Borbath Ivan, Garcia-Carbonero Rocio, Bikmukhametov Damir, Jimenez-Fonseca Paula, Castaño Angel, Barkmanova Jaroslava, Sedlackova Eva, Kollár Attila, Christ Emanuel, Kaltsas Gregory, Kos-Kudla Beata, Maasberg Sebastian, Verslype Chris, Pape Ulrich-Frank
Hepato-gastroenterology Unit, Cliniques Universitaires Saint-Luc, Bruxelles, Belgium.
Oncology Department, Hospital Universitario 12 de Octubre, IIS Imas12, UCM, CNIO, CIBERONC, Madrid, Spain.
Eur J Cancer. 2022 Jun;168:80-90. doi: 10.1016/j.ejca.2022.03.007. Epub 2022 Apr 23.
Neuroendocrine neoplasms (NENs) are rare tumours with variable clinical behaviour. Their natural history is ideally best approached in large, multicentre and multinational registries with long-term patients' follow-up. The European Neuroendocrine Tumour Society registry aims to obtain information regarding NEN outcomes and prognostic factors in a European frame.
We collected data from 7 national NEN registries (Belgium, Czech Republic, Germany, Greece, Poland, Spain, Switzerland), representing 10,102 patients. Anonymised/pseudonymised data were collected in a secured server. Descriptive statistical methods were applied, as well as Kaplan-Meier survival curves and multivariable analyses for prognostic factors of overall survival (OS).
median age of the study population was 60 years (range: 18-102), 48% were female. Common primary tumour sites were pancreas (27%) and small intestine (21%). Stage 4 disease was found in 47% of patients, while 26/10/16% had stage 1/2/3 disease, respectively. Grading (n = 6952) was G1/2/3 in 48/37/15% of the patients, respectively. Surgery was the main treatment, provided to 71% of patients, followed by somatostatin analogues (32%), chemotherapy (20%), Peptide receptor Radionuclide Therapy (PRRT) (9%) and targeted therapies (8%). OS at 5 years was 74%, influenced by grade, stage and tissue of origin in multivariate analysis. A Ki67 cut-off value set at 55% within the G3 group allowed to separate 2 groups with a meaningful different OS.
We report the first analysis of the European Neuroendocrine Tumour Society registry, comprising 10,102 patients with NEN from 7 European countries. This large cohort study describes prognostic factors for the survival of NENs throughout Europe, including primary tumour site, grade, stage and treatment.
神经内分泌肿瘤（NENs）是临床行为各异的罕见肿瘤。其自然病程最好在大型、多中心和跨国登记处对患者进行长期随访的情况下进行研究。欧洲神经内分泌肿瘤学会登记处旨在获取欧洲范围内有关NENs结局和预后因素的信息。
我们收集了来自7个国家NEN登记处（比利时、捷克共和国、德国、希腊、波兰、西班牙、瑞士）的10102例患者的数据。匿名/化名数据收集于安全服务器中。应用了描述性统计方法，以及Kaplan-Meier生存曲线和总生存（OS）预后因素的多变量分析。
研究人群的中位年龄为60岁（范围：18 - 102岁），48%为女性。常见的原发肿瘤部位是胰腺（27%）和小肠（21%）。47%的患者为4期疾病，而26/10/16%的患者分别为1/2/3期疾病。分级（n = 6952）的患者中，G1/G2/G3分别占48/37/15%。手术是主要治疗方式，71%的患者接受了手术，其次是生长抑素类似物（32%）、化疗（20%）、肽受体放射性核素治疗（PRRT）（9%）和靶向治疗（8%）。5年总生存率为74%，多变量分析显示其受分级、分期和起源组织的影响。在G3组中，将Ki67临界值设定为55%可将患者分为两组，其总生存率有显著差异。
我们报告了对欧洲神经内分泌肿瘤学会登记处的首次分析，该登记处包含来自7个欧洲国家的10102例NEN患者。这项大型队列研究描述了整个欧洲NENs生存的预后因素，包括原发肿瘤部位、分级、分期和治疗。
