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Eur J Cancer. 2013 Jul;49(11):2565-78. doi: 10.1016/j.ejca.2013.02.029. Epub 2013 Mar 27.

Rare neuroendocrine tumours: results of the surveillance of rare cancers in Europe project.

Author information

Department of Registry and Research, Comprehensive Cancer Centre the Netherlands, Catharijnesingel 55-h, Utrecht, The Netherlands. Electronic address:
Department of Preventive and Predictive Medicine, Fondazione IRCSS, Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy. Electronic address:
Department of Registry and Research, Comprehensive Cancer Centre the Netherlands, Catharijnesingel 55-h, Utrecht, The Netherlands. Electronic address:
Basque Country Cancer Registry, San Sebastian, Spain; Consortium for Biomedical Research in Epidemiology and Public Health (CIBERESP), San Sebastian, Spain. Electronic address:
Department of Cancer Epidemiology, Istituto Superiore di Sanità, Viale Regina Elena 299, Rome, Italy. Electronic address:
Girona Epidemiology Unit and Cancer Registry, Oncology Planning, Department of Health, Girona, Passatge Farinera Teixidor, núm 1 1r-2a, 17005 Girona, Spain; Catalan Institute of Oncology, Institute of Biomedical Research Investigation, Av. França s/n, 17007 Girona, Spain. Electronic address:
Department of Oncology and Anatomic Pathology and, General Hospital of Treviso, Piazza Ospedale 1,Treviso, Italy. Electronic address:
Institut Gustave-Roussy, 39 Rue Camille Desmoulins, Villejuif Cedex, France. Electronic address:
Aintree University Hospital, Longmoor Lane, Liverpool L9 7AL, United Kingdom. Electronic address:
Department of Internal Medicine - Endocrinology, University Medical Hospital Groningen, Hanzeplein 1 Postbus 30.001, 9700 RB Groningen, The Netherlands. Electronic address:


Because of the low incidence, and limited opportunities for large patient volume experiences, there are very few relevant studies of neuroendocrine tumours (NETs). A large population-based database (including cancer patients diagnosed from 1978 to 2002 and registered in 76 population-based cancer registries [CRs]), provided by the project 'surveillance of rare cancers in Europe' (RARECARE) is used to describe the basic indicators of incidence, prevalence and survival of NETs, giving a unique overview on the burden of NETs in Europe. NETs at all cancer sites, excluding lung, were analysed in this study. In total over 20,000 incident cases of NETs were analysed and a data quality check upon specific NETs was performed. The overall incidence rate for NETs was 25/1,000,000 and was highest in patients aged 65 years and older with well differentiated endocrine carcinomas (non-functioning pancreatic and gastrointestinal) (40 per 1,000,000). We estimated that slightly more than 100,000 people were diagnosed with NETs and still alive in EU27 at the beginning of 2008. Overall, NETs had a 5 year relative survival of 50%; survival was low (12%) for poorly differentiated endocrine carcinoma, and relatively high (64%) for well differentiated carcinoma (not functioning of the pancreas and digestive organs). Within NETs, endocrine carcinoma of thyroid gland had the best 5-year relative survival (82%). Because of the complexity and number of the different disciplines involved with NETs (as they arise in many organs), a multidisciplinary approach delivered in highly qualified reference centres and an international network between those centres is recommended.

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