Ivan Borbath1, Rocio Garcia-Carbonero2, Damir Bikmukhametov3, Paula Jimenez-Fonseca4, Angel Castaño5, Jaroslava Barkmanova6, Eva Sedlackova7, Attila Kollár8, Emanuel Christ9, Gregory Kaltsas10, Beata Kos-Kudla11, Sebastian Maasberg12, Chris Verslype13, Ulrich-Frank Pape14. 1. Hepato-gastroenterology Unit, Cliniques Universitaires Saint-Luc, Bruxelles, Belgium. Electronic address: ivan.borbath@saintluc.uclouvain.be. 2. Oncology Department, Hospital Universitario 12 de Octubre, IIS Imas12, UCM, CNIO, CIBERONC, Madrid, Spain. Electronic address: rgcarbonero@gmail.com. 3. Charité University Hospital Berlin, Gastroenterology Department, Berlin, Germany. Electronic address: damirbikm@gmail.com. 4. Hospital Universitario Central de Asturias, Medical Oncology, Oviedo, Asturias, Spain. Electronic address: palucaji@hotmail.com. 5. Hospital Universitario de Fuenlabrada, Pathology Unit, Madrid, Spain. Electronic address: angel.castano@salud.madrid.org. 6. Charles University, Oncological Clinic, General Faculty Hospital and 1.st Medical Faculty, Praha, Czech Republic. Electronic address: jaroslava.barkmanova@vfn.cz. 7. Charles University, Oncological Clinic, General Faculty Hospital and 1.st Medical Faculty, Praha, Czech Republic. Electronic address: eva.sedlackova@vfn.cz. 8. Department of Oncology, Inselspital, Bern University Hospital, University of Bern, Switzerland. Electronic address: attila.kollar@insel.ch. 9. Universitätsspital Basel, Endocrinology, Diabetology and Metabolism, Basel, Switzerland. Electronic address: emanuel.christ@usb.ch. 10. National and Kapodistrian University of Athens, Department of General Medicine and Endocrinology, Athens, Attica, Greece. Electronic address: gregory.kaltsas@gmail.com. 11. Medical University of Silesia, Department of Pathophysiology and Endocrinology, Katowice, Slaskie, Poland. Electronic address: beatakos@ka.onet.pl. 12. Asklepios Kliniken Hamburg, Department of Internal Medicine and Gastroenterology, Hamburg, Germany. Electronic address: s.maasberg@asklepios.com. 13. Katholieke Universiteit Leuven, UZ Leuven, Digestive Oncology - Hepatology, Leuven, Belgium. Electronic address: chris.verslype@uzleuven.be. 14. Asklepios Kliniken Hamburg, Department of Internal Medicine and Gastroenterology, Hamburg, Germany. Electronic address: ul.pape@asklepios.com.
Abstract
BACKGROUND: 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. PATIENTS AND METHODS: 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). RESULTS: 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. CONCLUSION: 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.
BACKGROUND: 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. PATIENTS AND METHODS: 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). RESULTS: 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. CONCLUSION: 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.