1723 Esophageal Granular Cell Tumour: An Unusual Polypoid Lesion

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INTRODUCTION: Esophageal granular cell tumours (GCTs) account for 1% of all Esophageal tumors and up to two-third occur in the distal third of Esophagus. Since first reported in 1931 approximately 400 cases have been reported so far. We report a case of Esophageal GCT incidentally found on endoscopic evaluation for resistant GERD. CASE DESCRIPTION/METHODS: A 42-year-old Hispanic male was seen for evaluation of refractory GERD and occasional dysphagia to solids of 3-month duration. There were no alarm symptoms or use of NSAIDs. Physical examination and labs were unremarkable. EGD was performed that showed a 4mm yellowish, sessile, subepithelial nodule with intact mucosa in the distal Esophagus (Figure 1). Biopsy showed clusters of polygonal cells with abundant granular cytoplasm that was Periodic acid–Schiff (PAS) positive suggestive of GCT. Immunohistochemical staining was positive for S100, CD 68, CD56 and Vimentin, thus conforming the diagnosis of GCT (Figure 2). EUS performed subsequently showed a 4mm, submucosal, hypoechoic, homogenous mass with no extension into muscularis propria (Figure 3); completely removed by hot snare technique with pathological conformation. Patient was discharged home with close endoscopic surveillance. DISCUSSION: Granular Cell Tumors (GCTs) arise from Schwann cells of the nerve sheath with incidence of about 0.03% and has a predilection towards skin, oral cavity and breasts. Of all GCTs, 6-8% occur in the GI tract with esophagus (65%) being the most common site. It has predilection towards females and African Americans between 40-60 years of age. Generally asymptomatic and found incidentally during endoscopy. However, GERD and dysphagia are associated with GCTs of more than 2 cm in size. EUS is invaluable in differentiating GCTs from other subepithelial tumors, determining size, depth and invasion. Biopsy/FNA can be performed during the initial endoscopy or during EUS. Definitive diagnosis is by histopathology and immunohistochemical staining. The treatment of esophageal GCTs is controversial. Conservative management is recommended for tumors <1 cm, asymptomatic, no deep tissue invasion or malignancy. However, resection is warranted for symptomatic tumors, >1 cm size, with deep tissue invasion or malignancy. Endoscopic removal is preferred over surgery for tumors without deep tissue infiltration or malignant. Endoscopic and histologic surveillance every 1-2 years is recommended.