Key Points

• Benign esophageal tumors are rare.
• Most are asymptomatic and probably do not require any treatment other than observation and follow-up.
• Accurate diagnosis is the key to effective management of benign tumors.
• If removal of the tumor is required, then extramucosal resection by enucleation is usually sufficient.

Benign esophageal tumors are rare, and many are asymptomatic. They will be found with increasing frequency as radiologic and endoscopic investigations become more commonplace, and they may result in significant morbidity if untreated or treated inappropriately. The exact incidence is difficult to determine because so many of these tumors are asymptomatic. In one autopsy series only 90 cases of a benign esophageal tumor were found in nearly 20,000 autopsies over a 50-year period.[1] From 11,000 patients complaining of dysphagia, only 15 cases were found in another series.[2] Benign tumors probably account for less than 1% of all esophageal tumors and account for less than 10% of all surgically resected esophageal tumors.[3]

Benign esophageal tumors are probably best classified according to their location within the wall of the esophagus (i.e., intraluminal, submucosal, intramural, or extraesophageal) (Table 1). They may also be classified according to their histologic type (i.e., epithelial, nonepithelial, or heterotopic). The smooth muscle leiomyoma is by far the most common benign tumor of the esophagus and is almost always intramural. Extraesophageal congenital cysts and duplications are the second most common lesions, but these are not true neoplasms. Intraluminal esophageal polyps are the next most common tumor overall. All other types are quite rare. Gastrointestinal stromal tumors (GISTs) are well-defined mesenchymal tumors of the gastrointestinal tract that are quite distinct from esophageal leiomyoma and are not discussed extensively here.[4]

The majority of these benign tumors are located in the middle and lower thirds of the thoracic esophagus. Tumors arising from the cervical esophagus are less common except for the fibrovascular polyps, which are more common in the cervical esophagus.