Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to a gastrinoma.
Diagnosis of ZES is often delayed by 5-7 years after the onset of symptoms 2.
Gastrinomas are usually multiple and typically located in the duodenum (more common) or pancreas (less common). These tumours secrete gastrin that results in hypersecretion of gastric acid, which in turn results in diarrhoea, gastritis, severe gastro-oesophageal reflux disease and peptic ulcer disease 1.
- increased gastrin levels in fasting patients (but not specific, and some data suggest that clinical assays may be unreliable 6)
- multiple endocrine neoplasia (MEN) type 1: ZES occurs when gastrinoma is functional
On double-contrast upper gastrointestinal studies the following features may be seen 4-5:
- thickened rugal folds
- multinodular gastric contour
- erosions and ulcers, especially in atypical locations
- barium may be diluted by the high volume of fluid in the stomach
- negative contrast may be used to distend the stomach
- thickened rugal folds
- multiple gastric nodules/masses 4
Treatment and prognosis
Surgery plays a vital role 8. Death from complications of ZES (e.g. perforation, haemorrhage) can occur.
History and etymology
It is named after Robert M. Zollinger and Edwin H. Ellison, who in April 1956 described two cases of severe, multifocal ulcerative lesions of the proximal gastrointestinal tract, which were remittent, refractory to surgery and associated with tumours in the adjacent pancreas 7.
Possible differential considerations include:
- 1. Ito T, Cadiot G, Jensen RT. Diagnosis of Zollinger-Ellison syndrome: increasingly difficult. World J. Gastroenterol. 2012;18 (39): 5495-503. doi:10.3748/wjg.v18.i39.5495 - Free text at pubmed - Pubmed citation
- 2. Plöckinger U. Diagnosis and Treatment of Gastrinomas in Multiple Endocrine Neoplasia Type 1 (MEN-1). Cancers (Basel). 2012;4 (4): 39-54. doi:10.3390/cancers4010039 - Free text at pubmed - Pubmed citation
- 3. Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology. 2008;135 (5): 1469-92. doi:10.1053/j.gastro.2008.05.047 - Free text at pubmed - Pubmed citation
- 4. Levy AD, Sobin LH. From the archives of the AFIP: Gastrointestinal carcinoids: imaging features with clinicopathologic comparison. Radiographics. 2007;27 (1): 237-57. Radiographics (full text) - doi:10.1148/rg.271065169 - Pubmed citation
- 5. Gore RM, Levine MS. High Yield Imaging: Gastrointestinal: Expert Consult - Online and Print, 1e (HIGH YIELD in Radiology). Saunders. ISBN:1416055444. Read it at Google Books - Find it at Amazon
- 6. Ito T, Igarashi H, Jensen RT. Zollinger-Ellison syndrome: recent advances and controversies. Curr. Opin. Gastroenterol. 2013;29 (6): 650-61. doi:10.1097/MOG.0b013e328365efb1 - Pubmed citation
- 7. Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann. Surg. 2003;142 (4): 709-23. Free text at pubmed - Pubmed citation
- 8. Epelboym I, Mazeh H. Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist. 2014;19 (1): 44-50. doi:10.1634/theoncologist.2013-0369 - Free text at pubmed - Pubmed citation