A case of gangliocytic paraganglioma in the ampulla of Vater

Abstract

Background: Duodenal gangliocytic paraganglioma is an extremely rare tumor and few cases have been reported to date.

Case presentation: The authors report a case of gangliocytic paraganglioma verified by post-op pathology after pancreaticoduodenectomy for a tumor in the ampulla of Vater. The 56-year-old male patient concerned visited our emergency room with melena that started one week prior to hospitalization. The patient was diagnosed to have a tumor in the ampulla of Vater with bleeding on its surface. However post-op, he was diagnosed as having gangliocytic paraganglioma by immunohistochemistry.

Conclusion: This tumor has precise clinical implications, and if continuous follow up is conducted after careful diagnosis and surgical treatment, invasive major operations, such as, radical pancreaticoduodenectomy can be avoided.

Figure 1

Esophagogastroduodenoscopic findings showing the periampullary submucosal tumor and surface ulcer bleeding.

Figure 2

(A) The mass lesion showed a triphasic pattern comprised of epithelioid cell nests, neurofibromatous spindle cells and ganglion cells. (×200) (B) Carcinoid-like epithelioid cell nests with surrounding spindle cells. (×400).

Figure 3

Immunohistochemical stains for (A) neuron-specific enolase, (B) synaptophysin, and (C) S-100: Epithelioid cell components were positive for neuron-specific enolase and synaptophysin, and neurofibromatous spindle cell components were positive for S-100.