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Duodenal cancer and desmoid tumors are the most common causes of death in patients with familial adenomatous polyposis (FAP) who have undergone proctocolectomy to prevent colorectal cancer. The prevalence of duodenal adenomas in patients with FAP seems to be increasing in clinical practice.
To study cancer risk in this population, researchers reviewed data from 1019 patients with FAP in a British polyposis registry. From 1969 to 2005, 20 patients (60% men) from 18 kindreds were diagnosed with cancer (9 duodenal and 11 ampullary) at a median age of 53. Twelve of the 20 developed cancer before the era of endoscopic surveillance of the duodenum; 17 died of cancer at a median age of 57.
All the ampullary and duodenal cancers for which endoscopic …