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

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Context: 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. Methods: 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. Results: 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. Conclusion: 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.

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