Surveillance of Duodenal Adenomas in Familial Adenomatous Polyposis Reveals High Cumulative Risk of Advanced Disease Jean-Christophe Saurin, Christelle Gutknecht, Bertrand Napoleon, Annick Chavaillon, René Ecochard, Jean-Yves Scoazec, Thierry Ponchon, and Jean-Alain Chayvialle A B S T R A C T Purpose The development of high-grade dysplasia (HGD) on duodenal or jejunal adenomas and of late-stage (stage IV) duodenal polyposis are major clinical events for familial adenomatous polyposis (FAP) patients. Our aim was to determine their respective frequency, risk factors, and cumulative risk. Patients and Methods A prospective, optimized, endoscopic surveillance protocol was applied to 58 FAP patients in a university hospital. The number, size, and histology of duodenojejunal polyps were assessed, and the Spigelman’s score was calculated at each endoscopy. Cox regression and linear regression analysis were used to determine risk factors for HGD development and the cumulative risk of stage IV duodenal polyposis, respectively. Results During a median ( standard deviation) follow-up of 47.9  15.6 months, 35 patients with at least two consecutive examinations had 107 duodenojejunal examinations. The Spigelman’s score increased in 21 patients (60.0%), and HGD developed in 12 patients (34.2%). High initial Spigelman’s score ( 7 points), but not age or APC mutation site, was a risk factor for HGD development. Estimated cumulative risk of developing stage IV duodenal polyposis was of 42.9% at age 60 (95% CI, 35.7% to 50.0%) and 50.0% at age 70 (95% CI, 42.9% to 57.1%). Conclusion This prospective series shows a higher duodenal polyposis progression rate and cumulative risk of late-stage (stage IV) duodenal polyposis in FAP patients compared with previous series. These results suggest that current modalities for surveillance and management of these patients need revision. J Clin Oncol 22:493-498. © 2004 by American Society of Clinical Oncology INTRODUCTION Proximal small bowel cancer is one of the two leading causes of death (the other being desmoid tumors) in familial adenomatous polyposis (FAP) patients with previous co- lectomy [1,2]. Cancer of the proximal small bowel in FAP develops from pre-existing adenomas, which are present in approxi- mately 100% of patients in the duodenum. These duodenal adenomas can be classified through macroscopic and histologic criteria in five stages (0 to IV) following the Spigelman’s classification. In a large series, 44% of FAP patients presented with a severe duodenal polyposis (stage III or IV) and were considered at higher risk of developing duodenojejunal cancer. Currently, two strategies are under discussion in FAP pa- tients with duodenal polyposis. First, pa- tients with advanced adenomas (larger than 1 cm) or with high-grade dysplasia (HGD) may be referred for endoscopic treatment by several means, the most common being mu- cosectomy [3]. Second, these patients may be simply followed up until they develop very advanced duodenal polyposis (stage IV) or duodenal cancer and treated at that time by pancreaticoduodenectomy (PD) [4,5]. The selection of the appropriate strat- From the Federation des Specialites Digestives and Service d’Anatomie Pathologique, Hopital E. Herriot; and Departement de Biostatistiques, Hos- pices Civils de Lyon, Lyon, France. Submitted June 6, 2003; accepted November 24, 2003. The study has been presented in part as an oral communication at the Diges- tive Disease Week in Orlando, FL, May 18, 2003. Authors’ disclosures of potential con- flicts of interest are found at the end of this article. Address reprint requests to J.C. Saurin, MD, Service d’Hepatogastroenterologie, Pavillon I, Hôpital Edouard Herriot, 5 Place d’Arsonval, 69437 Lyon cedex 03, France; e-mail: saurin@lyon.inserm.fr. © 2004 by American Society of Clinical Oncology 0732-183X/04/2203-493/$20.00 DOI: 10.1200/JCO.2004.06.028 JOURNAL OF CLINICAL ONCOLOGY O R I G I N A L R E P O R T VOLUME 22  NUMBER 3  FEBRUARY 1 2004 493 Downloaded from ascopubs.org by 3.236.66.163 on June 6, 2022 from 003.236.066.163 Copyright © 2022 American Society of Clinical Oncology. All rights reserved.