Esophageal cancer is cancer that occurs in the esophagus, the long tube that allows food to pass from the throat to the stomach. The two main types of esophageal cancer are squamous cell carcinoma and adenocarcinoma.

Esophageal cancer is usually discovered after individuals begin experiencing symptoms from the disease. The most common symptom of this type of cancer is difficulty swallowing (dysphagia).  The disease also may cause pain in the throat or chest and unexplained weight loss.

Tobacco use, excessive consumption of alcohol and a condition called gastroesophageal reflux disease (GERD) are all risk factors in the development of esophageal cancer. The disease is much more likely to affect men than women, and different forms of the disease affect whites and Africa-Americans to varying degrees.

Esophageal cancer is diagnosed using two primary methods: barium x-ray and upper endoscopy, in which a thin, lighted tube with a miniature camera is inserted into the esophagus.

Treatment of esophageal cancer depends on the type, location and stage of the cancer. A patient’s age, overall health and personal preferences are also taken into account. In many cases, combinations of treatment are more effective than individual therapies.

Esophageal cancer is a disease that affects the esophagus, the long tube that connects the throat to the stomach. The chief symptom is difficulty swallowing (dysphagia), although this usually does not occur until the disease has advanced.

The esophagus is typically between 10 and 13 inches (25 and 32 centimeters) long and 3/4 inches (1.9 cm) wide at its smallest point. The walls of the esophagus are composed of specialized tissue layers, including:

• Mucosa. Inner lining of thin, flat cells (squamous or adenomatous).
  
  
• Submucosa. Layer below the inner lining containing mucus-secreting glands.
  
  
• Muscularis and advential layers. Rarely give rise to malignancy but are often invaded by esophageal cancers.
  
  
• Upper and lower esophageal sphincters. Thick bands of muscle tissue.

The esophagus plays an important role in swallowing and digestion. When a person eats or drinks, a muscle in the upper esophagus (known as the upper esophageal sphincter) relaxes. This movement allows food and liquid to enter the tube. The esophagus then closes to prevent food from backing up. A series of rhythmic contractions in the esophageal wall move the food down the tube. This process is called peristalsis, and it takes between four and 10 seconds.

The food continues to move down to the junction between the esophagus and the stomach, a ring of muscle known as the lower esophageal sphincter. This ring opens to allow food into the stomach and then quickly closes to prevent stomach acids and digestive enzymes from backing up into the esophagus

Cancer of the esophagus usually starts on the innermost layer before spreading outward to other layers. Eventually, it can grow large enough to create an obstruction that causes swallowing difficulties. It can also spread (metastasize) to surrounding tissues and organs.

Esophageal cancer is most dangerous after it has spread to nearby lymph nodes or other parts of the body. The most common sites for metastases include the lymph nodes, lungs, liver, brain, adrenal glands and bones. Survival rates for esophageal cancer remain low, but they are improving. Today, 17 percent of white patients and 12 percent of African-American patients live at least five years after being diagnosed with the disease, according to the American Cancer Society (ACS).

MOre than 15,500 new cases of esophageal cancer will be diagnosed in 2007, according to the ACS. The disease is three to four times more common in men than in women and about 50 percent more common in African-Americans than in whites. It is far more common in some countries than others. For example, rates of esophageal cancer are between 10 to 100 times higher in Iran, northern China, India and southern Africa than in the United States. 

In the United States, the ACS estimates that nearly 14,000 deaths will occur from esophageal cancer in 2007. Most people eventually die of this disease because it is usually detected after it has reached an advanced stage; however, survival rates are improving. The chances of survival typically increase if the cancer is detected in the earlier stages.

Esophageal cancer typically begins in the inner layer of the esophagus, and can occur anywhere along the length of the 10-inch tube. It is classified according to the types of cells in which it originates:

• Squamous cell or epidermoid carcinoma. Develops in the thin, flat squamous cells that line the esophagus. This cancer can appear anywhere along the esophagus, but most often develops in the upper and middle part of the esophagus. It is the most common esophageal cancer in African-Americans and the most common esophageal cancer throughout the world.
  
  
• Adenocarcinoma. Develops in glandular (secretory) cells in the lower esophagus near the stomach. Glandular cells produce and release fluids such as mucus. It is more common in whites than in African-Americans. The incidence of adenocarcinoma is the fastest-growing among esophageal cancers in the United States. Approximately two-thirds of esophageal cancers diagnosed now fall into this category, according to the American Cancer Society (ACS). Experts attribute this growth to the rapid rise of acid reflux disease.
  
