FAP is characterised by innumerable adenomatous polyps throughout the colorectum and inevitable development of colorectal carcinoma usually by the fifth decade of life, if colectomy is not performed. Duodenal adenomas are found in 30-70% of FAP patients. The lifetime risk of duodenal adenoma development is virtually 100%. FAP patients have a 100-330-fold higher risk of developing duodenal cancer compared with the general population and an absolute lifetime risk of about 5%. No clear genotype-phenotype correlation exists, although mutations in the 3′ end of the APC gene (exon 15) appear to cause more severe duodenal manifestations. First screening for upper gastrointestinal adenoma is recommended at age 25-30 years. After baseline endoscopy, screening for duodenal polyposis is recommended as per Spigelman stage (see table 3). Recurrence of duodenal lesions after local endoscopic or surgical excision is common. Pancreaticoduodenectomy is the appropriate treatment for Spigelman stage IV duodenal polyposis and can be considered for stage III. Results of chemoprevention/regression studies for duodenal adenomas are equivocal or disappointing.
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