A Rare Case of Duodenal Gangliocytic Paraganglioma Treated With Ampullectomy

Introduction: Gangliocytic paraganglioma (GP) is a rare neuroendocrine tumor. Though, it is considered by other authors as a hamartoma developing in misplaced embryonic pancreatic tissue. Around 200 cases have been reported so far since 1957. The mean age of patients is 52 years, mostly men with man to woman ratio of 1.5:1. Mean size is 25 mm. Most lesions are in the duodenum (90%). The most common symptom is gastrointestinal bleeding (45.1%). Recurrence of tumor after treatment is extremely rare and Prognosis is excellent with no death reported in all cases. Case Description/Methods: A 46-year-old woman presented with chronic epigastric abdominal pain. Otherwise, no symptoms. She had a normal physical exam and normal laboratory testing. Upper endoscopy with endoscopic ultrasound showed (EGD-EUS) showed a single medium submucosal nodule in the major papilla (Figure 1A). The lesion was hypoechoic and homogenous originating from the deep mucosa (Layer 2) and submucosa (Layer 3). The outer margins were well-defined without invasion to other adjacent structures, and no lymphadenopathy was seen (Figure 1B). The biopsy result confirmed granulocytic paraganglioma. (Figure 1 C, D) Endoscopic ampullectomy was performed using a hot snare. Postoperatively patient didn’t have any complications. On follow-up visit she expressed the resolution of her epigastric pain. Discussion: Duodenal gangliocytic paraganglioma, is a rare tumor neuroendocrine tumor. Most common location in the third part of the duodenum. Most of the reported cases were treated surgically. This is the second reported case in which this tumor was treated endoscopically via ampullectomy. Overall prognosis is excellent with no documented recurrence or death after treatment.Figure 1.: Single medium submucosal nodule in the major papilla (A). Hypoechoic and homogenous originating from the deep mucosa (Layer 2) and submucosa (Layer 3) with well-defined outer margin without invasion to other adjacent structure and no lymphadenopathy seen (B) Biopsy result confirming granulocytic paraganglioma (C, D).