Esophageal Cancer

views updated May 14 2018

Esophageal Cancer

Definition

Esophageal cancer is a malignancy that develops in tissues of the hollow, muscular canal (esophagus) along which food and liquid travel from the throat to the stomach.

Description

Esophageal cancer usually originates in the inner layers of the lining of the esophagus and grows outward. In time, the tumor can obstruct the passage of food and liquid, making swallowing painful and difficult. Since most patients are not diagnosed until the late stages of the disease, esophageal cancer is associated with poor quality of life and low survival rates.

Squamous cell carcinoma is the most common type of esophageal cancer, accounting for 95% of all esophageal cancers worldwide. The esophagus is normally lined with thin, flat squamous cells that resemble tiny roof shingles. Squamous cell carcinoma can develop at any point along the esophagus but is most common in the middle portion.

Adenocarcinoma has surpassed squamous cell carcinoma as the most common type of esophageal cancer in the United States. Adenocarcinoma originates in glandular tissue not normally present in the lining of the esophagus. Before adenocarcinoma can develop, glandular cells must replace a section of squamous cells. This occurs in Barrett's esophagus, a pre-cancerous condition in which chronic acid reflux from the stomach stimulates a transformation in cell type in the lower portion of the esophagus.

A very small fraction of esophageal cancers are melanomas, sarcomas, or lymphomas.

There is great variability in the incidence of esophageal cancer with regard to geography, ethnicity, and gender. The overall incidence is increasing. About 13,000 new cases of esophageal cancer are diagnosed in the United States each year. During the same 12-month period, 12,000 people die of this disease. It strikes between five and ten North Americans per 100,000. In some areas of China the cancer is endemic.

Squamous cell carcinoma usually occurs in the sixth or seventh decade of life, with a greater incidence in African-Americans than in others. Adenocarcinoma develops earlier and is much more common in white patients. In general, esophageal cancer occurs more frequently in men than in women.

Causes and symptoms

The exact cause of esophageal cancer is unknown, although many investigators believe that chronic irritation of the esophagus is a major culprit. Most of the identified risk factors represent a form of chronic irritation. However, the wide variance in the distribution of esophageal cancer among different demographic groups raises the possibility that genetic factors also play a role.

Several risk factors are associated with esophageal cancer.

  • Tobacco and alcohol consumption are the major risk factors, especially for squamous cell carcinoma. Smoking and alcohol abuse each increase the risk of squamous cell carcinoma by five-fold. The effects of the two are synergistic, in that the combination of smoking and alchohol increases the risk by 25- to 100- fold. It is estimated that drinking about 13 ounces of alcohol every day for an extended period of time raises the risk of developing esophageal cancer by 18%. That likelihood increases to 44% in individuals who also smoke one or two packs of cigarettes a day. Smokeless tobacco also increases the risk for esophageal cancer.
  • Gastroesophageal reflux is a condition in which acid from the stomach refluxes backwards into the lower portion of the esophagus, sometimes causing symptoms of heartburn. In some cases of gastroesophageal reflux, the chronic exposure to acid causes the inner lining of the lower esophagus to change from squamous cells to glandular cells. This is called Barrett's esophagus. Patients with Barrett's esophagus are roughly 30 to 40 times more likely than the general population to develop adenocarcinoma of the esophagus.
  • A diet low in fruits, vegetables, zinc, riboflavin, and other vitamins can increase risk of developing to esophageal cancer.
  • Caustic injury to the esophagus inflicted by swallowing lye or other substances that damage esophageal cells can lead to the development of squamous cell esophageal cancer in later life.
  • Achalasia is a condition in which the lower esophageal sphincter (muscle) cannot relax enough to let food pass into the stomach. Squamous cell esophageal cancer develops in about 6% of patients with achalasia.
  • Tylosis is a rare inherited disease characterized by excess skin on the palms and soles. Affected patients have a much higher probability of developing esophageal cancer than the general population. They should have regular screenings to detect the disease in its early, most curable stages.
  • Esophageal webs, which are protrusions of tissue into the esophagus, and diverticula, which are out-pouchings of the wall of the esophagus, are associated with a higher incidence of esophageal cancer.