  
• Other forms. Although less common, other forms of esophageal cancer include:
  
  
• Sarcoma. A malignant tumor arising from connective tissues or muscle.
  
  
• Lymphoma. A malignant tumor that arises in the lymph nodes or in other lymphoid tissue.
  
  
• Small cell carcinoma. A highly malignant carcinoma composed of small round or egg-shaped cells with little cytoplasm.
  
  
• Spindle cell carcinoma. A carcinoma composed of elongated cells. It is frequently a poorly differentiated squamous cell carcinoma.

Cancer that starts in the breast or lung can also spread (metastasize) through the blood stream or lymph system or by direct extension to the esophagus.

Many causes of esophageal cancer remain unknown to scientists. However, the following risk factors have been associated with the condition:

• Sex. Men are three times more likely to develop esophageal cancer than women, according to the American Cancer Society (ACS).
  
  
• Age. The risk of being diagnosed with esophageal cancer grows as a person ages. Most people with the disease are between ages 55 and 85, with nearly half of diagnosed cases occurring in individuals over the age of 70.
  
  
• Race and ethnicity. Esophageal cancer is 50 percent more likely to develop in African-Americans. Squamous cell carcinoma is the most common type of cancer to occur in African-Americans while adenocarcinoma is the most common form in whites.
  
  
• Heavy alcohol consumption. In Western nations, heavy drinking is the chief source of the majority of esophageal squamous cell carcinomas. Chronic alcohol abuse irritates the lining of the esophagus, which leads to inflammation and potentially malignant changes in the cells.
  
  
• Tobacco use. Smoking of any kind and chewing tobacco increase the risk of esophageal squamous cell carcinoma. risk factors increase over time and rise substantially for people who both drink and smoke.
  
  
• Gastroesophageal reflux disease (GERD). This condition occurs when the lower esophageal sphincter consistently relaxes abnormally or weakens, which allows caustic stomach acids to back up into the esophagus and cause heartburn. This can lead to Barrett’s esophagus, a condition in which abnormal cells similar to the stomach’s glandular cells develop in the lower esophagus. Such cells have a high potential for malignancy, and reflux is the most common cause of esophageal adenocarcinomas. Reflux problems are associated with smoking, obesity and high-sodium diets.
  
  
• Diet. Eating too few fruits and vegetables may contribute to esophageal cancer, especially in diets lacking vitamins A, C, B1 (riboflavin), beta carotene and the mineral selenium. In particular, low levels of selenium have been associated with elevated risk of Barrett’s esophagus. On the other hand, high levels of selenium can be toxic, so it is best to get selenium from foods such as fish, whole-grain bread, Brazil nuts and walnuts. Frequent ingestion of very hot liquids also may raise the risk of esophageal cancer.
  
  
• obesity. People who weigh 20 to 30 pounds more than their ideal weight are at higher risk for adenocarcinoma. Obesity in men increases the risk of dying from this disease by approximately 50 percent.
  
   
• Exposure to silica dust. A primary component of sandstone and granite, silica dust has been linked with an increased risk of esophageal cancer. Miners, construction workers and people working in the pressurized spaces, such as building tunnels, are most at risk.
  
  
• Achalasia. Disorder in which food collects at the bottom of the esophagus due to an inability of the esophagus to move food along or because the lower esophageal sphincter does not properly relax to allow food into the stomach. For reasons that remain unclear, achalasia appears to increase the risk of cancer. About 6 percent of patients with achalasia develop squamous cell-type esophageal cancer, according to the ACS.
  
  
• Esophageal webs. Abnormal protrusions of thin tissue in the esophagus. Some cause no symptoms, but others can make swallowing difficult. About 10 percent of patients with this condition will develop esophageal cancer.
  
  
• Tylosis. A rare, inherited disorder that causes excess skin formation on the soles and palms. Scientists believe a genetic defect is responsible for both tylosis and esophageal cancer. People with tylosis have a 40 percent risk of developing esophageal cancer, according to the ACS.
  
  
• Lye ingestion. Lye is a chemical found in industrial and household cleaners, such as drain openers. It is corrosive and destroys cells. Children who accidentally swallow cleaners with lye have a higher chance of developing esophageal cancer as an adult. Cancer typically occurs about 40 years after ingestion.
  
