A Case of Gangliocytic Paraganglioma of the Papilla of Vater

Ikuo Kokufu, Katsumi Yamada, Masayuki Yamamoto, Mitsugu Kobayashi, Hidemine Ho, Yosuke Akiyama, Hirofumi Hirai, Hidetake Kitano, Kensuke Kadone, Motomu Tsuji*

Departments of Surgery and Pathology*, Itami City Hospital

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 2 cm 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.

Key words
gangliocytic paraganglioma of the papilla of Vater, duodenal gangliocytic paraganglioma, duodenal tumor

Reprint requests
Ikuo Kokufu Department of Surgey, Itami City Hospital
1-100, Koyaike, Itami, 664 JAPAN

Accepted
July 10, 1996

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