Makindo Medical Notes

Most patients experience severe refractory heartburn and epigastric pain, often accompanied by profound diarrhoea.

About

• Zollinger-Ellison Syndrome (ZES) is a condition characterized by peptic ulcer disease caused by excessive gastrin production from a gastrinoma.
• The tumour is usually located in the pancreas or the duodenum and is often associated with Multiple Endocrine Neoplasia Type 1 (MEN 1).

History

• Dr. Robert M. Zollinger and Dr. Edwin H. Ellison first described the syndrome in 1956, based on two cases of patients with severe, recurrent, multifocal peptic ulcers.
• The patients presented with ulcerative lesions affecting the proximal gastrointestinal tract.

Aetiology

• Gastrin is a hormone produced by G cells in the gastric pits, primarily in the antrum of the stomach.
• Excessive gastrin production in Zollinger-Ellison Syndrome leads to overstimulation of acid production, causing peptic ulcer disease.
• This results in gastric mucosal hypertrophy and increased risk of ulceration.

Clinical Features

• Patients often present with persistent epigastric pain, refractory heartburn, and diarrhoea due to excess acid secretion.
• Other symptoms include anaemia from gastrointestinal bleeding, melaena (black tarry stools), and shock in severe cases.
• If the tumour has metastasized to the liver, hepatomegaly may be present.

Investigations

• Endoscopy typically reveals multiple large peptic ulcers, often located in the duodenum or proximal small intestine.
• Fasting Gastrin Levels (> 1000 pg/ml) are used as a screening test. A low gastric pH (< 2) supports the diagnosis of Zollinger-Ellison Syndrome.
• Secretin stimulation tests are used to confirm elevated gastrin levels.
• Elevated serum calcium may indicate an association with MEN-1 and hyperparathyroidism.
• Somatostatin receptor scintigraphy (SRS) is useful for localizing gastrinomas, especially metastatic disease.
• CT scan or MRI can be used to localize the tumour and assess for metastasis.

MEN-1 Screening

• Screening for MEN-1 involves testing levels of prolactin, calcium, parathyroid hormone (PTH), and pancreatic polypeptide to evaluate for other endocrine tumours.

Management

• Zollinger-Ellison Syndrome is known to resist conventional treatment for peptic ulcer disease.
• High doses of proton pump inhibitors (PPIs) are necessary to control gastric acid hypersecretion.
• Surgical resection of the primary gastrinoma is indicated when the tumour is localized and non-metastatic, along with aggressive management to control metastatic spread if present.

References

• Zollinger-Ellison Syndrome: Classical Considerations and Current Controversies