Incidence and survival of patients with malignant pancreatic neuroendocrine neoplasms in Germany, 2009–2021
Andreas Stang, Ina Wellmann, Bernd Holleczek, Soo-Zin Kim-Wanner, Jacqueline Müller‐Nordhorn, Eunice Sirri, Ian Wittenberg, Jens T. Siveke, Hiltraud Kajüter
Abstract
Due to the rarity of pancreatic neuroendocrine neoplasms, only few population-representative studies on incidence and survival have been conducted. The aim was to provide up-to-date nationwide incidence and relative survival estimates of neuroendocrine (NE) neoplasms overall, NE tumors (NETs), NE carcinomas (NECs), and mixed NE neoplasms (MiNEN). We distinguished between pancreatic NETs (functioning versus non-functioning), NECs and mixed NE neoplasms and analyzed data from 2009 through 2021 from all German cancer registries covering a population of more than 80 million. We calculated crude and age-standardized incidence rates and 5-year relative survival estimates (RS). Overall 6474, 4217, and 243 patients with pancreatic NETs, NECs, and mixed NE neoplasms, respectively were registered. While the age-standardized incidence of NETs has increased (+16.4 % per year, 95 %CI 12.2;20.7), the incidence of NEC has fallen (about −6.4 % per year, 95 %CI −8.0; −4.8). The crude RS was 77.7 % (standard error [SE] 0.9) for non-functioning NETs, 90.3 % for functioning NETs (SE 3.9), and 18.5 % (SE 3.9) for MiNEN. Large and small cell NECs had a low RS (9.1 % and 6.9 %, respectively). RS for G1 NETs was 88.2 %, while it was only 36.6 % for G3 NETs. Localized NETs had a RS of 92.8 %, while distant metastatic NETs had a RS of 45.0 %. The incidence of pancreatic NETs has increased markedly in Germany in the period 2009–2021. Subgroups of NETs (G1 grading or localized stage) have an excellent prognosis. RS of MiNEN is more similar to NECs than NETs. • The incidence of neuroendocrine tumors (NET), small and large cell neuroendocrine carcinomas (NEC) has increased over time. • Relative 5-year survival (5 yr-RS) was 78 % for non-functioning NETs, 90 % for functioning NETs. • Large and small cell NECs had a very low 5-yr RS (9 % and 7 %, respectively). • 5-yr RS for G1 NETs was 88 %, while it was only 37 % for G3 NETs. • Localized NETs had a 5-yr RS of 93 %, while distant metastatic NETs had a 5-yr RS of 45 %.