Background : Gangliocytic paraganglioma is a rare tumor mostly located in the ampulla or periampullary region of the duodenum. These tumors have a triphasic cellular distribution, often containing a pre-dominant epithelioid component, as well as spindle and ganglion cells of varying proportions. It is usually considered a benign tumor, but cases with lymph node metastasis and distant metastasis have been reported.

Methods : We report a rare case of a 46-year-old female with upper gastrointestinal bleeding from an ampullary mass which was successfully treated with pylorus preserving pancreaticoduodenectomy.

Results : Histological and immunohistochemistry (IHC) findings were diagnostic of gangliocytic paraganglioma. Our case had lymph node metastases despite the tumor having a low Ki67 proliferation index, and the metastatic tumor showed all three tumor components.

Conclusions : In terms of treatment, When the tumor was confined to the ampulla and there was no regional lymph node spread, endoscopic mucosal resection (EMR) and ampullary resection are both viable options, but in the case of local or lymphovascular infiltration, more aggressive surgery should be performed, such as pancreaticoduodenectomy