An upper gastrointestinal endoscopy showed a submucosal tumor of the papilla of Vater for a 38 year-old woman. Endoscopic ultrasonography (EUS) showed a mixed echoic mass (12mm) in the submucosal layer of papilla of Vater and showed no remarkable findings in the common bile duct and the main pancreatic duct. Endoscopic biopsy was performed, but pathologic findings showed normal duodenal mucosa. Abdominal computed tomography showed a well-enhanced tumor in the papilla of Vater. These findings led us to suspect a neuroendocrine tumor or gangliocytic paraganglioma. EUS-FNA was not performed because of high level of catecholamine and a transduodenal extirpation of the tumor was performed. The yellowish tumor was located in the duodenal submucosal layer. We identified three components in the tumor : epithelioid cells, ganglion-like cells, and spindle cells. The tumor was finally diagnosed as a gangliocytic paraganglioma of the duodenum according to the histological findings. Gangliocytic paraganglioma is a rare disease in the duodenum. Therefore, we report a case of gangliocytic paraganglioma with a review of the literature.
