N80: An International Study Of Duodenal Disease In MAP: Incidence Of Polyposis And Cancer

L. E. Thomas1, A. Alonso Sanchez2, M. R. Aznárez2, A. Backman3, 4, J. Bjork3, 4, G. Capella5, S. K. Clark6, 7, C. Colas8, E. Dekker9, S. Dolwani10, Z. Ghorbanoghli11, M. Gonn3, 4, S. Gonzalez Romero5, F. J. Hes12, J. J. Hurley13, H. Jundi1, A. Latchford6, H. Leon Brito2, E. Meuser1, M. E. Mork14, M. Mort1, M. Navarro Garcia5, M. Neilsen12, Y. Parc15, M. T. Ricci16, J. C. Saurin17, K. van der Tuin12, H. Vasen11, E. Vilar14, 18, O. Vinet17, S. J. Walton6, 7, H. D. West1, J. R. Sampson1

1 – Division of Cancer and Genetics, Cardiff University, School of Medicine, Cardiff, CF14 4XN, UK. 2 – GI Department, Complejo Hospitalario de Navarra, Pamplona, Spain. 3 – Hereditary Cancer Unit, Cancer Division, Karolinska University Hospital Stockholm, Sweden. 4 – Institution of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden. 5 – Programa de Càncer Hereditari, Institut Català d’Oncologia, L’Hospitalet de Llobregat, Spain. 6 – The Polyposis Registry, St Marks Hospital, Watford Road, Harrow HA1 3UJ, UK. 7 – Department of Surgery and Cancer, Faculty of Medicine, Imperial College, London SW7 2AX, UK. 8 – Hopital Saint-Antoine AP-HP, Université Pierre et Marie Curie, Paris, France. 9 – Department of Gastroenterology and Hepatology, Academic Medical Centre, Amsterdam, The Netherlands. 10 – Division of Population Medicine, Cardiff University School of Medicine, Cardiff CF14 4XN, UK. 11 – Department of Gastroenterology & Hepatology, Leiden University Medical Centre, Leiden, The Netherlands. 12 – Leiden University Medical Center (LUMC), Department of Clinical Genetics, Leiden, The Netherlands. 13 – Dept. of Gastroenterology, Prince Charles Hospital, Merthyr Tydfil, CF47 9DT, UK. 14 – Clinical Cancer Genetics Program, UT MD Anderson Cancer Center, USA. 15 – Hopital Saint-Antoine AP-HP, Université Pierre et Marie Curie, Paris, France. 16 – Unit of Hereditary Digestive Tract Tumors, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 17 – Digestive Department, Edouard Herriot Hospital, Lyon, France. 18 – Department of Clinical Cancer Prevention, UT MD Anderson Cancer Center, USA.


Aim: Duodenal polyposis and cancer represent significant disease manifestations in patients with FAP and MAP. This study aims to determine the extent and incidence of duodenal disease in patients with MAP to establish whether upper GI surveillance recommendations developed for patients with FAP are also appropriate for MAP.

Method: A long-term prospective collaboration has been established. Demographic and genotype information and details of endoscopic surveillance and therapy has been collected on 394 MAP patients to date.

Results: 63/394 had duodenal disease at index endoscopy (16%) at a median age of 54 years (range; 33-81): this was Spigelman stage I in 37 patients (58.7%), stage II in 12 (19%), stage III in 10 (15.9%), stage IV in 1 patient and three patients had cancer (4.8%). During 1417 follow up years, five further patients progressed to stage IV disease at a median age of 63 (range; 51-67) and one patient developed cancer.

Conclusion: Patients with MAP appear to develop fewer duodenal polyps at a more advanced age than is reported in FAP. Nonetheless, progression to advanced disease and cancer may occur despite surveillance. We are collecting prospective data that may inform development of a more appropriate surveillance strategy for upper GI disease in MAP.