Sporadic ganglioneuromatosis of esophagogastric junction in a patient with gastro-esophageal reflux disorder and intestinal metaplasia

World Journal of Gastroenterology : WJG
Richard SideritsJanusz-J Godyn

Abstract

A 58-year-old female with a recurrent history of upper abdominal pain and intermittent dysphagia underwent endoscopic evaluation that demonstrated an irregular and nodular esophago-gastric (EG) junction and grade I erosive esophagitis. Biopsies showed prominent intestinal metaplasia of Barrett's type without dysplasia, chronic inflammation and multiple aggregates of large cells within the mucosal lamina propria, some with spindle shaped nuclei. Immunohistochemistry stains for keratins AE-1/AE-3 were negative, while S-100 and NSE were positive. This, together with routine stains, was diagnostic for mucosal ganglioneuromatosis. The background of chronic inflammation with intestinal type metaplasia was consistent with long-term reflux esophagitis. No evidence of achalasia was seen. Biopsies of gastric antrum and fundus were unremarkable, without ganglioneural proliferation. Colonoscopy was unremarkable. No genetic syndromes were identified in the patient including familial adenomatous polyposis and multiple endocrine neoplasia type IIb (MEN IIb). Iansoprazole (Prevacid) was started by oral administration each day with partial relief of symptoms. Subsequent esophagogastroscopy repeated at 4 mo showed normal appearing EG junction. E...Continue Reading

References

Oct 1, 1978·Australian and New Zealand Journal of Medicine·J A CuthbertJ R Turtle
Feb 23, 1978·Gastrointestinal Radiology·M E GershwindL R Ferraro
Nov 14, 1997·The Annals of Thoracic Surgery·R Lee, W A Williamson

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Citations

Oct 9, 2018·World Journal of Clinical Cases·Yu MiaoSheng-Qiang Cai
Nov 26, 2008·World Journal of Gastroenterology : WJG·J-Elliot Carter, Javier-A Laurini

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