Esophageal Cancer

Source: American Cancer S0ciety

 

What is cancer of the esophagus?

The esophagus

The esophagus is a muscular tube that connects the throat to the stomach. It lies behind the windpipe (trachea) and in front of the spine and in adults is about 10-13 inches long. At its smallest point, it is a little less than one inch wide. It carries food and liquids to the stomach.

The wall of the esophagus has several layers. Cancer of the esophagus starts in the inner layer and grows outward into deeper layers.

In the lower part of the esophagus that connects to the stomach, a sphincter muscle opens to allow food to enter the stomach. This muscle also closes to keep stomach acid and juices from backing up into the esophagus. When stomach juices escape into the esophagus, it is called gastroesophageal reflux disease (GERD) or just reflux. In many cases, reflux can cause symptoms such as heartburn or a burning feeling spreading out from the middle of the chest. But sometimes, reflux can happen without any symptoms at all.

Long-term reflux of stomach acid into the esophagus can lead to problems. It can change the cells in the lower end of the esophagus. They become more like the cells that line the stomach. When these cells change, the person has a condition called Barrett’s esophagus. These altered cells can change into cancer, so the person has a much higher risk of cancer of the esophagus and should be closely watched by a doctor. Still, most people with Barrett’s esophagus do not go on to get cancer of the esophagus.

Esophageal cancer

There are 2 main types of cancer of the esophagus. One type grows in the cells that form the inside layer of the lining of the esophagus. These are called squamous cells, and cancer that starts there is called squamous cell carcinoma. Squamous cell cancer can grow anywhere along the length of the esophagus. It accounts for less than half of all cancers of the esophagus.

Cancers that start in gland cells are called adenocarcinomas. This type of cell is not normally part of the inner lining of the esophagus. Before an adenocarcinoma can develop, glandular cells must replace an area of squamous cells. This happens in Barrett’s esophagus, so these cancers are mainly in the lower esophagus.

Cancers that start at the place where the esophagus joins the stomach (called the GE junction) or the first part of the stomach tend to behave like esophagus cancers (and are treated like them, as well), so they are grouped with esophagus cancers.

 

What are the risk factors for cancer of the esophagus?

We don’t know the exact cause of esophageal cancer, but we do know some of the risk factors that make this cancer more likely. A risk factor is anything that affects a person’s chance of getting a disease like cancer. Some risk factors, such as smoking, can be controlled. Others, like a person’s age or race, can’t be changed.

But risk factors don’t tell us everything. Having a risk factor, or even several, does not mean that you will get the disease. Many people with risk factors never get esophagus cancer, while others with this disease may have few or no known risk factors.

Age

The risk of this cancer goes up with age. Less than 15% of cases are found in people younger than age 55.

Sex

Compared with women, men have a more than 3 times higher rate of this cancer.

Barrett’s esophagus

This is caused by long-term reflux of acid from the stomach into the lower esophagus. Most people with Barrett’s esophagus have had symptoms of “heartburn,” but many have no symptoms at all. Over time, reflux can change the cells in the esophagus. This raises the risk of adenocarcinoma of the esophagus. But not everyone with Barrett’s esophagus will get cancer of the esophagus.

Reflux

Reflux (or GERD: gastroesophageal reflux disease) of acid and gastric juices from the stomach into the esophagus can cause symptoms such as heartburn or pain that seem to come from the middle of the chest. In some cases though, reflux doesn’t cause any symptoms at all. GERD can cause Barrett’s esophagus, but it also increases the risk of this cancer even without Barrett’s esophagus. The risk goes up based on how long the reflux has been going on and how severe the symptoms are.

Tobacco and alcohol

Using any form of tobacco (cigarettes, cigars, pipes, chewing tobacco) raises the risk of this cancer. The longer a person uses tobacco, the greater the risk. The risk of esophageal cancer goes down if tobacco use stops.

Drinking alcohol also increases the risk of esophageal cancer. The chance of getting esophageal cancer goes up the more a person drinks.

Those who both smoke and drink alcohol raise their risk of esophageal cancer much more than using either alone.

Overweight

The risk of esophageal cancer is higher for people who are overweight or obese. This may be because people who are obese are more likely to have esophageal reflux.

