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

Rosentraeger, M Johannes; Garbrecht, Nele; Anlauf, Martin; Raffel, Andreas; Knoefel, Wolfram T; Wiedenmann, Bertram; Klöppel, Günter

doi:10.1007/s00428-015-1890-9

Benutzer: Gast

journal article

Titel:: Syndromic versus non-syndromic sporadic gastrin-producing neuroendocrine tumors of the duodenum: comparison of pathological features and biological behavior.
Dokumenttyp:: Journal Article; Article
Autor(en):: Rosentraeger, M Johannes; Garbrecht, Nele; Anlauf, Martin; Raffel, Andreas; Knoefel, Wolfram T; Wiedenmann, Bertram; Klöppel, Günter
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.
Zeitschriftentitel:: Virchows Arch
Jahr:: 2016
Band / Volume:: 468
Heft / Issue:: 3
Seitenangaben Beitrag:: 277-87
Sprache:: eng
Volltext / DOI:: doi:10.1007/s00428-015-1890-9
PubMed:: http://view.ncbi.nlm.nih.gov/pubmed/26649731
Print-ISSN:: 0945-6317
TUM Einrichtung:: Institut für Allgemeine Pathologie und Pathologische Anatomie
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