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This Article Accepted manuscript (PDF) All Versions of this Article: ERC-08-0017v1 15/4/1083    most recent Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Google Scholar Articles by Pape, U.-F. Articles by Wiedenmann, B. PubMed PubMed Citation Articles by Pape, U.-F. Articles by Wiedenmann, B.

U Pape, Hepatology and Gastroenterology, Campus Virchow Clinic, Charite, University Medicine Berlin, Berlin, Germany
U Berndt, Hepatology and Gastroenterology, Campus Virchow Clinic, Charite, University Medicine Berlin, Berlin, Germany
J Mueller-Nordhorn, Institute for Social Medicine, Epidemiology and Health Economics, Campus Charite Mitte, Charite, University Medicine Berlin, Berlin, Germany
M Boehmig, Hepatology and Gastroenterology, Campus Virchow Clinic, Charite, University Medicine Berlin, Berlin, Germany
S Roll, Institute for Social Medicine, Epidemiology and Health Economics, Campus Charite Mitte, Charite, University Medicine Berlin, Berlin, Germany
M Koch, Institute for Pathology, Campus Charite Mitte, Charite, University Medicine Berlin, Berlin, Germany
S Willich, Institute for Social Medicine, Epidemiology and Health Economics, Campus Charite Mitte, Charite, University Medicine Berlin, Berlin, Germany
B Wiedenmann, Hepatology and Gastroenterology, Campus Virchow Clinic, Charite, University Medicine Berlin, Berlin, Germany

Correspondence: Ulrich-Frank Pape, Email: ulrich-frank.pape{at}charite.de

Abstract

Neuroendocrine tumours (NET) of the gastroenteropancreatic system (GEP) 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 center-based patient cohort of 399 patients with histologically proven NET. Data were analyzed 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 analyzed 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. 1.8% had an extra-intestinal and 10.5% an unknown primary tumour. 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 a significantly shorter in pancreatic as compared to 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.