Abstract:Objective To investigate the clinicopathologic features of intestinal ganglioneuromas (GNs). Methods To retrospectively analyze the clinicopathological features of the cases diagnosed as intestinal GNs by pathology in the Department of Pathology, West China Hospital, Sichuan University, from September 2008 to September 2021 and review the relevant literature. Results Nine patients with intestinal GNs, five males and four females, with a median age of 53 years (21~64 years). There were 6 cases of polypoid ganglioneuroma (PG), 1 case of ganglioneuromatous polyposis (GP) with tubular adenoma, 2 cases of diffuse ganglioneuromatosis (DG), and 2 cases of diffuse ganglioneuromatosis (DG), 1 of which was associated with a Neurosheath tumor. Microscopically, ganglioneuromas were composed of ganglion cells and spindle-shaped Schwann cells. Immunohistochemically, ganglion cells expressed neuron-specific enolase (NSE), synaptophysin(Syn), S-100 protein, neuron-specific nuclear protein (NeuN), and neurofilament protein (NF); spindle Schwann cells express S-100 protein and neurofilament protein (NF); and Ki-67 proliferation index was <1%. 9 cases of intestinal GNs were not combined with hereditary syndromes. Conclusion Intestinal GNs are maturely differentiated benign neurogenic tumors, which can be divided into three subtypes: PG, GP and DG. Each subtype has slightly different clinicopathological features and has a better prognosis, but should be closely followed up