Case Report
Copyright ©2007 Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. May 14, 2007; 13(18): 2629-2632
Published online May 14, 2007. doi: 10.3748/wjg.v13.i18.2629
Effect of sunitinib on metastatic gastrointestinal stromal tumor in patients with neurofibromatosis type 1: A case report
M Emin Kalender, Alper Sevinc, Ediz Tutar, Akif Sirikci, Celalettin Camci
M Emin Kalender, Alper Sevinc, Celalettin Camci, Department of Medical Oncology, Gaziantep University, School of Medicine, Gaziantep Oncology Hospital, Gaziantep TR-27310, Turkey
Ediz Tutar, Department of Pathology, Gaziantep University, School of Medicine, Sahinbey Medical Center, Gaziantep, TR-27310, Turkey
Akif Sirikci, Department of Radiology, Gaziantep University, School of Medicine, Sahinbey Medical Center, Gaziantep, TR-27310, Turkey
Author contributions: All authors contributed equally to the work.
Correspondence to: Dr. Alper Sevinc, Gaziantep University, School of Medicine, Department of Medical Oncology, Gaziantep Oncology Hospital, TR-27310 Gaziantep, Turkey. sevinc@gantep.edu.tr
Telephone: +90-342-4720711 Fax: +90-342-4720718
Received: December 29, 2006
Revised: December 30, 2006
Accepted: March 23, 2007
Published online: May 14, 2007
Abstract

Gastrointestinal stromal tumor (GIST) represents the most common mesenchymal malignancy of the gastrointestinal (GI) tract. In neurofibromatosis (NF), the increased incidence of tumor needs to be considered even in non-symptomatic individuals. Patients with neurofibromatosis NF type 1 have an increased risk of developing GI tumors including rare types such as GIST. We report a case of GIST in a 53-year-old male patient with neurofibromatosis. The patient was diagnosed with NF four years ago and his medical history revealed that he was hospitalized 5 times with a provisional diagnosis of massive lower gastrointestinal bleeding. GIST was diagnosed at explorative laparotomy and the tumor was 21 cm × 13 cm × 7 cm in size. Immunohistochemical examination showed that vimentin, actin and CD117 were positive. Computerized tomography showed peritoneal implants three months later. Imatinib mesylate (600 mg/d) was initiated. However, control computerized tomography revealed liver and omental metastasis. The dosage was elevated to 800 mg/d. Despite high dosage, the progression of the metastatic lesions continued in the liver and omentum. The patient started oral sunitinib malate (Sutent® Pfizer Inc, New York, NY, USA) 50 mg per day for 4 consecutive weeks, followed by 2 wk off per treatment cycle. The metastatic lesions in the liver and omentum were decreased in size after four courses, suggesting that sunitinib is also an effective treatment modality for metastatic GIST in NF patients.

Keywords: Neurofibromatosis; Gastrointestinal stromal tumors; Imatinib; Sunitinib