Zollinger-Ellison syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2] Mohamad Alkateb, MBBCh [3]
Overview
If mild cases if left untreated, patients with Zollinger-Ellison syndrome may progress to develop abdominal pain, diarrhea, heartburn. In severe cases if left untreated results in refractory peptic ulcer disease, severe gastroesophageal reflux disease, diarrhea and finally death, primarily due to the complications of the refractory peptic ulcer disease. [1] .[2] Common complications of Zollinger-Ellison syndrome include upper gastrointestinal bleeding, anemia, and duodenal ulcer perforation. Prognosis is generally good, and the 5 and 10-year survival rate of patients with Zollinger-Ellison syndrome is approximately 94% and 75%, respectively.[3]
Natural History
- ZES is a clinical syndrome due to the ectopic secretion of gastrin by a neuroendocrine tumor (gastrinoma), located primarily in the duodenum (60%-80%) or pancreas (10%-40%), resulting in gastric acid hypersecretion, which if left untreated results in refractory peptic ulcer disease, severe gastroesophageal reflux disease, diarrhea and finally death, primarily due to the complications of the refractory peptic ulcer disease. [1]
- In mild cases if left untreated, patients with Zollinger-Ellison syndrome may progress to develop abdominal pain, diarrhea, and heartburn.[2]
- The symptoms of Zollinger-Ellison syndrome usually develop in the third decade of life. Without treatment, the patient will develop symptoms of gastrinoma, which may eventually lead to anemia and death.[4]
Complications
Common complications associated with Zollinger-Ellison syndrome include:
Prognosis
The prognosis of Zollinger-Ellison syndrome is good with treatment and the 5 and 10-year survival rate of patients with Zollinger-Ellison syndrome is approximately 94% and 75%, respectively. Without treatment, Zollinger-Ellison syndrome may result in weight loss, anemia, and death.[3]
References
- ↑ 1.0 1.1 Ito T, Cadiot G, Jensen RT (2012). "Diagnosis of Zollinger-Ellison syndrome: increasingly difficult". World J Gastroenterol. 18 (39): 5495–503. doi:10.3748/wjg.v18.i39.5495. PMC 3482635. PMID 23112541.
- ↑ 2.0 2.1 Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL; et al. (2000). "Zollinger-Ellison syndrome. Clinical presentation in 261 patients". Medicine (Baltimore). 79 (6): 379–411. PMID 11144036.
- ↑ 3.0 3.1 Melvin WS, Johnson JA, Sparks J, Innes JT, Ellison EC (1993). "Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia". Surgery. 114 (6): 1183–8. PMID 7903006.
- ↑ Riegert-Johnson D, Gleeson FC, Westra W, et al. Peutz-Jeghers Syndrome. 2008 Jul 18 [Updated 2008 Aug 9]. In: Riegert-Johnson DL, Boardman LA, Hefferon T, et al., editors. Cancer Syndromes [Internet]. Bethesda (MD): National Center for Biotechnology Information (US); 2009-. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1826/