Gastrointestinal Stromal Tumor of the Stomach in a Patient with Neurofibromatosis Type 1: Case Report

Abstract

Neurofibromatosis Type 1 (NF-1) is a neurocutaneous disease characterized by abnormal skin pigmentations, neurofibromas, skeletal dysplasias and Lisch nodules. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Although GISTs occur frequently sporadically, their frequency in NF-1 patients is increasing. A 79-year-old woman with NF-1 presented to emergency room with complaints of abdominal pain and bloody vomiting. Physical examination of the patient was unremarkable except epigastric tenderness. Upper gastrointestinal system endoscopy revealed a submucosal tumoral mass in the fundus of the stomach. Laparoscopic gastric wedge resection was performed. She was discharged on 5th day. A GIST arising from gastric fundus in size of 9x6x5 cm and two GISTs in sizes of 0.7 and 0.1 cm in approximately 1 cm neighborhood of the tumor were detected at histopathological examination. Patients with NF-1 have an increased risk of GIST. Laparoscopic surgery can be used for the treatment.