Diet

A diet high in fruits and vegetables is linked to a lower risk of esophageal cancer. The exact reasons for this are not clear, but fruits and vegetables provide a number of vitamins and minerals that may help prevent cancer. It is also possible, although it has not yet been proven, that a diet high in processed meat may increase the risk of esophageal cancer. (Processed meats are things like deli meats, hot dogs, and bacon.)

Overeating, which leads to being overweight, also raises the risk.

Drinking a lot of very hot liquids might increase the risk of this cancer, too.

Achalasia

In this disease, the muscle at the bottom of the esophagus does not relax to release food into the stomach. So the lower end of the esophagus expands. Food collects there instead of moving into the stomach. Over time, this raises the risk for esophageal cancer.

Tylosis

This 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 also develop small growths (papillomas) in the esophagus and are at a very high risk for esophageal cancer. They should be seen by a doctor regularly to watch for this cancer. Often this means having upper endoscopies (described in “How is cancer of the esophagus found?“).

Esophageal webs

A web is an abnormal bulge of tissue that causes the esophagus to become narrow. Most esophageal webs do not cause any problems, but larger webs may cause food to get stuck in the esophagus, which can lead to problems swallowing. People who have these webs may have a syndrome (called Plummer-Vinson syndrome or Paterson-Kelly syndrome) that causes other symptoms, too, like problems with the tongue, fingernails, spleen, and other organs. About 1 in 10 people with this syndrome will get cancer of the esophagus.

Workplace exposure

Chemical fumes in certain workplaces may lead to an increased risk of esophageal cancer. Some studies have found that dry cleaning workers have a higher rate of cancer of the esophagus.

Injury to the esophagus

Lye is a chemical found in strong cleaners such as drain cleaners. Lye can burn and destroy cells. Sometimes small children mistakenly drink from a lye-based cleaner bottle. The lye causes a severe chemical burn in the esophagus. As the injury heals, the scar tissue can cause an area of the esophagus to become very narrow (called a stricture). People with these strictures have an increased rate of the squamous cell type of esophageal cancer as adults. The cancers occur on average about 40 years after the lye was swallowed.

Other cancers

People who have had certain other cancers such as lung cancer, mouth cancer, and throat cancer have a high risk of getting esophageal cancer, too. This may be because all of these cancers can be caused by smoking.

Human papilloma virus

Genes from human papilloma virus (HPV) have been found in up to one-third of esophagus cancer tumors from patients living in Asia and South Africa. Signs of HPV infection have not been found in esophagus cancers from patients living in the other places, including the US.

 

How is cancer of the esophagus found?

Looking for a disease in someone without symptoms is called screening. Screening the general public for esophageal cancer is not recommended at this time. This is because no screening test has been shown to lower the risk of dying from esophageal cancer in people who are at normal risk.

Testing for people at high risk

People at higher risk for esophageal cancer, such as those with Barrett’s esophagus, are often watched closely to look for signs that could mean that the cells lining the esophagus have changed. Many experts recommend that they have a test called upper endoscopy regularly (this test is discussed later in this section). Often, samples of tissue are removed (biopsies) and checked to see if they contain abnormal or even cancer cells. If they do, the patient may need to be treated. This is discussed in more detail in our document, Esophagus Cancer.

Signs and symptoms of cancer of the esophagus

In most cases, esophageal cancer is found because of the symptoms it causes. But often these symptoms don’t appear until the cancer is advanced, making a cure less likely. If esophagus cancer is suspected, tests will be needed to confirm the diagnosis.

Trouble swallowing (dysphagia)

This is the most common symptom of cancer of the esophagus. It means you feel like food gets stuck in your throat or chest. This is often mild when it starts, and then gets worse over time. Solid foods like bread and meat often get stuck. People with dysphagia may 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 have lots of thick mucus or saliva. If the cancer keeps growing, at some point even liquids will not be able to pass.

Chest pain

In some cases, 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. Swallowing may become painful when the cancer is large enough to limit the passage of food down the esophagus.

Weight loss

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. They may also find they don’t feel like eating.

Other symptoms

Other possible symptoms include hoarseness, constant cough, hiccups, pneumonia, bone pain, and bleeding into the esophagus, which can turn stools dark or black. Over time, this blood loss can lead to low red blood cell levels, which may make a person feel tired and weak.

