Zollinger-Ellison (ZE) syndrome (gastrinoma)
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Etiology
- gastrin secreting tumor
- associated with multiple endocrine neoplasia I in 25%
Pathology
- non-beta pancreatic islet cell or duodenal G cell tumor secreting gastrin
- 70% are malignant
* images[7]
Genetics
may occur in familial form (ask about family history)
Clinical manifestations
- epigsastric pain, epigastric tenderness[7]
- GERD, esophagitis
- watery diarrhea, resistant to treatment
- peptic ulcer
Laboratory
- fasting serum gastrin
- serum gastrin > 1000 pg/mL in patients who produce gastric acid is diagnostic
- high gastric acid levels are also seen in patients with pernicious anemia
- gastric pH < 2[7]
- secretin stimulation test
- exclude MEN 1
- tumor localization
- see ARUP consult[6]
Diagnostic procedures
* images[7]
Radiology
- gastrinomas are vascular tumors, thus angiogram useful*
- Indium-labeled octreotide = OctreoScan*
- abdominal CT detects 50% of tumors*
- positron-emission tomography identifies metastases ,
* exception is gastric neuroendocrine tumor resected in the presence of autoimmune gastritis (pernicious anemia) only observation indicated[5]
Management
- medical:
- proton pump inhibitor
- omeprazole 60 mg/day
- H2 antagonist
- octreotide reduces gastrin secretion
- proton pump inhibitor
- metastatic workup
- surgical resection of tumor
- preferred treatment
- lymph nodes may be positive[7]
- gastrin itself promotes tumor growth
- if gastric neuroendocrine tumor resected in the presence of autoimmune gastritis (pernicious anemia) only observation is indicated[5]
- surgery contraindicated in patients with MEN-1
- chemotherapy for patients with metastatic disease
- streptozocin & 5-fluorouracil
- effective in 50% of patients
More general terms
Additional terms
- gastrin (GAST, GAS)
- hypergastrinemia
- multiple endocrine neoplasia type-1 (MEN-1); multiple endocrine adenomatosis; Wermer Syndrome
References
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 327
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 360
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 288
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 585
- ↑ 5.0 5.1 5.2 Medical Knowledge Self Assessment Program (MKSAP) 15, 17, 18. American College of Physicians, Philadelphia 2009, 2015, 2018.
- ↑ 6.0 6.1 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 - ↑ 7.0 7.1 7.2 7.3 7.4 7.5 Binet Q, Borbath I. Images in Clinical Medicine. The Zollinger-Ellison Syndrome N Engl J Med 2022; 387:1699. Nov 3. PMID: https://www.ncbi.nlm.nih.gov/pubmed/36322847 https://www.nejm.org/doi/full/10.1056/NEJMicm2203797
- ↑ Zollinger-Ellison Syndrome https://www.niddk.nih.gov/health-information/digestive-diseases/zollinger-ellison-syndrome
Patient information
Zollinger-Ellison (ZE) syndrome (gastrinoma) patient information