Zollinger-Ellison syndrome What we need to know

Zollinger-Ellison syndrome was first reported in 1955 when Ohio State University surgeons Robert Zollinger and Edwin Ellison described the disease separately. In all cases there were ulcers in the mucosa of the small intestine (the beginning of the small intestine) associated with excessive secretion of gastric juice. The final finding was a pancreatic head tumor (non-beta-cell), with hypersecretion of gastric juice, hyperacidity and peptic ulcers.

 

Zollinger-Ellison syndrome is a rare condition when a gastrinoma develops in the pancreas (pancreas) or duodenum. Gastrinoma is a neuroendocrine tumor that overproduces the hormone gastrin. It can occur in isolation or in combination with other neuroendocrine tumors. Gastrinoma is malignant in 12% of cases. The disease is more common in men between the ages of 30 and 30. In 50-25% of cases, it develops as part of a rare genetic syndrome - multiple endocrine neoplasia type I.

Multiple endocrine neoplasia type I - It is a rare condition (1:30), with equal frequency in women and men. Tumors appear in two or more endocrine glands. Tumors of the parathyroid glands, pituitary gland, thyroid gland, and adrenal glands are observed along with gastrinoma.

 

 

Mechanism of Zollinger-Ellison syndrome

 

Stomach, duodenum and pancreas are constituent organs of digestive tract. The stomach and pancreas produce gastrin, which activates the production of gastric juice and other digestive enzymes.

Physiologically, gastric cells control the production of gastrin. In Zollinger-Ellison syndrome, the control mechanisms are disturbed, the tumor formed in the mouth secretes gastrin in large quantities. Massive secretion of gastrin stimulates pancreatic secretion and intestinal peristalsis (movement), causing chronic diarrhea. A high concentration of hydrochloric acid damages the walls of the stomach, blocks the release of pancreatic enzymes and contributes to the formation of an acute ulcerative process.

 

 Diagnosis

 

  • The first step in the diagnostics of Zollinger-Ellison syndrome is a family history to detect type I multiple endocrine neoplasia. This syndrome is inherited in an autosomal dominant way, so 90% of cases are familial and only 10% occur as a result of a de novo mutation.
  • An increase in gastrin is a warning sign in patients with Zollinger-Ellison syndrome. In healthy patients, the fasting gastrin level does not exceed 100 pg/ml, and in Zollinger-Ellison syndrome it is much higher than the upper limit of the norm.
  • Basal gastrinemia >1000 pg/mL confirms the diagnostics of Zollinger-Ellison syndrome.

Gastroscopy and endoscopic ultrasound determine the presence of visual lesions - ulcers or tumors.

 

 

Treatment

 

In most cases of Zollinger-Ellison syndrome, treatment is surgical - extirpation of the tumor. In the case of mass ulceration, total resection of the stomach is also possible. In moderate cases, it is possible to reduce the gastrin level and the size of the tumor through medication.

 

To diagnose Zollinger-Ellison syndrome, Laboratory "Synevo" We offer the following studies:

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Health care starts with accurate analyzes

Resources

https://www.synevo.ro/sindromul-zollinger-ellison-boala-pancreasul/

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