A Gangliocytic Paraganglioma of the Ampulla of Vater Removed by Endoscopic Resection / 대한췌담도학회지

ABSTRACT

Gangliocytic paraganglioma (GP) is an extremely rare tumor that mostly occurs in the periampullary area of the duodenum. It is characterized by benign behavior and favorable outcomes, but sometimes shows regional lymph node dissemination. GP consist of three characteristic histological components: epithelioid, spindle, and ganglion cells. Therefore, it is often misdiagnosed as a neuroendocrine tumor when only endoscopic forceps biopsy is performed. The clinical management of GP has not yet been standardized. This case report describes an asymptomatic patient who was initially diagnosed with a grade-1 neuroendocrine tumor, but was confirmed as having benign GP after endoscopic papillectomy. Complete en-bloc resection was performed for this patient, without any significant adverse events. At a 6-month follow-up assessment, the patient remained asymptomatic and there was no evidence of recurrence.