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

Simone Luecke; Christian Fottner; Harald Lahner; Henning Jann; Dominik Zolnowski; Detlef Quietzsch; Patricia Grabowski; Birgit Cremer; Sebastian Maasberg; Ulrich-Frank Pape; Hans-Helge Mueller; Thomas Matthias Gress; Anja Rinke; The Members Of The German Net Registry

doi:10.3390/cancers14112718

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

Cancers (Basel). 2022 May 31;14(11):2718. doi: 10.3390/cancers14112718.

Authors

Affiliations

¹ UKGM Marburg, Department of Gastroenterology, Philipps University Marburg, 35037 Marburg, Germany.
² Department of Internal Medicine I, Endocrinology, University Hospital Mainz, 55131 Mainz, Germany.
³ Department of Endocrinology and Metabolism, University Hospital of Essen, 45147 Essen, Germany.
⁴ Department of Gastroenterology and Hepatology, Campus Virchow Klinikum, University Medicine Charité, 10117 Berlin, Germany.
⁵ Department of Oncology, Klinikum Chemnitz, 09116 Chemnitz, Germany.
⁶ Praxis Dr. med. habil. Diener, 09376 Oelsnitz/Erzgebirge, Germany.
⁷ Klinikum Havelhöhe, Campus Virchow Klinikum, Institute of Medical Immunology, MVZ Oncology, University Medicine Charité, 10117 Berlin, Germany.
⁸ Department of Oncology, University Hospital of Cologne, 50923 Cologne, Germany.
⁹ Department of Internal Medicine and Gastroenterology, Asklepios Klinik St. Georg, 20099 Hamburg, Germany.
¹⁰ Institute of Medical Biometry and Epidemiology, Philipps University Marburg, 35037 Marburg, Germany.
¹¹ German NET Registry, German Society of Endocrinology, 90518 Altorf, Germany.

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.

Keywords: Ki67; chemotherapy; neuroendocrine carcinoma; neuroendocrine neoplasia; neuroendocrine tumor G3; prognosis.

Grants and funding

The German NET Registry received financial support from Esteve Pharma, Ipsen Pharma GmbH, Novartis Pharma GmbH and Pfizer GmbH. This specific study received no additional external funding.