Duodenal somatostatinomas associated with Von Recklinghausen's disease. Two case reports

Somatostatinomas are rare neuroendocrine tumors: they are essentially locat ed in the pancreas and in the duodenum. The association with a neurofibroma tosis type I is especially observed when the tumor is located in the ampull a of Vater. These tumors are not associated with a "somatostatin syndrome" but often present with gastrointestinal bleeding abdominal pain and obstruc tive jaundice. The diagnosis is confirmed by immunohistochemical studies. T he aim of this study is to report 2 cases of metastazing duodenal periampul lary somatostatinomas associated with von Recklinghausen's disease and to d iscuss the prognosis of these rumors. Future generic researchs are necessar y as point out the familial feature of this association in one of oar cases .