“Ampullary Gangliocytic Paraganglioma:” A Rare Neuroendocrine Tumor Can Be Safely Treated with Ampullectomy

ABSTRACT

Ampullary Gangliocytic paraganglioma is a rare gastro-entero-pancreatic neuroendocrine tumor with a characteristic histologic appearance involving epitheliod, spindle and ganglion cells. Endoscopic ultrasound is useful to determine depth of invasion and regional metastasis. Endoscopic resection, Ampullectomy and pancreato-duodenenectomy have been described as management options in literature but no consensus has been established. The index case describes an asymptomatic 42 y/o lady with a background of plexiform neurofibromatosis referred for cholestatic derangement of liver function. She was found to have a dilated biliary system with a periampullary mass which proved to be a Gangliocytic Paraganglioma without local lymph node metastasis. She underwent successful endoscopic ampullectomy with complete normalization of LFT and decompression of the biliary system with no clinical or endoscopic recurrence at 2 years follow up. We advocate that Partial Ampullectomy could be done safely as a treatment of localized Ampullary GP allowing long term resolution of symptoms.

Keywords

Ampullary lesion - ampullectomy - biliary dilatation - endoscopic ultrasound - gangliocytic paraganglioma - neuroendocrine tumor - Whipples surgery

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