Gangliocytic paraganglioma, a rare ampullary tumour treated with open transduodenal resection and sphincteroplasty

Abstract

Gangliocytic paragangliomas are rare neuroendocrine tumours residing in the gastrointestinal tract, most commonly the periampullary region. Most are benign tumours with a low malignancy rate. We report a 50-year-old man who presented with acute onset of left-upper quadrant abdominal pain with radiation to the back. An intraluminal mass at the junction of the duodenum with normal pancreatic/hepatic parenchyma was discovered on abdominal CT. Following upper endoscopy and MRI revealing a periampullary lesion, fine-needle aspiration and biopsies were. Immunohistochemistry was positive for synaptophysin and S100HU, consistent with gangliocytic paraganglioma. The benign nature of this tumour and unique anatomy of a separate opening of the pancreatic and common bile ducts led to transduodenal excision with sphincteroplasty, thereby avoiding extensive surgery. Surveillance CT every 6 months and upper endoscopy initially every 6 months (now yearly) revealed no evidence of endoscopic or histological recurrence at 3 years follow-up.

Endoscopic resection of a periampullary gangliocytic paraganglioma of the duodenum: A case report

“…In a follow up period from 12 to 96 months no death from GP was observed. Furthermore neither Rafiullah nor Yang observed any relapses of duodenal GPs in their case reports during a long term follow up of 3 years [9], [10]. Moreover Okubo et al performed a multi-institutional retrospective study in japan [7].…”

Gangliocytic Paraganglioma of the Minor Papilla of the Duodenum

“…It rarely develops in other organs, including the esophagus, mediastinum ( 6 ), thymus ( 7 ), lung ( 8 ), and ovary ( 9 ). According to a search of the PubMed database, 45 cases of the duodenal GP have been reported during the last decade (2007-2016) ( Table 2 ) ( 2 , 3 , 10 - 48 ). This tumor frequently develops in middle-aged and elderly individuals (mean, 51.2 years; range, 16-92 years) and predominantly affects men (27 men vs. 18 women).…”

“…To date, GP of the duodenal major papilla has been reported in a large number of cases, whereas that of the minor papilla is quite rare. We herein describe the case of a patient with a GP protruding from the minor papilla who was successfully treated by laparotomic window surgery ( 3 ).…”