Granular Cell Tumor of the Esophagus

Abstract

Introduction: Granular cell tumor (GCT) is a rare soft tissue neoplasm originating from the nervous system. Most of them are benign and only 1 to 2% are malignant. GCT is relatively rare in the gastrointestinal tract, accounting for approximately 8% of all GCT’s. Of these only about 2% occur in the esophagus. A 58-year-old asymptomatic man underwent Esophagogastroduodenoscopy (EGD) for evaluation of iron deficiency anemia. EGD revealed submucosal appearing nodule in the distal esophagus apart from mild antral erythema. Antral Biopsy was positive for H. pylori while initial biopsy from the esophageal nodule showed changes indefinite for atypia. Endoscopic ultrasound was performed for further evaluation which showed an 8 millimeter nodule with subtle thickening of the upper one-third of the submucosal layer. Biopsy of the nodule showed sheets of uniform epitheloid cells with abundant eosinophilic cytoplasm and fairly small nuclei in the subepithelial connective tissue. The epitheloid cells showed strong diffuse positivity for S100. CD 117 highlighted scattered mast cells, but were negative within the epitheloid cells. Actin highlighted bundles of smooth muscle, but were negative within the epitheloid cells. The histomorphological features and staining profile were consistent with granular cell tumor of esophagus. As patient was asymptomatic and tumor size was less than 1 cm, no intervention was performed and he was scheduled for follow up EGD after one year. Endoscopy ultrasonography (EUS) is not only of great help in identification of the size and location of the lesion, but also in determining the choice of treatment. A lesion that is asymptomatic and less than 1 cm in diameter requires the conservative approach with the regular endoscopy and EUS follow-up; whereas endoscopy or surgical resection should be performed for tumors size 1 cm or greater, tumor with rapid recent growth, suspicion of malignancy, transmural infiltration or tumor producing symptoms such as dysphagia.Figure 1: EGD and EUS images of the granular cell tumor.