Esophageal submucosal gland duct adenoma with multiple surface ductal opening

Esophageal submucosal gland duct adenomas (ESGDAs) are extremely rare benign tumors originating from submucosal gland (SMG) duct clusters, with only 26 reported cases that account for less than 1% of all esophageal tumors. Since esophageal SMGs are located deep in the submucosa, ESGDA with multiple surface ductal opening is even rarer, with only a few cases reported, and it exhibits very distinctive endoscopic, and pathological features. Unlike previously reported cases, our case had several pinhole-like orifices on the surface, which corresponded to the openings of the SMG ducts in pathology and indicated it communicating with the overlying squamous epithelium. Because of the rarity and the lack of specific clinical, endoscopic and ultrasound manifestations, none of the reported ESGDAs could be correctly diagnosed before pathological analysis and they are easily mistaken for even adenocarcinomas. We present a clinically instructive and exceptionally rare ESGDA with multiple surface ductal opening removed by endoscopic submucosal dissection. The diagnosis was definitively established through the systematic application of endoscopic imaging, endoscopic ultrasound manifestation and histopathological analysis. We describe this case, aiming to enhance the understanding of this rare tumor and prevent misdiagnosis and over-treatment.