Autoimmune Esophagitis: IgG4-related Tumors of the Esophagus |
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Authors: | James Lopes Steven N. Hochwald Nicholas Lancia Lisa R. Dixon Kfir Ben-David |
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Affiliation: | (1) Department of Surgery, University of Florida College of Medicine, 1600 SW Archer Road, P.O. Box 100109, Gainesville, FL 32610, USA;(2) Department of Pathology, Immunology and Laboratory Medicine, University of Florida College of Medicine, Gainesville, FL 32610, USA; |
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Abstract: | We present a case of a 23-year-old gentleman who presented with dysphagia, weight loss, and recurrent esophageal strictures requiring multiple dilatations. An endoscopic ultrasound with esophagogastroduodenoscopy revealed a mass present in the distal esophagus. Fine needle aspiration suggested that the mass in the lower esophagus resembled a gastrointestinal stromal tumor. After surgical resection, final pathologic analysis revealed that the tumor was comprised of benign-appearing fibroinflammatory cells with an increase and predominance of IgG4-positive plasma cells. The microscopic appearance was consistent with a benign condition as a result of an IgG4-related process. He did not, however, have any other symptoms indicative of systemic autoimmune disease or connective tissue disorders. We present the pre-operative imaging, operative management, pathologic diagnosis, and literature review of this rare condition and the first known report of autoimmune esophagitis as part of the IgG4 spectrum of diseases. |
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