Advanced duodenal disease in familial adenomatous polyposis: how frequently should patients be followed up after successful therapy?

• Published in: Familial cancer Vol. 11; no. 4; pp. 553 - 557
• Main Authors: Balmforth, Damian C., Phillips, Robin K. S., Clark, Susan K.
• Format: Journal Article
• Language: English
• Published: Dordrecht Springer Netherlands 01.12.2012; Springer Nature B.V
• Subjects: Adenocarcinoma - etiology; Adenocarcinoma - pathology; Adenomatous Polyposis Coli - complications; Adenomatous Polyposis Coli - genetics; Adult; Biomedical and Life Sciences; Biomedicine; Cancer Research; Disease Progression; Duodenal Diseases - etiology; Duodenal Diseases - pathology; Duodenoscopy; Epidemiology; Female; Follow-Up Studies; Human Genetics; Humans; Long-Term Care; Male; Middle Aged; Neoplasm Staging; Original Article; Prognosis; Prospective Studies; Duodenal adenomas; Duodenoscopy; Spigelman staging; Familial adenomatous polyposis
• ISSN: 1389-9600; 1573-7292
• DOI: 10.1007/s10689-012-9546-2

Duodenal polyposis is found in the majority of patients with familial adenomatous polyposis. Endoscopic surveillance programmes grade the severity of duodenal disease according to the Spigelman classification (stages 0–IV) to identify patients at risk of developing adenocarcinoma. To evaluate the progression of duodenal polyposis in patients with a previous diagnosis of Spigelman stage IV disease who have been downstaged by endoscopic or pharmacological means. A database search of a large polyposis registry identified patients who had been downstaged from stage IV disease and had further opportunity for disease progression. These patients were divided into three groups according to their new Spigelman stage. A measure of a patient’s disease progression was obtained by the increase in stage over the recommended follow up time period for their new, reduced, Spigelman stage. Group 1 (n = 16) were downstaged to stage III disease, with 50 % progressing back to stage IV over the recommended 1-year follow up period. Group 2 (n = 19) were downstaged to stage II disease, with 84 % progressing over the recommended 3-year follow up period. Group 3 (n = 6) were downstaged to stage I disease, with 100 % progressing over the recommended 5-year follow up period. Patients downstaged from Spigelman stage IV demonstrate an increased rate of disease progression in comparison to reported rates of primary disease progression. An amendment to the current endoscopic surveillance protocol is recommended to ensure that once a patient has been diagnosed with stage IV disease they are treated as a high-risk patient in perpetuity.