Background : Gangliocytic paraganglioma (GP) is an extremely rare neoplasm originating in the hindgut, predominantly arising in the second part of the duodenum, with rare local recurrence or metastasis to regional lymph nodes.

Methods : The authors reported the case of gangliocytic paraganglioma in duodenum

Results : A male-62 year old patient underwent routine check for gastroduodenoscopy. The tumor was 2.8 cm size in the 2nd portion of duodenum. The overlying mucosa was intact. The mass was arterial enhancing lesion on abdominal CT exam, hypermetabolic mass in PET CT scan. The authors had a plan for transduodenal ampullectomy and proceeding for pancreaticocduodenectomy in case of malignancy on frozen biopsy. However, the mass was proximal to ampulla, transduodenal tumor resection was performed, ganglioblastoma was suspicious on frozen biopsy. Final pathology was GP with the triphasic cellular differentiation; epithelioid neuroendocrine cells, spindle cells with Schwann cell?like differentiation, and ganglion cells.

Conclusions : Duodenal Gangliocytic paraganglioma is the most common among the periampullary GP. Local excision or endoscopic resection is the treatment of choice without evidence fo metastasis wheras pancreaticoduodenectomy is recommended for those with features suggestive of malignancy such as large tumor size, submucosal extent, or pancreatic GP.