A patient with von Recklinghausen syndrome presented with multiple attacks of pancreatitis. Evaluation using computed tomography, endoscopic retrograde cholangiopancreatography, and endosonography revealed an ampullary carcinoid. The patient underwent a pylorus-sparing Whipple resection, and is doing well after 30 months of follow-up. This is the second reported case of ampullary carcinoid presenting with pancreatitis in a patient with von Recklinghausen syndrome.
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