These symptoms can be caused by other problems, too. Still, if you have any of these symptoms, especially trouble swallowing, have them checked by a doctor so that the cause can be found and treated, if needed.

If certain symptoms suggest that you may have esophagus cancer, your doctor will use one or more tests to find out if the disease is really present. After asking questions about your health and symptoms and doing a physical exam, your doctor will tell you which of the tests below you will need. You may also be referred to a gastroenterologist (a doctor who is an expert in diseases of digestive tract).

Imaging tests

Imaging tests use different methods to create pictures of the inside of your body. These tests may be done for a number of reasons both before and after a diagnosis of esophageal cancer

Barium swallow or upper GI x-rays

This is a series of x-rays taken after you swallow barium, a dense liquid that shows up on x-rays. Barium coats the surface of the esophagus and helps make 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.

CT scan (computed tomography)

A CT (or CAT) scan is a type of x-ray that takes many pictures of the part of your body being studied. These pictures are combined by a computer to give a detailed view of your insides.

A CT scanner has been described as a large donut, with a narrow table in the middle “hole”. You will need to lie still on the table while the scan is being done. CT scans take longer than regular x-rays, and you might feel a bit confined by the ring while the pictures are being taken.

Before any pictures are taken, you may be asked to drink 1 to 2 pints of a liquid dye. This helps outline the esophagus and intestines so that certain areas are not mistaken for tumors. If you are having any trouble swallowing, you need to tell your doctor before the scan. You may also get an IV (intravenous) line through which you get a different kind of contrast dye (IV contrast).

The dye can cause some redness and warm feeling that may last hours to days. A few people are allergic to the dye and get hives. Rarely, more serious problems like trouble breathing and low blood pressure can happen. You can be given medicine to prevent and treat allergic reactions. Be sure to tell your doctor if you have any allergies or have ever had such a reaction.

A CT scan can be helpful in finding out the where and how big the cancer is. This test can help the doctor decide whether surgery is a good treatment option. CT scans can also be used to guide a biopsy needle (see below) into a place that might be cancer. The needle is used to remove a sample of tissue for study in the lab.

MRI (magnetic resonance imaging) scan

MRI scans use radio waves and strong magnets instead of x-rays to take pictures. They are a little more uncomfortable than CT scans. First, MRI scans take longer – often up to an hour. Also, you have to be placed inside a narrow, tube-like machine, which can upset people who fear enclosed spaces. Special, more open MRI machines can sometimes help with this if needed, although the pictures may not be as sharp in some cases.

A contrast material might be put into a vein. This contrast is different than the one used for CT scans, so being allergic to one doesn’t mean you are allergic to the other. The MRI machine makes thumping and clicking noises. Some places provide earplugs to block this out. MRI scans are very helpful in looking at the brain and spinal cord.

PET scan (positron emission tomography)

For this test, a special radioactive sugar is put into a vein. The tissues with cancer quickly take up the sugar. Then a scanner can spot those areas. This test may be useful for finding cancer that has spread if nothing is found on other imaging tests. Special machines combine a PET scan with a CT scan.

Endoscopy

Endoscopy is an important test for finding esophageal cancer. An endoscope is a thin, tube that can bend. It has a light and video camera on the end. The doctor uses it to look at the inside of the esophagus and the stomach. Several tests that use endoscopes can help find esophageal cancer or show how much it has spread. If there are any areas of concern, a small piece of tissue can be removed through the tube to see if the area is cancer. (This is called a biopsy.)

Upper endoscopy

You will first be given drugs to make you sleepy (a sedative) and then the back of your throat will be sprayed with something to numb it. Then the tube is passed through your mouth and down your esophagus into your stomach.

This test is useful because:

  • The doctor can see the esophagus clearly.
  • A tissue sample can be taken to find out if there is cancer.
  • If the cancer is blocking the opening of the esophagus, the opening can be made bigger to help food and liquids pass through to the stomach.
  • The doctor can learn more about whether the cancer can be removed with surgery.

Endoscopic ultrasound

Ultrasound tests use sound waves to take pictures of parts of the body. For an endoscopic ultrasound, the probe that gives off the sound waves is at the end of an endoscope (see above). This allows the probe to get very close to the cancer. The ultrasound can show how far the cancer has grown into the esophagus to help in making choices about surgery. It can also be used to guide the doctor when getting biopsy samples of nearby lymph nodes.

