背景:由于胰腺神经内分泌肿瘤的罕见,仅开展了少数人群代表性的发病率和生存率研究.目的是提供神经内分泌(NE)肿瘤总体的最新全国发病率和相对生存率估计,NE肿瘤(NET),NE癌(NEC),和混合NE肿瘤(MiNEN)。
方法:我们区分了胰腺NETs(功能性和非功能性),NEC和混合NE肿瘤,并分析了2009年至2021年来自德国所有癌症登记处的数据,涵盖了超过8000万的人口。我们计算了粗和年龄标准化的发病率和5年相对生存估计(RS)。
结果:总共6474、4217和243例胰腺NETs患者,NEC,和混合的NE肿瘤,分别注册。虽然NETs的年龄标准化发病率有所增加(每年+16.4%,95CI12.2;20.7),NEC的发病率下降了(每年约-6.4%,95CI-8.0;-4.8)。无功能NET的粗RS为77.7%(标准误差[SE]0.9),90.3%用于正常运行的NET(SE3.9),和18.5%(SE3.9)的MiNEN。大细胞和小细胞NEC的RS较低(9.1%和6.9%,分别)。G1NET的RS为88.2%,而G3NET只有36.6%。本地化NET的RS为92.8%,而远处转移性NETs的RS为45.0%。
结论:2009-2021年期间,德国胰腺NETs的发病率显著增加。NETs亚组(G1分级或局部分期)预后良好。MiNEN的RS比NETs更类似于NECs。
BACKGROUND: 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).
METHODS: 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).
RESULTS: 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 %.
CONCLUSIONS: 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.