Synonyms in a broader sense
Zollinger-Ellison Syndrome
Definition
Gastrinoma (Zollinger-Ellison syndrome) is a tumor of the gastrointestinal tract that produces large amounts of the hormone gastrin. This hormone is a messenger substance that also occurs in the body of healthy people and, once released, stimulates the stomach to produce digestive enzymes and stomach acid. This makes sense in healthy people, as gastrin is only released in a controlled manner in response to certain stimuli, for example after eating, and thus helps to process food that has been eaten. In people with gastrinoma, however, gastrin is produced in a very uncontrolled and increased manner. As a consequence, there is also a strong increase in the production of gastric acid, which is ultimately responsible for the development of various complaints and the gastrinoma.
Frequency
Gastrinomas occur very rarely, with 5 to 10 cases per million people per year. Mostly the age group of 30-50 years is affected, occasionally the disease also occurs in early childhood. Gastrinomas occur twice as frequently in men as in women.
Localization
The largest part (about 80%) of all gastrinomas (Zollinger-Ellison syndrome) is located in the pancreas. This is also known as an “ectopic” localization, as gastrin-producing G-cells are not normally found in the pancreas in healthy adults. In children, however, the presence of G-cells in the pancreas is normal.
Gastrinomas also occur in the stomach and in parts of the small intestine, namely the duodenum and the jejunum. The increased production of the hormone gastrin primarily leads to an increased synthesis of gastric acid. Gastrin acts directly on certain cells of the stomach, the supporting cells, which are stimulated to produce hydrochloric acid.
The increased hydrochloric acid level can lead to the stomach and/or intestinal wall being attacked and ulcers (med. : ulcerations), i.e. deep defects of the mucous membrane, forming, which cause pain. If the pain occurs directly during eating, it is usually a stomach ulcer, but if the pain occurs during the night or when fasting and subsides during eating, it is more likely to be a duodenal ulcer.
In addition to the pain associated with gastrinoma, affected patients may also experience a feeling of fullness, inappetence, nausea and vomiting. Due to the increased production of gastric acid and the associated damage to the intestinal wall, diarrhea occurs in about one third of those affected, in addition to ulcers. This is due to the fact that the damaged intestinal wall can no longer adequately absorb components of the food, so that the food is excreted more or less unchanged together with the absorbed fluid.
In addition to the damage to the intestinal wall, another phenomenon plays a role in the development of diarrhoea: Normally, the food components in the intestine are broken down by certain enzymes from the stomach and pancreas and thus made usable. However, due to the increased acid level, the enzymes are changed in their structure (denatured) and damaged in such a way that they can no longer perform their function. Fats and carbohydrates therefore remain unchanged in the intestine and could not be absorbed even if the intestinal wall was intact.
