Abstract
An upper gastrointestinal endoscopy revealed a swelling of the papilla of Vater for a 79 year-old man. Abdominal computed tomography showed an enhanced, well-demarcated mass, and endoscopic ultrasonography showed a low echoic mass (11×10 mm) in the papilla of Vater. Endoscopic retrograde cholangiopancreatography showed dilatation of the papilla of Vater, the common bile duct, and the main pancreatic duct. Based on these findings, an ampullary cancer was suspected, and a pylorus preserving pancreaticoduodenectomy was performed. Macroscopically, a hard, yellowish-white tumor of 1.8 cm in diameter was identified. The tumor was well-demarcated, but not encapsulated, occupying the papilla of Vater and the duodenal submucosal layer, and involving the sphincter of Oddi and duodenal epithelia. Microscopically, the tumor was composed of three components: the major component showing a paraganglioma-like appearance, and spindle cell proliferation similar to Schwannoma and isolated ganglion-like cells as minor components. Based on the histological appearances and immunohistochemical findings, the tumor was diagnosed as a gangliocytic paraganglioma.