[Duodenal somatostatinomas associated with von Recklinghausen disease]

A case of von Recklinghausen's disease with double somatostatin producing neuroendocrine tumour of the ampulla and duodenum is reported. A search of the world's literature revealed 28 patients with immunohistologically proven duodenal somatostatinoma associated with type I neurofibromatosis. These tumours are seldom associated with a recognizable "somatostatin syndrome", but often present with obstructive jaundice, duodenal obstruction, weight loss or gastrointestinal bleeding. Histologically, psammoma bodies are frequently encountered in the glandular lumina of duodenal somatostatinomas (66%), whereas their presence in other neuroendocrine tumours of the gastrointestinal tract is very rare. Metastatization is rare (27%) and mainly confined to lymph nodes (88%). In the world literature duodenal somatostatinoma is associated with von Recklinghausen's disease in 50%.