Case Report
Copyright ©2007 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Jun 28, 2007; 13(24): 3384-3387
Published online Jun 28, 2007. doi: 10.3748/wjg.v13.i24.3384
Multiple gastrointestinal stromal tumors and bilateral pheochromocytoma in neurofibromatosis
Klaus Kramer, Cornelia Hasel, Andrik J Aschoff, Doris Henne-Bruns, Peter Wuerl
Klaus Kramer, Doris Henne-Bruns, Peter Wuerl, Department of Surgery, University Hospitals of Ulm, Ulm, Germany
Cornelia Hasel, Department of Pathology, University Hospitals of Ulm, Ulm, Germany
Andrik J Aschoff, Department of Diagnostic and Interventional Radiology University Hospitals of Ulm, Ulm, Germany
Author contributions: All authors contributed equally to the work.
Correspondence to: Klaus Kramer, MD, Department of General-, Visceral- and Transplant-Surgery, University Hospital Ulm, Steinhoevelstr. 9, 89077 Ulm, Germany. kramer.k@web.de
Telephone: +49-731-50053517 Fax: +49-1212-600621210
Received: March 6, 2007
Revised: March 25, 2007
Accepted: March 31, 2007
Published online: June 28, 2007
Abstract

The coincidence of a gastrointestinal stromal tumor (GIST) and a neuroendocrine tumor (NET) in neurofibromatosis type 1 (NF1) is described only five times within the literature. We report on a 63 year old Caucasian female with the rare condition of neurofibromatosis type 1 coinciding with recurrent gastrointestinal stromal tumor plus bilateral pheochromocytoma (PCC). After a history of palpitations and dizziness that lasted for years, a left adrenal mass was detected by CT. Laparotomy revealed a pheochromocytoma of the left adrenal gland while an ileoterminal GIST was found incidentally intraoperatively. After six months contralateral PCC and multiple recurrent GIST were resected again. After four years the patient is doing well without any signs of further recurrent tumors. Discussion includes review of the literature.

Keywords: Gastrointestinal stromal tumor; Neuroendo-crine tumor; Neurofibromatosis; Pheochromocytoma; Coincidence