Case Report
Copyright ©2007 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. May 21, 2007; 13(19): 2761-2763
Published online May 21, 2007. doi: 10.3748/wjg.v13.i19.2761
Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen’s disease
Rossella Bettini, Massimo Falconi, Stefano Crippa, Paola Capelli, Letizia Boninsegna, Paolo Pederzoli
Rossella Bettini, Massimo Falconi, Stefano Crippa, Letizia Boninsegna, Paolo Pederzoli, Department of Surgery, University of Verona, Policlinico “GB Rossi”, Verona, Italy
Paola Capelli, Department of Pathology, University of Verona, Policlinico “GB Rossi”, Verona, Italy
Author contributions: All authors contributed equally to the work.
Supported by Fondazione Cariverona 2005, Verona (Italy); Ministero della Salute, Regione Marche e Ministero dell’Università No. PRIN 2005069205
Correspondence to: Dr. Massimo Falconi, Department of Surgery, University of Verona, Chirurgia Generale B-Policlinico “GB Rossi”. Piazzale LA Scuro, 10-37134 Verona, Italy. massimo.falconi@univr.it
Telephone: +39-45-8074553 Fax: +39-45-8201294
Received: August 10, 2006
Revised: December 11, 2006
Accepted: March 19, 2007
Published online: May 21, 2007
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.

Keywords: Endocrine tumour; Gastrointestinal stromal tumour; Neurofibromatosis; Somatostatinoma; Pancreatic neoplasm