Familial Adenomatous Polyposis Complicated with Intraabdominal Desmoid, Thyroid Papillary Cancer and Duodenal Cancer in Major Papilla

A 41-year-old female, having a history of sigmoidectomy at the age of 21 years and total colectomy for familial adenomatous polyposis at the age of 31 and undergoing periodical surveillance examinaton, was found to have neck tumors. She also had undergone a palliative resection of intraabdominal desmoid tumor at the age of 32. Ultrasonography and aspiration biopsy cytology revealed papillary cancer of both lobes of the thyroid gland. Upper gastrointestinal endoscopy and biopsy revealed tubular adenomas with a diameter of 2.2 cm in the major papilla of the duodenum. She underwent total thyroidectomy with modified neck dissection and pancreaticoduodenectomy. Histological examination revealed multiple papillary carcinomas in the thyroid gland and well-differentiated tubular adenocarcinoma of the papilla Vater. Once invasive cancer of the duodenal papilla has developed, high mortality rate is expected. Therefore, it is important to repeat follow-up endoscopic examinations with biopsy for duodenal papilla. In addition, regular examination of the thyroid gland including palpation and ultrasound examination should be a part of the routine follow-up for a familial adenomatous polyposis patient. The case of familial adenomatous polyposis complicated with intraabdominal desmoid, duodenal cancer, and thyroid papillary cancer is very rare.