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Zollinger-Ellison (ZE) syndrome (gastrinoma)

Etiology: 1) gastrin secreting tumor a) 90% are in head of pancreas or duodenum b) 10% are in tail of pancreas 2) associated with multiple endocrine neoplasia I in 25% Pathology: 1) non-beta pancreatic islet cell or duodenal G cell tumor secreting gastrin 2) 70% are malignant * images [7] Genetics: may occur in familial form (ask about family history) Clinical manifestations: 1) epigsastric pain, epigastric tenderness [7] 2) GERD, esophagitis 3) watery diarrhea, resistant to treatment 4) peptic ulcer a) duodenal bulb ulcer (most common presentation) b) large or multiple ulcers in distal duodenum or jejunum c) recurrent ulceration after otherwise adequate treatment Laboratory: 1) fasting serum gastrin a) serum gastrin > 1000 pg/mL in patients who produce gastric acid is diagnostic b) high gastric acid levels are also seen in patients with pernicious anemia 2) gastric pH < 2 [7] 3) secretin stimulation test a) measures increase in gastrin in response to secretin b) useful when fasting gastrin in equivocal c) large increase in gastrin occurs in patients with ZE 4) exclude MEN 1 5) tumor localization 6) see ARUP consult [6] Special laboratory: - upper GI endoscopy (esophagogastroduodenoscopy) - esophagitis, gastric antrum erosions, duodenal ulcers * images [7] Radiology: 1) gastrinomas are vascular tumors, thus angiogram useful* 2) Indium-labeled octreotide = OctreoScan* 3) abdominal CT detects 50% of tumors* 4) positron-emission tomography identifies metastases , * exception is gastric neuroendocrine tumor resected in the presence of autoimmune gastritis (pernicious anemia) only observation indicated [5] Management: 1) medical: a) proton pump inhibitor - omeprazole 60 mg/day b) H2 antagonist c) octreotide reduces gastrin secretion 2) metastatic workup 3) surgical resection of tumor a) preferred treatment b) lymph nodes may be positive [7] c) gastrin itself promotes tumor growth d) if gastric neuroendocrine tumor resected in the presence of autoimmune gastritis (pernicious anemia) only observation is indicated [5] e) surgery contraindicated in patients with MEN-1 4) chemotherapy for patients with metastatic disease a) streptozocin & 5-fluorouracil b) effective in 50% of patients

Related

gastrin (GAST, GAS) hypergastrinemia multiple endocrine neoplasia type-1 (MEN-1); multiple endocrine adenomatosis; Wermer Syndrome

General

amine precursor uptake & decarboxylation tumor (APUDoma) islet cell tumor; pancreatic neuroendocrine neoplasm; nesidioblastoma

Database Correlations

OMIM 131100

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 327
  2. Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 360
  3. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 288
  4. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 585
  5. Medical Knowledge Self Assessment Program (MKSAP) 15, 17, 18. American College of Physicians, Philadelphia 2009, 2015, 2018.
  6. ARUP Consult: Zollinger-Ellison Syndrome - Gastrinoma The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/zollinger-ellison-syndrome - Zollinger-Ellison Syndrome Testing Algorithm https://arupconsult.com/algorithm/zollinger-ellison-syndrome-testing-algorithm
  7. Binet Q, Borbath I. Images in Clinical Medicine. The Zollinger-Ellison Syndrome N Engl J Med 2022; 387:1699. Nov 3. PMID: 36322847 https://www.nejm.org/doi/full/10.1056/NEJMicm2203797
  8. Zollinger-Ellison Syndrome https://www.niddk.nih.gov/health-information/digestive-diseases/zollinger-ellison-syndrome