V6 - Sphincterotomy-Aided Endoscopic Submucosal Dissection (ESD) of a Periampullary Duodenal Paraganglioma

We present a case of a duodenal periampullary paraganglioma that was removed endoscopically with a combination of ERCP and ESD techniques. ERCP with sphincterotomy separated the ductal openings at the ampulla from the lesion and allowed ESD to achieve en-bloc resection of the lesion.

Case Description/

Methods: A 60-year-old male with polycystic kidney disease (PCKD), obstructive sleep apnea (OSA), cerebrovascular accident (CVA), VP shunt on chronic antibiotics underwent CT abdomen for follow-up of PCKD. An incidental polypoidal mass was seen in the second portion of the duodenum. An upper endoscopy with endoscopic ultrasound showed a 3 cm subepithelial mass around the ampulla. Biopsies confirmed paraganglioma. After multidisciplinary discussion, endoscopic resection was planned.

With the duodenoscope, the mass was confirmed to be involving the ampulla and extending inferior to the ampulla. Biliary and pancreatic cannulation followed by sphincterotomy and prophylactic PD stent were placed. With the forward viewing gastroscope, there were limited views of the sub-epithelial mass. Traction with double-loop floss and clips were applied to the lateral wall. Using saline immersion, endoscopic submucosal dissection (ESD) was performed initially in retroflexed view and later in forward view to achieve complete resection of the mass. The ampullary complex was divided below the biliary and pancreatic orifices facilitated by prior sphincterotomy. The ESD defect was closed using through-the-scope (TTS) sutures (X-Tack) and endoclips.

Successful en-bloc endoscopic resection was achieved. There were no post-procedure complications. Pathology confirmed R0 resection of gangliocytic paraganglioma with neuroendocrine cells staining positive for chromogranin and synaptophysin, spindle cells and ganglion cells. Multidisciplinary tumor board review recommended ongoing endoscopic/imaging surveillance.

Discussion: Periampullary sub-epithelial lesions pose a significant challenge in management. Endoscopic resection can be offered where the goal is local resection. Paragangliomas are benign lesions with low malignant potential. Hence, endoscopic resection was offered for this patient.

Ampullary subepithelial lesions have been removed endoscopically through various techniques. ESD of ampulla carries high risk of pancreatitis and perforation. By combining ERCP sphincterotomy and saline immersion ESD with traction, we were able to successfully remove this large sub-epithelial lesion with no adverse events.