Abstract
Familial adenomatous polyposis (FAP) is characterized by autosome-dominant inheritance and has a high tendency of cancerous change. Prophylactic total colectomy decreases the chance of developing colorectal cancer in FAP patients. The presence of extracolonic lesions, particularly duodenal papilla cancer, also affects the prognosis. A 19-year-old male, whose father died because of FAP, was diagnosed as having FAP by a genetic examination at 6 years of age. Multiple polyps in the colon were confirmed by colonoscopy when he was 13 years of age. At the same time, multiple polyps in the stomach and a polyp of Vater papilla were found by upper gastrointestinal endoscopy. Total colectomy was performed at 14 years of age. After the operation, he was admitted again because of acute pancreatitis. An enlarged Vater papillary tumor seemed to be the cause of the pancreatitis. Transduodenal papilla resection was performed. The tumor was revealed to be a well-differentiated tubular adenocarcinoma. After the papilla resection, the tumor near the bile duct orifice and pancreatic duct orifice increased in size. We suspected the recurrence of the cancer and performed pylorus-preserving pancreaticoduodenectomy 1 year and 10 months after the first operation. No sign of recurrence was found 2 years after the surgery. The occurrence of Vater papilla cancer at a young age is rare in FAP patients. To the best of our knowledge, this is the youngest patient with this condition in Japan.
