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  • Title: Gangliocytic paraganglioma, a rare ampullary tumour treated with open transduodenal resection and sphincteroplasty.
    Author: Rafiullah, Tanimu S, Onitilo AA.
    Journal: BMJ Case Rep; 2014 Jul 02; 2014():. PubMed ID: 25100803.
    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.
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