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

• Main Authors: Manik Sharma, Syed Adnan Mohiuddin, Saad Al Kaabi, Imad Bin Mujeeb, Jacques Deviere
• Format: Article
• Language: English
• Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2018-04-01
• Series: Journal of Digestive Endoscopy
• Subjects: ampullary lesion; ampullectomy; biliary dilatation; endoscopic ultrasound; gangliocytic paraganglioma; neuroendocrine tumor; whipples surgery
• Online Access: http://www.thieme-connect.de/DOI/DOI?10.4103/jde.JDE_66_17
• ISSN: 0976-5042; 0976-5050

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.