Treatment Approaches and Outcome of Patients with Neuroendocrine Neoplasia Grade 3 in German Real-World Clinical Practice

Abstract

Background: Neuroendocrine neoplasia grade 3 (NEN G3) represents a rare and heterogeneous cancer type with a poor prognosis. The aim of our study was to analyze real-world data from the German NET Registry with a focus on therapeutic and prognostic aspects. Methods: NEN G3 patients were identified within the German NET Registry. Demographic data and data on treatments and outcomes were retrieved. Univariate analyses were performed using the Kaplan–Meier-method. Multivariate analysis was performed using a Cox proportional hazard model. Results: Of 445 included patients, 318 (71.5%) were diagnosed at stage IV. Well-differentiated morphology (NET G3) was described in 31.7%, 60% of cases were classified as neuroendocrine carcinoma (NEC), and the median Ki67 value was 50%. First-line treatment comprised chemotherapy in 43.8%, with differences in the choice of regimen with regard to NET or NEC, and surgery in 41.6% of patients. Median overall survival for the entire cohort was 31 months. Stage, performance status and Ki67 were significant prognostic factors in multivariate analysis. Conclusions: The survival data of our national registry compare favorably to population-based data, probably mainly because of a relatively low median Ki67 of 50%. Nevertheless, the best first- and second-line approaches for specific subgroups remain unclear, and an international effort to fill these gaps is needed.

Efficacy of first-line chemotherapy based on primary site of tumor versus etoposide-platinum in advanced gastroenteropancreatic neuroendocrine carcinoma

“…Given that some of these neoplasms share similar immunohistochemical characteristics with small cell lung cancer (SCLC), chemotherapy of etoposide-platinum (EP), the standard of care for SCLC, is widely used in gastroenteropancreatic NECs (GEP-NECs) ( 7 , 8 ). Several retrospective studies evaluated the efficacy of EP as first-line chemotherapy in GEP-NECs and found a median response rate of 42.95% (28–62.5%), a median progression-free survival (PFS) of 5.65 months and a median OS of 11.67 months ( 9 - 11 ). However, according to National Comprehensive Cancer Network (NCCN) guidelines ( 12 ), the recommendation for EP regimen in GEP-NECs is only grouped into category 2A, which means the adoption of this regimen was based upon only low-level evidence, indicating that there is a lack of optimal treatment regimen for GEP-NECs.…”

Site‐specific trends in gastroenteropancreatic neuroendocrine neoplasms in Bavaria, Germany

“…Published data from Germany and other European countries have often focused on retrospective analysis of oncological studies, 19 mostly obtained from referral centers, or the analysis of specific subgroups. 20,21 Due to national restrictions and data protection rules, large population-based data such as provided by the Surveillance, Epidemiology, and End Results (SEER) Program in the United States 5,22 are often not available for Europe. In particular, regarding the more recent changes in the WHO and TNM classifications, population-based incidence data are needed.…”

“…Uncertainty exists whether the observed increase in NEN incidence is due to improved detection strategies of these tumors, altering risk factors, or changes in tumor biology. Published data from Germany and other European countries have often focused on retrospective analysis of oncological studies, 19 mostly obtained from referral centers, or the analysis of specific subgroups 20,21 . Due to national restrictions and data protection rules, large population‐based data such as provided by the Surveillance, Epidemiology, and End Results (SEER) Program in the United States 5,22 are often not available for Europe.…”