Rising Incidence of Neuroendocrine Neoplasms in Northern Switzerland-Data From the Cancer Registry

Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors that arise in multiple organs and encompass pheochromocytomas/paragangliomas (PPGLs). Adrenocortical carcinoma (ACC), though distinct, is a rare endocrine malignancy with a poor prognosis. We analyzed incidence and survival trends across NENs and ACC over 4 decades.

We conducted a population-based study using cancer registry data from the Canton of Zurich (1980-2022). NENs were classified by site and histology. Age-standardized incidence rates (ASIRs) were calculated per 100,000 person-years (European standard population). Joinpoint regression estimated the annual percent change (APC) for each trend segment and the average APC (AAPC) for the period.

A total of 2723 patients with a diagnosis of a NEN (n = 2647) or ACC (n = 76) between 1980 and 2022 were extracted from the database. ASIR of gastrointestinal NENs rose from 1.4 in 1980 to 11.3 per 100,000 in 2022 [AAPC +5.07%, 95% confidence interval (CI) 4.20-6.50%], with the most pronounced increases in rectal and appendiceal NENs, particularly since the early 2000s. Pancreatic NEN incidence also increased, especially from 2004 to 2022 (0.6-2.7 per 100,000; APC 5.36%, 95% CI 3.13-16.97%). ASIR of PPGLs rose from 0 in 1980 to 0.4 per 100,000 by 2022, while the ASIRs of ACC remained stable with ASIR of 0.2 per 100,000 in 2022.

The incidence of gastroenteropancreatic NENs and PPGLs continues to rise, with a pronounced acceleration since the early 2000s. These trends underscore the need for a deeper understanding of risk factors underlying NEN development.