Zollinger-Ellison syndrome, short-form is ZE syndrome also famous as gastrinoma. It is a rare disorder affecting the digestive tract.
It is characterized by a triad of severe peptic ulceration, hypersecretion of gastric acid, and a non-beta islet tumor of the pancreas that is gastrinoma.
Multiple presence of duodenal ulcers and may be associated with perforation, bleeding, and pyloric stenosis.
Esophagitis and esophageal stricture may be present due to the ascending reflection of gastric acid into the esophagus.
About one-third of patients have a functioning tumor with hypercalcemia. Tumors may also present in the pituitary, adrenal gland, and thyroid. The condition is also known as Multiple Endocrine Neoplasia Type 1 (MEN1)
Hypersecretion of acid under basal condition is measured by injection of pentagastrin.
Fasting plasma gastrin concentration:
After injection of hormone secretin, there is a dramatic increase in circulating gastrin in plasma. It can be as high as 1000 folds. Normally no change or a slight decrease in normal individuals.
Multiple x-rays are taken after giving thick fluid containing barium. Picture shows the presence of gastric mucosal folds in barium swallow study.
CT scan, MRI, or arteriography can localize the tumor. Endoscopy and endoscopic ultrasound can also diagnose the condition.
Symptoms resemble symptoms of peptic ulcers such as abdominal especially epigastric pain, bloating and burping, nausea, vomiting, weight loss, and anorexia. Diarrhea may also present.
Treatment of ZE syndrome
Medical management is done to reduce acid secretion with Proton pump inhibitors, H2-receptor blockers, and antacids.
Surgical Management is done to remove the tumor. But it can be difficult as tumors can be multiple at a different location and small in size which can be hard to locate.