Bronchoscopy

This test is much like an endoscopy except that the doctor passes the scope into the windpipe (trachea) and the tubes leading into the lungs to see if the cancer has spread there. The mouth and throat are sprayed first with a numbing medicine. You may also be given medicine into a vein line to make you feel relaxed. A biopsy sample might also be taken.

Thoracoscopy and laparoscopy

These are methods that allow the doctor to see lymph nodes and other organs inside the chest or belly (abdomen) using a hollow lighted tube with a small camera. The doctor can also remove lymph nodes through the same tube to test them for cancer. This information is helpful in telling whether surgery is a good option. For these tests the patient is in the hospital and is put into a deep sleep (general anesthesia). A small cut is then made in the side of the chest wall (for thoracoscopy) or the belly (for laparoscopy) to insert the tube.

Lab testing of biopsy samples

A spot seen on endoscopy or on an imaging test may look like cancer, but the only way to know for sure is to do a biopsy. For a biopsy, the doctor takes out a small piece of tissue from the area that looks like it could be cancer. The tissue is looked at under the microscope to see if cancer is present and to find out what type of cancer cells there are. It usually takes at least a few days to get the results.

If esophageal cancer is found but is too advanced for surgery, your doctor might have your biopsy samples tested for the HER2 gene or protein. Some people with esophageal cancer have too much of this gene or protein on the surface of their cancer cells, which helps the cells grow. But a drug that targets the HER2 protein may help treat these cancers when used along with chemotherapy.

Other tests

A doctor may order a blood test called a complete blood count (CBC) to look for anemia (which could be caused by bleeding inside the body). A stool sample may be checked to see if it contains unseen (occult) blood.

If esophageal cancer is found, the doctor may recommend other tests, especially if surgery may be an option.

 

How is cancer of the esophagus treated?

This information represents the views of the doctors and nurses serving on the American Cancer Society’s Cancer Information Database Editorial Board. These views are based on their interpretation of studies published in medical journals, as well as their own professional experience.

The treatment information in this document is not official policy of the Society and is not intended as medical advice to replace the expertise and judgment of your cancer care team. It is intended to help you and your family make informed decisions, together with your doctor.

Your doctor may have reasons for suggesting a treatment plan different from these general treatment options. Don’t hesitate to ask him or her questions about your treatment options.

About treatment

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. Give yourself time to think about the information you have been given.

You may want to get a second opinion. A second opinion can give you more information and help you feel good about the treatment you choose.

The main options for treatment of cancer of the esophagus include:

Other treatments may also be used for early cancers and pre-cancers of the esophagus. Some of these help relieve symptoms such as pain and blockage.

You may have different types of doctors on your treatment team. These doctors might include:

  • A thoracic surgeon: a doctor who treats diseases of the chest with surgery.
  • A radiation oncologist: a doctor who treats cancer with radiation.
  • A medical oncologist: a doctor who treats cancer with medicines such as chemotherapy.
  • A gastroenterologist: a doctor who specializes in treatment of diseases of the digestive system.

Many other specialists may be involved in your care as well.

 

Some questions to ask your doctor about cancer of the esophagus

As you cope with cancer and cancer treatment, you need to have honest, open talks 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. Be sure to add your own questions as you think of them. Nurses, social workers, and other members of the treatment team may also be able to answer many of your questions.

  • Would you please write down the type of esophagus cancer I have?
  • Has my cancer spread beyond the esophagus?
  • What is the stage of my cancer and what does that mean in my case?
  • Are there other tests that need to be done before we can decide on treatment?
  • Are there other doctors I need to see?
  • How often have you treated this type of cancer?
  • What treatment choices do I have?
  • What do you recommend? Why?
  • What is the goal of the treatment?
  • What are the chances my cancer can be cured with these options?
  • What are the risks or side effects that I should expect? How long are they likely to last?
  • How quickly do we need to decide on treatment?
  • What should I do to be ready for treatment?
  • Should I follow a special diet?
  • How long will treatment last? What will it involve? Where will it be done?
  • What are the chances my cancer will come back with this treatment plan?
  • What would we do if the treatment doesn’t work or if the cancer recurs?
  • What type of follow-up will I need after treatment?
  • Where can I get more information and support?

