Authors: Rafiullah, Tanimu S, Onitilo AA
Title: Gangliocytic paraganglioma, a rare ampullary tumour treated with open transduodenal resection and sphincteroplasty.
Journal: BMJ Case Rep 2014:-
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.
Last Updated: 02 Mar 2015