Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen's disease

World J Gastroenterol. 2007 May 21;13(19):2761-3. doi: 10.3748/wjg.v13.i19.2761.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Ampulla of Vater / pathology*
  • Common Bile Duct Neoplasms / diagnosis
  • Common Bile Duct Neoplasms / etiology*
  • Common Bile Duct Neoplasms / pathology
  • Gastrointestinal Stromal Tumors / diagnosis
  • Gastrointestinal Stromal Tumors / etiology*
  • Gastrointestinal Stromal Tumors / pathology
  • Humans
  • Jejunal Neoplasms / diagnosis
  • Jejunal Neoplasms / etiology*
  • Jejunal Neoplasms / pathology
  • Laparotomy
  • Male
  • Middle Aged
  • Neurofibromatosis 1 / complications*
  • Somatostatinoma / diagnosis
  • Somatostatinoma / etiology*
  • Somatostatinoma / pathology