title: 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.
authors.affiliation: Department of Internal Medicine, Saint Clair's Hospital, Weston, Wisconsin, USA.
authors.lastname: Rafiullah
authors.affiliation: Department of Gastroenterology, Marshfield Clinic, Weston, Wisconsin, USA.
authors.forename: Sabo
authors.initials: S
authors.lastname: Tanimu
authors.affiliation: Department of Oncology/Hematology, Marshfield Clinic, Weston, Wisconsin, USA.
authors.forename: Adedayo A
authors.initials: AA
authors.lastname: Onitilo
mesh_terms: D014670:Ampulla of Vater; D044963:Biopsy, Fine-Needle; D002763:Cholecystectomy / Q000379:methods; D003937:Diagnosis, Differential; D004379:Duodenal Neoplasms / Q000175:diagnosis / Q000601:surgery*; D004384:Duodenoscopy; D019160:Endosonography; D005500:Follow-Up Studies; D006801:Humans; D007813:Laparotomy / Q000379:methods*; D008297:Male; D008875:Middle Aged; D010235:Paraganglioma / Q000175:diagnosis / Q000601:surgery*; D009803:Sphincter of Oddi / Q000601:surgery*; D013105:Sphincterotomy, Transduodenal / Q000379:methods*; D014057:Tomography, X-Ray Computed
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