[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%.
Blaser
A
A
Service de chirurgie générale, Hôpital régional de Nyon. ablaser@worldcom.ch
Vajda
P
P
Rosset
P
P
D014670:Ampulla of Vater / Q000473:pathology; D002455:Cell Division / Q000502:physiology; D003138:Common Bile Duct Neoplasms / Q000150:complications* / Q000473:pathology; D004379:Duodenal Neoplasms / Q000150:complications* / Q000473:pathology; D004386:Duodenum / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000150:complications* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000473:pathology; D013005:Somatostatinoma / Q000150:complications* / Q000473:pathology