Symptoms

Unfortunately, symptoms generally don't appear until the tumor has grown so large that the patient cannot be cured. Dysphagia (trouble swallowing or a sensation of having food stuck in the throat or chest) is the most common symptom. Swallowing problems may occur occasionally at first, and patients often react by eating more slowly and chewing their food more carefully and, as the tumor grows, switching to soft foods or a liquid diet. Without treatment, the tumor will eventually prevent even liquid from passing into the stomach. A sensation of burning or slight mid-chest pressure is a rare, often-disregarded symptom of esophageal cancer. Painful swallowing is usually a symptom of a large tumor obstructing the opening of the esophagus. It can lead to regurgitation of food, weight loss, physical wasting, and malnutrition. Anyone who has trouble swallowing, loses a significant amount of weight without dieting, or cannot eat solid food because it is too painful to swallow should see a doctor.

Diagnosis

A barium swallow is usually the first test performed on a patient whose symptoms suggest esophageal cancer. After the patient swallows a small amount of barium, a series of x rays can highlight any bumps or flat raised areas on the normally smooth surface of the esophageal wall. It can also detect large, irregular areas that narrow the esophagus in patients with advanced cancer, but it cannot provide information about disease that has spread beyond the esophagus. A double contrast study is a barium swallow with air blown into the esophagus to improve the way the barium coats the esophageal lining. Endoscopy is a diagnostic procedure in which a thin lighted tube (endoscope) is passed through the mouth, down the throat, and into the esophagus. Cells that appear abnormal are removed for biopsy. Once a diagnosis of esophageal cancer has been confirmed through biopsy, staging tests are performed to determine whether the disease has spread (metastasized) to tissues or organs near the original tumor or in other parts of the body. These tests may include computed tomography, endoscopic ultrasound, thoracoscopy, laparoscopy, and positron emission tomography.

Treatment

Treatment for esophageal cancer is determined by the stage of the disease and the patient's general health. The most important distinction to make is whether the cancer is curable. If the cancer is in the early stages, cure may be possible. If the cancer is advanced or if the patient will not tolerate major surgery, treatment is usually directed at palliation (relief of symptoms only) instead of cure.

Staging

Stage 0 is the earliest stage of the disease. Cancer cells are confined to the innermost lining of the esophagus. Stage I esophageal cancer has spread slightly deeper, but still has not extended to nearby tissues, lymph nodes, or other organs. In Stage IIA, cancer has invaded the thick, muscular layer of the esophagus that propels food into the stomach and may involve connective tissue covering the outside of the esophagus. In Stage IIB, cancer has spread to lymph nodes near the esophagus and may have invaded deeper layers of esophageal tissue. Stage III esophageal cancer has spread to tissues or lymph nodes near the esophagus or to the trachea (windpipe) or other organs near the esophagus. Stage IV cancer has spread to distant organs like the liver, bones, and brain. Recurrent esophageal cancer is disease that develops in the esophagus or another part of the body after initial treatment.

Surgery

The most common operations for the treatment of esophageal cancer are esophagectomy and esophagogastrectomy. Esophagectomy is the removal of the cancerous part of the esophagus and nearby lymph nodes. This procedure is performed only on patients with very early cancer that has not spread to the stomach. Esophagogastrectomy is the removal of the cancerous part of the esophagus, nearby lymph nodes, and the upper part of the stomach. The resected esophagus is replaced with the stomach or parts of intestine so the patient can swallow. These procedures can significantly relieve symptoms and improve the nutritional status of more than 80% of patients with dysphagia. Although surgery can cure some patients whose disease has not spread beyond the esophagus, but more than 75% of esophageal cancers have spread to other organs before being diagnosed. Less extensive surgical procedures can be used for palliation.

Chemotherapy

Oral or intravenous chemotherapy alone will not cure esophageal cancer, but pre-operative treatments can shrink tumors and increase the probability that cancer can be surgically eradicated. Palliative chemotherapy can relieve symptoms of advanced cancer but will not alter the outcome of the disease.

Radiation

External beam or internal radiation, delivered by machine or implanted near cancer cells inside the body, is only rarely used as the primary form of treatment. Post-operative radiation is sometimes used to kill cancer cells that couldn't be surgically removed. Palliative radiation is effective in relieving dysphagia in patients who cannot be cured. However, radiation is most useful when combined with chemotherapy as either the definitive treatment or preoperative treatment.

Palliation

In addition to surgery, chemotherapy, and radiation, other palliative measures can provide symptomatic relief. Dilatation of the narrowed portion of the esophagus with soft tubes can provide short-term relief of dysphagia. Placement of a flexible, self-expanding stent within the narrowed portion is also useful in allowing more food intake.

Follow-up treatments

Regular barium swallows and other imaging studies are necessary to detect recurrence or spread of disease or new tumor development.

Alternative treatment

Photodynamic therapy (PDT) involves intravenously injecting a drug that is absorbed by cancer cells and kills them after they are exposed to specific laser beams. PDT can be used for palliation, but it also cured some early esophageal cancers during preliminary studies. Researchers are comparing its benefits with those of more established therapies.

