CT scans or upper endoscopy. Bronchoscopy is somewhat like an endoscopy except that the doctor looks into the windpipe and the tubes leading into the lungs to see if the cancer has spread there. The patient will be made drowsy for this test. A PET scan (positron emission tomography) uses a special radioactive sugar that is injected into a vein. The tissues with cancer quickly take up the sugar. This test is useful for finding cancer that has spread to nearby lymph nodes or other places in the body. Thoracoscopy and laparoscopy are methods that allow the doctor to see lymph nodes inside the chest or abdomen with a hollow lighted tube. The doctor can also remove lymph nodes for testing through the same tube. This information is helpful in telling whether surgery is a good option. A biopsy (the removal of a sample of tissue) is the most important test. The biopsied tissue is looked at under the microscope in order to see if cancer is present and to determine the type of cancer.

Staging

Staging is the process of finding out how far cancer has spread. This is very important because your treatment and the outlook for your recovery depend on the stage of your cancer. The three ways that cancer spreads in the body are: 1) cancer invades the surrounding normal tissue, and/or 2) cancer invades the lymph system and travels through the lymph vessels to other places in the body, and/or 3) cancer invades the veins and capillaries and travels through the blood to other places in the body. When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. Esophageal cancer is staged using the results of the tests described above. Stages are often labeled using Roman numerals 0 through IV (0-4). In general, the lower the number, the less the cancer has spread. A higher number, such as stage IV (4), means a more serious cancer. After looking at your test results, the doctor will tell you the stage of your cancer. Be sure to ask your doctor to explain your stage in a way you understand. This will help you both decide on the best treatment for you. After the cancer is found and staged, your doctor will talk to you about a treatment plan. There is a lot for you to think about when choosing the best way to treat or manage your cancer. There may be more than one treatment to choose from.

As esophageal cancer progresses from Stage 0 to Stage IV, the cancer cells grow through the layers of the esophagus wall and spread to lymph nodes and other organs.

Treatment

You may feel that you need to make a decision quickly. But give yourself time to absorb the information you have learned. Talk to your doctor. Patients often have special nutritional needs during treatment for esophageal cancer. Some patients may receive nutrients directly into a vein. Others may need a feeding tube (a flexible plastic tube that is passed through the nose or mouth into the stomach) until they are able to eat on their own.

Surgery There are two common types of operations for this cancer. With esophagectomy the part of the esophagus with cancer and nearby lymph nodes are removed. The esophagus is attached either to the stomach or the surgeon may replace the removed part of the esophagus with a piece of the small or large intestine. The other surgery, esophagogastrectomy , is done to remove part of the lower esophagus, nearby lymph nodes, and the upper part of the stomach. The esophagus is again connected to the remaining part of the stomach. Surgery can cure some patients whose cancer has not spread beyond the esophagus. It’s important to know the goal of surgery: is it to try to cure the cancer or to ease symptoms? Some surgeons are able to do the operation using a laparoscopic approach. This means that small cuts are made through which the doctor uses a tiny instrument something like a telescope to look inside the body. The surgery itself is done through even smaller incisions.

Radiation Therapy Radiation therapy is treatment with high energy rays (such as x-rays) to kill or shrink cancer cells. External radiation uses a beam from outside the body. This is the kind most often used for cancer of the esophagus. For internal or implant radiation, radioactive “seeds” are placed directly inside the body near the cancer. Radiation therapy by itself will not cure esophageal cancer. Often it is combined with surgery and/or chemotherapy. It is also used to relieve problems with swallowing, pain, or other symptoms of this cancer.

Chemotherapy Chemotherapy refers to the use of drugs to kill cancer cells. Usually the drugs are given into a vein or by mouth. Once the drugs enter the bloodstream, they spread throughout the body. Chemotherapy alone cannot cure cancer of the esophagus unless radiation treatment (and in some cases surgery) are also used. Chemotherapy is used in three ways: 1) it can be used to ease the symptoms of advanced cancer, and 2) it can be given along with radiation to shrink the cancer, and 3) it can be used before surgery to shrink a tumor.

Photodynamic therapy (PDT) Photodynamic therapy (PDT) may be used when the cancer has been found very early or has come back after other treatment. First, a harmless chemical is injected into the bloodstream. It collects in the tumor for a few days. Then a special type of laser light is focused on the cancer through an endoscope. The light changes the chemical into a new chemical that can kill cancer cells. PDT is useful because it can kill cancer cells with very little harm to normal cells. But because the light must be used, it can reach only cancers near the surface of the esophagus. It doesn’t work for cancers that have spread deeper or into other organs. Side effects of PDT include redness or discoloration of the skin and sensitivity to the sun or other light sources. Because of this, people having this treatment may be told to stay indoors for 6 weeks. At this time, the main use of PDT is to relieve symptoms of advanced cancer that are blocking the esophagus. It is also being used to treat Barrett esophagus and very early cancers found in Barrett esophagus. Researchers are looking at the use of PDT in comparison to other treatments such as surgery or the use of lasers.

