Sporadic diffuse gastric ganglioneuromatosis causing gastric outlet obstruction in an adult patient: A rare case report and literature review

Abstract

: Gastrointestinal ganglioneuromatosis is a rare benign neural neoplasm, consists of hyperplasia of nerve fibers, ganglion cells, and schwann cells in the wall of stomach and intestine. The disease may affect any part of the gastrointestinal tract but involvement of stomach is extremely unusual. Mainly are of three types: polypoid ganglioneuromas, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. To our knowledge, only two cases were reported as diffuse ganglioneuromatosis located in the stomach.: 27-years male patient, admitted with complaint of dysphagia for both solid and liquid and recurrent non-bilious vomiting since 1 week underwent Billroth - 1 procedure and antral segment of stomach was sent for histopathological examination. A histopathological diagnosis of Diffuse Gastric Ganglioneuromatosis with muscular mural hypertrophy was made. The presence of ganglion cells and positive staining for S-100 and NSE confirms the diagnosis.Obstructive gastrointestinal symptoms with a possibility of diffuse ganglioneuromatosis is mainly a disease of young children and infant. But an adult patient, which is an unusual age group, presenting with obstructive gastrointestinal symptoms, the rare possibility of Diffuse ganglioneuromatosis and intestinal neuronal dysplasia should be considered and investigated in order to avoid unnecessary and extensive surgical intervention.