Endoscopic laser therapy involves delivering short, powerful laser treatments to the tumor through an endoscope. It can improve dysphagia, but multiple treatments are required, and the benefit is seldom long-lasting.

Prognosis

Since most patients are diagnosed when the cancer has spread to lymph nodes or other structures, the prognosis for esophageal cancer is poor. Generally, no more than half of all patients are candidates for curative treatment. Even if cure is attempted, the cancer can recur.

Prevention

There is no known way to prevent esophageal cancer.

Resources

BOOKS

Heitmiller, Richard F., Arlene A. Forastiere, and Lawrence R. Kleinberg. "Esophagus." In Clinical Oncology, edited by Martin D. Abeloff, 2nd ed. New York: Churchill Livingstone, 2000.

Zwischenberger, Joseph B., Scott K. Alpard, and Mark B.Orringer. "Esophageal Cancer." In Sabiston Textbook of Surgery, edited by Courtney Townsend, Jr., 16th ed. Philadelphia: W. B. Saunders Company, 2001.

ORGANIZATIONS

American Cancer Society. 1599 Clifton Road NE, Atlanta, GA 30329. (800)ACS-2345. http://www.cancer.org.

National Coalition for Cancer Survivorship. 1010 Wayne Avenue, 5th Floor, Suite 300, Silver Spring, MD 20910. Telephone: 1-888-650-9127.

KEY TERMS

Computed tomography A radiology test by which images of cross-sectional planes of the body are obtained.

Endoscopic ultrasound A radiology test utilizing high frequency sound waves, conducted via an endoscope.

Laparoscopy Examination of the contents of the abdomen through a thin, lighted tube passed through a small incision.

Positron emission tomography A radiology test by which images of cross-sectional planes of the body are obtained, utilizing the properties of the positron. The positron is a subatomic particle of equal mass to the electron, but of opposite charge.

Synergistic The combined action of two or more processes is greater than the sum of each acting separately.

Thoracoscopy Examination of the contents of the chest through a thin, lighted tube passed through a small incision.

Esophageal Cancer

views updated May 11 2018

Esophageal cancer

Definition

Esophageal cancer is a malignancy that develops in tissues of the hollow, muscular canal (esophagus) along which food and liquid travel from the throat to the stomach.

Description

Esophageal cancer usually originates in the inner layers of the lining of the esophagus and grows outward. In time, the tumor can obstruct the passage of food and liquid, making swallowing painful and difficult. Since most patients are not diagnosed until the late stages of the disease, esophageal cancer is associated with poor quality of life and low survival rates.

Squamous cell carcinoma is decreasing in incidence in the United States and Europe but is far more prevalent in developing countries. The esophagus is normally lined with thin, flat squamous cells that resemble tiny roof shingles. Squamous cell carcinoma can develop at any point along the esophagus but is most common in the middle portion.

Adenocarcinoma has been increasing, and, among white males in the United States and Western Europe, incidence of adenocarcinoma is more commonly diagnosed than squamous cell carcinoma. Adenocarcinoma originates in glandular tissue not normally present in the lining of the esophagus. Before adenocarcinoma can develop, glandular cells must replace a section of squamous cells. This occurs in Barrett's esophagus, a precancerous condition in which chronic acid reflux from the stomach stimulates a transformation in cell type in the lower portion of the esophagus.

A very small fraction of esophageal cancers are melanomas, sarcomas, or lymphomas.

Demographics

There is great variability in the incidence of esophageal cancer with regard to geography, ethnicity, and gender. The overall incidence was increasing as of 2008. It is the eighth most common malignancy in the world. About 16,470 new cases of esophageal cancer were anticipated to be diagnosed in the United States in 2008. It was estimated that 14,260 Americans would die of this disease in 2008. In the United States, cancers of the esophagus constitute 1% of all cancers and about 6% of gastrointestinal cancers. In some areas of Asia, southern and eastern Africa, and Northern France the cancer is endemic.

Squamous cell carcinoma usually occurs in the sixth or seventh decade of life, with a greater incidence in African Americans than in others. Adenocarcinoma develops earlier and is much more common in white patients. In general, esophageal cancer occurs more frequently in men than in women. In Western Hemisphere countries, such as the United States, the most common site of esophageal cancers has shifted to the lower third of the esophagus, where it often involves the gastroesophageal junction.

