title: Large somatostatin-producing endocrine carcinoma of the ampulla of vater in association with GIST in a patient with von Recklinghausen's disease. Case report and review of the literature.
abstract: BACKGROUND
  Somatostatin-producing endocrine tumors of the duodenum are very rare neoplasms of the gastrointestinal tract. These tumors may be associated with von Recklinghausen's disease.
  
  
  METHODS
  We present the case of a 49-year-old female patient with von Recklinghausen's disease and an incidentally diagnosed ampullary neoplasm. The patient was treated with a classical pancreaticoduodenectomy. At surgery, a mass was found in the greater curve of the stomach which was resected using the classic Whipple procedure. Histology and immunohistochemistry showed that the duodenal tumor was an ampullary somatostatin-producing endocrine carcinoma while the gastric tumor was a gastrointestinal stromal tumor (GIST). The postoperative course was uneventful and the patient is alive, without tumor recurrence, six years after surgery.
  
  
  CONCLUSIONS
  Somatostatin-producing endocrine tumors of the duodenum are rare tumors, often associated with von Recklinghausen's disease; these neoplasms should be treated aggressively using radical surgical resection. Although local resection may be appropriate for small duodenal somatostatin-producing tumors, a pancreaticoduodenectomy is usually required for larger tumors.
authors.affiliation: Fourth Department of Surgery, Athens University Medical School, Attikon University Hospital, Athens, Greece. georgesakorafas@yahoo.com
authors.forename: George H
authors.initials: GH
authors.lastname: Sakorafas
authors.forename: George A
authors.initials: GA
authors.lastname: Giannopoulos
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Parasi
authors.forename: George
authors.initials: G
authors.lastname: Konstantoudakis
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Tzanakis
authors.forename: Spiridon
authors.initials: S
authors.lastname: Stergiopoulos
authors.forename: George
authors.initials: G
authors.lastname: Peros