ZOLLINGER–ELLISON SYNDROME : (Gastrinoma)
- A rare disorder caused by a non–beta islet cell, gastrin-secreting tumor of the pancreas that stimulates the acid-secreting cells of the stomach to maximal activity, with consequent gastrointestinal mucosal ulceration
- characterized by the triad of severe peptic ulceration, gastric acid hypersecretion and a non – beta cell islet tumor of the pancreas (Gastrinoma).
- It is most common between 30 and 50 years of age.
Pathology:
-
The gastrinoma produces enormous amounts of the hormone gastrin, due to which the parietal cell mass increases 3 – 6 folds and secrete to their maximal capacity.
-
The pancreatic lipase is inactivated and bile acids get precipitated, which results in diarrhea and steatorrhoea.Z
-
An increase in the acidity of the stomach contents, which can lead to a decrease in the pH of the small intestine. This acidic environment can inactivate pancreatic lipase, leading to malabsorption of fats and other nutrients.
-
In 90 percent cases gastrinoma is situated in the pancreatic head. The size varies between 1 mm to 20 cm.
-
Approximately half to two–thirds of the cases are malignant.
-
Multiple endocrine neoplasia (MEN), Type I is associated in 20 – 60 percent of patients. with hyperparathyroidism and pituitary adenomas. //
Diagnosis:
-
This is done by the demonstration of hypergastrinemia and concurrent gastric hyperacidity.
-
The tumor localization may be performed by endoscopic ultrasound.
-
It is seen that 80 – 90 percent of gastrinomas would be found within the gastrinoma triangle.
Treatment:
The goals of treatment:
-
Medical control of gastric acid hypersecretion
-
Surgical resection of the tumor
-
Surgical resection of the tumor is undertaken.
-
Omeprazole is given in high dose of 60 to 80 mg/day
-
Total gastrectomy is advised only, if medical therapy fails.
