Duodenal somatostatinoma presenting with complete somatostatinoma syndrome

Somatostatinomas are the rarest pancreatic endocrine tumors and can arise i n the pancreas or duodenum. Duodenal somatostatinomas are less common than, and are distinguished from, their pancreatic counterparts by a frequent as sociation with type I neurofibromatosis, the presence of psammoma bodies. t he less frequent presence of metastatic disease, and the absence of somatos tatinoma syndrome (diabetes mellitus. steatorrhea, and cholelithiasis). We report a case of somatostatinoma with metastases and psammoma bodies presen ting with all three features of the syndrome in a patient with neurofibroma tosis. Although several reports have documented portions of the syndrome in patients with duodenal somatostatinomas. to our knowledge, this is the fir st report of the complete syndrome associated with a duodenal lesion.