title: Prognostic factors of long-term outcome in gastroenteropancreatic neuroendocrine tumours.
abstract: Neuroendocrine tumours (NET) of the gastroenteropancreatic system comprise a malignant entity with a low incidence. Only limited information is available on long-term clinical outcome and clinically applicable prognostic factors. We performed a retrospective analysis of a large, well-characterized centre-based patient cohort of 399 patients with histologically proven NET. Data were analysed according to epidemiological, clinical and histopathological characteristics. Detailed survival analyses using the Kaplan-Meier method were performed. Prognostic factors were tested by log-rank testing and independent risk factors were analysed using a Cox regression model. In the studied cohort, primary tumours originated in the fore-, mid- and hindgut in 46.1, 37.1 and 4.5% respectively. Extra-intestinal or unknown primary tumours were present in 8.4 and 10.5% respectively. Distant metastasis was present at initial diagnosis in 69.4%. Most frequent metastatic sites were liver (85%), peritoneal cavity (18%), bones (8%), other intra-abdominal sites (6%) and lungs (4%). Overall, 5- and 10-year survival rates were 78 and 63% respectively. Time to progression after initial diagnosis was significantly shorter in pancreatic as compared with ileal NET. Survival analysis revealed significantly better clinical outcome for primary tumours smaller than 25 mm, absence of metastasis, absence of any clinical symptoms, positive immunohistochemical staining for chromogranin A and a lower Ki67 index. These results were confirmed as independent by multivariate analysis. Therefore, this large retrospective analysis of a well-documented cohort of patients with NET demonstrates several prognostic factors of clinical relevance and wide availability, which should be considered for risk stratification in the management of NET.
authors.affiliation: Department of Hepatology and Gastroenterology, Charité, Campus Virchow Klinikum, University Medicine Berlin, Berlin, Germany. ulrich-frank.pape@charite.de
authors.forename: Ulrich-Frank
authors.initials: UF
authors.lastname: Pape
authors.forename: Uta
authors.initials: U
authors.lastname: Berndt
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Müller-Nordhorn
authors.forename: Michael
authors.initials: M
authors.lastname: Böhmig
authors.forename: Stephanie
authors.initials: S
authors.lastname: Roll
authors.forename: Martin
authors.initials: M
authors.lastname: Koch
authors.forename: Stefan N
authors.initials: SN
authors.lastname: Willich
authors.forename: Bertram
authors.initials: B
authors.lastname: Wiedenmann
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D015331:Cohort Studies; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000453:epidemiology / Q000401:mortality / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D018358:Neuroendocrine Tumors / Q000453:epidemiology / Q000401:mortality / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000453:epidemiology / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D055815:Young Adult