Causes and symptoms

Causes

The exact cause of esophageal cancer is unknown, although many investigators believe that chronic irritation of the esophagus is a major culprit. Most of the identified risk factors represent a form of chronic irritation. However, the wide variance in the distribution of esophageal cancer among different demographic groups raises the possibility that genetic factors also play a role.

Several risk factors are associated with esophageal cancer.

  • Tobacco and alcohol consumption are major risk factors, especially for squamous cell carcinoma. Smoking and alcohol abuse each increase the risk of squamous cell carcinoma five-fold. The effects of the two are synergistic, in that the combination of smoking and alcohol increases the risk by 25- to 100-fold. It is estimated that drinking about 13 ounces of alcohol every day for an extended period of time raises the risk of developing esophageal cancer by 18%. That likelihood increases to 44% in individuals who also smoke one or two packs of cigarettes a day. Smokeless tobacco also increases the risk for esophageal cancer.
  • Gastroesophageal reflux is a condition in which acid from the stomach refluxes backwards into the lower portion of the esophagus, sometimes causing symptoms of heartburn. In some cases of gastroesophageal reflux, the chronic exposure to acid causes the inner lining of the lower esophagus to change from squamous cells to glandular cells, a condition called Barrett's esophagus. Patients with Barrett's esophagus are roughly 30 to 40 times more likely than the general population to develop adenocarcinoma of the esophagus. The biggest risk factors for adenocarcinoma of the esophagus are the presence of gastroesophageal reflux disease (GERD) and Barrett's esophagus.
  • A diet low in fruits, vegetables, zinc, riboflavin, and other vitamins can increase risk of developing to esophageal cancer.
  • Caustic injury to the esophagus inflicted by swallowing lye or other substances that damage esophageal cells can lead to the development of squamous cell esophageal cancer in later life.
  • Achalasia is a condition in which the lower esophageal sphincter (muscle) cannot relax enough to let food pass into the stomach. Squamous cell esophageal cancer develops in about 6% of patients with achalasia.
  • Tylosis is a rare inherited disease characterized by excess skin on the palms and soles. Affected patients have a much higher probability of developing esophageal cancer than the general population. They should have regular screenings to detect the disease in its early, most curable stages.
  • Esophageal webs, which are protrusions of tissue into the esophagus, and diverticula, which are outpouchings of the wall of the esophagus, are associated with a higher incidence of esophageal cancer.

Symptoms

Unfortunately, symptoms generally do not appear until the tumor has grown so large that the patient cannot be cured. Dysphagia (trouble swallowing or a sensation of having food stuck in the throat or chest) is the most common symptom. Swallowing problems may occur occasionally at first, and patients often react by eating more slowly and chewing their food more carefully and, as the tumor grows, switching to soft foods or a liquid diet . Without treatment, the tumor will eventually prevent even liquid from passing into the stomach. A sensation of burning or slight mid-chest pressure is a rare, often-disregarded symptom of esophageal cancer. Painful swallowing is usually a symptom of a large tumor obstructing the opening of the esophagus. It can lead to regurgitation of food, weight loss , physical wasting, and malnutrition . Anyone who has trouble swallowing, loses a significant amount of weight without dieting, or cannot eat solid food because it is too painful to swallow should see a doctor.

Diagnosis

A barium swallow is usually the first test performed on a patient whose symptoms suggest esophageal cancer. After the patient swallows a small amount of barium, a series of x rays can highlight any bumps or flat raised areas on the normally smooth surface of the esophageal wall. It can also detect large, irregular areas that narrow the esophagus in patients with advanced cancer, but it cannot provide

Clinical staging

Stage 0 is the earliest stage of the disease. Cancer cells are confined to the innermost lining of the esophagus. Stage I esophageal cancer has spread slightly deeper, but still has not extended to nearby tissues, lymph nodes, or other organs. In stage IIA, cancer has invaded the thick, muscular layer of the esophagus that propels food into the stomach and may involve connective tissue covering the outside of the esophagus. In stage IIB, cancer has spread to lymph nodes near the esophagus and may have invaded deeper layers of esophageal tissue. Stage III esophageal cancer has spread to tissues or lymph nodes near the esophagus or to the trachea (windpipe) or other organs near the esophagus. Stage IV cancer has spread to distant organs such as the liver, bones, and brain. Recurrent esophageal cancer is disease that develops in the esophagus or another part of the body after initial treatment.

Treatment

Treatment for esophageal cancer is determined by the stage of the disease and the patient's general health. The most important distinction to make is whether the cancer is curable. If the cancer is in the early stages, cure may be possible. If the cancer is advanced or if the patient will not tolerate major surgery, treatment is usually directed at palliation (relief of symptoms only) instead of cure.

