ORIGINAL PAPER Epidemiology of neuroendocrine cancers in an Australian population Colin Luke • Timothy Price • Amanda Townsend • Christos Karapetis • Dusan Kotasek • Nimit Singhal • Elizabeth Tracey • David Roder Received: 26 August 2009 / Accepted: 4 February 2010 / Published online: 24 April 2010 Ó Springer Science+Business Media B.V. 2010 Abstract Objective The aim was to explore incidence, mortality and case survivals for invasive neuroendocrine cancers in an Australian population and consider cancer control implications. Methods Directly age-standardised incidence and mor- tality rates were investigated from 1980 to 2006, plus disease-specific survivals. Results Annual incidence per 100,000 increased from 1.7 in 1980–1989 to 3.3 in 2000–2006. A corresponding mortality increase was not observed, although numbers of deaths were low, reducing statistical power. Increases in incidence affected both sexes and were more evident for female lung, large bowel (excluding appendix), and unknown primary site. Common sites were lung (25.9%), large bowel (23.3%) (40.9% were appendix), small intes- tine (20.6%), unknown primary (15.0%), pancreas (6.5%), and stomach (3.7%). Site distribution did not vary by sex (p = 0.260). Younger ages at diagnosis applied for lung (p = 0.002) and appendix (p \ 0.001) and older ages for small intestine (p \ 0.001) and unknown primary site (p \ 0.001). Five-year survival was 68.5% for all sites combined, with secular increases (p \ 0.001). After adjust- ing for age and diagnostic period, survivals were higher for appendix and lower for unknown primary site, pancreas, and colon (excluding appendix). Conclusions Incidence rates are increasing. Research is needed into possible aetiological factors for lung and large- bowel sites, including tobacco smoking, and excess body weight and lack of exercise, respectively; and Crohn’s disease as a possible precursor condition. Keywords Incidence Á Mortality Á Survival Á Aetiology Á Risk factors Introduction Neuroendocrine cancers are rare malignancies of the neu- roendocrine system, reported in population and institu- tionally based studies to account for fewer than one per cent of all invasive cancers in Western populations [1, 2]. Annual incidence rates vary by study from around one to five per 100,000 persons [2–8]. Importantly, incidence has increased in the USA over time, and there appear to be variations by race with Afro-Americans having higher incidence rates than the white population [1, 7–9]. C. Luke (&) Epidemiology Branch, South Australian Department of Health, PO Box 6, Rundle Mall, Adelaide, SA 5000, Australia e-mail: jowen@cancersa.org.au T. Price Á A. Townsend Department of Haematology-Oncology, Queen Elizabeth Hospital, Woodville, SA, Australia C. Karapetis Department of Medical Oncology, Flinders Medical Centre, Bedford Park, SA, Australia D. Kotasek Ashford Cancer Centre, Ashford, SA, Australia N. Singhal Cancer Centre, Royal Adelaide Hospital, Adelaide, SA, Australia E. Tracey NSW Cancer Institute, Everleigh, NSW, Australia D. Roder Research and Information Science, Cancer Council South Australia, Eastwood, SA, Australia 123 Cancer Causes Control (2010) 21:931–938 DOI 10.1007/s10552-010-9519-4