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Syndromic versus non-syndromic sporadic gastrin-producing neuroendocrine tumors of the duodenum: comparison of pathological features and biological behavior.
Virchows Arch. 2016 Mar; 468(3):277-87.VA

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.

Authors+Show Affiliations

First Department of Medicine, University Hospital Schleswig-Holstein, Christian-Albrechts University, Arnold-Heller-Str. 3, Kiel, 24105, Germany. johannes.rosentraeger@uksh.de.Department of Pathology, University Hospital Schleswig-Holstein, Christian-Albrechts University, Kiel, Germany.Institute of Pathology and Cytology, St. Vincenz Hospital, Limburg, Germany.Department of General, Visceral and Pediatric Surgery, University Hospital of Düsseldorf, Düsseldorf, Germany.Department of General, Visceral and Pediatric Surgery, University Hospital of Düsseldorf, Düsseldorf, Germany.Department of Hepatology and Gastroenterology, Charité, Campus Virchow Clinic, Berlin, Germany.Department of Pathology, Consultation Center for Pancreatic and Endocrine Tumors, Technical University, Munich, Germany.

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

26649731

Citation

Rosentraeger, M Johannes, et al. "Syndromic Versus Non-syndromic Sporadic Gastrin-producing Neuroendocrine Tumors of the Duodenum: Comparison of Pathological Features and Biological Behavior." Virchows Archiv : an International Journal of Pathology, vol. 468, no. 3, 2016, pp. 277-87.
Rosentraeger MJ, 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. 2016;468(3):277-87.
Rosentraeger, M. J., Garbrecht, N., Anlauf, M., Raffel, A., Knoefel, W. T., Wiedenmann, B., & Klöppel, G. (2016). Syndromic versus non-syndromic sporadic gastrin-producing neuroendocrine tumors of the duodenum: comparison of pathological features and biological behavior. Virchows Archiv : an International Journal of Pathology, 468(3), 277-87. https://doi.org/10.1007/s00428-015-1890-9
Rosentraeger MJ, et al. Syndromic Versus Non-syndromic Sporadic Gastrin-producing Neuroendocrine Tumors of the Duodenum: Comparison of Pathological Features and Biological Behavior. Virchows Arch. 2016;468(3):277-87. PubMed PMID: 26649731.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Syndromic versus non-syndromic sporadic gastrin-producing neuroendocrine tumors of the duodenum: comparison of pathological features and biological behavior. AU - Rosentraeger,M Johannes, AU - Garbrecht,Nele, AU - Anlauf,Martin, AU - Raffel,Andreas, AU - Knoefel,Wolfram T, AU - Wiedenmann,Bertram, AU - Klöppel,Günter, Y1 - 2015/12/09/ PY - 2015/07/25/received PY - 2015/11/23/accepted PY - 2015/10/04/revised PY - 2015/12/10/entrez PY - 2015/12/10/pubmed PY - 2016/8/18/medline KW - Duodenum KW - Gastrin production KW - Gastrinoma KW - Neuroendocrine tumors KW - Zollinger-Ellison syndrome SP - 277 EP - 87 JF - Virchows Archiv : an international journal of pathology JO - Virchows Arch. VL - 468 IS - 3 N2 - 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. SN - 1432-2307 UR - https://www.unboundmedicine.com/medline/citation/26649731/Syndromic_versus_non_syndromic_sporadic_gastrin_producing_neuroendocrine_tumors_of_the_duodenum:_comparison_of_pathological_features_and_biological_behavior_ L2 - https://dx.doi.org/10.1007/s00428-015-1890-9 DB - PRIME DP - Unbound Medicine ER -