SURGERY The most common operations for the treatment of esophageal cancer are esophagectomy and esophagogastrectomy. Esophagectomy is the removal of the cancerous part of the esophagus and nearby lymph nodes. This procedure is performed only on patients with very early cancer that has not spread to the stomach. Esophagogastrectomy is the removal of the cancerous part of the esophagus, nearby lymph nodes, and the upper part of the stomach. The resected esophagus is replaced with the stomach or parts of intestine so the patient can swallow. These procedures can significantly relieve symptoms and improve the nutritional status of more than 80% of patients with dysphagia. Although surgery can cure some patients whose disease has not spread beyond the esophagus, more than 75% of esophageal cancers have spread to other organs before being diagnosed. Less extensive surgical procedures can be used for palliation.

QUESTIONS TO ASK YOUR DOCTOR

  • What type of esophageal cancer do I have?
  • How will my cancer be treated?
  • What are the side effects associated with my treatment?
  • How long will my treatments last?
  • Will I need a feeding tube or IV nutrition?
  • What is my prognosis?

CHEMOTHERAPY Oral or intravenous chemotherapy alone will not cure esophageal cancer, but preoperative treatments can shrink tumors and increase the probability that cancer can be surgically eradicated. Palliative chemotherapy can relieve symptoms of advanced cancer but will not alter the outcome of the disease.

RADIATION External beam or internal radiation, delivered by machine or implanted near cancer cells inside the body, is only rarely used as the primary form of treatment. Post-operative radiation is sometimes used to kill cancer cells that could not be surgically removed. Palliative radiation is effective in relieving dysphagia in patients who cannot be cured. However, radiation is most useful when combined with chemotherapy as either the definitive treatment or preoperative treatment.

Palliation

In addition to surgery, chemotherapy, and radiation, other palliative measures can provide symptomatic relief. Dilatation of the narrowed portion of the esophagus with soft tubes can provide short-term relief of dysphagia. Placement of a flexible, self-expanding stent within the narrowed portion is also useful in allowing more food intake.

Follow-up treatments

Regular barium swallows and other imaging studies are necessary to detect recurrence or spread of disease or new tumor development.

Clinical trials

Researchers are searching for more effective chemotherapeutic agents and radiation treatment regimens. Many studies are aimed at defining the most beneficial combination of surgery, chemotherapy, and radiation in the treatment of esophageal cancer. In 2008, there were over 100 active clinical trials in progress related to the treatment of esophageal cancer.

Alternative and complementary therapies

Photodynamic therapy (PDT) involves intravenously injecting a drug that is absorbed by cancer cells and kills them after they are exposed to specific laser beams. PDT can be used for palliation, but it also cured some early esophageal cancers during preliminary studies. As of 2008 researchers were comparing its benefits with those of more established therapies.

Endoscopic laser therapy involves delivering short, powerful laser treatments to the tumor through an endoscope. It can improve dysphagia, but multiple treatments are required, and the benefit is seldom long-lasting.

Nutrition and dietetic concerns

Difficulty swallowing and weight loss are the most common problems associated with esophageal cancer. Assessment of swallowing capability is paramount. Individuals who are able to swallow soft foods and/or liquid foods may benefit from high calorie liquid food supplements. Patients who are no longer able to swallow may require placement of feeding tubes or placement of special catheters that allow nutritional replacement intravenously. Patients are monitored closely to ensure their caloric and fluid needs are met.

Prognosis

Since most patients are diagnosed when the cancer has spread to lymph nodes or other structures, the prognosis for esophageal cancer is poor. While esophageal cancer is treatable, it is generally not curable. Even with treatment, five year survival rates ranged from 5% to 30% in 2008.

Prevention

There is no known way to prevent esophageal cancer. However, risk for esophageal cancer can be decreased by avoiding tobacco and excessive alcohol consumption, and by increasing the amounts of fruits and vegetables in the diet. Maintaining a healthy weight and prompt treatment for GERD may also decrease risk.

KEY TERMS

Computed tomography —A radiology test by which images of cross-sectional planes of the body are obtained.

Endoscopic ultrasound —A radiology test using high frequency sound waves, conducted via an endoscope.

Laparoscopy —Examination of the contents of the abdomen through a thin, lighted tube passed through a small incision.

Positron emission tomography (PET) —A radiology test by which images of cross-sectional planes of the body are obtained, using the properties of the positron. The positron is a subatomic particle of equal mass to the electron, but of opposite charge.

Synergistic —The combined action of two or more processes is greater than the sum of each acting separately.

