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Syndromic versus non-syndromic sporadic gastrin-producing neuroendocrine tumors of the duodenum: comparison of pathological features and biological behavior

Abstract

Sporadic gastrin-producing neuroendocrine tumors of the duodenum present either with the Zollinger-Ellison syndrome (ZES) or with unspecific symptoms. While syndromic gastrin-producing neuroendocrine tumors often show metastases at the time of diagnosis, those without a syndrome do not. The aim of the study was to search for clinicopathological features that may distinguish the two categories of gastrin-producing duodenal tumors. In a retrospective study, we analyzed the clinical and pathological data in a series of 41 patients with syndromic (i.e., gastrinomas) or non-syndromic duodenal gastrin-producing neuroendocrine tumors (ns-gas-NETs). Twenty-four (59 %) of the 41 patients had tumors that were associated with a ZES and were classified as gastrinomas. These tumors showed a higher Ki-67 index than that of the ns-gas-NETs (1.74 vs. 0.85 %, p = 0.012). In addition, they had more lymph node metastases (75 vs. 6 %, p < 0.001) and showed liver metastases and thus presented much more frequently in TNM stage ≥III (75 vs. 6 %; p < 0.001) than their non-syndromic counterparts. Gastrinomas were removed surgically, ns-gas-NETs endoscopically. We did not observe any significant differences in overall survival or recurrence of disease. Duodenal gastrinomas show no clear morphological features that distinguish them from their non-syndromic counterparts. However, the patients with gastrinomas present in a more advanced stage of disease and need surgical treatment, while non-syndromic gastrin-producing duodenal NETs may be cured by complete endoscopical removal.

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Acknowledgements

The authors are grateful to Maike Pacena, Anja Bredtmann, and Sonja Schmid for their excellent technical assistance. We are indebted to Waldemar Strauss for the photo documentation and Katherine Dege for carefully reading the manuscript. We thank all colleagues who supported this study: Prof. Dr. H.-M. Barten, Rostock; Prof. Dr. D. B. von Bassewitz, Münster; Dr. R. Berndt., Ansbach; Dr. H.-H. Bernsdorff, Lütjenburg; Dr. Bertling, Speyer; Dres. B. Bier/E. Eltze/O. Scherr, Saarbrücken; Dr. M. W. M. Brandt, Wesel; Dr. Burow, Celle; Dr. Bürkle, Göppingen; Prof. Dr. R. Brückner, Norden; Dr. T. Darnedde, Braunschweig; Prof. Dr. Dietel, Berlin; Dr. J. Druschke, Hildburghausen; Prof. Dr. Dürr, Ortenau; Dres. S. Eidt/Hake, Köln-Hohenlind; Dr. D. Eppenstein, Geislingen; Dr. P. Flemming, Celle; Prof. Dr. Fölsch, Kiel; Prof. Dr. Friemann, Lüdenscheid; Prof. Dr. Dr. H. Gabbert, Düsseldorf; Dr. J. Gottberg, Hamburg; Dr. I. Göken, Oldenburg; Dr. P. Heising, Velpke; Dr. H. Herrmann, Brunsbüttel; Dres. A. Jacobs/V. Stoll, Wuppertal; Dr. K. Jessen, Kiel; Dr. T. Jork, Brunsbüttel; Dr. Khoury, Hof; Prof. Dr. J. Klempnauer, Hannover; Prof. Dr. H.-H. Kreipe, Hannover; Prof. Dr. Krieglstein, Köln; Prof. Dr. W. Kruis, Köln; Dr. Kühne, Nortorf; Prof. Dr. S. Liebe, Rostock; Dr. D. Löhmann, Streufdorf; Dr. W. Matschl, Hallstadt; Dres. Meyer-Tassler/Stöckmann, Münster; Dres. A. Neuber/H. Graupner, Wesel; Dr. P. Otte, Zweibrücken; Dr. R. Pauli, Brandenburg; Dr. N. Pittlik, Werdohl; Prof. Dr. C. Röcken, Kiel; PD Dr. P. Schmiegelow, Solingen; Prof. Dr. S. Schröder/Dr. Beckmann, Hamburg; Prof. Dr. N. T. Schwarz, Neumünster; Dr. M. Seelinger, Speyer; Prof. Dr. G. Seitz, Bamberg; Dr. G. Stöhr, Münster; A. Szöke, Köln; Dr. L.-S. Tschirch, Kiel; Prof. Dr. M. Vieth, Bayreuth; Dr. Vössing, Köln; Dr. R. Walzel, Ansbach; Dr. W. Wehler, Köln; Dr. Wicht, Brandenburg/Havel; Dr. Woziwodzki, Aurich; Dr. H. Zimmermann, Freudenberg; and Prof. C. F. Eisenberger, Köln-Holweide.

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Correspondence to M. Johannes Rosentraeger.

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Johannes Rosentraeger and Nele Garbrecht both contributed equally to this study. The results of this study are part of Johannes Rosentraeger’s MD thesis. Nele Garbrecht has a fellowship sponsored by the Hensel Stiftung, Germany.

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Rosentraeger, M.J., Garbrecht, N., Anlauf, M. et al. Syndromic versus non-syndromic sporadic gastrin-producing neuroendocrine tumors of the duodenum: comparison of pathological features and biological behavior. Virchows Arch 468, 277–287 (2016). https://doi.org/10.1007/s00428-015-1890-9

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Keywords

  • Neuroendocrine tumors
  • Duodenum
  • Gastrin production
  • Gastrinoma
  • Zollinger-Ellison syndrome