N Begum1, S Maasberg2, U Plöckinger3, M Anlauf4, A Rinke5, G Pöpperl6, H Lehnert7, J R Izbicki8, M Krausch9, Y K Vashist8, A Raffel9, C G Bürk1, J Hoffmann10, P Goretzki11, U F Pape2. 1. Klinik für Chirurgie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland. 2. Medizinische Klinik m. S. Hepatologie & Gastroenterologie, Charité-Universitätsmedizin, Campus Virchow Klinikum, Berlin, Deutschland. 3. Interdisziplinäres Stoffwechsel-Centrum, Charité-Universitätsmedizin, Berlin, Deutschland. 4. Institut für Pathologie, Universitätsklinikum Düsseldorf, Düsseldorf, Deutschland. 5. Klinik für Gastroenterologie, Endokrinologie und Stoffwechsel, Klinikum der Philipps-Universität, Marburg, Deutschland. 6. Klinik für Nuklearmedizin, Katharinenhospital, Stuttgart, Deutschland. 7. Medizinische Klinik I, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland. 8. Klinik und Poliklinik für Allgemein-, Viszeral- und Thoraxchirurgie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland. 9. Klinik für Allgemein-, Viszeral- und Kinderchirurgie, Universitätsklinikum Düsseldorf, Düsseldorf, Deutschland. 10. Chirurgische Klinik, Campus Großhadern, Klinikum der Universität , München, München, Deutschland. 11. Chirurgische Klinik I, Städtische Kliniken Neuss, Lukaskrankenhaus GmbH, Neuss, Deutschland.
Abstract
BACKGROUND: Neuroendocrine tumours (NET) are rare and heterogeneous neoplasia. To obtain valid data on epidemiology, diagnostics, therapy, prognosis and risk factors is the aim of the German NET registry. PATIENTS AND METHODS: Data from 2009 histologically proven NET were collected from 35 NET centres between 1999 and 2010. Data collection has been performed prospectively since 2004. Results: Median follow-up was 34.5 months and median age at diagnosis 56.4 years. Primary tumour localisations were pancreas (34.2%), midgut (5.8%), stomach (6.5%), bowel (6.9%), duodenum (4.8%) and neuroendocrine CUP (12.6%). Synchronous metastases were seen in 46% and second malignancies in 12%. From 860 patients, 402 (46.7%) had functional tumours with the following hormone excess syndromes: carcinoid syndrome (19.1%; n = 164), persistent hyperinsulinaemic hypoglycaemia (17.7%; n = 152), Zollinger- Ellison syndrome (7.1%; n = 61), glucagonoma (0.7%; n = 15), Verner-Morrison syndrome (0.4%; n = 8) and somatostatinoma syndrome(0.1%; n = 2). Surgical therapy was performed in 78%, therapy with somatostatin receptor analogues(SSA) in 28%, peptide radioreceptor therapy (PRRT) in 19%, chemotherapy in 18% and interferon therapy in 6.5%. Only surgery was done in 47%, whereas 53% received a second therapy. General mortality rate during follow-up was 14.9%. The tumour-specific survival rates for 2, 5 and 10 years were 94, 85 and 70%. The 5-year survival is dependent on the surgical or non-surgical therapy (82 versus 61%, p < 0.001) and also on the primary tumour site (90/30% for midgut, 85/65% for pancreas, p < 0.001). Grading (G1, G2, G3) based on proliferation index Ki-67 recommended by the ENETS guidelines and WHO classification is highly correlated to the 5-year survival rate (88, 82, 33%, p < 0.001). CONCLUSION: The German NET registry provides valid multicentric data on NET in Germany. Surgical therapy is the most frequent and important therapy with good clinical outcome. In non-resectable, metastatic tumours, systemic therapies are common. Continuation and evaluation of the new WHO and TNM classifications for NET and their therapies will be a future focus of the registry.
BACKGROUND:Neuroendocrine tumours (NET) are rare and heterogeneous neoplasia. To obtain valid data on epidemiology, diagnostics, therapy, prognosis and risk factors is the aim of the German NET registry. PATIENTS AND METHODS: Data from 2009 histologically proven NET were collected from 35 NET centres between 1999 and 2010. Data collection has been performed prospectively since 2004. Results: Median follow-up was 34.5 months and median age at diagnosis 56.4 years. Primary tumour localisations were pancreas (34.2%), midgut (5.8%), stomach (6.5%), bowel (6.9%), duodenum (4.8%) and neuroendocrine CUP (12.6%). Synchronous metastases were seen in 46% and second malignancies in 12%. From 860 patients, 402 (46.7%) had functional tumours with the following hormone excess syndromes: carcinoid syndrome (19.1%; n = 164), persistent hyperinsulinaemic hypoglycaemia (17.7%; n = 152), Zollinger- Ellison syndrome (7.1%; n = 61), glucagonoma (0.7%; n = 15), Verner-Morrison syndrome (0.4%; n = 8) and somatostatinoma syndrome(0.1%; n = 2). Surgical therapy was performed in 78%, therapy with somatostatin receptor analogues(SSA) in 28%, peptide radioreceptor therapy (PRRT) in 19%, chemotherapy in 18% and interferon therapy in 6.5%. Only surgery was done in 47%, whereas 53% received a second therapy. General mortality rate during follow-up was 14.9%. The tumour-specific survival rates for 2, 5 and 10 years were 94, 85 and 70%. The 5-year survival is dependent on the surgical or non-surgical therapy (82 versus 61%, p < 0.001) and also on the primary tumour site (90/30% for midgut, 85/65% for pancreas, p < 0.001). Grading (G1, G2, G3) based on proliferation index Ki-67 recommended by the ENETS guidelines and WHO classification is highly correlated to the 5-year survival rate (88, 82, 33%, p < 0.001). CONCLUSION: The German NET registry provides valid multicentric data on NET in Germany. Surgical therapy is the most frequent and important therapy with good clinical outcome. In non-resectable, metastatic tumours, systemic therapies are common. Continuation and evaluation of the new WHO and TNM classifications for NET and their therapies will be a future focus of the registry.
Authors: Nehara Begum; Ulrich Wellner; Christoph Thorns; Georg Brabant; Martin Hoffmann; Conny Georg Bürk; Hendrik Lehnert; Tobias Keck Journal: World J Surg Date: 2015-06 Impact factor: 3.352
Authors: M Anlauf; B Sipos; I Boeck; S E Baldus; S Heikaus; M Krausch; W T Knoefel; N Begum; P Goretzki; M Schott; C J Auernhammer; B Cremer; A Rinke; S Ezziddin; C Fottner; G Pöpperl; H Lahner; D Hörsch; H E Gabbert; P Komminoth; A Perren; G Klöppel; B Wiedenmann; M Pavel; U Pape Journal: Pathologe Date: 2014-05 Impact factor: 1.011
Authors: Dimitrios Schizas; Aikaterini Mastoraki; George I Kirkilesis; Athanasios D Sioulas; Ioannis S Papanikolaou; Evangelos P Misiakos; Nikolaos Arkadopoulos; Theodore Liakakos Journal: J Gastrointest Cancer Date: 2017-12