Stenting If the esophagus is partly blocked by the tumor, an expandable metal stent (tube) may be placed inside the esophagus to help keep it open.

Esophageal stent. A device (stent) is placed in the esophagus to keep it open to allow food and liquids to pass through into the stomach.

Laser Therapy Laser therapy is a cancer treatment that uses a laser beam (a narrow beam of intense light) to kill cancer cells.

Electrocoagulation Electrocoagulation is the use of an electric current to kill cancer cells.

Esophagectomy. A portion of the esophagus is removed and the stomach is pulled up and joined to the remaining esophagus.

Clinical Trials

Studies of promising new treatments are known as clinical trials. A clinical trial is done only when there is some reason to believe that the new treatment may be of value to the patient. Clinical trials are needed in order to find new and better ways to treat cancer. Treatments used in clinical trials are often found to have real benefits. Clinical trials are carried out in steps called phases. Each phase is designed to answer certain questions. Phase I clinical trials look at the best way to give a new treatment and how much of it can be given safely. The main purpose of a phase I study is to test the safety of the new drug. Phase II clinical trials are designed to see if the drug works. Patients are given the highest dose that does not cause serious side effects and then watched closely to see if there is an effect on the cancer. Phase III clinical trials compare the new treatment with standard treatment. Large numbers of patients are divided into two groups. The control group receives standard treatment and the other group receives the new treatment. Everyone is closely watched to see which treatment is more effective. The study is stopped if the side effects are too severe or if one group has much better results than the other. If you are in a clinical trial, you will have a team of experts watching your progress very carefully. However, there are some risks. No one knows in advance if the treatment will work or exactly what side effects will occur. That is what the study is designed to find out. Keep in mind, though, that even standard treatments have side effects. Taking part in a clinical trial is completely up to you. Even after joining a clinical trial, you are free to drop out of the study at any time, for any reason. Taking part in the study will not prevent you from getting other medical care you may need.

Complementary and Alternative Treatments

You may hear about different kinds of treatment from your family and friends. You can find people who will offer all sorts of things, such as vitamins, herbs, stress reduction, acupuncture, and more. There is a great deal of interest today in complementary and alternative treatments for cancer. Before changing your treatment or adding any of these methods, be sure to talk to your doctor or nurse. Some methods can be safely used along with standard medical treatment. Others, however, can interfere with standard treatment or cause serious side effects. That is why it's important to talk openly with your doctor.

Communications

You need to have honest, open discussions with your doctor. You should feel free to ask any question that's on your mind, no matter how small it might seem. Here are some questions you might want to ask:

1. Would you please write down the type of esophageal cancer I have?

2. Has my cancer spread beyond the esophagus?

3. What is the stage of my cancer and what does that mean in my case?

4. What treatment choices do I have?

5. What do you recommend? Why?

6. What are the risks or side effects that I should expect?

7. What are the chances my cancer will come back with this treatment plan?

8. Based on what you've learned about my cancer, how long will I survive?

9. What should I do to be ready for treatment?

Follow-up

Tests such as the upper GI, barium swallow and CT scans may be done to see if the cancer has come back or if you have a new tumor. It is important that you report any new symptoms to the doctor right away, especially if they include trouble swallowing or chest pain. Early treatment can relieve many symptoms and may improve your quality of life. Cancer of the esophagus often causes trouble with swallowing. For this reason, weight loss and weakness due to poor nutrition are common problems. Your doctor can help you find the information you need to cope with nutrition problems. If you have pain from your cancer, be sure to tell your doctor. There are many ways to control cancer pain. If you smoke, then it is very important to quit. Your appetite will improve and so will your overall health. And quitting helps reduce the chance of getting a new cancer. Remember that your body is unique, and so are your emotional needs and your personal circumstances. In some ways, your cancer is like no one else's. No one can predict how your cancer will respond to treatment. Statistics can paint an overall picture, but you may have special strengths such as a healthy immune system, a strong family support system, or a deep spiritual faith. All of these have an impact on how you cope with cancer. Your doctor or nurse can suggest other resources that might help you during your recovery from treatment. There are many support groups that provide emotional support, friendship, and understanding. If at any time you are having trouble coping, talk with your doctor, nurse, or social worker about your concerns. They may also suggest a therapist or mental health professional to help you.

Resources

National Coalition for Cancer Survivorship
Telephone: 1-877-NCCS-YES (1-877-622-7937)
Internet Address: www.canceradvocacy.org

National Cancer Institute
Telephone: 1-800-4-CANCER or 1-800-422-6237
TYY: 1-800-332-8615
Internet Address: www.cancer.gov

American Cancer Society
Telephone: 1·800·ACS·2345
Internet Address: www.cancer.gov

Updated: 7/30/08 by Robert F. Marschke, Jr., M.D.; Front Range Cancer Specialists