title: 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.
authors.affiliation: First Department of Medicine, University Hospital Schleswig-Holstein, Christian-Albrechts University, Arnold-Heller-Str. 3, Kiel, 24105, Germany. johannes.rosentraeger@uksh.de.
authors.forename: M Johannes
authors.identifier: http://orcid.org/0000-0001-5893-1033
authors.initials: MJ
authors.lastname: Rosentraeger
authors.affiliation: Department of Pathology, University Hospital Schleswig-Holstein, Christian-Albrechts University, Kiel, Germany.
authors.forename: Nele
authors.initials: N
authors.lastname: Garbrecht
authors.affiliation: Institute of Pathology and Cytology, St. Vincenz Hospital, Limburg, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Anlauf
authors.affiliation: Department of General, Visceral and Pediatric Surgery, University Hospital of Düsseldorf, Düsseldorf, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Raffel
authors.affiliation: Department of General, Visceral and Pediatric Surgery, University Hospital of Düsseldorf, Düsseldorf, Germany.
authors.forename: Wolfram T
authors.initials: WT
authors.lastname: Knoefel
authors.affiliation: Department of Hepatology and Gastroenterology, Charité, Campus Virchow Clinic, Berlin, Germany.
authors.forename: Bertram
authors.initials: B
authors.lastname: Wiedenmann
authors.affiliation: Department of Pathology, Consultation Center for Pancreatic and Endocrine Tumors, Technical University, Munich, Germany.
authors.forename: Günter
authors.initials: G
authors.lastname: Klöppel
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004379:Duodenal Neoplasms / Q000378:metabolism* / Q000473:pathology; D005260:Female; D015408:Gastrinoma / Q000378:metabolism*; D005755:Gastrins / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000378:metabolism*; D018358:Neuroendocrine Tumors / Q000378:metabolism*; D010190:Pancreatic Neoplasms / Q000378:metabolism / Q000473:pathology; D012189:Retrospective Studies
chemical_list: D005755:Gastrins
keywords: Duodenum; Gastrin production; Gastrinoma; Neuroendocrine tumors; Zollinger-Ellison syndrome