title: [Neuroendocrine tumours of the GI tract--data from the German NET Registry].
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.
  
  
  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).
  
  
  CONCLUSIONS
  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.affiliation: Klinik für Chirurgie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland.
authors.forename: N
authors.initials: N
authors.lastname: Begum
authors.affiliation: Medizinische Klinik m. S. Hepatologie & Gastroenterologie, Charité-Universitätsmedizin, Campus Virchow Klinikum, Berlin, Deutschland.
authors.forename: S
authors.initials: S
authors.lastname: Maasberg
authors.affiliation: Interdisziplinäres Stoffwechsel-Centrum, Charité-Universitätsmedizin, Berlin, Deutschland.
authors.forename: U
authors.initials: U
authors.lastname: Plöckinger
authors.affiliation: Institut für Pathologie, Universitätsklinikum Düsseldorf, Düsseldorf, Deutschland.
authors.forename: M
authors.initials: M
authors.lastname: Anlauf
authors.affiliation: Klinik für Gastroenterologie, Endokrinologie und Stoffwechsel, Klinikum der Philipps-Universität, Marburg, Deutschland.
authors.forename: A
authors.initials: A
authors.lastname: Rinke
authors.affiliation: Klinik für Nuklearmedizin, Katharinenhospital, Stuttgart, Deutschland.
authors.forename: G
authors.initials: G
authors.lastname: Pöpperl
authors.affiliation: Medizinische Klinik I, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland.
authors.forename: H
authors.initials: H
authors.lastname: Lehnert
authors.affiliation: Klinik und Poliklinik für Allgemein-, Viszeral- und Thoraxchirurgie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland.
authors.forename: J R
authors.initials: JR
authors.lastname: Izbicki
authors.affiliation: Klinik für Allgemein-, Viszeral- und Kinderchirurgie, Universitätsklinikum Düsseldorf, Düsseldorf, Deutschland.
authors.forename: M
authors.initials: M
authors.lastname: Krausch
authors.affiliation: Klinik und Poliklinik für Allgemein-, Viszeral- und Thoraxchirurgie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland.
authors.forename: Y K
authors.initials: YK
authors.lastname: Vashist
authors.affiliation: Klinik für Allgemein-, Viszeral- und Kinderchirurgie, Universitätsklinikum Düsseldorf, Düsseldorf, Deutschland.
authors.forename: A
authors.initials: A
authors.lastname: Raffel
authors.affiliation: Klinik für Chirurgie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland.
authors.forename: C G
authors.initials: CG
authors.lastname: Bürk
authors.affiliation: Chirurgische Klinik, Campus Großhadern, Klinikum der Universität , München, München, Deutschland.
authors.forename: J
authors.initials: J
authors.lastname: Hoffmann
authors.affiliation: Chirurgische Klinik I, Städtische Kliniken Neuss, Lukaskrankenhaus GmbH, Neuss, Deutschland.
authors.forename: P
authors.initials: P
authors.lastname: Goretzki
authors.affiliation: Medizinische Klinik m. S. Hepatologie & Gastroenterologie, Charité-Universitätsmedizin, Campus Virchow Klinikum, Berlin, Deutschland.
authors.forename: U F
authors.initials: UF
authors.lastname: Pape
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy; D004067:Digestive System Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006729:Hormones, Ectopic / Q000097:blood*; D006801:Humans; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D018358:Neuroendocrine Tumors / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D011379:Prognosis; D012042:Registries; D013577:Syndrome; D055815:Young Adult
chemical_list: D006729:Hormones, Ectopic