Thoracoscopy —Examination of the contents of the chest through a thin, lighted tube passed through a small incision.

Caregiver concerns

Many cancer patients have found it helpful to discuss cancer and treatment with other cancer patients and survivors in support groups. Guidance from a nutritionist may be helpful to maintain a balanced diet and to ensure that the patient is receiving adequate nutritional support. The hospital staff and treatment team may be valuable resources for locating support groups and other community resources.

Resources

BOOKS

Yarbro, C. H., M. H. Frogge, and M. Goodman. Cancer Nursing Principles and Practice, 6th ed. Boston: Jones and Bartlett, 2005.

OTHER

“Esophageal Cancer Treatment.” National Cancer Institute (NCI) February 21, 2008 [April 9, 2008]. http:www.cancer.gov/cancertopics/pdq/treatment/esophageal/HealthProfessional/page2.

ORGANIZATIONS

American Cancer Society, PO Box 22718, Oklahoma City, OK, 73123-1718, (800) ACS-2345, https://www.cancer.org/.

National Cancer Institute, 6116 Executive Blvd., Room 3036A, Bethesda, MD, 20892-8322, (800) 422-6237, http://www.cancer.gov/.

National Coalition for Cancer Survivorship, 1010 Wayne Avenue, 5th Floor, Suite 300, Silver Spring, MD, 20910, (888) 650-9127, http://www.canceradvocacy.org/.

Maureen Haggerty

Kevin O. Hwang M.D.

Melinda Oberleitner R.N., D.N.S.

Esophageal Cancer

views updated May 23 2018

Esophageal cancer

Definition

Esophageal cancer is a malignancy that develops in tissues of the hollow, muscular canal (esophagus) along which food and liquid travel from the throat to the stomach.

Description

Esophageal cancer usually originates in the inner layers of the lining of the esophagus and grows outward. In time, the tumor can obstruct the passage of food and liquid, making swallowing painful and difficult. Since most patients are not diagnosed until the late stages of the disease, esophageal cancer is associated with poor quality of life and low survival rates.

Squamous cell carcinoma is the most common type of esophageal cancer, accounting for 95% of all esophageal cancers worldwide. The esophagus is normally lined with thin, flat squamous cells that resemble tiny roof shingles. Squamous cell carcinoma can develop at any point along the esophagus but is most common in the middle portion.

Adenocarcinoma has been increasing, and, among white males in the U.S., incidence of adenocarcinoma is almost equal to that of squamous cell carcinoma. Adeno-carcinoma originates in glandular tissue not normally present in the lining of the esophagus. Before adenocarcinoma can develop, glandular cells must replace a section of squamous cells. This occurs in Barrett's esophagus , a precancerous condition in which chronic acid reflux from the stomach stimulates a transformation in cell type in the lower portion of the esophagus.

A very small fraction of esophageal cancers are melanomas, sarcomas , or lymphomas.

Demographics

There is great variability in the incidence of esophageal cancer with regard to geography, ethnicity, and gender. The overall incidence is increasing. About 13, 000 new cases of esophageal cancer are diagnosed in the United States each year. During the same 12-month period, 12, 000 people die of this disease. It strikes between five and ten North Americans per 100, 000. In some areas of China the cancer is endemic.

Squamous cell carcinoma usually occurs in the sixth or seventh decade of life, with a greater incidence in African-Americans than in others. Adenocarcinoma develops earlier and is much more common in white patients. In general, esophageal cancer occurs more frequently in men than in women.

Causes and symptoms

Causes

The exact cause of esophageal cancer is unknown, although many investigators believe that chronic irritation of the esophagus is a major culprit. Most of the identified risk factors represent a form of chronic irritation. However, the wide variance in the distribution of esophageal cancer among different demographic groups raises the possibility that genetic factors also play a role.

Several risk factors are associated with esophageal cancer.

