Association of multiple gastrointestinal stromal tumor (GIST) and gastric schwannoma in a patient with type 1 neurofibromatosis

Neurofibromatosis is a common autosomal dominant disease associated with a higher incidence of neoplasms than in the general population. We report the case of a 39-old-man affected by neurofibromatosis type 1 (NF-1) who was admitted for anemia and melena. Endoscopy of the upper part of the gastrointestinal tract demontrated polypoid formation with central ulceration of the jejunum. Biopsies were inconclusive. On laparotomy, main jejunal tumor and numeros mural nodules in the stomach and intestine with numerous small nodules in the peritoneum were found. A wedge resection of the largest tumor taking the mucosa was performed. Only three other small nodules from the jejunum and stomach were resected. Pathological exam concluded to GISTs (including the largest tumor) and one schwannoma. Our case highlight the fact that all lesions encountred in NF-1 patients must be as possible as resected for histopathological examination.