 

Moving on after treatment

For some people with esophagus cancer, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about cancer growing or coming back. (When cancer comes back after treatment, it is called recurrence.) This is a very common concern in people who have had cancer.

It may take a while before your fears lessen. But it may help to know that many cancer survivors have learned to live with this uncertainty and are living full lives. To learn more, see our document, Living With Uncertainty: The Fear of Cancer Recurrence.

For other people, the esophagus cancer may never go away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other treatments to help keep the cancer in check. Learning to live with cancer as an on-going (chronic) disease can be difficult and very stressful. It has its own type of uncertainty. Our document, When Cancer Doesn’t Go Away, talks more about this.

Follow-up care

If you have finished treatment, your doctors will still want to watch you closely. It is very important to keep all these follow-up visits. Your doctors will ask about symptoms, examine you, and may order blood tests, upper endoscopy, or imaging tests such as barium swallows or CT scans. These tests are described in the section, “How is cancer of the esophagus found?“) Follow-up is needed to check for cancer that has come back or spread, and to look for possible side effects of certain treatments.

Almost any cancer treatment can have side effects. Some may last for a few weeks or months, but others can last for the rest of your life. It is very important to 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 improve your quality of life. Use this time to ask your health care team questions and discuss any concerns you might have. You may also want to see our document, When Your Cancer Comes Back: Cancer Recurrence.

Help for trouble swallowing, nutrition, and pain

There are treatments aimed at helping to relieve the symptoms of esophagus cancer, rather than trying to cure the cancer (palliative treatments). In some cases they are used along with other treatments that focus on curing the cancer, but palliative treatments are often used in people with advanced cancer to help improve their quality of life.

Cancer of the esophagus often causes trouble swallowing. For this reason, weight loss and weakness due to poor nutrition are common problems. Your doctor and others can work with you to help you eat well and maintain your weight.

There are many ways to control pain caused by cancer of the esophagus. If you have pain, please tell your cancer care team right away, so they can make sure you get good relief.

Seeing a new doctor

At some point after your cancer is found and treated, you may find yourself seeing a new doctor who doesn’t know about your cancer. It is important that you be able to give your new doctor the exact details of your diagnosis and treatment. Make sure you have this information handy and always keep copies for yourself:

  • A copy of your pathology report from any biopsy or surgery
  • Copies of imaging tests (CT or MRI scans, etc.), which is often stored on a CD, DVD, etc.
  • If you had surgery, a copy of your operative report
  • If you stayed in the hospital, a copy of the discharge summary that the doctor wrote when you were sent home
  • If you had radiation treatment, a summary of the type and dose of radiation and when and where it was given
  • If you had chemotherapy or targeted therapies, a list of your drugs, drug doses, and when you took them

 

What’s new in esophagus cancer research?

Research on the causes, prevention and treatment of this cancer is now being done at many places across the nation.

Genetics

Research has found that certain gene changes are more common in people with Barrett’s esophagus. Once more is known about this, we might be able design new tests for finding the people who are likely to get Barrett’s esophagus and esophageal cancer earlier, so that these problems can be prevented. Knowing about these changes may also lead to new targeted treatments that overcome the effects of these abnormal genes.

Screening and prevention

The rate of adenocarcinoma of the esophagus has risen sharply in recent decades. Efforts are now being made to reduce obesity, a major risk factor for this form of cancer (and several other types as well).

In people with Barrett’s esophagus, researchers are trying to find out if newer tests can tell which patients are likely to go on to develop cancer. This may help doctors decide which patients need intense follow-up and which ones may be examined less often.

Researchers are also looking for ways to help stop Barrett’s cells from turning into pre-cancer or cancer. Drugs such as proton pump inhibitors (that lower stomach acid) and aspirin are now being studied for this purpose. There are many proton pump inhibitors, such as omeprazole (Prilosec®) and lansoprazole (Prevacid®).

Drug treatment

Many studies are being done on new ways to combine chemotherapy drugs to get the best results. Drugs that target certain substances in the cancer cell are becoming available. This is known as targeted therapy and it has been successful in some other cancers. It is now being tested in esophagus cancer.