Last updated: 02/17/2009

Esophageal cancer is diagnosed in about 13,000 people annually in the United States; it is responsible for approximately 1.5–5% of cancer deaths each year. Although it is not as prevalent as breast and colon cancer, its rate of occurrence is increasing....

This rise is thought to be related to an increase in gastroesophageal reflux disease, or GERD.

The esophagus has a muscular opening, or sphincter, at the entrance to the stomach, which usually keeps acid from passing upward. In people with GERD, the esophageal sphincter allows partially digested food and excess stomach acid to flow back into the esophagus. This occurrence is known as regurgitation. Regurgitation continually exposes the lining of the esophagus to large amounts of acid, causing repetitive damage to the cells of the esophageal lining. The result is Barrett's esophagus, a condition in which the normal cells (squamous cells) of the esophageal lining are replaced by the glandular type of cells that normally line the stomach. Glandular cells are more resistant to acid damage but at the same time, they can more readily develop into cancer cells. Studies at New York's Memorial Sloan-Kettering Hospital have shown that only 30% of people diagnosed with Barrett's esophagus will later be diagnosed with cancer; the other 70% will not develop dysplasia, the precancerous condition. Effective medical treatment of acid reflux is thought to be a factor in the low incidence of cancer in people with Barrett's esophagus. Other types of cancer can also occur in the esophagus, including melanoma, sarcoma, and lymphoma.

The risk factors for esophageal cancer include:

• Use of tobacco. The highest risk for esophageal cancer is the combination of smoking and heavy alcohol use.
• Abuse of alcohol.
• Barrett's esophagus as a result of long-term acid reflux disease.
• A low-fiber diet; that is, a diet that is low in fruits and vegetables, and whole grains that retain their outer bran layer. Other dietary risk factors include such vitamin and mineral deficiencies, as low levels of zinc and riboflavin.
• Accidental swallowing of cleaning liquids or other caustic substances in childhood.
• Achalasia. Achalasia is an impaired functioning of the sphincter muscle between the esophagus and the stomach.
• Esophageal webs. These are bands of abnormal tissue in the esophagus that make it difficult to swallow.
• A rare inherited disease called tylosis, in which excess layers of skin grow on the hands and the soles of the feet. People with this condition are almost certain to develop esophageal cancer.

Cancer of the esophagus begins in the inner layers of the tissue that lines the passageway and grows outward. Cancer of the top layer of the esophageal lining is called squamous cell carcinoma; it can occur anywhere along the esophagus, but appears most often in the middle and upper portions. It can spread extensively within the esophagus, requiring the surgical removal of large parts of the esophagus. Adenocarcinoma is the type of cancer that develops in the lower end of the esophagus near the stomach. Both types of cancer may develop in people with Barrett's esophagus. Prior to 1985, squamous cell carcinoma was the most common type of esophageal cancer, but adenocarcinoma of the esophagus and the upper part of the stomach is increasing more rapidly than any other type of cancer in the United States. Up to 83% of patients undergoing esophagectomy have been shown to have adenocarcinoma. This development may be related to such changes in risk factors as decreased smoking and alcohol use as well as increased reflux disease. People at high risk for esophageal cancer should be examined and tested regularly for changes in cell types.

Esophageal cancer is classified in six stages determined by laboratory examination of tissue cells from the esophagus, nearby lymph nodes, and stomach. The six stages are:

• Stage 0. This is the earliest stage of esophageal cancer, in which cancer cells are present only in the innermost lining of the esophagus.
• Stage I. The cancer has spread to deeper layers of cells but has not spread into nearby lymph nodes or organs.
• Stage IIA. The cancer has invaded the muscular layer of the esophageal walls, sometimes as far as the outer wall.
• Stage IIB. The cancer has invaded the lymph nodes near the esophagus and has probably spread into deeper layers of tissue.
• Stage III. Cancer is present in the tissues or lymph nodes near the esophagus, especially in the trachea (windpipe) or stomach.
• Stage IV. The cancer has spread to more distant organs, such as the liver or brain.

Unfortunately, the symptoms of esophageal cancer usually don't appear until the disease has progressed beyond the early stages and is already metastatic. Without early diagnostic screening, patients may wait to consult a doctor only when there is little opportunity for cure. The symptoms of esophageal cancer may include difficulty swallowing or painful swallowing; unexplained weight loss; hiccups; pressure or burning in the chest; hoarseness; lung disorders; or pneumonia.

