455 Esophageal Granular Cell Tumors: A Retrospective Institutional Analysis

Abstract

INTRODUCTION: Granular Cell Tumor (GCT) is generally a benign neoplasm derived from Schwann cells. Less than ten percent of GCTs are found in the gastrointestinal (GI) tract and most common site in the GI tract has been reported to be the esophagus. The current existing literature is limited. Given the rarity of these lesions, the management of GCTs remain a clinical challenge for gastroenterologists. Here we present a case series of thirteen esophageal GCTs and their characteristics, diagnosis and management. METHODS: We conducted a retrospective review of all cases of pathologically confirmed GCTs from 2010 to 2018 at our institution from our endoscopic pathology database. Data were collected regarding demographics, endoscopic, endosonographic appearance and presence of any malignant transformation. RESULTS: Thirteen total patients were identified. Mean age was 43.6 years. Six patients were females. All esophageal lesions were noted to be in the mid-distal esophagus. Majority of the lesions were subcentimeter. Mean size of the lesions was 8.2 mm. Most common endoscopic features were nodularity (11/13 cases) and white-yellow appearance (8/13). Endoscopic ultrasound (EUS) was conducted in 11/13 cases and the lesions appeared hypoechoic in the majority of cases (6/11). Extension of the lesion was limited to the submucosa in all except one case. Endoscopic mucosal resection (EMR) was performed in 8/13 cases. Pathologic diagnosis of GCT was confirmed using immunohistochemical stain of S100 in 8/13 cases. None of the cases showed any evidence of malignancy from the initial endoscopic resection or biopsies. CONCLUSION: Our institutional review of esophageal cases of GCTs not only highlights the rarity of GCTs, but provides endoscopic and endosonographic features of esophageal GCT. Based on our cohort of patients, GCTs most commonly present in mid-distal esophagus with a white-yellowish hue. Patients are generally asymptomatic. EUS findings suggest lesions are hypoechoic and arise from the submucosal layer. Given this location, lesions can be removed in entirety through EMR, which will also allow for greater depth of resection. Overall, mucosectomy may serve as an adequate modality for removal of these apparent benign lesions. Our preliminary finding delineates the success of en bloc resectability of esophageal GCTs less than 2 cm in size. Additional studies would be needed to better establish a more definitive management algorithm of esophageal GCTs and the role of endoscopic mucosal resection.