A duodenal cancer associated with combined syndrome of juvenile polyposis and hereditary hemorrhagic Telangiectasia

Abstract

A 51-year-old man was followed up due to hereditary hemorrhagic telangiectasia (HHT). We pointed out a polyposis lesion of the duodenum by gastroscopy. A duodenal polyp located on the opposite side of the major duodenal papilla was diagnosed as adenocarcinoma by biopsy. We also pointed out a polypotic lesion of the colon by colonoscopy. As the polypectomy for colonic polyposis was difficult to treat, we performed subtotal stomach-preserving pancreaticoduodenectomy for the duodenal cancer, and partial resection of the cecum and descending colon for the colonic polyposis. Although the hepatic artery was dilated because of HHT, we safely achieved a curative operation. Histological diagnosis revealed a well differentiated adenocarcinoma in juvenile polyposis (JP) of the duodenal lesion. Juvenile polyps were also seen in the colon. To the best of our knowledge, this is the first case undergoing pancreaticoduodenectomy for combined syndrome of juvenile polyposis and hereditary hemorrhagic telangiectasia in Japan.