A Resected Case of Carcinoid Tumor of Vater’s Papilla in a Patient with von Recklinghausen’s Disease

Abstract

It has been known that von Recklinghausen’s disease (VRD) is often associated with gastrointestinal disease. We report a very rare case of carcinoid of Vater’s papilla in a patient with VRD. A 50-year-old man visited our hospital to investigate the cause of the elevation in serum hepatobiliary enzymes. Abdominal CT scan demonstrated the dilation of intrahepatic bile duct. A 15-mm tumor was found at Vater’s papilla by endoscopy and an endoscopic biopsy was performed. No malignant findings were detected pathologically, but clinically, there was a possibility of carcinoma or carcinoid of Vater’s papilla. He underwent a pylorus-preserving pancreatoduodenectomy. Pathologic examination of the isolated specimen revealed a carcinoid tumor in Vater’s papilla. In addition, he had café-au-lait spots and multiple neurofibromatosis at the precordia region, and carcinoid tumor of Vater’s papilla associated with VRD was diagnosed.