Esophageal cancer is the sixth leading cause of cancer death worldwide. The incidence varies substantially in different countries. The highest rates are seen in China, Singapore, Iran, South Africa, France, and Puerto Rico.

In the United States, the incidence of esophageal cancer for the year 2006 was 14,550 new cases and 13,770 deaths. It is the seventh leading cause of cancer death in American men. In the last 25 years, there has been a significant increase in the incidence of adenocarcinomas of the distal esophagus and gastroesophageal junction. Also, in the last 30 years, the incidence of adenocarcinoma of the esophagus has increased in men and the incidence of squamous carcinoma of the esophagus has declined.

The incidence of esophageal carcinoma increases with age. The median age of onset is 69 years. Males are 2 to 4 times more likely to develop esophageal cancer compared to females. Squamous cell carcinoma is more common in African Americans and adenocarcinoma is more common among Caucasians. The incidence rates within the United States are higher in the Northeast and urban areas.

Approximately 10% to 15% squamous cancers originate in the upper third, 35% to 40% in the middle third, and 40% to 50% in the distal third of the esophagus. Adenocarcinomas arise predominately in the distal esophagus and are commonly associated with Barrett’s esophagus.

Esophageal cancers may develop as second primary tumors in patients with other primary tumors of the upper aerodigestive tract. Between 5% and 12% of patients with esophageal cancer are found to have synchronous or metachronous aerodigestive tract cancer.

Other less common tumors of the esophagus include lymphoma, carcinosarcoma, pseudosarcoma, squamous adenocarcinoma, melanoma, mucoepidermoid carcinoma, squamous cell papilloma, primary small cell carcinoma, verrucous carcinoma, and malignant carcinoid tumor the esophagus. Local spread from the lung and thyroid and metastasis from distant cancers may occur but are rare.

Chronic use of alcohol and smoking are associated with esophageal carcinoma. This may be due to chronic irritation of the esophageal mucosa with these agents. Other conditions with increased prevalence of esophageal carcinoma are lye strictures, achalasia, previous exposure to ionizing radiation, head and neck cancer, Plummer-Vinson syndrome, tylosis, celiac sprue, and Barrett’s epithelium.

Squamous cell carcinoma accounts for less than one half of esophageal carcinomas. Adenocarcinomas of the esophagus, which used to account for less than 10% of esophageal cancers, now account for greater than two thirds of all esophageal malignancies in the United States. Adenocarcinoma usually arises from metaplastic columnar epithelium (Barrett’s epithelium) and rarely from esophageal glands. Adenocarcinoma of the stomach may spread to the esophagus by extension.

Gastroesophageal reflux disease (GERD) is thought to be the major risk factor for esophageal adenocarcinoma. Recurrent symptoms of GERD seem to increase the risk of esophageal adenocarcinoma by eightfold. The annual incidence of cancer in Barrett’s epithelium is approximately 0.8%. Anticholinergic calcium channel blockers, nitrates, and theophyllines, by decreasing the lower esophageal sphincter tone, are thought to increase the risk of adenocarcinomas of the esophagus. In addition, obesity, which increases intraabdominal pressure and GERD, is an added risk factor.

The 5-year survival rate for all patients is approximately 16%. The 5-year survival even with the earliest stages of cancer is only 50% to 80% and with lymph node involvement it drops to below 25%. With locally advanced esophageal cancer the survival drops to 5% to 10% (with radiation or surgery alone) and with chemoradiation and surgery, it may be 25% to 27%.

Esophageal cancers grow extensively locally and invade adjacent structures. The tumor has the propensity to spread longitudinally via lymphatic channels within the esophageal wall to mediastinal cervical and celiac lymph nodes. There may also be hematogenous spread to lungs, liver, and other organs. Esophagobronchial or esophagopleural fistulas may form and manifest as recurrent pneumonia or abscess. Erosion into the aorta may result in exsanguination.

Progressive dysphagia for less than a year, first with solids then with semisolids and liquids, is the most common symptom. Substernal pain, usually steady, radiating to the back may also be present and may suggest periesophageal spread of the tumor. Most patients complain of anorexia and profound weight loss. Patients may have iron-deficiency anemia from blood loss from the lesion, but brisk bleeding is rare. Hoarseness may result from involvement of the recurrent laryngeal nerve. If the lesion is obstructive, patients may aspirate esophageal contents and may present with aspiration pneumonia and pleural effusion. Horner’s syndrome, cervical adenopathy, hpeatoroegalys, boney pain, and paraneoplastic syndromes including hypercalcemia, inappropriate ACTH, and gonadotropins may be present.