Singapore Med J 2011; 52(4) : 246 Original Article Department of Colorectal Surgery, Singapore General Hospital, Outram Road, Singapore 169608 Chew MH, MBBS, MRCSE, FRCSE Associate Consultant Quah HM, MBBS, FRCSE, FAMS Consultant Loi TTC, MSc HCM, GDipGenetCouns Genetic Counsellor Eu KW, MBBS, FRCSE, FAMS Senior Consultant Tang CL, MBBS, FRCSE, FAMS Senior Consultant and Head Yong Loo Lin School of Medicine, National University of Singapore, 1E Kent Ridge Road, Singapore 119228 Teh KL Medical Student Correspondence to: Dr Hak-Mien Quah Tel: (65) 6321 4677 Fax: (65) 6226 2009 Email: quah.hak. mien@sgh.com.sg Twenty years of familial adenomatosis polyposis syndromes in the Singapore Polyposis Registry: an analysis of outcomes Chew M H, Quah H M, Teh K L, Loi T T C, Eu K W, Tang C L ABSTRACT Introduction: The Singapore Polyposis Registry (SPR) was established in 1989 at the Singapore General Hospital. This initiative was aimed at providing a central registry service to facilitate identification, surveillance and management of families and individuals at high risk of colorectal cancer. The aim of the present study was to provide a comprehensive review of all patients with familial adenomatous polyposis (FAP) syndrome in the SPR. Methods: All patients diagnosed with FAP in 1989–2009 were analysed. Data was extracted from a prospectively collected database. Results: 122 patients from 88 families were analysed. The median age of this cohort was 29 (range 10–68) years. 97 percent of the cases were FAP and 3 percent were attenuated FAP. 92 patients tested positive for adenomatous polyposis coli gene. 42 percent of patients were diagnosed with colorectal cancer, of which 78 percent were diagnosed at an advanced stage. 73 percent of patients underwent restorative proctocolectomy and 21 percent had total colectomy. The median age at operation was 30 years. At median follow-up of 98 months, ten-year overall survival was 75.6 percent (95 percent confidence interval 67.0–84.2) and the median age at death was 40 years. For cancer cases, the overall recurrence was 13.5 percent. Recurrence and disease-free survival were not significant for the type of surgery performed (p-value is 0.486). Conclusion: The SPR plays an important and integral part in counselling patients and families with FAP. Improved surveillance programmes may be required to detect the development of cancers in these patients at an earlier stage. Keywords: colorectal cancer, familial adenomatous polyposis, outcomes Singapore Med J 2011; 52(4): 246-251 INTRODUCTION The irst polyposis registry, the St Mark’s Hospital Polyposis Register, was conceived by JP Lockhart- Mummery and C Dukes in 1924. (1) It was only around 1950 that a policy for cancer prevention and early detection of polyposis evolved with the improved knowledge of the epidemiology and incidence of polyposis cancers. (1) Since then, many national and regional registries have been set up, with considerable impact on the reduction of colorectal cancer (CRC) in familial adenomatous polyposis (FAP). These include registries in Europe, (2-5) Canada (5) as well as Asia, (6, 7) and number more than 50 worldwide. The Singapore Polyposis Registry (SPR) was established in 1989 at the Singapore General Hospital (SGH). (8) The aim of this initiative was to provide a central registry service to all doctors in Singapore in order to facilitate identiication, surveillance and management of families and individuals at high risk of CRC. From an initial emphasis on FAP and hereditary non-polyposis CRC, patients with other polyposis types, such as Peutz Jeghers, juvenile polyposis and hereditary mixed polyposis syndromes, have also been included. The SPR has also evolved and is currently backed by molecular biology, cell-kinetic and anorectal physiology laboratories. Patients are thus assured of accurate pre- or post-clinical diagnosis, genetic testing and detailed post- surgical functional assessments for those who undergo pouch operations. Various research projects performed using data from the SPR have also resulted in important genetic testing and identiication of at-risk individuals. This has led to important data that improve diagnosis as well as survival and genetic counselling for patients. (9,10) The aim of the present study was to provide a comprehensive review of all high-risk individuals with FAP in the SPR, and to evaluate phenotypic