Aims: The aim of this study was to investigate the epidemiology of histology-proven
Neuroendocrine neoplasms (NENs) in an Italian area.
Background: NENs are a rare and poorly known disease and the global incidence and prevalence appear
to be increasing over the past decades.
Objective: The objectives of this study were to estimate the incidence and trends of NENs in a
250,000-inhabitant area in the North-East of Italy in the 1998-2018 period and to compare them with
Methods: This retrospective cohort study was based on the analysis of anonymous health administrative
databases, linked with each other at individual patient level through an anonymous stochastic key.
NENs were identified from the anatomical pathology database.
The standardized incidence rate (2010ESP and US2000) ± 95% CI per 100,000 were calculated, both
annually and globally, for the whole period. Incidence was also calculated for specific anatomical sites
and by gender.
Trends for the considered periods and sites were summarized through the annual percent change (APC)
and average increase (cases per 100,000 per year).
Results: In the 1998-2018 period, the standardized incidence rate of NENs in the area of Udine was
2.49 (APC 3.33). A total of 162 cases were observed (51.2% males). Differences in incidence and
trend were observed between sexes. The obtained results were consistent with those reported in other
countries, confirming a significant and steady increase in NENs incidence in the last twenty years.
Conclusion: This study provides new epidemiological data on NENs in Italy. The observed sex differences
deserve further investigations.