Zollinger-Ellison Syndrome was found in 5-Minute Clinical Consult which helps you diagnose, treat, and follow up on over 900 medical conditions seen in everyday practice.
To view this entire topic, please sign in or purchase a subscription.
Explore 5-Minute Clinical Consult - view these FREE monographs:
-- The first section of this topic is shown below --
Basics
Description
- Zollinger-Ellison Syndrome triad:
- Markedly elevated gastric acid secretion
- Peptic ulcer disease
- A gastrinoma or non-β islet cell tumor of the pancreas or duodenal wall that produces gastrin
- Gastrinomas (at the time of diagnosis) may be single or multiple (1/2–2/3), large or small, benign or malignant (2/3), sporadic (70–75%) or associated with MEN1 (multiple endocrine neoplasia type 1) (25–30%).
- System(s) affected: Endocrine/Metabolic; Gastrointestinal
- Synonym(s): Z-E syndrome; Pancreatic ulcerogenic tumor syndrome; Multiple endocrine neoplasia, partial; Ulcerogenic islet cell tumor
Epidemiology
Incidence
- 1–3 per million per year
- Predominant age: Middle age (30–65 years)
- Predominant sex: Male > Female (1.3:1)
Pediatric Considerations
Very aggressive cases have been reported in teenagers.
Pregnancy Considerations
Cases reported; influences medication choices and surgical timing
Risk Factors
- MEN1
- Family history of ulcer disease
Genetics
~25–30% of cases occur in association with the MEN1 syndrome.
General Prevention
Screen 1st-degree relatives of patients with MEN1.
Etiology
- Gastrinoma is equally distributed between the head of the pancreas and the 1st or 2nd portion of the duodenum; if in the pancreas, the lesion is more likely to metastasize to the liver.
- Also may be found rarely in the mesentery, peritoneum, spleen, skin, or mediastinum (possibly metastasis with primary not identified)
Commonly Associated Conditions
- MEN1: Hyperparathyroidism, prolactinomas, other pituitary tumors
- Insulinoma
- Carcinoid tumors
-- To view the remaining sections of this topic, please sign in or purchase a subscription --
