CC BY-NC-ND 4.0 · Journal of Digestive Endoscopy 2018; 09(02): 082-084
DOI: 10.4103/jde.JDE_66_17
Case Report
Society of Gastrointestinal Endoscopy of India

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

Manik Sharma
Department of Gastroenterology, Erasme University Hospital, Brussels, Belgium
,
Syed Adnan Mohiuddin
Department of Gastroenterology, Erasme University Hospital, Brussels, Belgium
,
Saad Al Kaabi
Department of Gastroenterology, Erasme University Hospital, Brussels, Belgium
,
Imad Bin Mujeeb
1  Department of Anatomic Pathology, Hamad Medical Corporation, Doha, Qatar
,
Jacques Deviere
2  Department of Gastroenterology, Erasme University Hospital, Brussels, Belgium
› Author Affiliations
Further Information

Publication History

Publication Date:
24 September 2019 (online)

  

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.