Zollinger Ellison Syndrome

Definition

• Gastrointestinal changes secondary to a gastrin producing neoplasm with resultant small intestinal ulceration

Diagnostic Criteria

• Changes are secondary to a gastrin secreting neoplasm
• 50-70% are duodenal and remainder are pancreatic
• Duodenal neoplasms may be very small (< 1 mm)
• Hyperplasia of parietal cells
  • Symptoms primarily related to ulcers due to increased acid secretion
    • Ulcers throughout small intestine
      • Removal of gastric body may lead to long term resolution of symptoms even if gastrin producing neoplasm cannot be removed
• Hyperplasia of endocrine cells in gastric body is uniformly seen
• Gastric carcinoids develop only if associated with MEN1
• Foveolar layer normal

Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting :xxxxx

Classification/Lists

Gastrointestinal Endocrine Cell Proliferations and Neoplasms

• Well differentiated processes including carcinoids
  • Esophagus
    • Very rare
  • Stomach
    • Gastric neuroendocrine cell hyperplasia and dysplasia
    • Carcinoid
  • Duodenum and proximal jejunum
    • Predominantly ampullary
      • Somatostatinoma
      • Gangliocytic paraganglioma
    • Duodenum NOS and proximal jejunum
      • Gastrin cell neoplasm see also Zollinger Ellison syndrome
  • Ileum and distal jejunum and cecum
    • Carcinoid, predominantly enterochromaffin cell, serotonin producing
  • Appendix
    • Carcinoid, predominantly enterochromaffin cell, serotonin producing
    • Goblet cell carcinoid
      • Goblet cell carcinoid (GCC), pure
      • Carcinoma ex GCC, signet ring cell type
      • Carcinoma ex GCC, poorly differentiated type
  • Large intestine
    • Cecum (see Ileum)
      • Carcinoid, predominantly enterochromaffin cell, serotonin producing
    • Rectum and distal colon
      • Carcinoid, predominantly L cell, glucagon-like peptide and pancreatic peptide (PP) producing
  • Anus
    • Very rare
• Poorly differentiated neuroendocrine carcinoma, all sites
  • Small cell
  • Large cell

Bibliography

• Riddell RH, Petras RE, Williams GT, Sobin LH. Tumors of the Intestines, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 32, 2003.
• Hamilton SR, Aaltonen LA eds. Pathology and genetics of tumours of the digestive system. World Health Organization classification of tumours, Vol. 2. Lyon: IARC Press 2000.