2019 Volume 80 Issue 11 Pages 2018-2022
Two years ago, a 57-year-old woman with neurofibromatosis type 1 (NF1) underwent an abdominal hysterectomy for a uterine myoma and a wedge-shaped excision of the jejunum for gastrointestinal stromal tumor (GIST). A follow-up computed tomography revealed a 32-mm tumor in the duodenum, and endoscopic findings revealed a submucosal tumor on the contralateral and anal side of the duodenal papilla. Hence, a diagnosis of duodenal GIST was made and an open surgery was performed. A part of the jejunum (15 cm in length) was removed using local excision and a jejunal mucosal patch was grafted. After the surgery, no deformation or stenosis of the surgical lesion was found following fluoroscopy. In general, 7% of NF1 patients have GISTs, and 68% of the lesions often occur in multiples in the small intestine. The malignancies are often low grade. There is an increased risk in surgery and reconstruction when a large lesion is located in the duodenum ; however, it is possible to prevent deformation and stenosis using a jejunal mucosal patch for the duodenal defect.