The decision to perform an esophageal resection will be made when staging tests have confirmed the presence of cancer and its stage. Two-thirds of people who undergo endoscopy, a close examination of the inside lining of the esophagus, and biopsies (testing esophageal tissue cells) will already have cancer, which can progress rapidly. Some will be treated with surgery and others with medical therapy, depending on the stage of the cancer, the patient's general health status, and the degree of risk. Removing the esophagus or the affected portion is the most common treatment for esophageal cancer; it can cure the disease if the cancer is in the early stages and the patient is healthy enough to undergo the stressful surgery. Esophagectomy will be recommended if early-stage cancer or a precancerous condition has been confirmed through extensive diagnostic testing and staging. Esophagectomy is not an option if the cancer has already spread to the stomach. In this case an esophagogastrectomy will usually be performed to remove the cancerous part of the esophagus and the upper part of the stomach.

Esophagectomy

An esophagectomy takes about 6 hours to perform. The patient will be given general anesthesia, keeping him or her unconscious and free of pain during surgery. One of several approaches or incisional strategies will be used, chosen by the surgeon to gain adequate access to the upper abdomen and remove the esophagus or the tumor and the nearby lymph nodes. The four common incisional approaches are: transthoracic, which involves a chest incision; Ivor-Lewis, a side entry through the fifth rib; three-hole esophagectomy, which uses small incisions in the chest and abdomen to accommodate the use of instruments; and transhiatal, which involves a mid-abdominal incision. The approach chosen depends on the extent of the cancer, the location of the tumor or obstruction, and the overall condition of the patient.

In a minimum-access laparoscopic and thorascopic procedure, the surgeon makes several small incisions on the chest and abdomen through which he or she can insert thin telescopic instruments with light sources. The abdomen will be inflated with gas to enlarge the abdominal cavity and give the surgeon a better view of the procedure. First, the camera-tipped laparoscope will be inserted through one small incision, allowing images of the organs in the abdominal area to be displayed on a video monitor in the operating room. If the surgeon is going to use a portion of the stomach to replace the resected esophagus, he or she will first locate the fundus, or upper portion of the stomach. The fundus will be manipulated, stapled off, and removed laparoscopically, to be sutured in place (gastroplasty) as a replacement esophagus.

Next, the surgeon will pass thorascopic instruments into the chest through another incision. The esophagus or cancerous portion of the esophagus will be visualized, manipulated, cut and removed. Lymph nodes in the area will also be removed. Then the surgeon will either pull up a portion of the stomach and connect it to the remaining portion of the esophagus (anastomosis), or use a piece of the stomach or intestine, usually the colon, to reconstruct the esophagus. Either procedure will allow the patient to swallow and pass food and liquid to the stomach after recovery. As discussed above, other approaches may be used to gain access to the affected portion of the esophagus.

There are several variations of an esophagectomy, including:

• Standard open esophagectomy. This technique requires larger incisions to be made in the chest (thoracotomy) and in the abdomen so that the surgeon can dissect the esophagus or cancerous portion and remove it along with the nearby lymph nodes. The esophagus can then be reconnected to the stomach using a portion of either the stomach or the colon.
• Laparoscopic esophagectomy. This is a less invasive technique performed through several small incisions on the chest and abdomen with the camera-tipped laparoscope and a video monitor to guide removal of the esophagus or tumor along with nearby lymph glands.
• Vagal-sparing esophagectomy. This procedure preserves the branches of the vagus nerve that supply the stomach, with only minimal alteration of the size of the stomach and the nerves that control acid production and digestive functions.

Esophagogastrectomy

An esophagogastrectomy is also major surgery performed with the patient under general anesthesia. The surgeon will choose the incisional approach that allows the best possible access for resecting the lower portion of the esophagus and the upper portion of the stomach. The surgeon's decision will depend on the extent of the cancer, the amount of the esophagus that must be removed, and the patient's overall health status. An esophagogastrectomy can be performed as an open procedure through large incisions, or as a laparoscopic procedure through small incisions.

In a minimum-access laparoscopic procedure, several small incisions are made in the patient's abdomen. A laparoscope will be inserted through one small incision, allowing images of the abdominal organs to be displayed on a video monitor. As in an esophagectomy, gas may be used to inflate the abdominal cavity for better viewing and space for the surgeon to maneuver. The cancerous upper portion of the stomach will first be stapled off and resected. The cancerous portion of the esophagus will then be cut and removed along with nearby lymph nodes. Finally, a portion of the stomach will be pulled upward and connected to the remaining portion of the esophagus (anastomosis); or, if most of the esophagus has been removed, a piece of the colon will be used to construct a new esophagus. Sometimes the surgeon must make an incision in the neck in order to gain access to and resect the upper portion of the esophagus, followed by making an anastomosis between the esophagus and a portion of the stomach.