Details

Autor(en) / Beteiligte

• van der Zwan, Jan Maarten
• Trama, Annalisa
• Otter, Renée
• Larrañaga, Nerea
• Tavilla, Andrea
• Marcos-Gragera, Rafael
• Dei Tos, Angelo Paolo
• Baudin, Eric
• Poston, Graeme
• Links, Thera

Titel

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

Ist Teil von

• European journal of cancer (1990), 2013-07, Vol.49 (11), p.2565-2578

Ort / Verlag

Kidlington: Elsevier Ltd

Erscheinungsjahr

2013

Link zum Volltext

Quelle

MEDLINE

Beschreibungen/Notizen

• Abstract 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.