Système gastro-intestinal et digestif

Abstrait

Acute Gastrointestinal Bleeding with Hypovolemic Shock Induced by Gastrointestinal Stromal Tumor Coincident with Neurofibromatosis Type 1: A Case Report

Gha Na Jo, Kang Hong Lee, Byung Kyu Ahn

We report a case of young patient diagnosed as Neurofibromatosis type 1 (NF-1) five years ago. A 42-year-old male was admitted due to sudden onset of gastrointestinal bleeding. Contrast enhanced computed tomography (CT) scan revealed a lobulating unenhanced mass, originating from the small bowel in the pelvic cavity. The patient developed a fulminant gastrointestinal hemorrhage and emergency operation was performed. On operation, multilobulated submucosal mass was observed on the serosal surface of proximal ileum and this tumor with hemorrhagic and cystic change was the source of bleeding. Histopathological examination revealed this tumor was 5.5 cm × 5 cm × 4 cm in size and small bowel gastrointestinal stromal tumor (GIST) with moderate risk malignant potential. Immunohistochemical examination showed that C-kit and CD34 were positive. In neurofibromatosis, GISTs are more frequent and we should pay attention to gastrointestinal manifestation in such patients.