Clinical ResearchPeriampullary adenomas and adenocarcinomas in familial adenomatous polyposis: Cumulative risks and APC gene mutations☆,☆☆
Section snippets
Patients
The Swedish Polyposis Registry includes data on 467 patients with verified FAP from 154 Swedish FAP-families registered since the late 1950s.5 One hundred ten of the patients were dead before introduction of EGD surveillance, and another 62 were not screened because at the end of the study period they were younger than 30 years old, the recommended starting age for screening in Sweden. Thus, 265 patients remained, and the registry had EGD surveillance information for 180 (68%) of these patients
Duodenal adenomas
Duodenal adenomas were found in 134 (74%) of the FAP patients. As shown in Figure 1, the cumulative age-dependent risks of duodenal adenomatosis were 80% (95% CI, 72.9-87.1) and 98% (95% CI, 95-100) at ages 60 and 75 years, respectively.
Stage IV periampullary adenomas
As shown in Table 1, stage IV
Discussion
We found that 74% of the FAP patients screened by upper endoscopy had duodenal adenomas, compared with the 33%-92% reported in previous studies of 100 or more patients.8, 16, 17, 18, 19 However, the cumulative risk in our material was almost 100% at the age of 75 years, indicating that all FAP patients eventually develop duodenal adenomas. The malignant potential of the most common lesions of the duodenum, the sessile adenomatous polyps predominantly located at the mucosal folds, seems to be
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Address requests for reprints to: Jan Björk, M.D., Ph.D., The Swedish Polyposis Registry, Department of Gastroenterology and Hepatology, Karolinska Hospital, S-171 76 Stockholm, Sweden. e-mail: [email protected]; fax: (46) 8-51775768.
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Supported by grants from the Cancer Society in Stockholm and the Karolinska Institute and the King Gustav V Jubilee Clinic Cancer Research Foundation.