Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease.

Somatostatinomas are extremely rare periampullary malignant neuroendocrine tumors that may be associated with von Recklinghausen disease or type-I neurofibromatosis. Duodenal somatostatinomas are distinguished from pancreatic somatostatinomas by their frequent association with type-I neurofibromatosis and typically absence of somatostatinoma syndrome. We report a very rare and atypical case of malignant duodenal somatostatinoma presenting with somatostatinoma syndrome in association with type-I neurofibromatosis.