Zollinger-Ellison Syndrome: Symptoms, Causes, Treatment

In Zollinger-Ellison syndrome (synonyms: Gastrinoma; MEN; Multiple endocrine neoplasia (MEN) type I; ICD-10 E16.4: Abnormal gastrin secretion) is a neoplasia (neoplasm) that results in increased gastrin production and is therefore also called gastrinoma.
Gastrin is a hormone produced in the mucosa of the stomach. Its production is stimulated by a food stimulus. Gastrin, in turn, stimulates the production of gastric acid.

Gastrinoma is often localized in the pancreas (approximately 80%). It can be a benign (benign) tumor, but more often it is malignant (malignant; up to 70%). At the time of diagnosis, metastases (daughter tumors) have already occurred in 50% of those affected.

Gastrinoma is genetic in 25% of patients and then occurs in the setting of multiple endocrine neoplasia (MEN) type I. MEN-I syndrome is a disease leading to different endocrine tumors: the “three Ps” – pituitary, pancreas, parathyroid – describe the localization.

Peak incidence: The disease occurs predominantly between the ages of 30 and 60.

The incidence (frequency of new cases) is about 5-10 cases per 1,000,000 inhabitants per year (in Germany).

Course and prognosis: As long as the underlying gastrinoma has not metastasized, especially to the lymph nodes and liver, cure is possible. Gastrinoma is surgically removed, but may recur (return). If metastases are detectable, symptomatic therapy can still significantly improve the course. In most cases, the disease progresses slowly, so that survival is possible for years or decades. However, there are also aggressive courses with a severely shortened life expectancy.