Esophageal cancer usually develops in persons between 50 and 70 years of age. There were an estimated 18,440 new cases of esophageal cancer in the United States in 2020. The overall ratio of men to women is 3:1. There are two histologic types: squamous cell carcinoma and adenocarcinoma, and their incidence has significant geographic variation. Squamous cell carcinoma is associated with smoking, alcohol, poor nutritional status, and drinking hot beverages. It accounts for over 90% of cases of esophageal cancer in Eastern and Southeast Asia and sub-Saharan Africa. Adenocarcinoma is associated with obesity and gastroesophageal reflux disease, with the majority of cases developing as a complication of Barrett metaplasia due to chronic gastroesophageal reflux(eFigure 39–1). Adenocarcinomas make up the majority of new cases of esophageal cancer in North America and Northern and Western Europe. In the United States, squamous cell carcinoma is much more common in Blacks than in Whites. Additionally, in countries with lower incidence of squamous cell carcinoma, it is estimated that up to 90% of cases are due to modifiable risk factors such as smoking, alcohol, and diets low in fruits and vegetables. Most (90%) squamous cell carcinomas occur in the distal two-thirds of the esophagus(eFigure 39–2), whereas adenocarcinomas are more common at the gastroesophageal junction. The risk of squamous cell cancer is also increased in patients with tylosis (a rare disease transmitted by autosomal dominant inheritance and manifested by hyperkeratosis of the palms and soles), achalasia, caustic-induced esophageal stricture, and other head and neck cancers. There are conflicting data about whether HPV may play a role in the pathogenesis of some esophageal squamous cell carcinomas.
An ulcerated mass with submucosal extension is visible in the mid-esophagus amidst columnar epithelium (Barrett esophagus). Biopsies confirmed adenocarcinoma. (Used, with permission, from Y. Chen.)
An exophytic mass in visualized in the distal esophagus. Biopsies confirmed poorly differentiated squamous cell carcinoma. (Used, with permission, from Y. Chen.)
The majority (50–60%) of patients with esophageal cancer present with advanced, incurable disease. While early symptoms are nonspecific and subtle, over 90% eventually have solid food dysphagia, which progresses over weeks to months. Odynophagia is sometimes present. Significant weight loss is common. Local tumor extension into the tracheobronchial tree may result in a tracheo-esophageal fistula, characterized by coughing on swallowing or by pneumonia. Chest or back pain suggests mediastinal extension. Recurrent laryngeal nerve involvement may produce hoarseness. Physical examination is often unrevealing. The presence of supraclavicular or cervical lymphadenopathy or of hepatomegaly implies metastatic disease.