Periampullary gangliocytic paraganglioma

A33-year-old man presented with features of obstructive jaundice. Enterohepatic retrograde cholangiopancreatography examination revealed a periampullary growth. After pancreatoduodenectomy, histologic examination of the growth revealed three elements: a spindle cell element, large ganglion cells, and smaller paraganglioma-like cells arranged in small nests and groups. A diagnosis of gangliocytic paraganglioma was given. Gangliocytic paraganglioma is a rare benign duodenal tumor, with uncertain histogenesis. It is possible to identify the three components that are essential for its diagnosis on routine hematoxylin and eosin sections without the use of immunohistochemical markers. Because this lesion may present clinically as a periampullary carcinoma, it is important to recognize and diagnose this rare, but benign, entity.