“Ampullary Gangliocytic Paraganglioma:” A Rare Neuroendocrine Tumor Can Be Safely Treated with Ampullectomy
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, Amp...
Main Authors: | , , , , |
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Format: | Article |
Language: | English |
Published: |
Thieme Medical and Scientific Publishers Pvt. Ltd.
2018-04-01
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Series: | Journal of Digestive Endoscopy |
Subjects: | |
Online Access: | http://www.thieme-connect.de/DOI/DOI?10.4103/jde.JDE_66_17 |
Summary: | 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. |
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ISSN: | 0976-5042 0976-5050 |