Abstract
A 75-year-old female previously diagnosed as von Recklinghausen's disease (VRD) was underwent follow-up abdominal CT after operation for rectal carcinoid, thyroid cancer and intrahepatic cholangiocarcinoma. Endoscopic examination of the duodenum showed a submucosal tumor in the papilla of Vater. Because of the diagnosis as adenocarcinoma by biopsy specimen, pancreatoduodenectomy was performed. Histological examination showed collision tumor with adenocarcinoma and carcinoid in the papilla of Vater. We reported this case because collision tumor of the papilla with VRD is the first case in Japan, quintuple cancer is very rare and only 8 cases including our case have been reported in Japan.