  • Tobacco and alcohol consumption are the major risk factors, especially for squamous cell carcinoma. Smoking and alcohol abuse each increase the risk of squamous cell carcinoma by five-fold. The effects of the two are synergistic, in that the combination of smoking and alchohol increases the risk by 25-to 100-fold. It is estimated that drinking about 13 ounces of alcohol every day for an extended period of time raises the risk of developing esophageal cancer by 18%. That likelihood increases to 44% in individuals who also smoke one or two packs of cigarettes a day. Smokeless tobacco also increases the risk for esophageal cancer.
  • Gastroesophageal reflux is a condition in which acid from the stomach refluxes backwards into the lower portion of the esophagus, sometimes causing symptoms of heartburn. In some cases of gastroesophageal reflux, the chronic exposure to acid causes the inner lining of the lower esophagus to change from squamous cells to glandular cells. This is called Barrett's esophagus. Patients with Barrett's esophagus are roughly 30 to 40 times more likely than the general population to develop adenocarcinoma of the esophagus.
  • A diet low in fruits, vegetables, zinc, riboflavin, and other vitamins can increase risk of developing to esophageal cancer.
  • Caustic injury to the esophagus inflicted by swallowing lye or other substances that damage esophageal cells can lead to the development of squamous cell esophageal cancer in later life.
  • Achalasia is a condition in which the lower esophageal sphincter (muscle) cannot relax enough to let food pass into the stomach. Squamous cell esophageal cancer develops in about 6% of patients with achalasia.
  • Tylosis is a rare inherited disease characterized by excess skin on the palms and soles. Affected patients have a much higher probability of developing esophageal cancer than the general population. They should have regular screenings to detect the disease in its early, most curable stages.
  • Esophageal webs, which are protrusions of tissue into the esophagus, and diverticula, which are outpouchings of the wall of the esophagus, are associated with a higher incidence of esophageal cancer.

Symptoms

Unfortunately, symptoms generally don't appear until the tumor has grown so large that the patient cannot be cured. Dysphagia (trouble swallowing or a sensation of having food stuck in the throat or chest) is the most common symptom. Swallowing problems may occur occasionally at first, and patients often react by eating more slowly and chewing their food more carefully and, as the tumor grows, switching to soft foods or a liquid diet. Without treatment, the tumor will eventually prevent even liquid from passing into the stomach. A sensation of burning or slight mid-chest pressure is a rare, often-disregarded symptom of esophageal cancer. Painful swallowing is usually a symptom of a large tumor obstructing the opening of the esophagus. It can lead to regurgitation of food, weight loss , physical wasting, and malnutrition. Anyone who has trouble swallowing, loses a significant amount of weight without dieting, or cannot eat solid food because it is too painful to swallow should see a doctor.

Diagnosis

A barium swallow is usually the first test performed on a patient whose symptoms suggest esophageal cancer. After the patient swallows a small amount of barium, a series of x rays can highlight any bumps or flat raised areas on the normally smooth surface of the esophageal wall. It can also detect large, irregular areas that narrow the esophagus in patients with advanced cancer, but it cannot provide information about disease that has spread beyond the esophagus. A double contrast study is a barium swallow with air blown into the esophagus to improve the way the barium coats the esophageal lining. Endoscopy is a diagnostic procedure in which a thin lighted tube (endoscope) is passed through the mouth, down the throat, and into the esophagus. Cells that appear abnormal are removed for biopsy . Once a diagnosis of esophageal cancer has been confirmed through biopsy, staging tests are performed to determine whether the disease has spread (metastasized) to tissues or organs near the original tumor or in other parts of the body. These tests may include computed tomography , endoscopic ultrasound, thoracoscopy , laparoscopy , and positron emission tomography .

Treatment team

The treatment team includes the surgeon, radiolo-gist, radiation therapist, and oncologist. Nutrition therapists are also vital in optimizing a diet that the patient can swallow easily.

Clinical staging, treatments, and prognosis

Staging

Stage 0 is the earliest stage of the disease. Cancer cells are confined to the innermost lining of the esophagus. Stage I esophageal cancer has spread slightly deeper, but still has not extended to nearby tissues, lymph nodes, or other organs. In Stage IIA, cancer has invaded the thick, muscular layer of the esophagus that propels food into the stomach and may involve connective tissue covering the outside of the esophagus. In Stage IIB, cancer has spread to lymph nodes near the esophagus and may have invaded deeper layers of esophageal tissue. Stage III esophageal cancer has spread to tissues or lymph nodes near the esophagus or to the trachea (windpipe) or other organs near the esophagus. Stage IV cancer has spread to distant organs like the liver, bones, and brain. Recurrent esophageal cancer is disease that develops in the esophagus or another part of the body after initial treatment.

Treatment

Treatment for esophageal cancer is determined by the stage of the disease and the patient's general health. The most important distinction to make is whether the cancer is curable. If the cancer is in the early stages, cure may be possible. If the cancer is advanced or if the patient will not tolerate major surgery, treatment is usually directed at palliation (relief of symptoms only) instead of cure.

SURGERY.

