Zollinger-Ellison syndrome

Zollinger-Ellison syndrome - peptic ulcer of the stomach and duodenum [or gastroentero (duodeno) anastomosis after gastrectomy], caused by hypergastrinemia arising from hyperplasia of the G cells of the antral stomach (type I syndrome) or with the development of a tumor from the D-cells of the islet apparatus Pancreas, producing gastrin (type II syndrome - gastrinoma). Approximately 60% of tumors of the islet apparatus are malignant. In 30% of patients, single or multiple benign adenomas are detected and in 10% of the hyperplasia of the D-cells of the islet apparatus. Gastrinoma can be located outside the pancreas, more often in the wall of the duodenum. In 25% of patients with Zollinger-Ellison syndrome, adenomas of other endocrine organs - parathyroid glands, pituitary gland, adrenal glands (multiple endocrine adenomatosis) are found.

Symptoms, course. Abdominal pain and diarrhea. Pain in the abdomen is typical for peptic ulcer, however it is very intense and does not lend itself to antacid therapy. Diarrhea is pathogenetically associated with hypersecretion of hydrochloric acid: the degree of secretion of hydrochloric acid exceeds the possibility of neutralizing it in the duodenum, acidic content enters the jejunum and causes the development of enteritis. The emergence of diarrhea is also associated with inactivation of pancreatic enzymes; In addition, as a result of hypergastrinemia, peristalsis of the intestine is intensified, and the absorption of water in it decreases. The course of peptic ulcer in the syndrome of Zollinger-Ellison is very difficult: often ulcers are complicated by bleeding, perforation; Relapses of peptic ulcer occur even after surgical treatment. The localization of ulcers is very diverse: the descending section of the duodenum, even the jejunum.

In the diagnosis, the study of gastric secretion, which has a number of features, is important: over 12 hours the secretion of gastric juice exceeds 1500 ml; The level of basal secretion exceeds 15 mmol / h (the norm of 2-3 mmol / h); After the administration of the maximum dose of histamine, there is no increase in the production of hydrochloric acid. The most reliable diagnostic method is radioimmunochemical determination of gastrin in blood plasma. If a healthy human gastrin content in the plasma is 50-200 pc / ml, then with Zollinger-Ellison syndrome it can exceed 500 pg / ml. Stimulation of the secretion of gastrin with calcium or serotonin allows differentiating between types 1 and 2 of the syndrome.

Treatment. At 1 type of a syndrome (a hyperplasia of G-cells of an antral department of a stomach) resection of an antral department of a stomach is shown. In type II, the search for gastrinoma and its removal are justified. However, this is difficult to do, especially since 20% of patients have microadenomatosis and islet D-cell hyperplasia. Therefore, only the removal of the entire stomach (gastrectomy) completely eliminates the relapse of ulcers.

The forecast is always very serious. Even with an unsuccessful pancreatic tumor after gastrectomy, the level of gastrin is reduced to normal, there is a regression of distant metastases.