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Esophageal cancer affects approximately 16,700 people annually. Its incidence has steadily increased over the past 30 years. According to estimates by the American Cancer Society, in 2008, there were 16,700 new cases of esophageal cancer and 14,280 deaths. Although 5-year survival rates have gradually improved over time, much work still needs to be done to enhance the outlook for patients with advanced disease.
The stage of your disease at the time of diagnosis affects your prognosis. Prognosis is measured in terms of 5-year survival following treatment. Patients with early, or Stage 0, esophageal cancer (high grade dysplasia or carcinoma in situ) have a 95% 5-year survival; Stage I patients have up to 80% 5-year survival; Stage IIA have up to 40%; Stage IIB have up to 30%; Stage III have up to 15%; and Stage IV patients have less than 5% 5-year survival. Unfortunately, over 50% of patients present with advanced (stage III or IV) disease. However, even when the outlook is grim, individual patients with advanced disease can have success in fighting this cancer. It is impossible to know beforehand precisely which patients will do well. However, with continuing improvement in surgical and medical treatment for this disease, we hope to increase the number of individual success stories.
Squamous cell cancer accounts for about 90% of esophageal cancers in African-American males but only 50% in caucasian males. The etiology of squamous cell cancer is unclear, but the incidence of this histologic subtype has steadily decreased over the past two decades. Risk factors for this type of cancer include all forms of tobacco use, including cigarettes, cigars, and chewing tobacco. A high intake of alcohol over prolonged periods, a diet deficient in fruits and vegetables, a history of caustic injury to the esophagus, and a history of head and neck cancers or achalasia all appear to be associated with squamous cell cancers of the esophagus.
Adenocarcinoma is another type of esophageal cancer. While the incidence of squamous cell carcinoma has been decreasing in the United States, the incidence of adenocarcinoma has been rising. This switch in incidence may be related to certain lifestyle changes associated with Western cultures. Heartburn and obesity are strongly associated with adenocarcinoma. In fact, a combination of prolonged heartburn and obesity can increase the relative risk for esophageal cancer by 180 times. Barrett's esophagus, a type of esophageal cancer that is located near the GE junction, can develop in patients who have prolonged reflux disease. Prolonged exposure to reflux causes the cells that line the esophagus to change from a squamous epithelial type to a glandular cell type. Over time, these changes can lead to the development of 'high grade dysplasia.' Approximately 45% of patients diagnosed with high grade dysplasia have undiagnosed adenocarcinoma of the esophagus. Esophageal cancer at this stage is highly curable with surgical resection. Therefore, we recommend immediate surgical resection for any patient diagnosed with high grade dysplasia. Waiting to see if the high grade dysplasia will turn into cancer is risky and often results in more advanced disease by the time of surgery and a much poorer prognosis.
Generally speaking, patients with early stage esophageal cancer have minimal symptoms. Symptoms tend to become evident only with large, advanced tumors. Some typical presenting symptoms are listed below.
Making a diagnosis of esophageal cancer requires a tissue biopsy. Patients also may undergo one or several additional studies that aid in evaluating the location and extent of disease. Some of these studies are listed below.
Chemotherapy is often used before or after surgery or alone in the most advanced cases of cancer. It is given before surgery in locally advanced cancers that may or may not involve local lymph nodes. The purpose of chemotherapy is to shrink the tumor with the goal of making the tumor small enough that it can be surgically resected. It is often used in conjunction with radiation. Chemotherapy is a systemic treatment that targets all microscopic tumor cells in the body, including the lymph nodes.
Common chemotherapuetic regimens include cisplatin and 5-FU which is given in 4 monthly cycles or 5-FU and mitomycin which is given in 2 monthly cycles. Other medications include irinotecan or paclitaxel. Medications are administered on an outpatient basis and can be given at a local institution near your home. Many of these drugs are used in the context of a clinical trial. You should consult with your thoracic surgeon or oncologist about the availability of such trials.
