Background: The reviewing and assessment of epidemiological characteristics of neuroendocrine tumours (NETs) remains a challenge. Despite the fact that it is an uncommon family of neoplasms, several worldwide series have revealed an increasing incidence of this rare condition. However, the data are difficult to compare over time due to changes in classification.
Methods: We compared the data related to incidence, prevalence, stage of the disease at diagnosis and survival reported in several series, focusing on the differences and trying to examine some of the probable reasons that may explain the variations in the results between studies.
Results and conclusions: The incidence of NETs is increasing over time, and their incidental discovery due to improved and more frequent imaging does not seem to be enough to explain this rise. Significant differences can be found between geographic regions and races, suggesting that environmental or genetic factors may contribute to the clinical and biological behaviour of these tumours; increasing our knowledge of oncogenesis will be necessary to explain them. As with other rare diseases, creating specific databases and multidisciplinary working groups would improve the accuracy of the information gained.
© 2016 S. Karger AG, Basel.