Periampullary gangliocytic paraganglioma

A 33-year-old man presented with features of obstructive jaundice. Enterohe patic retrograde cholangiopancreatography examination revealed a periampull ary growth. After pancreatoduodenectomy, histologic examination of the grow th revealed three elements: a spindle cell element, large ganglion cells, a nd smaller paraganglioma-like cells arranged in small nests and groups. A d iagnosis of gangliocytic paraganglioma was given. Gangliocytic paragangliom a is a rare benign duodenal tumor, with uncertain histogenesis. It is possi ble to identify the three components that are essential for its diagnosis o n routine hematoxylin and eosin sections without the use of immunohistochem ical markers. Because this lesion may present clinically as a periampullary carcinoma, it is important to recognize and diagnose this rare, but benign , entity.