  
• Radiation therapy. A link has been established between breast cancer radiation treatments following mastectomy and a moderately increased risk of esophageal cancer. The risk is greatest 10 to 15 years after treatment. The increased risk does not appear to apply to women who have had a lumpectomy and radiation.

The most common symptom of esophageal cancer is difficulty swallowing (dysphagia) and a feeling that food is sticking in the throat or chest. It usually does not appear until the tumor has grown so large that it narrows the esophagus to about half its normal width of three-quarters of an inch (1.91 centimeters). Patients may find it impossible to swallow heavily textured foods, such as meats or breads. Many patients may unconsciously change their eating habits as a result of their swallowing problems.

Other symptoms associated with esophageal cancer include:

• Substantial, unintentional weight loss. Esophageal cancer both prevents the patient from eating properly and, when advanced, changes the way the body metabolizes nutrients. Both of these factors can lead to weight loss.
  
  
• Pain in the throat, mid-chest or between the shoulder blades. Some patients may feel pain during swallowing or discomfort and burning behind the breastbone.
  
  
• Hoarseness, chronic cough and coughing up blood. These symptoms usually do not appear until the cancer is in an advanced stage.
  
  
• Tracheoesophageal fistula. Occurs when a tumor creates a hole between the esophagus and the windpipe (trachea) and can cause aspiration of food into the lungs. This can create coughing and gagging when the patient tries to swallow and can lead to pneumonia. This condition usually does not occur unless the tumor is very far advanced.

Patients experiencing signs and symptoms of esophageal cancer should immediately notify their physician who will compile a thorough medical history and perform a complete physical examination. Certain screening tests are used to check for esophageal cancer in its early stages, before symptoms develop. People at high risk for the disease, especially those already diagnosed with Barrett’s esophagus or tylosis, are most likely to be screened.

Esophageal cancer is diagnosed using two primary methods:

• Barium swallow. The patient drinks a thick liquid (barium) that coats the lining of the esophagus, allowing it to be viewed on an x-ray. In some cases, air is blown into the esophagus to push the barium onto the esophageal walls. While this test – also known as an esophagram – can reveal growths including cancer of the esophagus, it may miss some lesions. The test cannot be used to determine how far the cancer may have spread (metastasized) beyond esophagus.
  
  
• Upper endoscopy. An endoscope – a thin, lighted tube with a miniature camera at the end – is used to examine the esophagus. Before the tube is inserted into the throat, a topical anesthetic is applied to make the procedure less uncomfortable. The camera sends back pictures of abnormalities that are viewed on a monitor. The physician can also use the endoscope to obtain tissue samples (biopsy) for analysis.
  
  In an endoscopic ultrasound, a probe may be inserted to determine the presence of and depth of penetration of the cancer. The probe sends out very sensitive sound waves that penetrate deep into the tissues to reveal close-up images of the esophagus and nearby tissues. Recent studies have suggested that this test may be more accurate than traditional endoscopy and CT scans in determining the size and shape of the cancer as well as the spread of the disease.

Although endoscopy and barium swallow are used for diagnosis, other tests may be performed to stage the disease. These tests include:

• Bronchoscopy. This test is similar to an esophagoscopy, except that the endoscope is used to examine the windpipe (trachea) and air passages leading to the lungs (bronchi). It can reveal whether or not the cancer has spread from the esophagus to these areas or visa versa.
  
  
• Computed axial tomography (CAT) scan.  A CAT scan uses multiple x-ray images and computerized technology to create cross-sectional images of the body. CAT scans can confirm the location of tumors in the esophagus and reveal whether the cancer has spread to the lymph nodes or other organs.
  
  
• Magnetic resonance imaging (MRI). Test that produces clear cross-sectional or three dimensional images of the body's tissues and organs. MRI can be used to help detect the location of cancer in the body.

• Positron emission tomography (PET) scan. During a PET scan, a small amount of radioactive tracer is injected into the body. Cancerous tumors absorb greater amounts of the tracer and these areas appear as bright spots on the scan.
  
  
• Thoracoscopy and laparoscopy. Allow the physician to see the lymph nodes and other organs near the esophagus in the chest (thoracoscopy) or in the abdomen (laparoscopy). A biopsy sample may be obtained using these methods, which require the patient to undergo general anesthesia.

Treatment of esophageal cancer depends on the type, location and stage of the cancer. A patient’s age, overall health and personal preferences are also taken into account. In many cases, combinations of treatment are more effective than individual therapies. For some patients, esophageal cancer may have advanced to the point that it is no longer curable. However, treatments may still help relieve symptoms associated with esophageal cancer (palliative care).

