Esophageal Granular Cell Tumor: A Benign Tumor or an Insidious Cause for Concern?

Esophageal granular cell tumors (GCTs) are rare, often benign tumors of neurogenic origin. GCTs most frequently occur in the skin and subcutaneous tissues but are found in the gastrointestinal (GI) tract in 6%-10% of cases, with the distal two-thirds of the esophagus being the most common site. Owing to the insidious nature of GCTs, presentation is typically asymptomatic. In fact, GCTs are often discovered incidentally during investigation of other GI disturbances.

We report the case of a 36-year-old white male who had a 2.3 × 2.0-cm submucosal mass of the midesophagus found during esophagogastroduodenoscopy (EGD) at an outside hospital for workup of chronic diarrhea. He was referred to us for further evaluation that led to a diagnosis of a large esophageal GCT.

Because of the rarity of GCTs in clinical practice and their poorly defined malignant classification, proper workup and management are essential to avoid the potential morbidity and mortality associated with large and/or malignant tumors. Although malignancy is uncommon, approximately 1%-2% of esophageal GCTs are malignant. Conservative management is tolerated for benign, asymptomatic lesions <10 mm in diameter, but endoscopic removal is recommended for large, symptomatic tumors or those with features suggestive of malignancy. Routine surveillance often includes EGD and/or esophageal ultrasonography to evaluate tumor size, location, and depth and to exclude malignancy or lymph node involvement.