A Case of Gangliocytic Paraganglioma of the Papilla of Vater

• Published in: Nippon Shokaki Geka Gakkai zasshi Vol. 29; no. 12; pp. 2284 - 2288
• Main Authors: Kokufu, Ikuo, Yamada, Katsumi, Yamamoto, Masayuki, Kobayashi, Mitsugu, Ho, Hidemine, Akiyama, Yosuke, Hirai, Hirofumi, Kitano, Hidetake, Kadone, Kensuke, Tsuji, Motomu
• Format: Journal Article
• Language: Japanese
• Published: The Japanese Society of Gastroenterological Surgery 1996
• Subjects: duodenal gangliocytic paraganglioma; duodenal tumor; gangliocytic paraganglioma of the papilla of Vater
• ISSN: 0386-9768; 1348-9372
• DOI: 10.5833/jjgs.29.2284

A 58-year-old woman was found to have an asymptomatic elevated lesion in the antrum of the stomach. Endoscopic examination revealed no elevated lesion in the stomach but rather an elevated mass in the second portion of the duodenum. Upper gastrointestinal series showed a smooth edged filling defect in the second portion of the duodenum. Abdominal CT, angiography and ERCP findings were negative. An endoscopic biopsy showed no malignancy. Under a possible diagnosis of carcinoid, pancreaticoduodenectomy was performed. The tumor was located in the papilla of Vater and measured 2cm in diameter. Histopathologically, epithelioid cells were arranged in nests and ganglion-like cells were scattered diffusely and surrounded by spindle cells. Immunohistochemical staining for neuron specific enolase, S-100 and pancreatic polypeptide showed positive results. Thus, the tumor was diagnosed as a gangliocytic paraganglioma. A case of gangliocytic paraganglioma has been presented and only 16 cases have been reported in Japan, including our case, to date.