Endocrine tumors of the duodenum. A study of 55 cases relative to clinicopathological features and hormone content

Hepatogastroenterology. 2004 Sep-Oct;51(59):1367-71.

Abstract

Background/aims: Study of prognosis of duodenal endocrine tumors.

Methodology: Retrospective study concerned 55 duodenal endocrine tumors discovered in biopsy or surgical specimens. Follow-up records available for 49 patients indicated that inconspicuous associated clinical manifestations were often found subsequently. Seven patients were classified as Zollinger-Ellison syndrome and seven as multiple endocrine neoplasia (6 MEN I and 1 MEN II).

Results: Tumors were small (mean 1.28cm) and located preferentially in the first and second part of the duodenum. Fifty-four were well-differentiated and one poorly differentiated. Immunochemistry revealed 30 G-cell tumors (54.6%), 15 D-cell (27.3%), two plurihormonal (EC cell and G cell), and one GRH-cell, whereas seven could not be classified. Fifteen patients died (five in relation to their disease). Twenty-one had metastases (liver, nodes, lung), eight of whom are still alive.

Conclusions: Eighty-eight percent of duodenal endocrine tumors were gastrinomas, small plurifocal tumors and somatostatinomas preferentially located in the ampullar region and diagnosed because of hematemesis or icterus. Size is an important prognostic factor in determining whether surgery is required. The prognosis is better for D- and G-cell tumors than pancreatic endocrine tumors. Duodenal endocrine tumors in multiple endocrine neoplasia have a good prognosis, but can be associated with pancreatic plurihormonal tumors and metastases.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers, Tumor / analysis
  • Biopsy
  • Duodenal Neoplasms / diagnosis
  • Duodenal Neoplasms / mortality
  • Duodenal Neoplasms / pathology
  • Duodenal Neoplasms / surgery*
  • Duodenum / pathology
  • Duodenum / surgery
  • Female
  • Follow-Up Studies
  • Gastrectomy
  • Humans
  • Male
  • Middle Aged
  • Multiple Endocrine Neoplasia Type 1 / diagnosis
  • Multiple Endocrine Neoplasia Type 1 / mortality
  • Multiple Endocrine Neoplasia Type 1 / pathology
  • Multiple Endocrine Neoplasia Type 1 / surgery*
  • Multiple Endocrine Neoplasia Type 2a / diagnosis
  • Multiple Endocrine Neoplasia Type 2a / mortality
  • Multiple Endocrine Neoplasia Type 2a / pathology
  • Multiple Endocrine Neoplasia Type 2a / surgery*
  • Neoplasm Staging
  • Pancreaticoduodenectomy
  • Prognosis
  • Survival Rate
  • Zollinger-Ellison Syndrome / diagnosis
  • Zollinger-Ellison Syndrome / mortality
  • Zollinger-Ellison Syndrome / pathology
  • Zollinger-Ellison Syndrome / surgery*

Substances

  • Biomarkers, Tumor