2001 Volume 62 Issue 2 Pages 400-405
A 53-year-old woman with von Recklinghausen's disease was admitted to the hospital because of abdominal pain and vomiting. She had been diagnosed as von Recklinghausen's disease at the age of two, and undergone removals of left and right retroperitoneal neurofibromas at the ages of 41 and 53respectively as well as spinal neurofibroma at the age of 41. Since 50 years of age, diarrea and intractable peptic ulcer appeared and at the same time, hypergastrinemia was noted. Zollinger-Ellison syndrome had been suspected. On the 4th hospital day, massive melena occurred. Endoscopic examination showed multiple duodenal ulcers and a submucosal tumor close to the Papilla Vater. As she had marked hypergastrinemia to 1160pg/ml, Zollinger-Ellison syndrome was suspected. Pancreaticoduodenectomy was performed, when a submucosal tumor close to the Papilla Vater and lymph node metatstasis were found. Pathological examination revealed doudenal cartinoid 4mm in diameter, which was positive for gastrin by immunohistochemical staining. Hypergastrinemia disappeared postoperatively.