title: Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen's disease.
abstract: Von Recklinghausen's disease is an autosomal dominant hereditary disease associated with a wide number of neoplasms. We report a case of a 47-year-old Caucasian male affected by Von Recklinghausen's disease who developed a malignant somatostatinoma of the papilla major and minor associated with jejunal gastrointestinal stromal tumour with uncertain behaviour. At laparotomy, multiple hepatic metastases were evident. Whipple pancreaticoduodenectomy, jejunal resection, extensive lymphadenectomy and multiple hepatic wedge resections were performed. The patient was alive without recurrence after 24 mo. This is the fourth case reported in the world literature of a patient with Von Recklinghausen's disease associated with periampullary somatostatinomas and jejunal stromal tumor. In patients with Von Recklinghausen's disease who complain of gastrointestinal symptoms, a high suspicion index for periampullary endocrine tumours and/or gastrointestinal stromal tumour is required. An aggressive surgical approach seems to give long term survival also in metastatic patients.
authors.affiliation: Department of Surgery, University of Verona, Chirurgia Generale B-Policlinico GB Rossi, Piazzale LA Scuro, 10-37134 Verona, Italy.
authors.forename: Rossella
authors.initials: R
authors.lastname: Bettini
authors.forename: Massimo
authors.initials: M
authors.lastname: Falconi
authors.forename: Stefano
authors.initials: S
authors.lastname: Crippa
authors.forename: Paola
authors.initials: P
authors.lastname: Capelli
authors.forename: Letizia
authors.initials: L
authors.lastname: Boninsegna
authors.forename: Paolo
authors.initials: P
authors.lastname: Pederzoli
mesh_terms: D014670:Ampulla of Vater / Q000473:pathology*; D003138:Common Bile Duct Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007580:Jejunal Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D007813:Laparotomy; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications*; D013005:Somatostatinoma / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology