title: [Von Recklinghausen's disease associated with pancreatic somatostatinoma].
abstract: BACKGROUND
  Pancreatic somatostatinoma is a rare entity and its association with Von Recklinghausen's disease has only been described on two occasions. We report a new observation, with clinical and evolving status differing from those described in isolated cases of somatostatinoma.
  
  
  METHODS
  A 28 year-old man presenting with familial Von Recklinghausen's disease, had suffered for 10 years from paroxysmic abdominal pain. He was hospitalized for intense pain. Imaging revealed a retroperineal tumoral formation. Following duodenopancreatectomy, somatostatinoma was diagnosed. Diffuse metastatic miliary was revealed and multiple glandular metastases.
  
  
  CONCLUSIONS
  Other than the rarity of the morbid association (pancreatic somatostatinoma and Von Recklinghausen's disease) described, this case is particular in that the patient was very young, symptomatology was unapparent and tumoral evolution was minimal.
authors.affiliation: Services de réanimation infectieuse, Hôpital Bichat, 46 rue H. Huchard, F75018 Paris.
authors.forename: P
authors.initials: P
authors.lastname: Thannberger
authors.forename: J M
authors.initials: JM
authors.lastname: Wilhelm
authors.forename: A
authors.initials: A
authors.lastname: Derragui
authors.forename: O
authors.initials: O
authors.lastname: Saraceni
authors.forename: P
authors.initials: P
authors.lastname: Kieffer
mesh_terms: D000328:Adult; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D010179:Pancreas / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D013004:Somatostatin / Q000097:blood; D013005:Somatostatinoma / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery
chemical_list: D013004:Somatostatin
vernacular_title: Maladie de Von Recklinghausen associée à un somatostatinome pancréatique.