Study of a rare benign gangliocytic paraganglioma within the ampulla of vater

Method: Gangliocytic paraganglioma (GP) is an extremely rare benign tumor with embryological origin commonly from the hindgut. Retrospective study of this rare presentation in a 64year old male done. 4/ 2016, Our patient had presented with malaena, vomiting and abdominal pain. Multiple investigations conducted (OGD, ERCP, CT abdomen and pelvis) demonstrated: A 2.4 by 3 cm, non-obstructing, hypodense, polypoidal enhancing tumour arising from the mesenteric aspect of the 3rd segment of the duodenum, with 2-3areas of ulceration alongwith a segment VII/VI liver lesion which an MRI liver pointed towards a haemangioma. Multi-Disciplinary Team Meeting conclusion: Likely Gastrointestinal Stromal Tumor. 9/2016 Serial scans: Stable duodenum mass and liver lesion with no retroperitoneal lymphadenopathy. 09/2016 Trans-duodenal ampulla excision with re-implantation of bile and pancreatic duct completed. Clearance Margin 0.2mm. Intra- op findings: 2.5-3cm ovoid mass in second part of duodenum prolapsing to 3rd part of duodenum. The lesion was excised and stalk revealed pancreatic and bile duct opening separately. Frozen section: negative for malignancy Results: Gangliocytic Paraganglioma is characterized by its triphasic cellular differentiation (epithelioid neuroendocrine cells, spindle cells with Schwann cell differentiation, ganglion cells) alongwith characteristic immunoprofiling. Known clinical Features:-Age 15 - 84 years.-M > F (1.5:1)-Approximately only7% metastasize to lymph nodes. (Only 23cases such cases reported by 2014. Conclusion: Here we describe a rare condition managed with a limited resection and reconstruction with no recurrence in 4years. Meta analyses of the known GP cases to identify differentiating features may be helpful in understanding this disease better.