Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes. A 92-year-old female presented with obstructive jaundice. On exam she had pale conjunctiva and icteric sclera. Abdominal examination revealed tenderness in the upper abdomen. Laboratory data was consistent with obstructive jaundice. Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct (CBD) with no evidence of liver lesions or pancreatic head mass. Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area, dilated CBD (9 mm), a prominent pancreatic duct (4.1 mm) and a hydropic gall bladder with no stones. Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection. Histopathology analyses with immunohistochemical stains were positive for cytokeratin, synaptophysin, S-100 protein, neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma. Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel. Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice. Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.
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