Depending on the stage and location of the cancer, surgery may be the first treatment option for esophageal cancer. A partial esophagectomy is the procedure typically used to remove the cancerous tissue. In this surgical procedure, a surgeon removes the portion of the esophagus that contains the cancer and, if the tumor is in the lower one-third, the upper portion of the stomach.  Nearby lymph nodes are also removed. The extent of the esophagus and stomach that is removed depends on the location and spread of the cancer. For some individuals, the remaining esophagus may be reconnected to the stomach so the patient can still swallow and ingest food. In others, a section of the large intestine or a plastic tube may be used to replace the missing part of the esophagus.

Esophagectomy is a complicated operation and the patient may be hospitalized up to two weeks following the surgery. There are several risks associated with these procedures, including infection, bleeding and leakage from the areas where the remaining esophagus is attached. In recent years, this procedure has been performed laparoscopically, which is less invasive than standard surgery. In laparoscopic surgery, the procedure is performed through a smaller incision with sepcialized instruments resulting in less recuperation time.

There are many different techniques and approaches to surgery for esophageal cancer. A patient’s physician can determine the best procedure based on the individual case. In addition to surgery, other therapies may be used to treat esophageal cancer. These treatments are often provided in combination with surgery and include:

• Chemotherapy. Useful powerful drugs to kill cancer cells and interfere with their ability to reproduce and spread throughout the body. It usually involves one or more drugs and is often used in conjunction with other therapies. This approach can help kill cancer cells that have spread beyond the esophagus. Chemotherapy may be used before surgery to shrink the size of the tumor or after surgery to destroy any remaining malignant cells. According to the American Cancer Society, 10 to 40 percent of patients respond to chemotherapy drugs with a significant reduction in tumor size. Chemotherapy without additional therapies (e.g., surgery, radiation) usually will not cure esophageal cancer.
  
  
• Radiation therapy. Uses a specific type of radiation to kill or shrink cancer cells. Radiation disrupts or destroys cancer cells' genetic material, preventing the cells from continuing to grow and spread throughout the body. This approach is most effective against esophageal cancer when it is used in combination with chemotherapy and is often used to relieve pain and improve swallowing. In most cases, the radiation comes from an external source. However, in some cases, radioactive "seeds" or wires may be implanted near the cancer cells of the esophagus or held in a tube within the esophagus for a limited time. This is known as internal radiation or brachytherapy.
  
  Recent research has indicated that chemoradiotherapy combined with surgery results in a better outcome than surgery alone. Patients who received all three therapies had a five year survival rate of 39 percent as compared to 16 percent for patients who only underwent surgery. Additional studies have not yet yielded these results.
  
  
• Photodynamic therapy. The patient receives an injection of a light-sensitive drug that remains in cancer cells longer than it does in healthy cells. A laser light is then focused through an endoscope and onto the esophagus, stimulating the production of an active form of oxygen that destroys cancer cells while leaving healthy cells alone. This treatment usually is used to relieve pain and obstruction in the esophagus, but is also being studied as a treatment for early-stage esophageal cancer.

Patients who have constrictions in their esophagus may benefit from certain procedures. In an esophageal dilation, a balloon-like device is inserted into the esophagus to stretch the opening. A stainless steel or plastic stent also may be used to push open the esophagus. In addition, patients with tracheosophageal fistulas may require surgery or the use of a stent to keep food or liquids from entering the windpipe and lungs.

Swallowing difficulties from esophageal cancer can lead to weight loss, weakness and malnutrition. Patients who develop dysphagia may benefit from swallowing therapy provided by a speech-language pathologist.

It is not always possible to prevent esophageal cancer. However, certain lifestyle changes can reduce a person’s risk of developing this disease. These include:

• Quitting smoking. Tobacco smoke contains carcinogens that damage the DNA (deoxyribonucleic acid) that regulates cell growth. In addition, smoking is a leading cause of gastroesophageal reflux disease (GERD), which causes cell changes that can lead to esophageal cancer.
  
  
• Limit alcohol consumption. Chronic alcohol use over a period of years is a major factor in the development of esophageal squamous cell carcinomas and adenocarcinomas. Drinking in moderation or abstaining from drinking can lower this risk.
  
  
• Explore the cause of heartburn. Patients diagnosed with GERD should take medications prescribed by their physician to prevent chronic acid reflux, a factor in esophageal cancer. Patients should also avoid consuming food and drinks that can cause heartburn and gastric reflux.
  
