Zollinger-Ellison syndrome is a rare disorder characterized by severe peptic ulcers, gastric acid hypersecretion, and a neuroendocrine tumor that secretes high amounts of gastrin. This excessive gastric acid causes ulcers in the stomach and small intestine, leading to diarrhea, steatorrhea, and poor response to standard ulcer treatments. Diagnosis involves finding elevated gastrin levels and localizing the gastrin-secreting tumor through imaging tests. Treatment consists of surgery to remove a single localized tumor when possible, or medications like PPIs and octreotide to reduce acid secretion and gastrin levels when the tumor is not resectable.

1. Zollinger-Ellison
syndrome
ALI NAJAT JABBAR
KIRKUK MEDICAL COLLAGE

2. INTRODUCTION
– Rare disorder characterized by the triad of sever peptic ulceration, gastric acid
hypersecretion and neuroendocrine tumour of the pancreas or duodenum.
– It accounts for about 0.1%of all cases of duodenal ulceration.
– It occurs in either sex at any age.
– Most commonly between 30 and 50 years of age.

3. Pathophysiology
– The tumour secretes gastrin, which stimulate acid secretion to its maximal
capacity and increases the parietal cell mass three to six fold.
– The acid output may be so great that it reaches the upper small intestine,
reducing the luminal PH to 2 or less (normally 5.5-7).
– Pancreatic lipase inactivated (because of hyperacidity) and bile acid precipitate
 diarrhea and steatorrhea result.
– Between 20%-60% of patients have multiple endocrine neoplasia type 1 (MEN1)

4. Clinical features
1. Multiple peptic ulcers
2. Poor response to standard ulcer therapy
3. Bleeding
4. Perforation
5. Nausea
6. Diarrhea
7. Loss of appetite

5. Investigations
1. Serum gastrin elevated (10-100 fold).
2. Normally, injection of hormone secretin causes no change or slight decrease
in circulating gastrin concentration, but in Zollinger syndrome it produces a
paradoxical and dramatic increase in gastrin.
3. Tumour localization is best achieved by a combination CT and EUS.
4. Radio-labelled somatostatin receptor scintigraphy and gallium DOTATATE PET
scanning may also be used.

6. Management
1. Surgery: for single and localized tumour but many are multifocal and
metastasized in which circumstances surgery is inappropriate unless there is
complication such as GIT obstruction
2. Medications:
i. omeprazole or other PPI can be used in healing ulcers and alleviating
diarrhea.
ii. Octreotide given by subcutaneous injection, reduces gastrin secretion