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
.