Multiple Esophageal Leiomyomas

Abstract

An 18-year-old male with profound developmental delay was evaluated for recurrent vomiting. Acid suppression therapy did not alleviate his symptoms. An upper endoscopy showed 5 polypoid lesions in the mid- and upper esophagus (Fig. 1). Cold biopsies showed stratified squamous epithelium with submucosal bland spindled cell proliferation (Fig. 2). Smooth muscle myosin staining was positive (Fig. 3). These findings were diagnostic of esophageal leiomoymas.FIG. 1: Polypoid lesions in the mid esophagus.FIG. 2: H&E staining show submucosal bland spindled cell proliferation.FIG. 3: Positive smooth muscle myosin staining (brown).Leiomyomas are the most common benign esophageal tumors usually affecting the distal two thirds of the esophagus. In 5% of patients, they can be multiple (1). This should be differentiated from leiomyomatosis, which is characterized by diffuse hypertrophy of the muscular layer (2). Although a leiomyoma is the most likely etiology of an esophageal polypoid lesion in a child, other diagnoses are possible that rarely include malignancy, so histological evaluation is often recommended (3,4). Esophageal leiomyomas are rarely symptomatic when small (<5 cm in diameter). Large tumors can present with dysphagia, chest pain, obstruction, regurgitation, and, rarely, bleeding. Histologically, leiomyomas are composed of bundles of interlacing smooth muscle cells with a connective tissue capsule covered by mucosa (5). Asymptomatic or small lesions can be followed periodically as they have a slow growth rate and negligible risk of malignant transformation. Surgical excision is recommended for symptomatic leiomyomas and those >5 cm (1).