The most common operations for the treatment of esophageal cancer are esophagectomy and esophagogastrectomy. Esophagectomy is the removal of the cancerous part of the esophagus and nearby lymph nodes. This procedure is performed only on patients with very early cancer that has not spread to the stomach. Esophagogastrectomy is the removal of the cancerous part of the esophagus, nearby lymph nodes, and the upper part of the stomach. The resected esophagus is replaced with the stomach or parts of intestine so the patient can swallow. These procedures can significantly relieve symptoms and improve the nutritional status of more than 80% of patients with dysphagia. Although surgery can cure some patients whose disease has not spread beyond the esophagus, but more than 75% of esophageal cancers have spread to other organs before being diagnosed. Less extensive surgical procedures can be used for palliation.

CHEMOTHERAPY.

Oral or intravenous chemotherapy alone will not cure esophageal cancer, but pre-operative treatments can shrink tumors and increase the probability that cancer can be surgically eradicated. Palliative chemotherapy can relieve symptoms of advanced cancer but will not alter the outcome of the disease.

RADIATION.

External beam or internal radiation, delivered by machine or implanted near cancer cells inside the body, is only rarely used as the primary form of treatment. Post-operative radiation is sometimes used to kill cancer cells that couldn't be surgically removed. Palliative radiation is effective in relieving dysphagia in patients who cannot be cured. However, radiation is most useful when combined with chemotherapy as either the definitive treatment or preoperative treatment.

Palliation

In addition to surgery, chemotherapy, and radiation, other palliative measures can provide symptomatic relief. Dilatation of the narrowed portion of the esophagus with soft tubes can provide short-term relief of dysphagia. Placement of a flexible, self-expanding stent within the narrowed portion is also useful in allowing more food intake.

Follow-up treatments

Regular barium swallows and other imaging studies are necessary to detect recurrence or spread of disease or new tumor development.

Prognosis

Since most patients are diagnosed when the cancer has spread to lymph nodes or other structures, the prognosis for esophageal cancer is poor. Generally, no more than half of all patients are candidates for curative treatment. Even if cure is attempted, the cancer can recur.

Alternative and complementary therapies

Photodynamic therapy (PDT) involves intravenously injecting a drug that is absorbed by cancer cells and kills them after they are exposed to specific laser beams. PDT can be used for palliation, but it also cured some early esophageal cancers during preliminary studies. Researchers are comparing its benefits with those of more established therapies.

Endoscopic laser therapy involves delivering short, powerful laser treatments to the tumor through an endoscope. It can improve dysphagia, but multiple treatments are required, and the benefit is seldom long-lasting.

Coping with cancer treatment

Many cancer patients have found it helpful to discuss cancer and treatment with other cancer patients and survivors in support groups. Guidance from a nutritionist may be helpful to maintain a balanced diet and to ensure that the patient is receiving adequate nutritional support . The hospital staff and treatment team may be valuable resources for locating support groups and other community resources.

Clinical trials

Researchers are searching more effective chemotherapeutic agents and radiation treatment regimens. Many studies are aimed at defining the most beneficial combination of surgery, chemotherapy, and radiation in the treatment of esophageal cancer.

Prevention

There is no known way to prevent esophageal cancer.

Resources

BOOKS

Heitmiller, Richard F., Arlene A. Forastiere, Lawrence R. Kleinberg. "Esophagus." In Clinical Oncology, edited by Martin D. Abeloff, second ed. New York: Churchill Livingstone, 2000, pp.1517-1539.

Zwischenberger, Joseph B., Scott K. Alpard, and Mark B. Orringer. "Esophageal Cancer." In Sabiston Textbook of Surgery, edited by Courtney Townsend Jr., 16th ed. Philadelphia: W.B. Saunders Company, 2001, pp.731-749.

ORGANIZATIONS

American Cancer Society. "Esophageal Cancer." <http://www3.cancer.org> 6 July 2001.

National Coalition for Cancer Survivorship. 1010 Wayne Avenue, 5th Floor, Suite 300, Silver Spring, MD 20910. Telephone: 1-888-650-9127.

Kevin O. Hwang, M.D.

KEY TERMS

Computed tomography

A radiology test by which images of cross-sectional planes of the body are obtained.

Endoscopic ultrasound

A radiology test utilizing high frequency sound waves, conducted via an endoscope.

Laparoscopy

Examination of the contents of the abdomen through a thin, lighted tube passed through a small incision.

Positron emission tomography

A radiology test by which images of cross-sectional planes of the body are obtained, utilizing the properties of the positron. The positron is a subatomic particle of equal mass to the electron, but of opposite charge.

Synergistic

The combined action of two or more processes is greater than the sum of each acting separately.

Thoracoscopy

Examination of the contents of the chest through a thin, lighted tube passed through a small incision.