Radiation involves the use of high energy beams to cause damage to cells. This is often used in conjunction with chemotherapy in the pre-operative setting to shrink the tumor. Both normal and cancer cells are damaged by radiation; hence, efforts are made to provide the highest amount of radiation to the tumor while minimizing radiation to normal tissue. Treatment with radiation often requires planning with CT scans and simulators to accurately map out the treatment area. Tattoos are placed on the skin to mark the area of interest. When treatment begins, you will not need any additonal preparation. The treatment only takes a few minutes, but the process requires daily treatments for up to 5 weeks.
Surgery is the most common treatment for esophageal cancer. The goal of surgery is to completely remove the cancer and all surrounding lymph nodes. Surgery is most effective with early disease, but can be used in conjunction with chemotherapy and radiation for advanced cancer. The goal is not only to remove the disease, but also to provide relief of symptoms such as obstruction and dysphagia. A key component of surgery is reconstruction of a new esophagus from a suitable graft.
There are several types of surgery for esophageal cancer. What is best for you will depend on the type, location, and extent of your disease, as well as your overall health and condition. Common surgical operations include transhiatal esophagectomy, three hole esophagectomy, Ivor-Lewis esophagectomy, thoracoabdominal esophagectomy, and minimally invasive esophagectomy. For all of these operations, the esophagus is removed and reconstructed by elongating the stomach. In some cases, however, the colon or small bowel may be used as an esophageal replacement. A feeding tube is placed during surgery to support your nutrition until you can eat adequately.
Most patients stay in the hospital for 10-14 days following surgery, including several days in the Intensive Care Unit (ICU). Pain is controlled with patient-controlled anesthesia (PCA), an epidural catheter, or some combination of both. The main concerns after surgery are pain control and early mobilization. Coughing, deep breathing, and walking are encouraged and supported. A barium swallow is performed about a week after surgery, and if no leak is found, an oral diet is started.
Since the stomach is now tubularized, this limits the amount of food that can be consumed in a single meal. Hence, most patients have 6 small meals per day as opposed to 3 large ones. With time, the stomach may distend somewhat to allow for larger meals, but this takes time. After you are discharged from the hospital, your diet may still be supported by tube feeds at night until your oral intake increases. Physical activity and exercise are required following discharge and should be gradually increased over time according to your doctor's guidelines. Depending on the type of surgery you undergo, you may be on pain medication at home for up to several weeks.
Key points to remember:
Esophageal resection is a stressful operation. Complications and mortality rates can be high. Therefore, it is important to have your surgery at an experienced center that performs a high volume of cases each year. A dedicated team of surgeons, anesthesiologists, and nurses is critical to minimizing risk. Inexperienced centers can have mortality rates up to 25%. In experienced centers, that rate drops to about 5-8%. The most common complications include anastomotic leak, chylothorax, pneumonia, respiratory failure, heart arrhythmia, heart attack, and vocal cord injury resulting in hoarseness or difficulty swallowing.
Common side effects from surgery include dysphagia which may be due to stricture formation at the anastamosis or at the connection between the esophagus and stomach. Often a simple dilation performed endoscopically on an outpatient basis can help relieve these symptoms. Dumping syndrome sometimes occurs, and is associated with symptoms such as flushing, palpitations, dizziness, sweating, cramping, or diarrhea after eating. These symptoms can be addressed with dietary modification. Reflux of stomach contents or bile can also occur secondary to loss of the valve at the gastroesophageal (GE) junction with esophageal replacement. Head position and diet modification can minimize this symptom.
After you are treated with surgery, chemotherapy, and/or radiation, you will require lifelong follow-up with your thoracic surgeon. Cancer can cause continued physical, psychological, emotional, and financial burdens. Recurrence of cancer is always a concern. Periodic assessment by your surgeon and oncologist will be required and some patients may undergo CT or PET scanning at regularly scheduled intervals.
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Division of Thoracic Surgery
Brigham and Women's Hospital
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Boston, MA 02115