Background
Benign tumors of the esophagus are rare lesions that constitute less than 1% of esophageal neoplasms. Nearly two thirds of benign esophageal tumors are leiomyomas; the others mostly are polyps and cysts. Thus, leiomyomas are the most common benign tumors of the esophagus. [1]
Pathophysiology
Leiomyomas represent a hyperproliferation of interlacing bundles of smooth muscle cells that are well-demarcated by adjacent tissue or by a smooth connective tissue capsule. They usually arise as intramural growths, most commonly along the distal two thirds of the esophagus. They are multiple in less than 3% of patients. [2]
The majority of leiomyomas have been discovered incidentally during evaluation for dysphagia or during autopsy. Bleeding rarely occurs in cases of benign disease but typically is observed with leiomyosarcoma, the malignant counterpart of this tumor. The potential for malignant degeneration of leiomyomas is extremely small. In the distal esophagus, leiomyomas may reach large proportions and may encroach on the cardia of the stomach.
In a retrospective review of the Cleveland Clinic pathology database, Jiang et al investigated the clinicopathologic features of esophageal leiomyomas. Analyzing 30 cases from 28 patients, the investigators found mean tumor sizes among symptomatic and asymptomatic patients to be 5.2 cm and 0.4 cm, respectively. In symptomatic patients, the most common complaint was dysphagia (71.4%). The majority of esophageal leiomyomas (69%) were found in the distal and middle thirds of the esophagus, with most (69.6%) developing from the muscularis propria layer. For the most part, the tumors were made up of bland spindle cells with low cellularity and lacked both nuclear atypia and mitotic activity; cells were found to be positive for smooth muscle actin and negative for the CD34 and CD117 proteins. Among the 22 patients (78.6%) for whom follow-up information was available, no leiomyoma-related adverse events were found. [1]
Epidemiology
Incidence
Esophageal leiomyomas comprise approximately 0.4% of all esophageal neoplasms. [3]
Age-, sex-, and race-related demographics
Esophageal leiomyomas typically occur in individuals aged 20-50 years; the male-to-female ratio is approximately 2:1. [4]
No known race predilection is recognized.