  
• Eat a balanced diet. Adding fruits and vegetables to a diet may help protect against esophageal cancer. Deep green and dark yellow or orange produce is best. Vegetables from the cabbage family are also a good choice for those concerned about esophageal cancer. Diets low in selenium have been implicated in helping trigger esophageal cancer. Foods with selenium include walnuts, fish and whole grains.
  
  
• Maintain an optimal weight. People who are obese are at a greater risk for esophageal cancer and other diseases. Steady weight loss of 1 or 2 pounds a week is considered the safest way to lose weight and keep it off.

There have been several new developments regarding esophageal cancer research and treatment in the following areas:

• Genetics. Changes in certain genes have been discovered that appear to be responsible for causing normal cells to develop into esophageal cancer.  Researchers hope to find ways through genetics to detect esophageal cancer earlier in a more curable stage.
  
  
• Drug treatment. Clinical trials are in progress that test new ways to combine medications to more effectively treat esophageal cancer. Other studies are examining the best ways to combine chemotherapy and radiation therapy. In addition, drugs are being developed that target certain substances in the cancer cell, while others try to block the process of blood vessel formation in the tumor.
  
  
• Immunotherapy. Esophageal cancer treatments that boost the immune system mostly involve monoclonal antibodies, which are made in the laboratory and injected into patients. Clinical studies are examining the use of antibodies to fight adenocarcinoma esophageal cancer.
  
  
• Prevention. Efforts are being made to reduce obesity, which is a major contributor to esophageal cancer. In addition, scientists are trying to develop tests that can help predict which patients with Barrett’s esophagus have the greatest risk of developing esophageal cancer.

When cancer has been diagnosed, the physician will order additional tests, such as a biopsy or imaging studies, to reveal whether or not the cancer has spread (metastasized). This process is known as staging, and it is used to help determine the most effective treatment plan and the patient's prognosis.

The prognosis (predicted outlook or chance of survival) of esophageal cancer depends on the cancer’s stage and grade. The stage indicates the extent of the cancer, or how widespread it is in the body. The grade measures how abnormal the cells look under a microscope. The grading and staging systems are combined into another system that allows the physician to discuss the pathology in layman’s terms. 

There are a variety of systems used for staging cancers. The TNM system of the American Joint Committee on Cancer (AJCC) is the most commonly used staging system. It is based on three important factors:

• T describes the original (primary) tumor (e.g., size, location).
  
  
• N describes whether or not the cancer has spread to neighboring lymph nodes.
  
  
• M describes whether or not there are distant metastases (spread of cancer to distant parts of the body).

Sometimes, additional letters will follow the T, N or M, such as “Tm,” which indicates the presence of multiple cancers or “Tis,” which indicates that the cancer is superficial (carcinoma in situ) and has not invaded surrounding tissues.

The stages of esophageal cancer are:

• Stage 0. Cancer is found only in the most superficial layer of cells lining the esophagus and is not capable of invading other parts of the body at present. Nonetheless, they should be removed or monitored closely to ensure they do not eventually become invasive.
  
  
• Stage I. Cancer has spread to the next layer of tissue in the wall of the esophagus.
  
  
• Stage II. Cancer has invaded deeper layers of the esophageal lining such as the esophageal muscle or the outer wall of the esophagus. Cancer may also have spread to nearby lymph nodes.
  
  
• Stage III. Cancer has spread more deeply into the outer esophageal wall and to nearby tissues or lymph nodes.
  
  
• Stage IV. Cancer has spread to other parts of the body, such as distant lymph nodes and organs.

Preparing questions in advance can help individuals to have more meaningful discussions with their physician regarding their condition. Patients may wish to ask their doctor the following questions about esophageal cancer:

1. What tests will I be given to diagnose my suspected cancer?
   
   
2. What is the type and stage of my cancer?
   
   
3. Am I a candidate for surgery and if so, what will it entail?
   
   
4. Can I have the surgery laparoscopically?
   
   
5. What are the risks associated with my surgery?
   
   
6. Will I need chemotherapy or radiation in addition to surgery?
   
   
7. Will I recieve these treatments before or after surgery?
   
   
8. What can I expect in terms of recovery?
   
   
9. Who can help me if I have swallowing problems?
   
   
10. How long will these problems last?
    
    
11. What are the chances that my cancer will return?
    
    
12. Am  I at risk for other cancers if I have esophageal cancer?
    
    
13. Can you recommend a support group?