Ampullary carcinoid tumor presenting as acute pancreatitis in a patient with von Recklinghausen's disease: case report and review of the literature.

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.