Duodenal somatostatinoma associated with von Recklinghausen's disease

A case of von Recklinghausen's disease with double somatostatin producing n euroendocrine tumour of the ampulla and duodenum is reported. A search of the world's literature revealed 28 patients with immunohistolog ically proven duodenal somatostatinoma associated with type I neurofibromat osis. These tumours are seldom associated with a recognizable "somatostatin syndr ome", but often present with obstructive jaundice, duodenal obstruction, we ight 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 Reckli nghausen's disease in 50%.