Abstract

Gangliocytic paraganglioma (GP) is a rare tumor. Until today, only few cases have been reported. Usually GPs are encountered in the second portion of the duodenum, commonly occurring as small submucosal lesions. Histologically, they are characterized by the presence of three different types of cells: epithelioid cells with endocrine growth pattern, resembling paraganglioma or carcinoid tumor cells, spindle cells reminiscent of Schwann cells and ganglia or ganglion-like cells. Generally this tumor has a benign clinical course. Rarely, it may recur or metastasize to regional lymph nodes. We present a case of gangliocytic paraganglioma of the ampulla of Vater in a patient who underwent partial pancreaticoduodenectomy.

Keywords: Duodenum, immunohistochemical findings, ampulla of Vater, gangliocytic paraganglioma, neuroendocrine neoplasms.