Gastrointestinal ganglioneuromatosis is a rare and benign proliferation of both ganglion and Schwann cells, which may present in a variety of clinicopathological characteristics including polypoid, localized and diffuse forms. It is characterized by the presence of a large number of dysplastic ganglion cells or hyperganglionosis. The affected patients are usually children and a number of them have intestinal neuronal dysplasia type B, which may overlap with ganglioneuromatosis. We report a very rare case of gastric ganglioneuromatosis with hyperganglionosis in a 34 year old male, who presented as a case of gastric outlet obstruction and marked gastric dilatation which was diagnosed both clinically and radiologically as a case of gastric obstruction due to gastric malignancy. Based on these findings and because of the patient’s deteriorating clinical situation; the patient underwent distal gastrectomy and the final diagnosis was made following the histopathological examination of the resected specimen. The clinicopathological features and diagnostic criteria of this rare disorder are described together with a literature review of other similar cases affecting different parts of the gastrointestinal tract.

Gastrointestinal ganglioneuromatosis is a rare and benign proliferation of both ganglion and Schwann cells, which may present in a variety of clinicopathological characteristics including polypoid, localized and diffuse forms. It is characterized by the presence of a large number of dysplastic g...